Synovial sarcoma (SS) is a malignant mesenchymal neoplasm that is relatively common in the distal extremities. Primary SS of bone is an extremely rare finding. Here in this report, we present a 44-year-old male patient referred with bone and later bone fracture that was finally diagnosed with primary SS of thumerus. So far, 13 documented cases of primary SS of the bone have been reported. The current case is the second known case of primary SS of humerus. Our case was treated with both neoadjuvant and adjuvant chemotherapies associated with surgical tumor removal and prosthesis implantation. Follow-up of the case demonstrated significant remission but with late metastasis and subsequent advanced chemotherapy regimens.

Non-healing diabetic foot ulcer (DFU) is one of the main complications in diabetic patients. This case reported a 65-year-old male with a neuropathic ulcer in the right foot came to Ahwaz Wound Clinic after the wound had not healed with routine treatments. In addition to the routine treatment program, we used tropical ozone therapy and autohemotherapy (blood ozone therapy) for 2 months. Zinc supplementation (50 mg) was also administered daily during the treatment. The DFU was clearly healed with diminishing inflammation and wound closing, and there were no side effects. Additionally, the C-reactive protein level was obviously decreased during the treatment indicating effective suppression of infection. This way indicates a helpful new intervention approach to the treatment of DFU.

Guillain-Barré syndrome is an autoimmune disorder presented by ascending paralysis and areflexia. The condition has been reported after many infections, but Guillain-Barré syndrome after spine surgery is rare. We, herein, present a case of Guillain-Barré syndrome after multiple spine surgeries for degenerative lumbar scoliosis. A 60-year-old woman with degenerative scoliosis underwent surgery for the third time and developed Guillain-Barré syndrome 3 weeks after the final operation. The patient received intravenous immune globulin therapy and needed mechanical ventilation and intensive care. She was discharged in good condition after 5 weeks.

In this article, a 75-year-old patient with pain in left knee and restricted range of motion following total knee arthroplasty (TKA) is presented. Serological evaluation and aspiration of knee joint suggested a fungal prosthetic joint infection. After the diagnosis was confirmed, treatment started with antifungal drugs, removing prosthesis, exhaustive debridement, and revision of TKA after efficient antifungal treatment. At one-year follow-up, she has a painless motion range of 10 to 90 degrees, and there was no recurrence of infection observed.

The aim of this study was to report a case of the treatment of hydatidiform mole in Coronavirus pandemic in Iranshahr. A 17-year-old primiparous woman with gestational age of 14 weeks presented with unilateral leg swelling and sudden abdominal distension beginning in the night before referring to the health center. In the abdominal examination of the patient by a healthcare provider, the baby's heartbeat was not heard and a mismatch was observed between gestational age and fundal height, which corresponded to approximately 24 weeks of gestation. She was conscious and pale with hematuria and uterine contractions. After inserting two IV lines, the patient immediately underwent monitoring and was visited by a gynecologist. Complete molar pregnancy was diagnosed with an enlarged heterogeneous uterus 180 cm × 90 cm in size and containing 170 mm × 80 mm cysts. The treatment began with vancomycin AMP, hydrocortisone AMP, oseltamivir CAP 75 mg, kaletra CAP 200 mg, and meropenem AMP.

Primary gastrointestinal small cell carcinoma is reported 0.1%–1.0% of malignant gastrointestinal tumors and predominantly in the esophagus. All reported cases are in the adult population. We present a 9-year-old boy with small cell neuroendocrine carcinoma of the esophagus with mediastinal lymph node involvement, whose chief complaint was progressive dysphagia. He survived for 22 months with chemoradiation but did not have resectional surgery. Although small cell neuroendocrine carcinoma of the esophagus is extremely rare in children, it should be considered in the differential diagnosis of any undifferentiated tumor of the esophagus in any age.

Avascular necrosis (AVN) of multiple carpal bones is a very rare condition. This case report presents a patient with concomitant AVN of the lunate and proximal pole of the scaphoid that had led to severe joint space narrowing and degenerative arthritis in the radiocarpal and distal radioulnar joints (DRUJs). She was known to have thalassemia minor hemoglobinopathy. Wrist arthrodesis and distal ulna hemiresection-interposition arthroplasty (Bowers technique) for a DRUJ were offered and performed. The patient was satisfied with the clinical outcomes.

Lipoma is an uncommon mesenchymal tumor of the colon. They are typically symptom free, the large lipomas within the colon are rare, and can cause obstruction, bleeding, or intussusception. Intussusception is comparatively common in pediatrics and in adults, it could be a rare entity. Pathologic lesions are usually found with a major percentage of malignancy. This is a retrospective evaluation of an adult with an intestinal intussusception who underwent oncosurgical treatment. He was diagnosed with surgically proven intussusception. Using ultrasound and colonoscopy as diagnostic studies, colocolic intussusception was discovered. The patient underwent oncosurgical exploration. Intestinal resection with abrupt anastomosis was the procedure of choice for the patient. The etiology was benign lipoma. Adult intussusception should be evaluated in any patient with subacute abdominal discomfort with bearing in mind the high rate of malignancy and a lower rate of benign tumors. intestinal resection without reducing is highly recommended for colonic intussusceptions.

Prurigo pigmentosa (PP) is a rare skin disorder presenting as erythematous urticarial papules on the chest and leaving reticulated pigmentation. Although the etiology of PP is unknown, conditions associated with ketosis such as diabetes mellitus, ketogenic diet (KD), and anorexia nervosa are implicated. Herein, we report a 21-year-old woman who developed PP after adhering to a KD and responded to resuming a regular diet.

Also known as intravascular papillary endothelial hyperplasia, Masson's tumor is a relatively rare soft-tissue vascular tumor that usually arises in the hand. Felon is an abscess formation in the distal phalanx that usually occurs following a penetrating microtrauma. We present a 30-year-old patient who was referred to our clinic with a palpable mass in the distal phalanx of the index finger after a needle stick injury. At first, the lesion was treated as a felon but finally and after treatment failure, a complete reevaluation revealed the lesion to be a Masson's tumor of the distal phalanx.

Ataxia–telangiectasia (AT) is a type of primary immunodeficiency characterized by an autosomal recessive mode of inheritance and usually presents with progressive cerebellar ataxia in early life. This complex disease is associated with humoral and cellular immune dysfunction and other features including characteristic oculocutaneous telangiectasia and increased predisposition to cancers, particularly lymphoma and leukemia. An 11-year-old Iranian girl presented with primary immunodeficiency and was diagnosed as having AT according to her clinical manifestations and molecular findings. She had a history of two types of non-Hodgkin's lymphoma and showed spontaneous regression of her diffuse large B-cell lymphoma without any specific treatment. Gene mutations and dysfunction in patients with AT result in different manifestations including abnormal development of the thymus, immunodeficiency, increased susceptibility to malignancies, and increased radiosensitivity. No standard treatment is available for these patients. The use of immunotherapeutic strategies in patients with primary immune deficiency disease-associated tumors is potentially important.

Diagnosing breast cancer (BC) in early stages increases the chances of treating this cancer in men. However, because BC is very rare in men, especially inflammatory BC (IBC), it is unlikely that screening men for BC by mammography or other tests would yield promising outcomes. The aim of this study was to report IBC in a 53-year-old man. The case was a 53-year-old man with a history of mass in the left breast and trauma to the same side as well as swelling and severe redness of the breast skin. The patient underwent neoadjuvant chemotherapy and relative responded to medical treatment. He then underwent modified mastectomy surgery and initial chest wall repair followed by radiotherapy. IBC in men is challenging due to its rarity, unknown biological behaviors, and difficulty in early diagnosis. This tumor is usually detected in advanced stages in the elderly and has a poor prognosis.

Parsonage-Turner syndrome (PTS) is a rare syndrome of unknown etiology; however, it is believed that an abnormality of immune response after a previous infection may be the cause of the disease. We report neuralgic amyotrophy in a patient with a history of kidney transplantation with severe acute respiratory distress syndrome coronavirus 2 infection. This literature is reviewed regarding clinical presentation, etiology, treatment, and prognosis of PTS after COVID-19 infection. We should consider PTS as another complication of COVID-19 infection.

Schwannoma is a rare tumor in the colon which originates from the peripheral nerve plexus. Most of the cases have been asymptomatic but occasionally present as an obstructive mass. Abdominal investigations are effective in some cases, but usually, they are not informative. A significant number of cases have been detected after their operation by histopathology examination. Immune and histochemical staining shows the spindle cells that have been positive for S-100 and vimentin, but negative for CD34 and smooth muscle actin. If the diagnosis of Schowannoma is confirmed preoperatively, segmental resection is recommended. In this case report, we presented a 58-year-old woman with pelvic mass and normal colonoscopy that mimic extramural large uterine myoma with extraluminal pressure effect on the rectosigmoid.

Usher syndrome is a heterogeneous genetic disease that is the most common cause of hereditary blindness–deafness. This syndrome is the most prevalent syndrome associated with retinitis pigmentosa. A 25-year-old man referred to us with hearing loss and vision diminution since childhood which has gradually worsened. Visual acuity of both eyes was 20/100. Slit lamp examination of the left eye revealed endothelial stellate keratic precipitates, mild anterior chamber reaction, iris heterochromia, ectropion of uvea, and mild posterior subcapsular cataract. There were also no crypts and abnormal vessels in the left eye iris. His intraocular pressure was 14 mmHg in the right eye and 18 mmHg in the left one. Funduscopy demonstrated waxy pallor optic nerve, marked arterial narrowing, and retinal bone spicule pigment formation in both eyes. We report for the first time a very rare association between Usher syndrome, Fuchs heterochromic uveitis (FHU), and ectropion uvea. To our knowledge, no association has been reported between ectropion uvea, FHU, and Usher syndrome.

Extraintestinal manifestations (EIMs) are common in patients with Crohn's disease (CD). Various reactive cutaneous conditions, including erythema nodosum and pyoderma gangrenosum frequently occur as a part of EIMs. However, cutaneous metastasis of CD is rarely encountered in CD patients. Here, we report a 28-year-old female patient presenting with discharging deep fissures on genital and intergluteal regions. The result of a skin biopsy showed noncaseating granulomas. After rule out all the other differential diagnoses for granulomatous skin lesions, we believe this patient may be a case of CD, presenting with skin metastasis and GI tract involvement has not been occurred during 1-year follow-up. We suggest including cutaneous (metastatic) CD in the list of dermatologic differential diagnoses for cutaneous lesions of these sites. These lesions can occasionally precede gastrointestinal (GI) involvement by months and years, therefore, an appropriate follow-up needs to be done to detect GI lesions as soon as they appear.

Retropubic midurethral sling (MUS) is safe and effective surgery used for the treatment of stress urinary incontinence in women. Bladder neck perforation is a rare intraoperative complication. If this complication missed in intraoperative cystoscopy may have serious morbidity. A 52-year-old woman underwent a retropubic MUS. She presented with early and unusual symptoms such as suprapubic and labial cellulitis and urine leakage through the suprapubic incision 1 week after surgery which was due to a missed bladder neck perforation during surgery. In cystoscopy after MUS revealed mesh traversing the bladder neck and it was removed. The missed bladder perforation may have early and unusual symptoms and cystoscopy must be done more carefully and obsessively in patients with risk factors.

Primary breast lymphomas are uncommon tumors and account for <1% of all malignant neoplasms of the breast. They are almost always of non-Hodgkin type, with B-cell lymphomas being the most common subtype. Anaplastic large cell lymphoma (ALCL) is a rare T-cell lymphoma that can involve the breast. Most of the articles in the literature describe ALCL in association with breast implants. We present a 48-year-old woman with a left breast enlargement and no history of an implant. Microscopic sections showed a high-grade CD30-positive lymphoid neoplasm with frequent giant cells, which turned out to be a primary ALCL of the breast, giant cell-rich pattern. To our knowledge, no cases of primary ALCL, giant cell-rich variant, have been reported in the breast in the absence of an implant making our case unique.

