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Acrodysostosis is a rare syndrome of peripheral dysostosis, neurodevelopment delay, and skeletal abnormalities. The most common bone changes are peripheral dysostosis with severe brachydactyly of the lesser toes. The first ray of the feet is often not affected or may present hyperplasia resulting in an unbalanced transverse arch of the forefoot, with potential negative effects on function. Additionally, the insufficiency of the lesser toes may be associated with complex congenital hallux valgus deformities. Surgical approaches include growth plate epiphysiodesis, bulk reduction procedures, bone shortening, osteotomies, or amputation. Here, we report a case of a 14-year-old girl with acrodysostosis and severe discrepancy of the first ray and hallux valgus deformity simultaneously treated by a modified scarf osteotomy. Levels of Evidence Level V: Case report

Silent Sinus Syndrome is a rare syndrome that usually involves the maxillary sinus. In this case report, we present a rare case diagnosed as Silent Sinus Syndrome with frontal, ethmoid and maxillary sinus involvement which was presented with periorbital complaints after the rhinoplasty operation performed in our clinic.

Roberts Syndrome is an extremely rare syndrome reporting about 150 cases in the literature, with a very low survival rate. The authors present a case of a female patient with Roberts Syndrome who also had a coronal craniosynostosis. The aim of this case report is to present a case of a patient with Roberts Syndrome with a brachycephaly that required management of fronto-orbital advancement. In conclusion Roberts Syndrome is a rare disease, which can have different skeletal variations. This syndrome can manifest itself with craniosynostosis, with the requirement of a comprehensive management to correct it and avoid compression of the brain with endocranial hypertension.

We report the case of a 63-year-old white man who, 3 days after stent removal of endoscopic drainage of pancreatic cysts, developed a penile necrosis due to purpura fulminans (PF) that has been misdiagnosed as Fournier's gangrene. Penile necrosis was rapidly followed by a lethal multiorgan failure due to disseminated intravascular coagulopathy (DIC), triggered by the subsequent development of a severe acute pancreatitis. PF describes a rare syndrome involving intravascular thrombosis and hemorrhagic infarction of the skin. Although reports of penile necrosis secondary to various causes are documented in the literature, penile necrosis secondary to PF in the setting of acute pancreatitis is a rare event. Histopathologic studies of the skin showing an occlusive nonvasculitic vasculopathy are the first step to achieve an accurate diagnosis.

Cerebro-costo-mandibular syndrome (CCMS) is a rare syndrome characterized by posterior rib ossification gaps and orofacial anomalies. Posterior rib ossification gaps are the hallmark of the disease and mimic multiple old rib fractures. The condition is caused by heterogeneous mutation of Small Nuclear Ribonucleoprotein Polypeptides B and B1 (SNRPB) gene on the chromosome 20p13. There is scanty literature regarding the extent of bony changes and the associated pulmonary-airway abnormalities on computed tomography (CT) imaging. In this case report, we presented a detailed CT imaging features of a case of CCMS in a male infant. This report highlighted on the nature of osseous defects, structural changes in the chest wall, airways, lungs, and a wide range of spinal anomalies. Some of our observations regarding the changes in the airway and chest wall are likely to have bearing on evaluating the prognosis and the need for respiratory support.

Introduction: Nutcracker syndrome (NCS) is a rare syndrome, where symptoms can be debilitating, severely affecting quality of life. Patients who fail conservative management can be offered...

Case We present a long-term follow-up on a woman with otospondylomegaepiphyseal dysplasia (OSMED). At the age of 46 years, she is one of the oldest patients with the syndrome in the literature to date. We focus on the musculoskeletal anatomy and orthopaedic interventions over her lifetime. Conclusion OSMED is a very rare syndrome. Arthritis and joint pains presented in her early adolescence and progressed to the point of requiring joint replacements by her 20s. Early intervention and monitoring improved the quality of life for this patient.