The COVID-19 pandemic has raised concerns among physicians and patients with autoimmune disorders about how this viral infection affects the patients receiving immunosuppressive drugs. There are speculations about a higher incidence and severity of COVID-19 in patients receiving a variety of immunosuppressant drugs. However, we reported the rapid recovery from COVID-19 in a 67-year-old male with granulomatosis with polyangiitis who did not experience severe symptoms of the COVID-19 as expected, despite having a history of serious lung involvement due to the autoimmune disease. He received conventional medications to treat COVID-19, though he had been receiving rituximab and corticosteroids before the onset of COVID-19 symptoms. Prevention of the cytokine storm caused by SARS-CoV-2 infection owing to taking the immunosuppressive drugs (rituximab and corticosteroids) could be a reason for these unexpected observations. Therefore, this case showed that taking immunosuppressive drugs is unlikely to be directly related to the increased severity of COVID-19.

Extrapulmonary tuberculosis (TB) is a well-recognized cause of pyrexia of unknown origin. However, clinical presentation of TB in children with isolated hematological abnormalities is extremely rare. Anemia, usually normocytic, normochromic, leukopenia, leukocytosis, thrombocytopenia, thrombocytosis, and monocytosis are more common complications of TB rather than pancytopenia. Only anecdotal case reports and small case series are available in this regard. We are reporting an 18-year-old boy who presented with on and off low-grade fever for 3 months and anorexia and progressive pallor for 1 month. After extensive workup, pancytopenia remained unexplained. Bone marrow (BM) examination revealed caseating granulomas, along with Mantoux positivity and contact with sputum-positive pulmonary TB. He responded favorably to antitubercular therapy (ATT) within 2 months. This report alerts clinicians to be vigilant regarding the rare possibility of BM TB while investigating unexplained pancytopenia, as it is completely reversible with ATT.

An 81-year-old female presented with a loss of consciousness and a history of fever, dry cough, dyspnea, and conjunctivitis during 5 days ago. On initial physical examination, the laboratory examination revealed high levels of lactate dehydrogenase and creatinine concomitant severe thrombocytopenia. Moreover, the peripheral blood smear showed schistocytes 3%, suggesting thrombotic thrombocytopenic purpura (TTP). A ground-glass pattern was reported in the high-resolution computed tomography of the lung. A positive polymerase chain reaction was reported for coronavirus disease 2019 (COVID-19). After initiating treatment for COVID-19, the patient received fresh frozen plasma and 24-h electrocardiogram monitoring in the emergency department. As the patient was being prepared for transfer to another hospital for plasmapheresis treatment, cardiac arrest occurred again, and the patient passed away. This study highlights the atypical behavior of this virus over the course of the disease including TTP with conjunctivitis, which could vary from case to case.

Dystroglycanopathy is a type of congenital muscular dystrophy caused by mutations causing defective glycosylation of a dystrophin-associated glycoprotein, dystroglycan and as such is a very rare disease entity. We are reporting a 1-year-old girl child with dystroglycanopathy who presented with motor predominant developmental delay. She had motor development quotient of 52, mental development quotient of 75, facial dysmorphism, mixed hypotonia with a global decrease in muscle power, and areflexia. Serum CPK level was elevated; magnetic resonance imaging brain revealed multiple intraparenchymal cysts in the cerebellum with disorganized folia. Next-generation sequencing revealed a homozygous missense mutation in exon 3 of the ISPD gene (p.Gln215His; ENST00000407010) consistent with the diagnosis of dystroglycanopathy muscle-eye-brain disease. Genetic counseling and prenatal diagnosis for subsequent pregnancies were advised for the family, apart from appropriate rehabilitation for the child.

Diffuse idiopathic skeletal hyperostosis (DISH) also known as Forestier disease is a noninflammatory, systemic skeletal disease of unknown etiology. DISH is usually asymptomatic but may compress the posterior wall of the aero digestive tract and lead to dysphagia, globus, hoarseness, stridor, dyspnea, and neurological problems. Although dysphagia is not uncommon among the presenting symptoms of DISH but dysphonia and stridor are rarely reported. We report a 68-year-old man who presented with a history of progressive dysphagia over 1 year and recent dysphonia and stridor secondary to cervical osteophytes. We discuss the symptoms, radiological features, and management of this uncommon case of DISH in conjunction with review of literature.

Anesthesia management has always been challenging in cardiac patients, especially patients with cardiomyopathy. There are a variety of cardiomyopathies such as unclassified cardiomyopathy as a complex type that can occur in many forms like left ventricular noncompaction (LVNC) that is an uncommon primary genetic cardiomyopathy typified by noticeable trabeculation of the left ventricular (LV) wall and intertrabecular recesses. We report anesthesia management in a 53-year-old female patient who admitted to the hospital for the transureteral lithotripsy surgery due to dysuria and urolithiasis with a medical history, and echocardiographic examination indicated the diagnosis of hypertension and unclassified cardiomyopathy (LVNC).

Suicide with cyanides is relatively rare but highly lethal. The lethal oral dose of cyanide salts is 200 mg, and concentrations >3 mg/L may be potentially lethal. The symptoms of poisoning are predominating in the central nervous system and cardiovascular system. We report the case of a 43-year-old goldsmith man who presented with self-poisoning by cyanide salt ingestion. Patient's symptoms included confusion, cardiac arrhythmias, hyperkalemia, leukocytosis, metabolic acidosis with high anion gap, hypotension, and then hypertension. The cause of the patients poisoning was not initially diagnosed. Intensive supportive treatment was performed. The patient died on the 3^rd day of admission following cardiac arrest. At autopsy, hemorrhagic gastritis and cherry-red discoloration of the chest muscles were observed. Forensic toxicology showed cyanide in the blood and tissues. Cyanide poisoning could appear in different forms, and like our case, the symptoms can last for several days with nonspecific symptoms.

Poisoning with any of the colchicine or chloroquine drugs is rare. These drugs exert therapeutic and toxic effects on tissues by different mechanisms. Colchicine is used to treat a number of rheumatologic diseases and heart problems. In addition, chloroquine is used to treat malaria and some inflammatory diseases. There is a small gap between the therapeutic and toxic doses of these drugs. Gastrointestinal symptoms are the initial causes of poisoning with these drugs and then widespread organ failure in later stages can lead to sudden cardiac death. We introduce a case of concurrent poisoning with both drugs, in which the patient presented with a headache, nausea, and vomiting several hours after suicide. On the 1^st day, the patient's status was stable, but on the 2^nd day, the patient suddenly becomes ill and died even though the patient received supportive therapy. Concurrent poisoning with chloroquine and colchicine is extremely lethal, and early aggressive management is recommended even in an apparently stable patient.

Prolactinomas are the most common type of functional pituitary tumors. Dopamine agonists is the most important drugs used in prolactinoma,have antagonistic effect with antipsychotic drugs used in schizophrenia. Conversely, dopamine antagonist drugs increase prolactin in patients with simultaneous schizophrenia. In the present case, we report a 29-year-old single male with schizophrenia who treated for 8 years with risperidone and presented with macroprolactinoma. Iatrogenic hyperprolactinemia is a well-known side effect of dopamine antagonist drugs for treatment in a patient with schizophrenia. On the other hand, it appears these drugs have the other side effects, such as drug- induced prolactinoma or boost growth.

Gastroenteritis is common among children and is usually caused by bacterial, viral, or parasitic gastrointestinal infections. The occurrence of gastroenteritis as the only symptom of coronavirus disease 2019 (COVID-19) is an uncommon condition. We present a 16-month-old girl that has recently been admitted to our hospital with vomiting, diarrhea, and lethargy, who was ultimately diagnosed with COVID-19. This case shows that the clinical manifestations of COVID-19 can be misleading in children.

Phlegmasia cerulea dolens is a severe form of deep venous thrombosis (DVT) characterized by severe venous outflow obstruction, marked limb swelling, pain, bluish discoloration, and even venous gangrene if the condition is untreated. In our case, 75-year-old woman, with general abdominal pain, which increases with eating and anorexia and 5 days of coldness and swelling of the left leg, was accepted. The patient had a history of Type II diabetes, ischemic heart disease, congestive heart failure, hyperlipidemic (HLP), hypertension, metastatic ovarian cancer, and previous DVT. She has undergone chemotherapy for the past 3 weeks due to ovarian cancers. Anticoagulation with intravenous administration of heparin and fluid resuscitation started immediately. The evidence of color Doppler sonography approved acute DVT in common femoral vein extending to the left external iliac. The patient did not consent for continuing the procedure in the hospital and succumbed to her illness on the 7^th day after discharge.

In tuberculosis (TB) endemic countries like India, this is a common practice to attribute patient's illness to TB even without microbiological confirmation. Furthermore, a false diagnosis may prove fatal as the underlying disease is left untreated, and the burden is amplified by avoidable potential side effects of antitubercular drugs. We present a case of a 42-year-old female who presented to us with a typical presentation of disseminated TB but found to have metastatic thyroid carcinoma.

Uveitis and erythema induratum are two uncommon extrapulmonary manifestations of tuberculosis (TB). In most circumstances, we cannot isolate mycobacterium from those sites, so diagnosis is difficult. In the presented case, panuveitis, erythema induratum, and persistent anogenital warts were found concurrently in a 19-year-old girl who had a history of pulmonary TB 10 years ago. Assessment of her immune condition ruled out any immunodeficiency state. Extrapulmonary TB and persistent warts responded dramatically to anti-TB drugs and interferon-gamma, respectively. Our case reveals that a constellation of these clinical manifestations may also occur in immunocompetent individuals.

The prevalence of uterine myomas during pregnancy is estimated to be small. However, a significant fraction of these could lead to pregnancy complications. Myomectomy is rarely performed during pregnancy because of fear of miscarriage and the risk of uncontrolled hemorrhage necessitating hysterectomy. This article reports on a case of myomectomy of subserous leiomyoma that was causing septic necrosis, leukocytosis, rise of inflammatory markers (erythrocyte sedimentation rate and C-reactive protein), and pressure symptom on the liver. Myomectomy was performed at 20 weeks of gestation. The pregnancy continued with no further problems, and at 39 weeks and 1 day of gestation, vaginal delivery resulted in a healthy baby. It is postulated that when myomectomy is performed in carefully selected patients, it prevents sepsis due to myoma torsion and necrosis and also protects surrounding organs against damage.

The present study aimed to present a rare case of successful pregnancy within 6 months from cranial mass surgery and postsurgical radiotherapy. A 32-year-old female with gestational age of 19 weeks had been refereed with a past medical history of recently treated brain astrocytoma. Close obstetric monitoring had been planned; the pregnancy was complicated with severe preeclampsia at the gestational age of 36 weeks, which leads to successful delivery. Pregnancy in patients with a history of cancer has been the focus of studies today and in many cancers it is recommended to delay pregnancy for at least 2 years. The prognosis of such a patient after unplanned pregnancy conception could be more complex; and the present case report aimed to explain about it. The goal of this presentation was to emphasis on the possibility of fertility preservation in the patient with malignancy even after cranial mass surgery and radiotherapy.

Mayer–Rokitansky–Küster–Hauser (MRKH) Syndrome is a female reproductive system disorder. It is characterized by a defect in the Müllerian ducts development, and it causes the absence of the uterus in variable degrees in upper vaginal hypoplasia. In addition, it is often associated with the unilateral renal dysplasia. Müllerian agenesis affects 1 in 4500 newborn girls and is considered as a sporadic anomaly. Women with MRKH Syndrome have a normal female chromosome pattern 46, XX with normal ovarian function. The presence of bilateral kidney agenesis with a pelvic pancake-shaped kidney is a rare condition, and a few cases have been reported in medical journals. This case study focuses on a case of MRKH Syndrome with bilateral renal agenesis and a pancake-shaped kidney.

Muscular hydatid cyst is a rare condition mainly secondary to the cysts from other organs. We hereby present an uncommon case of primary hydatid cyst found in the right biceps brachii muscle of a 36-year-old male. Magnetic resonance revealed no Echinococcus involvement in any other part of his body. Chest X-ray was normal, and no trace of cyst was found in the lung. In areas where echinococcosis is endemic, any tumor or mass in any part of the patient's body should be evaluated and examined for Echinococcus infestation. This paper is the second case report article on echinococcal biceps brachii infestation existing to this date.

Coarctation of aorta (CoA) is a congenital heart disease that can lead to heart failure during infancy and neonatal period. Several treatments have been proposed for this disease, including surgery and balloon angioplasty. The experiences of using stents in small infants or neonatal patients are very low. In this article, we report a 3 months old age infant who after balloon angioplasty, his CoA symptoms had return and stenting of CoA was performed for him.

Sickle cell disease (SCD) is a hereditary blood disorder that can increase complications during pregnancy and in turn negatively influence pregnancy outcomes. In addition to patients with SCD are at a high risk of been infected with hepatitis C infection. Furthermore in this study, we reported the clinical status of a pregnant woman with SCD who had hepatitis C virus infection.

Branchio-oculo-facial syndrome (BOFS), a rare, multiple-malformation congenital disorder, is characterized by facial anomalies, including associated cutaneous and ocular abnormalities. We report a new case of BOFS in an 11-year-old male child with bilateral cervical erythematous scaly linear plaque associated with scar formation and erosion. Although BOFS is very rare, physicians, especially dermatologists, should be aware of the cutaneous and histopathological features of BOFS due to impacts of the associated anomalies.

Primordial dwarfism (PD) is a group of rare genetically heterogeneous disorders consisted of disorders with intrauterine growth retardation continued through the life. SOFT syndrome with characteristics of short stature, onychodysplasia, facial dysmorphism, and hypotrichosis has been presented as a subtype of PD. Only 20 cases of SOFT syndrome have been reported in world to date, but none of them were not in Iran. Our case was 6.5-year-old girl with a complaint of growth retardation including height of 97 cm (Z = −4.6 standard deviation [SD]) and weight of 14 kg (Z = −4 SD) referred to growth clinic. She had a prominent forehead, triangular face, short limbs, malformed nails, and crowded teeth and her psychomotor function was normal. Laboratory and karyotype tests were normal while she was homozygous for c.G491A mutation of POC1A gene thus SOFT syndrome diagnosis was confirmed for her and recombinant growth hormone therapy was discontinued.

Carnitine membrane transporter deficiency or primary carnitine deficiency (PCD) is an autosomal recessive disorder of fatty acid oxidation, in which the transport of carnitine into cells is impaired. Carnitine plays an important role in transporting fatty acids into the mitochondria and carnitine deficiency block oxidation of long-chain fatty acids in the mitochondria that leads to heart and hepatic disease, myopathy, nonketotic hypoglycemia, and neurological complications. PCD has a wide range of symptoms and can reveal itself as symptomatic cardiomyopathy or even asymptomatic. In this study, we reported twin brothers with PCD. One of them had symptoms of disease and cardiomyopathy and was under treatment with carnitine. Another twin was asymptomatic and was diagnosed during follow-up period of his brother.

Patient with refractory seizure may undergo awake craniotomy when the area to be resected is immediately adjacent to eloquent cortex. An awake craniotomy in a 30-year-old man was conducted using a scalp block, dexmedetomidine, and remifentanil infusion without any airway device throughout the procedure. Dexmedetomidine administered at a loading dose of 1 μg/kg and maintenance dose of 0.2–0.7 μg/kg/h. Remifentanil dose was 0.02 μg/kg/min. The patient remained comfortable and hemodynamically stable throughout the procedure. Cortical electrical stimulation revealed motor cortex overlapped with seizure focus. So that surgical procedure was limited to subpial resection of the epileptic focus. Dexmedetomidine with concurrent scalp block appears to be an useful sedation for awake craniotomy when sophisticated neurological test is required.

Langerhans cell histiocytosis (LCH) is characterized by idiopathic monoclonal infiltration of Langerhans cells in different organs such as the skeleton, skin, pituitary gland, liver, spleen, lungs, and the hematopoietic system. Skin lesions are common in LCH and affect about 40% of cases. It is reported that skin lesions are usually the first manifestation of LCH in 80% of patients. Usually, cutaneous presentations of LCH in adults are generalized or seborrhea-like lesions and it is often the first manifestation of disease. Here, we describe a 45-year old female who was known case of hypothyroidism, systemic lupus erythematosus, and diabetes insipidus. In our patient, cutaneous involvement was unusual. It was single and presented in unusual site (ankle) and before developing such lesion, she had diabetes insipidus for several years due to the involvement of pituitary gland.

Thyroid follicular cancers are one of the thyroid gland cancers. This cancer can lead to metastases to various areas of the body. We describe a patient with thyroid follicular carcinoma who after total thyroidectomy had severe hypercalcemia, increased creatinine, and thyrotoxicosis due to extensive bone metastases. The patient was a 52-year-old man who had femoral neck fracture as the first manifestation of thyroid cancer. He was hospitalized for some time after orthopedic measures because of thyrotoxicosis and deep-venous thrombosis. The study found that the origin of metastatic lesions was thyroid follicular cancer, leading to extensive bone metastases. After administering of methimazole and control of thyrotoxicosis, he was subjected to total thyroidectomy. Methimazole was discontinued immediately after surgery. One month after surgery, ultrasound confirmed that the thyroid was completely removed. However, T3 (triiodothyronine) remained high; besides the patient had hypercalcemia and increased creatinine due to dehydration. The patient was retreated with methimazole due to thyrotoxicosis, and for hypercalcemia fluid therapy, intravenous zoledronic acid was prescribed. These measures led to the normalization of creatinine and glomerular filtration rate. The purpose of introducing this case report was that these symptoms are a rare manifestation of functional metastases of follicular thyroid carcinoma after total thyroidectomy. Bone metastases of follicular thyroid carcinoma may be functional and are lytic that can lead to hypercalcemia and its complications.

Subcorneal pustular dermatosis (SPD) or Sneddon-Wilkinson disease is a rare, benign, chronic, sterile pustular eruption which is associated with various systemic diseases including immunoglobinopathies, neoplasms, and autoimmune disorders. This paper reports a case of SPD in a patient with diffuse scleroderma in a 37-year-old woman. The hypothesis that immune dysregulation may play a role in the pathogenesis of SPD was supposed by the coexistence of diffuse scleroderma and SPD in our patient.

Heterotopic bone formation is a rare event in gastrointestinal lesions, especially in the pediatric population. Osseous metaplasia is exceedingly rare in colonic polyps. We present a case of rectal juvenile polyp with stromal osseous metaplasia in a 10-year-old male child. This histologic finding is very rare in children with colorectal polyps.

In this paper, we will present a case of a 63-year-old female with bifrontal epidermoid tumor who has gone under bilateral craniotomy. In a case report study, a 63-year-old female with a chief complaint of progressive headache that has been admitted to Department of Neurosurgery was studied. Magnetic resonance imaging was performed for better evaluation. After detection of bifrontal epidermoid cyst, the patient underwent surgery, and following the surgery, a cut of the tumor has been excised, sent for pathology sampling and reviewed for detection of cyst. Microscopic review of the resected part reported normal brain tissue along with sections containing parts of cyst wall covered by squamous epithelium and huge amount of irregularly stratified keratin within its lumen, which clearly emphasizes on diagnosis of a typical epidermoid tumor. Bifrontal epidermoid cyst is rare, and according to our study, the clinical symptoms and patients imaging were consistent with other studies.

Neu–Laxova syndrome (NLS) is an autosomal recessive disorder characterized by central nervous system anomalies, facial dysmorphic features, anomalies of limb and genitalia, intrauterine growth retardation, skin disorders, and other congenital abnormalities. In this article, we present a newborn infant who was born with facial dysmorphic features, flat nose, ichthyosis, rocker bottom feet, and fixed flexion contractures. We believe that these clinical findings in this patient are consistent with features of NLS.

Cellular therapy is being actively pursued as a therapeutic modality in many of the neurological diseases. A variety of stem cells from diverse sources have been studied in detail and have been shown to exhibit angiogenetic and immunomodulatory properties in addition to other neuroprotective effects. Published clinical data have shown bone marrow mononuclear cell (BMMNC) injection in neurological disorders is safe and possesses regenerative potential. We illustrate a case of 27-year-old male with traumatic brachial plexus injury, administered with autologous BMMNCs intrathecally and intramuscularly, followed by multidisciplinary rehabilitation. At the follow-up assessment of 3 and 7 months after first cell transplantation, improvements were recorded in muscle strength and movements. Electromyography (EMG) performed after the intervention showed a response in biceps and deltoid muscles suggesting the process of reinnervation at the site of injury. In view of the improvements observed after the treatment, the patient underwent second cell transplantation 8 months after the first transplantation. Muscle wasting had completely stopped with an increase in the muscle girth. No adverse effects were noted. Improvements were maintained for 4 years. A comprehensive randomized study for this type of injury is needed to establish the therapeutic benefits of cellular therapy.

Primary muscular hydatidosis is a very rare condition with an incidence of 1%–4% in endemic regions. This case report describes an unusual location of hydatid cyst in the sartorius muscle and the application of imaging in its preoperative diagnosis, choosing a treatment strategy, and monitoring.

Thrombotic thrombocytopenic purpura (TTP) secondary to diabetic ketoacidosis has been rarely reported and is considered as a rare complication. If left untreated, this condition could be life threatening with considerable morbidity and mortality. Herein, we report a 6-year-old girl with reduced consciousness and respiratory distress with a history of polydipsia and polyuria in the 2 weeks before hospitalization. The patient was initially diagnosed as diabetic ketoacidosis based on clinical and laboratory findings and treated accordingly. After treatment and during hospitalization although she had gained relative consciousness, she experienced seizure and reduced consciousness again. Considering laboratory and clinical findings and the patient's underlying conditions (thrombocytopenia, renal failure, and high lactate dehydrogenase), TTP was suspected although ADAMTS13 test could not be done. Treatment with plasmapheresis was initiated, and after 48 h, the patient was conscious, and laboratory indices became normal within a few days. The patient was discharged after full recovery. TTP should be considered as a rare complication of diabetic ketoacidosis in patients with thrombocytopenia, renal failure, and reduced consciousness and should be immediately treated.

Tyrosinemia type III is an autosomal recessive disorder caused by the deficiency of 4- hydroxyphenylpyruvate dioxygenase (4-HPPD). It is characterized by elevated levels of blood tyrosine and massive excretion of its derivatives into the urine. Clinical findings of tyrosinemia type III include neurological symptoms and mental retardation. Only a few patients presenting with this disease have been described, and the clinical phenotype remains variable and unclear. We present a case, who was admitted to the hospital at the age of 4 months for recurrent seizures. Two months later, she was admitted again with status epilepticus. Laboratory data showed increased level of tyrosine in the blood. She was treated with a diet low in tyrosine and phenylalanine and anamix formula that leading to catch-up growth and improvement of her symptoms. Plasma tyrosine level dropped to normal values. In any child who presents with the neurologic symptom, some rare diagnosis like tyrosinemia type III should be considered.

In this study, we report a 26-year-old female case of Niemann–Pick disease type C in association with Fuchs heterochromic iridocyclitis who was admitted with the complaint of ocular pain and redness following trauma. She had mild inflammatory signs and also vertical ocular motility limitations.