Among the multiple birth defects is fibular aplasia, which is a rare congenital frequency that is part of a low prevalence syndrome called FATCO, fibular aplasia (fibular aplasia), campomelia of the tibia (Campomelia tibial) and oligosyndactyly (Oligosyndactyly). We present the case of a male man of 31 months of age evaluated in the courtesy and prosthesis consultation in a rehabilitation house in the city of Bogota, Colombia, with a history of FATCO syndrome and the family history of malformations abroad. Given the paucity of reports on this rare syndrome and the lack of a standardized treatment approach, evaluation of the prosthetic rehabilitation process is important.

A large solitary intrathoracic mass was identified in a patient with concurrent immunodeficiency. The patient was found to have a type AB thymoma and was diagnosed with Good’s syndrome. A case report demonstrates the importance of timely surgical intervention for this rare syndrome.

The first report of an abdominal wall dyskinesia syndrome was published in 1990 by Iliceto et al. (1990), who were the first to use the “belly dancer dyskinesia” (BDD) nomenclature. BDD is a rare syndrome involving repetitive, involuntary, and continuous movements of the abdominal wall muscles (Iliceto et al., 1990). We describe the case of a 79-year-old, right-handed woman who showed symptoms of BDD syndrome after using levodopa due to parkinson's disease (PD). In this report, BDD was treated by stopping levodopa. After 15 days without levodopa, a positive outcome was achieved. The patient no longer exhibited BDD at the six-month and one-year follow-ups.

Pentalogy of Cantrell is a rare syndrome consisting of a constellation of congenital anomalies including omphalocoele, lower sternal defect, sdiaphragmatic defect, diaphragmatic pericardial defect and congenital heart disease. It is reported to be usually fatal in absence of timely treatment. We present a case where an untreated patient has miraculously survived into the sixth decade.

Ortner’s syndrome is rare syndrome characterised by hoarseness of voice due to recurrent laryngeal nerve involvement in cardiovascular disease.

Objective To demonstrate robot-assisted hysterectomy and bilateral orchidopexy in a patient with persistent Mullerian duct syndrome (PMDS). PMDS is a relatively rare disorder of sexual development, patients have a 46, XY karyotype and normal male external genitalia, but internal Mullerian duct structures. Usually these phenotypic males have unilateral or bilateral undescended testes, bilateral fallopian tubes, a uterus, and an upper vagina draining into a prostatic utricle. Methods We present a 48-year-old gentleman that was referred to our institute as a case of primary infertility and bilateral undescended testes. Results Diagnostic laparoscopy revealed a uterus, fallopian tubes with bilateral gonads that were biopsied and showed fibrotic seminiferous tubes. Due to the probable risk of malignant transformation of the remnant Mullerian structures, we counselled the patient for robot-assisted hysterectomy, bilateral salpingectomy and bilateral orchidopexy, upon which he agreed to proceed. Conclusion Our video demonstrates this rare syndrome and shows the feasibility of performing such a procedure robotically with good results.

Snapping elbow is a rare syndrome caused by dislocation of the medial head of the triceps. Simultaneous dislocation of the ulnar nerve occurs even less frequently. We report a case of ulnar nerve dislocation accompanied by dislocation of the triceps over the medial humeral epicondyle. The ulnar nerve was located in front of the medial epicondyle and did not change position with the elbow flexed or extended. At the point of insertion, the proximal end of the triceps muscle was displaced anteriorly. In this case, diagnosis by physical examination was challenging, but ultrasonography proved useful for diagnosis. During surgical treatment, partial resection of the triceps medial head was achieved, and the patient demonstrated a full recovery.