Adams–Oliver syndrome (AOS) is a rare congenital disorder with unknown etiology commonly presented with aplasia cutis and terminal limb defects. Central nervous and cardiopulmonary systems may also be affected. It is commonly inherited as an autosomal dominant disorder but autosomal recessive and sporadic cases have also been reported. Here, we present a 10-year-old boy with extensive aplasia cutis congenita and limb anomalies as well as mild pachygyria and focal acrania in neuroimaging. No other internal organ involvement was obvious in this patient. Family history was negative for this syndrome. AOS is a multisystem disorder, and so it is crucial to investigate for internal organ involvements.

Homocystinuria is the second most common treatable aminoacidopathy. Clinically, affected patients present with eye, skeleton, central nervous system, and most importantly, vascular system abnormalities. This autosomal recessive disorder leads to accumulation of homocysteine and its metabolites in the blood and urine. In this report, we present a case with clinical and biochemical findings of homocystinuria with stroke and a positive familial history of the disease in her brother. A 4-year-old girl was admitted to pediatric emergency ward because of acute onset of right hemiparesis and subsequent generalized tonic–clonic seizures. Cranial magnetic resonance imaging revealed acute infarct areas in the left cerebral hemisphere. Metabolic screening revealed elevated concentrations of serum homocysteine and methionine and a normal serum concentration of vitamin B12. These findings, along with a positive familial history led to the diagnosis of homocystinuria. In any child who presents with stroke, some rare condition such as homocystinuria should be considered in diagnosis.

Nonneoplastic demyelinating processes of the brain with mass effect on magnetic resonance imaging can cause diagnostic difficulties. It requires differential diagnosis between the tumefactive demyelinating lesion and the coexistence of neoplasm. We document the case of 41-year-old woman with clinical and radiological findings suggestive of multiple sclerosis. Additional investigations confirmed the coexistence of astrocytoma. This report emphasizes the importance of considering brain tumors in the differential diagnosis of primary demyelinating disease presenting with a cerebral mass lesion.

Hirayama's disease is a rare clinical variant of amyotrophic lateral sclerosis where distal muscles are involved more compared to proximal muscles and vice-versa occurs only in 10% cases and so it is differentiated from O'Sullivan McLeod syndrome which involves only small muscles of single limb. Here, we present a case of Hirayama's disease where disease achieved a plateau after 3 years with no further progression. His electrophysiological studies, and clinical picture, and magnetic resonance imaging findings were consistent with a diagnosis of Hirayama's disease.

Most of the vascular lesions on head and neck soft tissue are congenital, but a rare cause can be trauma. A 23-year-old man came to our clinic with a wide pulsatile tortuous mass in the left temporofrontal area. That mass was appeared since 10 years ago. Ten years before his coming to our clinic, he had a blunt trauma in that area. After that, a small wound appeared there and healed gradually. In clinical examination, mass was large and pulsatile, and a fine murmur was detected from it. In paraclinical examination, computed tomography scan with intravenous contrast and sonography revealed a vascular mass with arteriovenous (AV) fistula in soft tissue only in that area. We operated him and vascular mass completely excised without recurrency. Pathologic report was AV malformation (AVM). According to our study, also rare trauma is one of the causes of AVMs, and we able to resection it completely without recurrency.

An orbital defect causes severe facial asymmetry and disfigurement that result in an immense emotional trauma to the patient and is also associated with economic, esthetic, and psychological problems. A prosthetic replacement is the treatment of choice in helping individual to return to his normal life by producing an acceptable and lifelike appearance. This case report describes the critical areas of fabrication of ocular prosthesis for a patient with missing right eye due to trauma to eye ball in an accident. Patient had given a history of surgical enucleation of the eye 15 days after ocular trauma. A polymethyl methacrylate ocular prosthesis was planned. The technique described in this case report presents the use of both custom-made and stock eye shell in an attempt to include the benefits of both. A novel attempt was made to simulate eye movements and exact color matching to that of contralateral eye to provide a better and functional ocular prosthesis to the patient.

To report a case of upper eyelid swelling and ptosis caused by silicone oil migration in a patient after sutureless 23-gauge pars plana vitrectomy and silicone oil injection. A 36-year-old female was referred to eye clinic with total retinal detachment (RD) and superotemporal giant dialysis secondary to trauma in the left eye. Transconjunctival sutureless 23-gauge pars plana vitrectomy and intraocular silicone oil (1300 centistokes) injection were performed. After about 5 months, she came back with the left upper eyelid and periorbital swelling. Palpation of the eyelid revealed soft and mobile subcutaneous small lumps that were not attached to the skin. Computed tomography scan of the orbit showed an 11 mm × 8 mm soft tissue density mass in lateral aspect of the left orbit. Transcutaneously surgical exploration was performed. The patient was seen after 1 week postoperatively and there was a significant improvement of the ptosis. Eyelid swelling and ptosis caused by silicone oil migration after RD surgery are very rare and this is one of the few reports in literature.

Placental site throphoblastic tumor (PSTT) is a rare manifestation of gestational trophoblastic neoplasia that may complicate any type of pregnancy. The disease is unique from other type, and is defined by slow growth, low human chorionic gonadotropin (hCG) serum levels, the late-onset metastatic potential, and most significantly, insensitivity to chemotherapy. We describe a case of a 31-year-old woman with prolonged amenorrhea and slightly elevated serum beta hCG (βhCG) level, referred for termination of abnormal pregnancy. During curettage, necrotic tissue was removed and severs vaginal bleeding was controlled with medical therapy. Histology examination showed neoplastic intermediate trophoblastic cells with invasion to the vessel wall compatible with PSTT. After that, hysterectomy was down and serum βhCG declined to undetectable level 2 weeks after surgery and was followed for 2 years without complication.

Proteus syndrome is a rare sporadic disorder that appears with localized macrosomia, congenital lipomatosis, and slow flow vascular malformations, connective tissue nevus, and epidermal nevus. There are usually some manifestations at birth. The vascular abnormalities that have been reported in Proteus syndrome are capillary and slow flow venous malformation. We report a case of a 10-year-old boy with confirmed Proteus syndrome characterized by high flow vascular malformation (arteriovenous [AV] malformation) unlike the usual vascular malformations seen in this syndrome. This case adds a new perspective to the established clinical findings of the Proteus syndrome.

We report a case of malignant transformation of an intracranial extradural epidermoid cyst into squamous cell carcinoma (SCC), that presented with cerebrospinal fluid (CSF) leakage at the time of recurrence. Intracranial epidermoid cysts are histologically benign and slow-growing neoplasms. They are congenital lesions that develop from ectodermal remnants during neuroembryogenesis. Malignant transformation of epidermoid cysts into SCC is very rare. Various clinical presentations of these tumors after malignant transformation are mentioned in the literature. None of the previous cases, presented with CSF leakage as the recent case did. In cases of malignant transformation, surgical resection and then adjuvant radiation therapy are highly recommended.

Dentures are common accidentally ingested foreign bodies (FBs), especially in the aged population. It is usual for a FB to be swallowed in adults and lodge in the esophagus; however, it is unusual for a denture to remain in esophagus for a period of 9 months without any complication. We present, a 57-year-old deaf mute man swallowed his denture with the chief complaint of dysphagia and odynophagia only after 9 months. Although multiple attempts for removing the denture by rigid esophagoscopy were done, it entered the stomach, so gastrotomy was performed and the patient recovered uneventfully. In all cases with suspicion of esophageal FB, rigid esophagoscopy seems necessary, but in some unusual cases, large FBs may be remained in esophagus for a long time without perforation.

Congenital absence of the pericardium is a rare abnormality that can be diagnosed by cardiac imaging procedures. A 49-year-old male needed medical attention due to the appearance of palpitation with a systolic murmur, and a notable aortic arch deviation was seen in the chest X-ray. In the echocardiogram, a poor echo window was detected. A cardiac magnetic resonance imaging (MRI) showed a rare concomitant anomaly of partial absence of the pericardium including a rare defect of the right-sided aortic arch. Using cardiac MRI, the pericardium can be easily visualized, and thus, its absence more easily detected, aiding appropriate clinical decision-making.

Intratesticular arteriovenous malformation (AVM) is an extremely rare benign testicular lesion. Ultrasonography (US) usually reveals a hypoechoic solid vascular mass within the testicular parenchyma. Herein, we report our experience with a young patient in whom an intratesticular AVM was found incidentally by US during the workup of scrotal trauma.

Symmetrical peripheral gangrene (SPG) is a rare clinical entity, infective, and noninfective both types of etiologies are responsible. The basic underlying pathology in SPG is being disseminated intravascular coagulation and carries a high mortality. Here, we describe a 52-year-old male with dengue fever, who developed bilateral symmetrical dry gangrene of both hand and feet. His dengue IgM antibody was positive. All the peripheral pulses of the affected limbs were palpable. Color Doppler study of upper and lower limb vessels showed normal flow. The patient was managed with intravenous fluids, low molecular weight heparin, and fresh frozen plasma. His general condition was improved within 72 h with no further progression of gangrene. Clinician should suspect the possibility of SPG while dealing a case of dengue fever presenting as peripheral gangrene.

Fungal infections are as a cause of morbidity and mortality in immunocompromise patients. Because the respiratory tract is colonized with Candida, the presence of this agent in respiratory specimens makes the diagnosis of Candida pneumonia problematic. Candida pneumonia is a rare infection, and the majority of cases are secondary to hematogenous dissemination. Furthermore isolated Candida pneumonia originating from endotracheal inoculation is an extremely rare entity. We describe a case of isolated pulmonary candidiasis in the form multiple nodular lesions in a patient with long-term history of diabetes mellitus without evidence of fungemia or systemic involvement who responded to antifungal therapy.

We present a case of primary ectopic frontotemporal extradural craniopharyngioma. Primary ectopic craniopharyngiomas are very rare and have been reported involving the fourth ventricle, infrasellar region, lateral ventricle, temporal area, cerebellopontine angle, clivus, corpus callosum, and prepontine cistern. There was just 1 case of craniopharyngioma previously presented in the literature, with nearly same location as the presenting case.

Parkinson disease (PD) is a neurodegenerative disease of the central nervous system (CNS) with the highest prevalence in adults over 60 years of age On the other hand multiple sclerosis (MS), which mostly affects individuals between 20 and 40 years of age, is another neurodegenerative and autoimmune disease of the CNS, however, less common than PD. Here we aim to report the case of a 39-year-old woman, who developed PD 18 years after diagnosis of MS.

Hemorrhagic pleural effusion is a common clinical entity still diagnosis is often missed. An unusual and often over-looked cause of pleural effusion is an intra-abdominal process including complication arising due to pancreatitis. We report a rare case of massive left sided hemorrhagic pleural effusion in a patient due to pancreatic pathology.

Chorioretinal coloboma is a congenital defect of the eye caused by improper closure of the embryonic fissure. Optic nerve head drusen (ONHD) are white calcareous deposits that are generally asymptomatic. We report a very rare association of both in a healthy patient with no any systemic syndrome. A 16-year-old man was referred to our clinic from suffering blurred vision. Best corrected visual acuity of the right eye was 6/10 and 10/10 in the left one. External ocular and slit lamp examination were normal. Dilated ophthalmoscopy showed marked swelling in both optic nerves and chorioretinal coloboma in the right eye inferiorly. Ultrasonography showed an echodense structure with acoustic shadowing in both eyes consistent with buried ONHD. Visual field testing showed normal field in the left eye and moderate superior field depression in the right eye corresponding to inferior coloboma in funduscopy. Results of general medical and neurologic, cardiologic, and other examinations were normal. To the best our knowledge combination of bilateral ONHD and unilateral chorioretinal coloboma in a healthy patient with no any systemic syndrome has not been published in the literature. We reported this very rare association and recommended examine eyes and other body organs. In such cases that coloboma is associated with ONHD, we should keep in mind Noonan syndrome. The diagnosis of Noonan syndrome is clinical and confirm by the consultant pediatricians and clinical geneticists.