Highlights • This case is a rare presentation of C. sordellii toxic shock syndrome resulting from a Caesarian section. • This is the only obstetric case of C. sordellii to have survived. • Extreme levels of a leukocytosis and hypotension without fevers raises suspiscion for this severe infection. • Broad spectrum antibiotics, including coverage for anaerobic organisms, and surgical intervention is the standard of care. • At the moment, no early detection or antitoxins are commercially available., Introduction Clostridial toxic shock is a rare syndrome traditionally described in case studies and reviews as a uniformly fatal condition associated with post-partum and post-abortion. Clostridium sordellii was first mentioned in scientific literature as a human pathogen in 1922 when Argentinian microbiologist Alfredo Sordellii identified the organism in gangrenous wounds. Over the past century, there has been infrequent discussion and little awareness surrounding this serious condition. Information about patients who survived C. sordellii toxic shock is essentially nonexistent. Presentation of case A 31-year old post-partum female who underwent caesarian section 17 days prior, presented with abdominal pain and septic features. She underwent six operations/procedures over the next 17 days and was hospitalized for 25 days before discharge to her home with a successful outcome. Discussion This case provides insight on the process of identifying and managing complications associated with clostridial toxic shock syndrome. Out of now, 16 reported cases of C sordellii gynecologic infections in the world literature, our patient is the second patient to survive infection by this organism. More research is needed to provide clinicians with evidence-based approaches for managing these unique cases. Conclusion We present a case report of a rare toxic shock syndrome in a post-partum female caused by Clostridium sordellii. Her survival is notable among 16 patients with whom this infection now has a documented 87.5% mortality rate. Aggressive surgical measures and optimized intensive care management may have contributed to this patient’s survival.

Wyburn-Mason syndrome (WMS) is a widely recognized neurocutaneous syndrome in which the unilateral anterior venous malformation in its complete form is associated with eye, brain, and skin involvement. The term WMS also includes the incomplete form in which the patients show unilateral anterior venous malformation as a single anomaly not associated with other districts involvement. Neurologic complications are almost constantly reported in this syndrome. The aim of this report is to describe the neurologic course of two patients affected by the complete form of this very rare syndrome offering particular remarks whether convulsive episodes or epilepsy are leading features of this disorder. Based on the experience registered in the long follow-up in the present patients and in those reported in the literature, seizures and convulsive episodes are uncommon in WMS and, when present, mainly occur in consequence of acute or chronic disruptive events of the arteriovenous malformations.

A rare syndrome, Dyke-Davidoff-Masson Syndrome (DDMS), with a diagnostic conundrum, and the way it was solved is presented. DDMS is one among the syndromes associated with refractory epilepsy. We have come across a case of Dyke-Davidoff-Masson syndrome while investigating a case of refractory epilepsy. When cerebral hemi atrophy is associated with the radiological features of osseous hypertrophy of calvarium, hyper-pneumatisation of sinuses dilatation, DDMS is to be considered.Bangladesh J Medicine Jan 2014; 25 (1) : 31-34

Background Posterior reversible encephalopathy syndrome (PRES) is a well-recognized complication of hypertensive encephalopathy. Recently, pre-eclampsia, connective tissue disorders, and immunosuppressive drugs have been reported to be the etiologies of this rare syndrome. Methods We evaluated 9 cases of PRES whose diagnosis were confirmed based on clinical and radiologic evidence between July 2011 and December 2013 in a tertiary center, Imam Khomeini Hospital, Tehran, Iran. Results Immunosuppressive drugs, especially cyclosporine, and hypertension were the main precipitating factors. In this study, seizure was the most common clinical presentation (100%), whereas other common clinical presentations were confusion (78%), visual loss (67%), and headaches (67%). With conservative management and elimination of predisposing factor, the patients improved gradually except for 2 cases who experienced prolonged recovery period because of delayed diagnosis. Conclusions With timely diagnosis, PRES generally has a good prognosis with complete recovery. However, in missed conditions, it could be associated with catastrophic burden especially in organ transplantation after a prolonged time spending to find matched donors or in chronic immunosuppressive conditions. Thereupon, physicians should be aware of clinical and radiologic manifestations of this preventable but potentially disabling syndrome.

Key Clinical Message The Conradi–Hünermann Disease is a rare syndrome, which affects the cranial development and the anatomy of dental occlusion. After interdisciplinary treatment completion, the patient reached satisfactory facial anatomy, as well as regular occlusal relationship, attested 2 years of accompaniment.