Acute onset bulbar symptoms with respiratory failure and descending paralysis may occur in several neuromuscular disorders including variants of Guillain-Barre syndrome (GBS), diphtheria, botulism and toxins. We present a 51-year-old man who presented with complains of ptosis and dyspnea following pyrethroids spraying in an enclosed area for eradication of flea. Within 5-6 days of admission limb weakness, dysphagia, dysarthria, blurred vision, diplopia, tremor and respiratory distress added to previous symptoms. Temporal profile of events after exposure, development of similar symptoms in patient's son, electrodiagnostic findings and exclusion of other etiologies confirms intoxication etiology. We reviewed the literature and provide an extensive electrodiagnostic overview.

Childhood disintegrative disorder (CDD) is a rare autistic-like clinical condition with unknown etiology, in that previously acquired age-appropriate language, social and adaptive abilities deteriorate significantly in 2-10-year-old healthy children, although physical and neurological evaluations display no observable abnormality. Our case is a 22-year-old female born of a consanguineous marriage, with the appearance of CDD symptoms in her fifth year of age following normal mental and physical development during her initial four years of life. Without any precipitating factor, she gradually lost her language abilities, social relational skills, affectionate behavior, adaptive capacities, peer play and meaningful interest in her surrounding, friends and family members over a period of 4 years, reaching a plateau in her ninth year of age. The unique special clinical symptom in this case is a seasonal total mutism, which after the beginning of her CDD symptoms is revealing every year covering the spring. As no additional physical or psychological change accompanies her total seasonal speech loss, it cannot be attributed to any mental condition known as having a seasonal pattern. Because in the literature CDD is presented mostly as case reports with lacking of advanced research data, describing any new case is recommended to improve the knowledge about this rare condition, especially if it displays some new unusual signs, not reported till now.

Gabapentin is a common drug used as analgesic and anticonvulsant and also is prescribed for insomnia, depression, obsessive - compulsive disorder and panic attack. We report a case of a 48-year-old man who is prescribed gabapentin because of insomnia, headache, and depressed mood. In the first period of using the drug no complication has been seen. However in the next period, side-effects such as hyperesthesia, scaling and severe localized edema has been observed. After several laboratory tests and imaging, no reason was found for his edema. And after discontinuing gabapentin the pain and edema was quite relieved. We found out the brand of the drug has been switched in the second stage. The point which makes our study special is the incidence of side-effects such as severe edema, scaling and hyperesthesia for the first time because of using gabapentin and changing the drug combination.

Herpes zoster is an infectious disease with neurological complications caused by reactivation of varicella zoster virus in dorsal root ganglia of spinal cord which is also known as "Shingles." Suppression of immune system is the major predisposing factor for reactivation of latent virus. Disease is mainly characterized by rash, vesicles and pain along one or more dermatomes which are innervated from one or more spinal nerve roots. Complications may be present after a while despite of patient treatment. Motor involvement is included. Some previous studies showed segmental zoster paresis as a rare complication, a few weeks after first presentation, among immunocompetent individuals. We present post herpetic motor involvement of C5 and C6 in a 59-year-old woman who underwent chemotherapy and radiotherapy due to breast cancer, manifesting left upper limb weakness and paresis, 6 months after left partial mastectomy. Segmental paresis of zoster virus should be considered as a cause of motor impairment in an immunocompromised person suffering from shingles.

Cystic lymphangioma (CL) is a benign lymphatic malformation mostly seen in the head and neck of neonates and infants. Abdominal CL is an unusual entity which may present in omentum, mesentery, abdominal wall, or solid organs. The authors present an unusual case with two separate abdominal cystic lymphangiomas.

Schwartz-Jampel syndrome (SJS), first described in the United States in 1962, is a hereditary disorder characterized by facial dysmorphism and muscle stiffness. We describe the first case of a Persian 9-year-old boy with SJS and review the literature. The child had a short neck, blepharophimosis, flattened face, hypertrichosis of the eyelids, prominent eyebrows, high arched palate, low set ears, micrognathia, short stature, and skeletal deformities. He had proximal muscle hypertrophy, distal muscle wasting and generalized hyporeflexia. Bone X-ray revealed pseudofracture of humerus. Needle electromyography revealed continuous myotonic discharges at rest with no waxing and waning in all tested muscles. Based on clinical and electrodiagnostic findings, the diagnosis of SJS type 1B was made and procainamide was started which resulted in clinical improvement. The diagnosis of SJS should be suspected when a child presents with the triad of myotonia, facial dysmorphism and skeletal deformities.

46, XX gonadal dysgenesis without the phenotype of Turner's syndrome is described as "pure". Although, previous investigations obtained that commonly gonadal dysgenesis did not cause breast development as a result of low levels of circulating estradiol. However, in this study, we aimed to report a familial pure gonadal dysgenesis with and without normal secondary sexual characteristics. In this study, we reported three siblings with pure gonadal dysgenesis with and without normal secondary sexual characteristics. The elder two sisters had a normal female phenotype and the youngest had amenorrhea with no breast development (B1) and pubic hair. In addition, it seems that the absence of pubic hair occurred due to delayed constitutional puberty. According to results, it seems that clinicians should consider different presentations for pure gonadal dysgenesis with familial pattern.

Brucellosis endocarditis is a zoonosis infection of cardiovascular system with world-wide distribution, which is endemic in many provinces of the Iran. The present report describes an exceptional case of fistulization between the aorta and pulmonary artery by Brucella melitensis in a 34-year-old patient. He presented with the complaints of fever and weight loss and congestive heart failure. He was strongly positive for Brucellosis by serological reaction and conventional microbiological cultures from blood and valve tissue were positive. Echocardiography revealed aortic root abscess, cavity formation in aortic ring, large vegetation and native aortic valve destruction with aortic regurgitation and fistula from non-coronary sinus to pulmonary artery and pericarditis. The patient underwent open heart surgery with aortic valve replacement and transpulmonary fistula repair. The patient had uneven full postoperative recovery and with good general condition discharged to home in 16 ^th days of hospitalization.

This report describes the findings on the evaluation of a 9-year-old girl with disabling and pronounced increased writing and painting activities associated with Turner's syndrome and autistic spectrum disorder. She spent most of the time doing these activities which affected not only her academic performance, but also social relationships. A comprehensive treatment plan consists of both biological and psychological aspects, is the main point of this case. Low dose of risperidone (0.5 mg/day) was started to decrease the patient's stereotypic behaviors. Sertraline (12.5 mg/day) was prescribed for her phobia. She was also referred to an occupational therapist in order to improve her social skills.

Ependymomas are glial tumors derived from ependymal cells lining the ventricles and the central canal of the spinal cord. Two thirds of ependymomas arise in the infratentorial or intraventricles, whereas one-third are located in supratentorial space. But supratentorial "cortical" ependymomas are very rare. We report a case of a cortical ependymoma in a 17-year-old boy. The patient presented with transient recurrent right weakness and diplopia. This tumor was located in the left parieto-occipital region and he had gross total excision. Microscopy and immunohistochemistry showed grade III differentiation ependymoma.

Fifty-seven-year-old gentleman, who was a known victim of left-sided clear cell renal cell carcinoma (RCC), presented with isolated huge parietal swelling in left anterolateral aspect of abdomen. He had undergone open left radical nephrectomy 2 years back. Parietal swelling was widely excised and histopathology revealed clear cell RCC, nuclear Fuhrman grade 2.

Toxic epidermal necrolysis (TEN) is a rare and life-threatening allergic drug reaction. We report a 26-year-old young female with end-stage renal disease on maintenance hemodialysis developing TEN while on filgrastim and phenytoin. It was successfully treated with intravenous immunoglobulins and steroids.

The autoimmune disease myasthenia gravis (MG), can mimic a variety of neurological disorders leading to a delay in diagnosis and treatment. On occasions, misdiagnosis of MG could lead to unnecessary therapeutic interventions. We report the case of a 50 year-old man, in whom MG was mistaken for motor neuron disease (MND). Subsequently, correct diagnosis and optimal management resulted in saving his life and significant improvement in his functional status. We discuss the importance of considering MG as one of the potential differential diagnoses among cases of new onset or recurrent unexplained bulbar symptoms, despite exaggerated deep tendon reflexes. Also, a literature review on the misdiagnosis of MG and the potential pitfalls in MG diagnosis are discussed.

The intercostobrachial nerve (ICBN) is often encountered during axillary dissection for axillary lymph node dissection (ALND) for diagnostic and therapeutic surgery for mastectomy. The present report is a case observed in the Department of Anatomy at Vardhman Mahavir Medical College, Delhi during routine dissection of the upper extremity of a male cadaver for first year undergraduate medical students. On the right side , the medial cord of brachial plexus gave two medial cutaneous nerves of arm. Both the nerves were seen communicating with the branches of the ICBN. The ICBN and one of its branches were surrounding the termination of an alar thoracic artery. These peripheral neural connections of the ICBN with the branches of the medial cord can be a cause of sensory impairment during axillary procedures done for mastectomy or exploration of long thoracic nerves. The alar thoracic artery found in relation to the ICBN could further be a cause of vascular complications during such procedures.

Frontotemporal dementia (FTD) is a group of neurodegenerative disorders characterized by atrophy of the frontal and temporal lobes. Clinical features suggestive of FTD include pre-senile onset before the age of 65, behavioral changes, social and interpersonal disinhibition, fluent and nonfluent aphasia, and loss of insight. FTD and parkinsonism linked to chromosome 17 (FTDP-17) was defined during the International Consensus Conference in Ann Arbor, Michigan in 1996. FTDP-17 is an autosomally dominant inherited condition. Most genotypic alterations do not correlate with clinical phenotypes. However, mutations affecting exon 10 splicing are associated with parkinsonism. In the present study, a male case with FTDP who presented with insidious onset of speech difficulty at a young age that was associated with signs of parkinsonism and a positive family history of FTD with MAPT gene mutation at exon 13 has been reported.

Stenotrophomonas maltophilia (S. maltophilia) is a gram-negative bacillus emerging as an opportunistic, nosocomial pathogen associated with a high mortality rate. The organism has been shown to survive several biocides used in the hospital setting. Hospital water sources can serve as a reservoir for S. maltophilia. The transmission of S. maltophilia to susceptible individuals may occur through direct contact with the source or through the hands of health care personnel. S. maltophilia is usually resistant to third-generation cephalosporins, aminoglycosides and antipseudomonal penicillins. These microorganisms are intrinsically resistant to carbapenems, and exposure to these agents has been linked to selection of S. maltophilia. There have also been reports of the organism developing resistance to trimethoprim-sulfamethoxazole (TMP-SMX), which was initially considered as the drug of choice for S. maltophillia infections. We describe a case of nosocomial urinary tract infection (UTI) due to S. maltophilia in a diabetic patient, which the patient developed during treatment with meropenem for UTI due to Klebsiella pneumonia that was resistant to TMP-SMX.

Bronchioloalveolar carcinoma is a form of adenocarcinoma. Its clinical presentation spans the entire spectrum from asymptomatic solitary pulmonary nodule to full presentation with cough, hemoptysis and dyspnea. Clinical symptoms usually are in correlation with the extent of disease. The case we present here is a patient in late stage of disease with few symptoms regarding to the extent of disease involvement.