Takotsubo cardiomyopathy or apical ballooning is a condition characterized by transitory left ventricular dysfunction, affecting commonly postmenopausal females after foregoing acute emotional or physical stress. We report a case of a 63 year old female presenting with severe dyspnea and right-sided secondary spontaneous pneumothorax, initially treated with tube thoracostomy. Despite the fact that pneumothorax resolved, shortness of breath persisted and due to ST-segment elevation and increased Troponin I levels, she was admitted to cardiac catheterization. A significant coronary stenosis was ruled out and the diagnosis of a Takotsubo cardiomyopathy was established. Electrocardiographic findings were normalized within three days and attributable to prolonged air leakage. A thoracoscopic apex resection followed by a partial parietal pleurectomy was performed. Although Takotsubo cardiomyopathy is a rare syndrome, it should always be considered as a potential cardiac complication of a pneumothorax.

Epilepsia partialis continua (EPC) is a rare syndrome of continuous focal jerking of a body part, usually localized to a distal limb, occurring over hours, days or even years. Etiology is multiple and diverse as well as the outcome is also variable. Here we report two cases of EPC admitted almost in a same period in our hospital in Khulna with different etiology and outcome. The first one, an adult diabetic patient, developed status epilepticus followed by right sided epilepsia partialis continua over a period of three days after a viral illness. He responded well with treatment. The second case, a three year old girl, progressively developed epilepsia partialis continua over a period of six months with no antecedent event. Her treatment response is not satisfactory. DOI: http://dx.doi.org/10.3329/bmjk.v47i1-2.22559 Bang Med J (Khulna) 2014; 47 : 25-28

Acute esophageal necrosis (AEN) also known as “black esophagus” or necrotizing esophagitis is a rare syndrome characterized by a striking diffuse patchy or circumferential black appearance of the esophageal mucosa that preferentially affects the distal esophagus and terminates at the gastroesophageal junction. Only 88 patients over a span of 40 years have received this diagnosis, and the prevalence of this disease ranges from 0.001 to 0.2% of cases in literature. It more commonly affects men (4 : 1 ratio) in the sixth decade of life. It is associated with a high mortality rate, approaching 32%. We report a case of AEN presenting in the setting of diabetic ketoacidosis (DKA), affecting both the proximal and distal esophagus.

Painful legs and moving toes (PLMT) syndrome is characterized by spontaneous movements of the digits and pain in one or both lower extremities. Of the reported cases, a majority of the patients was female, and the mean age of onset was 58 years. Only one pediatric case has been reported so far. Herein, we report the first adolescent case of PLMT in Korea. A 16-year-old girl complained of tingling pain in the left leg and involuntary movement of the ipsilateral great toe one month after a second untethering surgery. Three years ago, she had undergone untethering surgery to correct lipomeningomyelocele at the S2 level of the conus medullaris. At that time, she was diagnosed with polyradiculopathy at the left L5 level with axonal involvement. We diagnosed her with PLMT syndrome and prescribed gabapentin. Her symptoms diminished within a day. Complete relief from involuntary movement of the toe was achieved within four months. PLMT is a rare syndrome but it should be considered in the differential diagnosis of children and adolescents with limb pain and spontaneous movement in their toes.

The ocurrence of a traumatic arteriovenous fistula after arthroscopic surgery of TMJ represents an extremely rare event. Specifically, this uncommon complication has been described only in a few case reports. In this light, the most frequent symptoms showed by this disease are thrills, bruits, pulsatile tinnitus, and an expansible vascular mass. Importantly, the severity of these symptoms is also dependent on the vessels involved. With regard to the management, is important to note that the vessel ligation with surgery as well as vessel emolization with endovascular procedures have been shown to be effective in the treatment of these cases. In view of that, the present study describes a case of superficial temporal arteriovenous fistula that arose as a postoperative complication of a bilateral arthroscopic eminoplasty of TMJ. The aim of the present report is to characterize this rare syndrome with the goal of proposing suitable treatments. Key words:Arteriovenous fistula, arthroscopic surgery, eminoplasty of TMJ, temporal vessels.