Hypochondriasis is a persistent preoccupation that despite appropriate medical evaluations and assurance of patient's physical health, the patient insists on having a serious disease. The case which is discussed in this article is a 39-years-old woman that hospitalized for half of her life and no one can perceive her disorder according to her assertions. The mentioned case is a "difficult patient"with fear of oxygen shortage and being choked (Pnigophobia) which leads to continuous tendency to wear oxygen device even during sleep. There is no benefit in exaggerating her condition for herself so there is no fictitious disorder considered. During the therapy she has been assured that she does not have a serious disease and she has learnt to decrease oxygen intake and breath with his mouth. The point that makes this study different from the others is that most of hypochondriacal patients have a fear of getting HIV, cancers, hepatitis and MS but our patient has phobia of pulmonary embolism.

Osteochondroma or exostosis is a bony developmental anomaly, which arises from exophytic outgrowth on bone surfaces in a characteristic manner. Osteochondroma is asymptomatic and grows away from the nearby joint. This paper reports an unusual presentation of osteochondroma in which the patient was surprisingly completely symptomatic. The lesion grew toward the nearby joint and the radiographic findings were not compatible with surgical findings

Erythema nodosum migrans (subacute nodular migratory panniculitis) is a panniculitis characterized by migrating subcutaneous nodules or plaque on the lower extremity. We describe a 75-year-old woman with idiopathic erythema nodosum migrans which was manifest centrifugally spreading, slightly morpheaform erythematous plaque on the lower left leg successfully treated with indomethacin. She was initially diagnosed and treated as a case with cellulitis and with poor clinical response. A biopsy specimen from this lesion showed that the septal was thickening; fibrous tissue was also seen with lymphohistiocytic infiltrate and occasional multinucleated giant cells with some inflammatory cells infiltrated into the periphery of the fat lobules. Erythema nodosum migrans should be kept in mind in the differential diagnosis of any morpheaform centrifugally expended plaque, especially in the lower extremities in cases of unknown etiology.

Cutaneous larva migrans (CLM), a serpiginous cutaneous eruption is the most commonly acquired tropical dermatosis. It is caused by infection with hookworm larvae in tropical and sub-tropical areas, and people who have a history of travel in these countries. The most frequent location of CLM is the distal lower extremities or buttocks. We describe a case of 57-year-old Iranian female patient with CLM of hand (unusual site) without traveling to endemic countries that was successfully treated with oral albendazole. To the best of our knowledge, this is the first report of CLM in Iran.

Nowadays one of the most challenging problems in Medicine is addiction - addiction to sedative drugs such as benzodiazepines. In this article, we are going to describe a case of diazepam addiction that has tolerated a high dose of intravenous diazepam.

We report a case of a 55-year-old woman, who presented with a vague pelvic pain and was found to have an ectopic pelvic kidney involved by a mass. Preoperative assessment was done by multi-slice CT and 3D-CT angiography. According to our knowledge only eight cases of pelvic kidney tumor have been reported in the literature and our case is the first report of using multi-slice CT and 3D-CT angiography in the preoperative evaluation of these cases.

Placenta increta during the first trimester of pregnancy is very rare. This report describes two cases of placenta increta that caused prolonged vaginal bleeding after a first-trimester abortion. We were encountered two cases of placenta increta in October 2012 and May 2013. Case I: A 35-year-old patient with continues vaginal bleeding from 2 months after curettage due to missed abortion in the first trimester. The uterus was large, the human chorionic gonadotropin (BHCG) level was 112 mUI/mL and ultrasound showed an echogenic mass in the lower segment of the uterine cavity. She was a candidate for curettage but received hysterectomy because of massive vaginal bleeding. Pathology reported placenta increta. Case II: A 32-year-old patient in the 12th week of gestation with missed abortion. After 6 weeks from curettage, she returned with continues vaginal bleeding, BHCG = 55 mUI/mL and sonography showing mixed echo lesion in the uterine cavity like hydatiform mole. Total abdominal hysterectomy was performed. Pathology reported placenta increta. In patients with a history of recent first-trimester abortion presenting with prolonged vaginal bleeding, uterine mass and low-level BHCG, a diagnosis of abnormal placentaion should be kept in mind.

The diagnostic accuracy of nuclear medicine reporting can be improved by awareness of these instrument-related artifacts. Both awareness and experience are also important when it comes to detecting and identifying normal (and abnormal) variants. We present a case of hot spot on the upper right chest in the region of right subclavicular region resulting from injection of radiotracer from central subclavian line. A 52-year-old woman with a history of left breast cancer and recent bone pain was referred to our nuclear medicine department for skeletal survey. Anterior views of chest show a focus of increased radiotracer uptake corresponding to anterior arch of one of the right second rib. The nuclear physician reported it as a focal rib bony lesion and recommended radiological evaluation. As technician later explained, physicians realized that injection site was a central subclavian line on the right side and hot spot on that region is due to injection site. The appearance of both skeletal and soft-tissue uptake depends heavily on imaging technique (such as the route of radiotracer administration) and the interpreting physicians should be aware of the impact of technical factors on image quality.

Actinomycosis is a chronic granulomatous suppurative disease having the propensity for extension to the contagious tissue with the formation of multiple discharging sinus tracts. Primary actinomycosis of extremity is a very uncommon clinical entity and is commonly considered as a soft-tissue infection. We report here, a case of primary actinomycosis of the upper extremity in a 24-year-old male who was treated successfully with surgical excision and extended period of antimicrobial treatment.

Oculosporidiosis caused by Rhinosporidium seeberi is a common infectious disease of south India. Here we are reporting a rare case of oculosporidial polyp containing another parasite Enterobius vermicularis as a co infection. This is the first report of its kind in literature. A nine year old school going girl from a rural area presented with a reddish polypoidal lesion from conjunctiva along with complaints of severe itching over it. On examination, the polyp measured 5 mm Χ 5 mm in size, reddish, granular with a tendency to bleed on touch. Her vision was normal. All the basic investigations were within normal limits. A differential diagnosis of conjunctival papilloma, foreign body granuloma and oculosporidiosis were considered clinically. The polyp was removed completely using electro cautery with healthy margins around the lesion. To our surprise histopathological examination of the swelling revealed the presence of two different parasites in the conjunctival polyp. Sections showed sporangia of Rhinosporidium seeberi, fragments of Enterobius vermicularis and its eggs. She was then treated with anti helminthic drugs. Two months of follow up till now showed no recurrence of the lesion.

Hallervorden-Spatz disease (HSD) is a rare disorder characterized by progressive extrapyramidal dysfunction and dementia. Hallervorden and Spatz first described the disease, in 1922 as a form of familial brain degeneration characterized by iron deposition in the brain. Here we present four HSD cases with different clinical pictures.

Erosive pustular dermatosis of the scalp (EPDS) is a rare inflammatory disease with unknown etiology that usually occurs in the elderly. It is characterized by multiple sterile pustules, chronic crusted erosions, cicatricial alopecia, and skin atrophy. It typically develops in aged or sun-damaged skin and is most often accompanied by a history of local trauma. Histopathologically, non-specialized change manifests as atrophic epidermis and chronic inflammation. Although this disease mainly occurs in elderly white women, we here report a case of EPDS in a 35-year-old man, following hair transplantation as a local trauma, that was successfully treated with topical steroid.

Neurofibromas are rare, benign, nerve sheath tumors in the peripheral nervous system. The solitary type is found in those who do not have neurofibromatosis. Solitary neurofibromas are too rare in the giant type. We report a rare case of a solitary giant neurofibroma of the anterior right thigh. The diagnostic criteria, characteristics of imaging studies, and operative approach are represented.

A young male presented to our clinic with 3 months history of shortness of breathness and progressive distension of abdomen. On investigations, patient had renal failure, polycythemia and nephromegaly. A diagnosis of non-Hodgkin's lymphoma was made on renal and lymph node biopsy. Serum erythropoietin concentrations were physiologically inappropriate. - Erythropoietin immunohistochemistry on renal tissue samples demonstrated positive staining for tumor cells. This patient was managed as a case of infiltrative lymphoproliferative disorder with kidney involvement having polycythemia owing to paraneoplastic Erythropoietin production and possibly local hypoxia produced by tumor cells. With maximum efforts, we could not find such an association in the literature.

Disseminated cryptococcal infection is an uncommon initial manifestation in immunocompromised patients. We report a rare case of a 40-year-old female presenting with fever and burning epigastrium. Peripheral blood film revealed a leukoerythroblastic picture with thrombocytopenia. Bone marrow aspiration showed granulomas along with cryptococcal yeast forms. The ELISA test for detection of human immunodeficiency virus (HIV) antigen was positive. Disseminated cryptococcosis can develop as the first manifestation of HIV infection in previously healthy individuals and granulomas in such bone marrow aspiration smears are a valuable clue to an underlying opportunistic infection.

Congenital micronychia may involve big toes or may involve other nails. The etiology of micronychia is not clear but amniotic bands, teratogens (drugs, alcohol), Nail Patella Syndrome etc. A 44-year-old woman with multiple isolated congenital micronychia over her hands and feet was selected. The major affected nails were thumbs and Index fingers. Surgical method were done step by step: Anesthesia of the area, extraction of short nail, elevation of nail bed, longitudinal nail bed incisions, suturing the lateral nail bed to the nail wall, covering the nail bed by a splint of plastic suction tube, bandage with gauze Vaseline. Finally, we hypnotized that in congenital micronychia, the main pathology is in nail bed; through this theory by nail bed expansion better outcomes are coming.

Traditionally, the management of chronic osteomyelitis emphasizes the excision of necrotic and infected material (sequestrectomy/debridement) followed by prolonged administration of antibiotics. Most children with chronic osteomyelitis undergo surgery with the inherent risk of damage to their growth plate. Treatment regimen based on findings of imaging with emphasis on antibiotics to potentially reduce the rate of surgical interventions is being increasingly reported. An 8-year-old thin built Indian boy belonging to lower socio-economic group presented to the orthopedic department with the chief complaints of pain in the left upper leg for the last 3 months. Radiograph of the affected limb showed features of chronic osteomyelitis with a large diaphyseal sequestrum on the medial cortex of tibia with incomplete involucrum. No surgery was performed; not even incision and drainage. The sinuses healed completely in 6 weeks time with appropritate antibiotics alone. Gradually, over a period of 8 months, the large tibial diaphyseal sequestrum got fully incorporated into the healthy diaphyseal bone indistinguishable from normal bony architecture with complete clinical remission of sepsis. Our rare case is an example of the evolving notion that antibiotics and supportive care alone may be sufficient enough in the treatment of chronic osteomyelitis even with large diaphyseal sequestrum in paediatric cases where excellent healing potential of the immune-competent child may potentially make surgical intervention redundant.

Leiomyoma is benign mesenchymal tumor, that frequently occur in uterus but it rarely happens in ovary. Ovarian leiomyomas are seen concomitantly with uterine leiomyoma in about 78% of cases. They often discover incidentally but their most clinical manifestations are abdominal pain and palpable mass. Herein, we reported a large ovarian leiomyoma in a 22-year-old woman with abdominal pain and palpable mass at lower abdominal region. Computed tomography scan revealed large adnexal mass. Microscopic appearance was typical for leiomyoma but because its rarity in ovary the immunohistochemical staining was done. Major differential diagnostic considerations for this tumor in ovary are fibroma/thecoma, sclerosing stromal tumor, and leiomyosarcoma. The immunohistochemical staining with desmin, inhibin, and α-smooth muscle actin are helpful to rule out this differential diagnosis.