UNLABELLED Superior canal dehiscence is a rare syndrome. The symptoms consist of hearing loss, dizziness and autophonia. The patient can be cured with surgery. A 28 year old woman went to several doctors for several months due to diminished hearing, dizziness and autophonia. The symptoms got worse. A work-up led to the diagnosis of superior canal dehiscence in the left ear. She underwent surgery and her symptoms improved. A latency in diagnosis is expected as the syndrome is rare. It's important to think of superior canal dehiscence when patients complain of these symptoms. KEY WORDS superior canal dehiscence, autophonia, dizziness, hearing deficit. Correspondence: Bryndis Baldvinsdottir, bryndisbaldvins@gmail.com.

Middle Aortic Syndrome is a rare vascular disorder consisting of narrowing or stenosis of the distal thoracic or abdominal aorta. It is described in the literature in the form of case studies and case series. The authors present an unusual case of Middle Aortic Syndrome attributed to Takayasu’s arteritis in a 60-year-old female who presented to the emergency department with uncontrolled hypertension. Traditional intervention involves open surgical bypass. This case study reviews the published literature on this rare syndrome and illustrates a successful alternative to open surgery through an endovascular approach.

Papillon-Lefevre syndrome (PLS) is a very rare syndrome of autosomal recessive inheritance characterized by progressive palmar-plantar keratoderma and early onset periodontitis, leading to premature loss of both primary and permanent dentitions and recurrent pyogenic infections. Patient Presented with a chief complaint of thickening of palms and soles extending on to knees and elbows.. On intraoral examination there is loss of both temporary and permanent teeth. Patient had recurrent pyogenic infections involving face and nose leading to nasal destruction. Histopathological examination of the specimen taken from the thickened skin was reported to be consistent with PLS1,2,3

Traumatic asphyxia is a rare syndrome caused by blunt thoracoabdominal trauma and characterized by cyanosis, edema, and subconjunctival and petechial hemorrhage on the face, neck, upper extremities, and the upper parts of the thorax. Traumatic asphyxia is usually diagnosed by history and inspection; however, the patient should be monitored more closely due to probable complications of thoracoabdominal injuries. Treatment is conservative, but the prognosis depends on the severity of the associated injuries. Herein we present a traumatic asphyxia due to an elevator accident in a 32-year-old male patient and discuss the diagnosis, treatment, and prognosis by reviewing the relevant literature.

Gorlin-Goltz syndrome or basal cell nevus syndrome is a comparatively rare syndrome characterized by basal cell nevi, odontogenic keratocysts, and skeletal anomalies. Diagnosis is based on the major and minor clinical and radiographic criteria. Dentist plays a major role in the diagnosis of this disease due to the oral and maxillofacial manifestations of the syndrome. In some cases, jaw cysts are diagnosed by routine radiographs advised by the dentists. Odontogenic keratocysts in such syndromic patients will be multiple and extensive and in some cases results in cortical expansion and facial disfigurement. Thorough clinical examination and investigations prompt an early confirmation of the syndrome, which is very essential to avoid morbidity associated with the syndrome. Here, we report a case of multiple odontogenic cysts in a 16-year-old patient which later was diagnosed as a case of Gorlin Goltz syndrome.

Sirenomelia is a lethal condition characterized by fusion of the lower limbs with severe malformations of the urogenital and lower gastrointestinal tract. We report a baby born with bilateral fused lower limbs, blind ended colon and single umbilical artery. Antenatal Diagnosis by ultrasonography may be difficult. Knowledge of this rare syndrome is important for health care providers.

Postpneumonectomy syndrome (PPS) is a rare syndrome whereby the airway and oesophagus become obstructed due to extreme rotation of the mediastinum after pneumonectomy. Our treatment of this condition involved mediastinal repositioning and placement of a saline filled tissue expander into the pleural space. This has not been reported in the plastic surgical literature and we describe technical difficulties and how they were overcome.