Ewing's sarcoma is a rare malignant neoplasm that comprises approximately 4-6% of primary bone tumors. In most cases, femur and pelvis are affected, and less commonly the head and neck areas (in the jaws, usually the mandible). These tumors have been reported more frequently in males, mostly aged 5-20 years old. Systemic symptoms and signs such as fever, weight loss, anemia, leukocytosis, and elevated erythrocyte sedimentation rate (ESR) may be the first signs in oral Ewing's sarcoma. Such signs and symptoms are also seen in odontogenic infections and abscess. In one case, the patient went to a dentist with pain, swelling, and abscess similar to odontogenic infection and patient's tooth was pulled due to misdiagnosis. This tumor has an aggressive clinical behavior and is identified with rapid growth and high probability of metastasis at diagnosis. Thus, it is necessary to differentiate it from a dental abscess. As for the treatment of Ewing's sarcoma, first the tumor must undergo chemotherapy to reduce its size and, eventually, it undergoes extensive surgery. This case report deals with a 16-year-old patient wrongly diagnosed with odontogenic infection and abscess, and hospitalized. As the symptoms did not remit, biopsy was carried out and the patient was operated on with Ewing's sarcoma diagnosis.

Neurogenic tumors, especially paraganglioma of larynx, are rare. In this article, we present a 64-year-old woman who complained of intermittent dysphagia to solid foods. Further evaluation revealed a supraglottic paraganglioma and she was treated successfully by total excision of tumor.

Systemic sclerosis sine scleroderma is a subtype of scleroderma, which is characterized by involvement of visceral organs, but no characteristic skin alteration. The involved organs could be kidneys, heart, gastrointestinal system, and lungs. Interstitial lung disease (ILD) is one of the pulmonary manifestations of sine scleroderma. We report a 38-year-old woman presenting with chill, fever, generalized malaise, dyspnea on exertion, and dry cough with a history of Raynaud's phenomenon, who was evaluated by physical examination, spirometry, and computed tomography scan, that all lead to the diagnosis of ILD. Combination of high-titer positive anti-nuclear antibody, high erythrocyte sedimentation rate, positive C-reactive protein, and ILD could be explained by sine scleroderma.

Osteosarcoma is the most common primary malignant tumor of the skeleton involving predominantly metaphysis of the long bones. Diaphyseal osteosarcoma is a rare form, which accounts for approximately 10% of all cases of osteosarcomas. Osteosarcoma contains a family of lesions with considerable diversity in histologic features and grade. We present a case of a 29-year-old male with diaphyseal osteosarcoma of the left tibia, who presented with pain and swelling over middle one-third of the left leg. The biopsy of the lesion revealed varied histomorphologic features in a small tissue studied. This prompted us to report the various histologic patterns of osteosarcoma.

Small colony variants (SCVs) of Staphylococcus aureus often cause persistant and relapsing infections. SCVs are characterized by a strong reduction in growth rate, atypical colony morphology and unusual biochemical characteristics. We here report a case of chronic oesteomyelitis caused by SCV of Staphyloccous aureus in a middle aged male patient.

Twenty-eight-year-old female while undergoing a medical termination of pregnancy (MTP) encounter complete urethral loss and massive bladder curettage. After resuscitation, she developed continuous urinary leakage followed by progressive decline in urine output. Case is highlighted here because of the massive vesico-urethral trauma because of MTP, leading to permanent urinary diversion.

Signet ring carcinoma is a common type of adenocarcinoma of stomach but its occurrence in ampulla of Vater is extremely rare. There are only a few previous reported cases of signet ring carcinoma of ampulla of Vater. Here we reported a 61-year-old woman with obstructive jaundice. Ultrasonography and computed tomography (CT scan) examination showed intra- and extrahepatic bile duct dilatation. Endoscopic examination with biopsies revealed a small-size mass in ampulla of Vater with diagnosis of signet ring carcinoma. On consequent pancreatoduodenectomy the tumor was diagnosed as T2N0M0, stage IB. Because of the specific site of signet ring carcinoma of ampulla of Vater, the tumor seems to present itself at an early stage of disease. We review in the literature to suggest our idea.

Inflammatory pseudotumor (IPT) is an uncommon tumor and its occurrence in spleen is rare. This tumor is composed of proliferation of spindle cells of unknown origin and etiology that mimic other tumors at clinical and histological evaluation. The most surmising etiology is Epstein Barr virus (EBV) and the most suspected origin is myofibroblasts, hence its synonym is "inflammatory myofibroblastic tumor." The clinical appearance of tumor is related to its location but the most ones are abdominal pain, fever and weight loss. Surgical removal for this lesion is treatment of choice and there is few reported case with recurrence and metastasis. Herein we report a 63-year-old female patient with and abdominal discomfort that primary paraclinical investigations had been showed splenic mass and therefore surgical treatment was performed for her. Microscopic examination suggest some different diagnosis such as IPT, thus immunohistochemical staining was perform to confirm the diagnosis and rule out the others.

Pulmonary arteriovenous malformations (PAVM) are rare pulmonary vascular anomalies. Although most patients are asymptomatic, right to left shunt produced by PAVM, could result in easy access of septic or non-septic emboli to systemic circulation, end to serious central nervous system (CNS) complication. Here we report a case of brain abscess in a young man. Its source was initially unknown but multiple arteriovenous malformations were detected incidentally in his thoracic CT, which was performed for ruling out embolism. Although the cases of brain abscesses associated with PAVM are very rare, the brain abscess could be an initial clinical manifestation in asymptomatic PAVM as in the case presented in this report.

Upper extremity deep vein thrombosis (UEDVT) is a life threatening rare condition. Among the patients with UEDVT, internal jugular vein is accepted as uncommon thrombosis. Since internal jugular vein thrombosis (IJVT) is mostly underdiagnosed, a great attention is needed to diagnose the disease. A 75-year-old woman with history of dyspnea on exertion (DOE), weight loss and anorexia with the stable vital sign and right-sided cervical adenopathy with suspected thyroid nodule was admitted. One day after admission, sudden dyspnea was occurring with unstable vital sign and hypotension. Chest X-ray showed a bilateral pleural effusion. Pleural tap was exudative, CT scan showed bilateral pulmonary emboli. CT scan of the neck showed right-sided cervical adenopathy, heterogeneous thyroid and internal jugular vein thrombosis concomitant with superior thyroid vein thrombosis. The patient was treated with thrombolytic agent and enoxaparin followed by warfarin. Thyroid FNA revealed papillary thyroid carcinoma that followed by total thyroidectomy, histopathological examination verified papillary thyroid carcinoma. Although lower extremity DVT is the main cause of pulmonary emboli, but IJVT may be proceed by pulmonary embolism. Due to fatal outcome of pulmonary emboli in IJVT, color duplex sonography, is recommended in documented tumors or suspected history of malignancy.

Streptomyces are saprophytic soil organisms rarely known to cause invasive infections. Streptomyces is the largest genus, producing antibacterial, antifungal and antiparasitic drugs. The case was a 24-year-old man, admitted for sudden dyspnea, fever and sputum and decreased sound in the left lung. The chest X-ray showed hydropneumothorax. After chest tube insertion, lung expansion did not happen. Pleural effusion was exudative with gram-positive bacillus and Streptomyces in culture. Owing to symptoms of Cushing in history, examination and laboratory work-up for Cushing was done and finally he underwent bilateral adrenalectomy. The patient was on antibiotic broad spectrum antibiotic and then was changed to antibiotic as organism was sensitive to and discharged with clarithromycin for 6 months. Streptomyces happens in immunodeficient patient. Diagnosis is based on culture and contamination was ruled out. Treatment period is longer for patients owing to slow growing nature.

Pulmonary arteriovenous fistula (PAVF) is a venous malformation that permits right to left shunting of blood, bypassing the pulmonary capillary bed. Often PAVFs are seen in association with hereditary conditions. On the other hand, isolated PAVFs are rare and asymptomatic. There have been few reports of isolated PAVF related complications. A patient was referred to us with dysarthria and diplopia and history of surgically-treated PAVF. Further evaluations revealed a stroke in thalamic region. We found an open PAVF in a case of thalamic stroke.

Tracheal chondrosarcoma is a rare malignant mesenchymal tumor and there are less than 15 reports in the literature. We report a rare case of laryngotracheal chondrosarcoma in a 74-year-old man. He gave a history of radioiodine therapy for thyroid papillary carcinoma about 24 years ago. Diagnostic steps, histological presentation, and therapy are described in detail.

Surgical treatment of brain tumors, especially those located in the eloquent areas such as anterior temporal, frontal lobes, language, memory areas, and near the motor cortex causes high risk of eloquent impairment. Awake craniotomy displays major rule for maximum resection of the tumor with minimum functional impairment of the Central Nervous System. These case reports discuss the use of awake craniotomy during the brain surgery in Alzahra Hospital, Isfahan, Iran. A 56-year-old woman with left-sided body hypoesthesia since last 3 months and a 25-year-old with severe headache of 1 month duration were operated under craniotomy for brain tumors resection. An awake craniotomy was planned to allow maximum tumor intraoperative testing for resection and neurologic morbidity avoidance. The method of anesthesia should offer sufficient analgesia, hemodynamic stability, sedation, respiratory function, and also awake and cooperative patient for different neurological test. Airway management is the most important part of anesthesia during awake craniotomy. Tumor surgery with awake craniotomy is a safe technique that allows maximal resection of lesions in close relationship to eloquent cortex and has a low risk of neurological deficit.

Primary splenic cyst is a relatively rare entity; they comprise only about 10% of benign non-parasitic cysts. Most of these are asymptomatic and are observed incidentally during abdominal ultrasonography. The number of diagnosed splenic cyst cases seems to have risen because of the increasing use of abdominal imaging techniques. However, definite diagnosis is possible only after splenectomy when epithelial lining is confirmed histologically. We report a case of a 14-year-old child who presented with fullness of abdomen and pain in the left hypochondrium since last few months. First impression made was in favor of a splenic lymphangioma of size 8 × 8 cm which was based on a Computerized tomography scan and the sonographic findings. Splenectomy was performed and was sent for histopathological examination which in combination with immunohistochemistry revealed findings suggestive of primary epithelial cyst. A large cystic mass with a relatively thin wall localized in the spleen is likely to be a primary or secondary cyst. The diagnosis of false cyst should be favored if there is a clear history of trauma, if the patient is older than fourth decade, if there is a hematoma elsewhere in spleen, or if cyst wall is calcified. This rare entity should be considered in the differential diagnosis in a patient presenting with left hypochondrial pain.

Clear cell hidradenoma or nodulocystic hidradenoma or acrospiroma are histologically distinct relatively rare tumors of sweat gland duct origin, found mainly in adults with a female preponderance. We report a case of eccrine hidradenoma in a 31-year-old man who presented with an asymptomatic, solitary nodule on occipital region. A few reports are available in literature regarding presence of this tumor on occipital region of young man and present case is being reported because of its rarity in this region of scalp and in this sex.

Gallbladder cancer is found in about 1-2% of patients after laparoscopic cholecystectomy and it is difficult to diagnose preoperatively. Laparoscopic cholecystectomy may disseminate gallbladder cancer to peritoneum and even port sites. Here, we present a case of a 59-year-old female patient operated for gallstone disease and her histopathology was suggestive of well-diffentiated gallbladder carcinoma (T1N0M0). Patient presented to us with port site and distant peritoneal metastases after 3 months.

Multiple sclerosis (MS) and celiac disease (CD) are considered to be T-cell-mediated autoimmune disease. We discuss about a known case of CD-showed relapsing - remitting neurological symptoms compatible with MS. In this rare co-occurrence subject, MS-CD patient, the interaction between MS - and CD-related inflammatory processes is open to discussion.

Peutz-Jeghers syndrome is a rare autosomal dominantly inherited condition, characterized by the presence of hamartomatous gastrointestinal polyps and mucocutaneous pigmentation. Patients with this syndrome can be associated with other neoplasms such as ovarian neoplasms known as sex-cord tumor with annular tubules that are associated in one third of the cases with this syndrome and other types of malignancies. We report a 42-year-old woman with a history of Peutz-Jeghers Syndrome and bilateral breast cancer that presented with abnormal uterine bleeding. Total abdominal hysterectomy with bilateral salpino-oophorectomy was done and an ovarian sex cord tumor with annular tubules was incidentally diagnosed. By reviewing literatures and in agreement with previous studies we suggest routine screening for malignancies in patients with Peutz-Jeghers syndrome.

Thalassemia is a hematologic disorder that causes ineffective hematopoiesis and is related to severe anemia, iron overload, extramedullary hematopoiesis, and hepatomegaly. Hepatomegaly is related to significant extramedullary hematopoiesis. The other sites that are involved in extramedullary hematopoiesis are spleen, lymph nodes, paraspinal regions, kidney, pleura, and intestine, but intracranial involvement is a rare presentation. We discuss about a case with intracranial medullary hematopoiesis in a thalassemic patient.

Tuberculous dactylitis is a distinctly uncommon, yet well recognized form of tuberculosis involving the small bones of the hand or foot. It occurs in young children in endemic areas under 5 years of age. Tuberculosis of the short tubular bones like phalanges, metacarpals or metatarsals is quite uncommon beyond 6 years of age, once the epiphyseal centers are well established. The radiographic features of cystic expansion have led to the name " Spina Ventosa" for tuberculous dactylitis of the short bones. Scrofuloderma is a mycobacterial infection affecting children and young adults, representing direct extension of tuberculosis into the skin from underlying structures e.g. lymph nodes. An 8-year-old malnourished girl had multiple axillary ulcers with lymphadenopathy. Tuberculous dactylitis with ipsilateral axillary scrofuloderma was suspected on clinical and radiological grounds. The suspicion was confirmed by histology and bacteriology. The patient responded to antitubercular drugs with progressive healing of the lesions without surgery. Concomitant presence of these dual lesions suggesting active disseminated tuberculosis in immune-competent child over 6 years is very rare and hardly reported.

Colloid milium is a rare cutaneous condition with at least three distinct subtypes, characterized clinically by the development of yellowish translucent papules or plaques on sun-exposed skin, and histologically by the presence of colloid in the dermal papillae. In this case report, we present a man with multiple small papules on dorsum of his hands that in pathology confirmed to be colloid milium. Colloid milium is more commonly observed in fair-skin patients and remain unchanged; however our patient had dark skin type (Fitzpatrick skin type III) and lesions were increasing in summer and decreasing in winter.

The classic presentation of malaria with paroxysms of fever is seen only in 50-70% of the patients. The development of immunity, the increasing resistance to anti-malarial drugs, and the indiscriminate use of anti-malarial drugs have led to malaria with the presentation of unusual features. Cerebellar ataxia, extrapyramidal rigidity and various psychiatric symptoms have been described either as early manifestations of cerebral malaria or as a part of post malaria neurological syndrome. In this case report, we will discuss one such patient of falciparum malaria infection who developed midline cerebellar signs, and responded to anti-malarial treatment.

Our proband is a 29-year-old man, who is affected with soft cleft palate and hypernasality. A study of about six generations of this family pedigree shows that cleft palate has repeatedly occurred in males, with probably a X-linked recessive pattern of inheritance. Interestingly, the sister of the proband is affected with hypernasality and she has an affected son. This is the first report of X-linked inheritance pattern of cleft palate in Iran.

Myoepitheliomas are benign neoplasms of salivary glands derived from myoepithelial cells. These tumors can occur at any age but are most common in young adults. This tumor is usually located in the parotid gland and the minor salivary glands of the soft palate and represents less than 1% of all salivary gland tumors. The myoepithelioma is classified in the follow cells types: spindle, plasmacytoid, reticular, epitheliod, and clear, additionally, mixed histological forms are described. The plasmacytoid myoepithelioma from palate salivary glands is considered as a rare entity. A 45-year-old lady presented with an asymptomatic, well-circumscribed, solid mass located on the hard palate, which was gradually increasing in size. A clinical impression of Pleomorphic Adenoma was made which on histopathological examination revealed cords, clusters, and sheets of homogenous, large cells with plasmacytoid characteristics and a prominent eosinophilic cytoplasm. Ductal and acinar differentiation were absent thus ruling out the pleomorphic adenoma, whereas, features consistent with plasmacytoid myoepithelioma were evident.

Eclampsia remains a problem in the developing countries despite improvements in antenatal care and emergency obstetric facilities. It is an important cause of maternal morbidity and mortality in Nigeria. A 26-year-old primipara, residing in an urban city in Nigeria with antenatal care facilities, booked for antenatal care at 36 weeks of gestation and was then diagnosed with severe pre-eclampsia. She initially refused therapy and was later booked for an emergency cesarean section. She had eclamptic fits during cesarean section under spinal anesthesia, and the seizure was aborted with intravenous diazepam. The postoperative period was uneventful. Progression of pre-eclampsia to eclampsia during cesarean section under spinal anesthesia is rare, but it can occur. Early booking for antenatal care to enable an early diagnosis and treatment are necessary to prevent the progression of pre-eclampsia to eclampsia. There is need to educate the populace on the importance of ante natal care so as to improve its utilization.

Wilson's disease (WD) or hepatolenticular degeneration is an inherited neurodegenerative disorder of copper metabolism (autosomal recessive, chromosome13). Psychiatric disorders in WD include dementia, characterized by mental slowness, poor concentration, and memory impairment. Symptoms may progress rapidly, especially in younger patients, but are more often gradual in development with periods of remission and exacerbation. Delusional disorder and schizophrenia-like psychosis are rare forms of psychiatric presentation. In this report, the patient with WD presented by psychosis symptoms and treated mistaken as schizophrenia for almost ten years. Although he has treated with antipsychotics, he had periods of remissions and relapses and never was symptoms free. Since psychosis can be the manifestation of medical diseases such as WD, overall view of these patients is necessary and medical diseases should be considered as a differential diagnosis.

Generally, skeletal peripheral metastases below the elbow and the knee are rare. Skeletal metastases to the hand or foot are very rare; but when they do it may be a revealing clinical finding. Purely lytic lesions are commonly seen in metastases from lung, renal, and thyroid tumors, but they are also known to occur in primary myeloma, brown tumor and lymphomas. A 70-year-old man was brought to the emergency department with acute painful swelling involving his right hand and the right knee. Due to significant accompanying soft tissue swellings cellulitis, acute osteomyelitis and gouty arthropathy were included in the initial differential diagnosis. Radiographs showed pure lytic bony lesion with complete disappearance of lower two third of the second metacarpal, trapezium and trapezoid bones of the right hand along with a lytic subarticular lesion of medial condyle of ipsilateral femur. Chest X-ray (CXR) was normal but sonography of the abdomen readily demonstrated a large renal mass, later confirmed at biopsy as renal cell carcinoma (RCC). Clinicians should be cognizant of the strong association between digital acrometastases and renal cell carcinoma in male patients with normal CXR findings. In suspected hand acrometastasis associated with a soft tissue component outside the contours of normal bone, screening the abdomen by sonography should be done prior to bone biopsy and before costly or time-consuming investigations are offered. Metastatic RCC should be included in the differential diagnosis of all unilateral expansile bony lesions of the digit. It is particularly important if such lesion/lesions are accompanied by local inflammation. Screening the abdomen by sonography may be of particular value in such elderly male patient when Chest X-ray shows no abnormality.

Linguatulosis occurs accidentally in human by ingestion of raw or undercooked visceral tissues of sheep, goat, camel, and other herbivores. In this case report, 2 patients from an Afghan family infested with this parasite in rural area of Isfahan, Iran are presented. Clinical signs and symptoms including pricking sensation in throat, coughing, sneezing, yellow nasal and ears discharges, which started 2-3 hours after eating raw goat liver. Nymphs of Linguatula serrata were isolated from their tongues and later, several more parasites were discharged by coughing and sneezing. An ancient belief about nutritional benefits of eating raw liver may causes more Halzoun syndrome in future.

Fibro-osseous pseudotumor of the digit is an unusual ossifying soft tissue lesion, which is usually an ill-defined soft tissue mass in radiography, with focal calcification, especially in the proximal phalanx. It predominantly affects young adults and, unlike myositis ossificans, is more common in women. The current case is a 30-year-old man who presented with pain and swelling on the dorsum of middle phalanx of the left index finger without history of trauma. Diagnosis of this lesion requires a high index of suspicion and should be differentiated from myositis ossificans, turret exostosis, and extra-skeletal osteosarcoma, which are discussed. This lesion is considered benign and has an excellent prognosis following complete removal and local recurrence is unusual. No cases of malignant change are on record.

Mucormycosis is a fatal invasive infection which mostly involves diabetic or immunosuppressed patients. Early diagnosis, improving immunosuppression, systemic antifungal therapy, and surgical debridement are necessary for successful treatment. In this case study, we represent a known case of Wegener's granulomatosis (WG), with concomitant sinusal mucormycosis mimicking vasculitic disease relapse, which was successfully treated with surgical debridement, amphotericine, and intravenous immunoglobuline.

Ureteropelvic junction obstruction (UPJO) and vesicoureteral reflux (VUR) are two of the most common urologic problems in children that sometimes coexist simultaneously in a patient. However, presentation of UPJO after VUR treatment is rare. We will present two cases and discuss diagnostic and therapeutic aspects of the condition.

One of disastrous complications of central venous cannulation (CVC) is malposition of central venous catheter. In this case report, we present an adult patient with history of multiple trauma and intracranial hemorrhage in whom the right axillary vein was accidentally cannulated during CVC insertion.

Leeches are blood-sucking hermaphroditic parasites that attach to vertebrate hosts, bite through the skin, and suck out blood. When leeches feed, they secrete an anticoagulant (hirudin), which helps them get a full meal of blood. This is the first report of leech removal from external auditory canal. Previous leech involvement cases were explained in nasopharynx, larynx, pharynx, eye, and gastrointestinal tract. Prominent sign of all cases was active bleeding from the leech attachment site; that stopped with leech removal. A 24-year-old man was presented to Al-Zahra hospital with left otorrhagia and otalgia from 2 days ago. After suction of ear a small soft foreign body was seen in the external ear near the tympanic membrane, then the ear filled with glycerine phenice, the patient explained decreased movement of foreign body. Four hours later the bloody discharge stopped and otalgia decreased. After suction of clots, a leech was extruded from external auditory canal by alligator. Leech infestation is a rare cause of otorrhagia and should be suspected in the endemic region in all of unusual bleeding; it can be diagnosed and treated by exact inspection and removal.

Developmental stuttering is a common disorder of speech dissiliency that is characterized by excessive repetitions of sounds, syllables, and monosyllabic words, as well as sound prolongations and complete blockages of the vocal tract. About 60 million people are affected and it is more common between the age of 3 and 6, when children begin forming sentences and connecting thoughts verbally. There are three types of stuttering known as developmental stuttering, neurogenic stuttering, and psychogenic stuttering. The exact pathophysiology of developmental stuttering is unknown; however, various family and twin studies have repeatedly implicated heredity as a major factor in the etiology of stuttering. It is clear that the genetic influence is not in the form of an exact single gene effect such as autosomal recessive, autosomal dominant, or x-linked in all families; however, in all of the inheritance forms it is influenced by sex with higher occurrence in males than females at a ratio of 4:1 in older children and adults. Recently special genetic locus has been determined on several autosomal chromosomes related to developmental stuttering. In this report, the proband is a 20-year-old boy was referred to our clinic for premarriage genetic counseling; he has been affected since 3 years and now is under cure. three generation study of his family show 13 individuals are affected by stuttering. For the first it occurred in the proband's grandfather and after this time about all of affected cases has been seen in consanguineous marriages. Therefore, the genetical inheritance of stuttering is crystal clear in this family and autosomal recessive inheritance pattern is proposed. Totally in such families with repeated occur of stuttering, we cannot account it as a multifactorial disorder.