The present report describes the case of a 29-year-old man referred to our service for TMJ pain and progressive reduction of the mouth opening. Differential diagnostics included rheumatologic diseases, monoarthritis and intraarticular lumps. In this line, a face CT scan and a MRI of TMJ were carried out in order to ensure a proper diagnosis. These tests showed a solid lesion into the joint cavity. In view of that, we decided to perform a diagnostic and therapeutic arthroscopy of TMJ. Histopathological studies confirmed the diagnosis of pigmented villonodular synovitis. The main aim of this report is to describe this rare syndrome with the goal of proposing suitable treatments. Moreover, we highlight the benefits of using arthroscopic procedures in the cases which the tumor is still confined to the joint. As far as we are aware, scientific literature documents only a single case of pigmented villonodular synovitis of TMJ treated with arthroscopic approach. Key words:Arthroscopic approach, pigmented villonodular synovitis, TMJ, mouth opening.

Patients with Werner’s syndrome frequently develop chronic leg ulcers that heal poorly. We present a patient who suffered from this rare syndrome and developed typical heel ulcers. Treatment of the ulcer is challenging, as flap options are limited over the lower third of the leg and skin grafting is not easy as there is a lack of healthy granulations. We successfully treated the ulcer with osteomyelitis by drilling the bone and applying an ultrathin split thickness skin graft with the thigh skin as the donor site.

Acute esophageal necrosis, or black esophagus, is a rare syndrome affecting sick elderly patients, with high mortality rate. Its clinical expression is gastrointestinal bleeding. Treatment includes hemodynamic support, nil-per-os, proton pump inhibitors and management of the coexisting clinical conditions. We report the case of a woman presenting with black esophagus as complication of a percutaneous gastrostomy, who received medical care at Hospital Pablo Tobon Uribe, in Medellin, Colombia.

Made available in DSpace on 2022-04-29T08:14:24Z (GMT). No. of bitstreams: 0 Previous issue date: 2016-10-01 The excessive unilateral tearing as a result of gustatory stimuli is a characteristic of crocodile tears syndromes. The aim of this study is to report 2 cases of Frey and crocodile tears syndromes, considering diagnosis, monitoring, and selection of appropriate treatment. A 74-year-old female patient presented with a history of Bell palsy, tearing on left eye at the moment of chewing, and absence of pain. The patient opted only for periodic case monitoring because the symptoms presented by the syndrome did not bother her, which made any invasive procedure unnecessary, and with the closed diagnosis she may be reassured and really know what was happening to her. So scientific divulgations about this syndrome should be made in order to increase the knowledge of dentists and oral and maxillofacial surgeons, because it is a rare syndrome and easily diagnosed. Due to the low knowledge in Brazil, the patient of this report had to wait about 9 years to get a diagnosis. Araçatuba ‡Medical Sciences *Department of Basic Sciences †Division of Oral and Maxillofacial Surgery Department of Surgery and Integrated Clinic Aracatuba Dental School University Estadual Paulista Júlio de Mesquita Filho Faculty of Medical Sciences University of Campinas-Unicamp Araçatuba ‡Medical Sciences *Department of Basic Sciences †Division of Oral and Maxillofacial Surgery Department of Surgery and Integrated Clinic Aracatuba Dental School University Estadual Paulista Júlio de Mesquita Filho Faculty of Medical Sciences University of Campinas-Unicamp

We describe a patient with Doege-Potter syndrome (solitary fibrous tumor of the pleura presenting with hypoglycemia) and illustrate several important lessons learned from the case. Seven years after the initial diagnosis, the tumor showed significant growth and developed a high-grade undifferentiated component. Solitary fibrous tumors do grow and cannot be deemed benign. Resection should be considered in all patients who are candidates for operation upon diagnosis. Our case also serves as a reminder of this rare syndrome, inasmuch as early recognition of the association of hypoglycemia with these tumors may have allowed for earlier diagnosis and avoidance of extensive tests in our patient.

Asplenia syndrome is a syndrome of right isomerism or attempted bilateral right sidedness. It includes congenital asplenia in association with complex congenital cyanotic heart disease and situs anomalies of the other thoracoabdominal organs. Herein we report a case of asplenia syndrome so as to highlight and refocus attention on the possibility of this rare syndrome that must be considered in a neonate presenting with congenital cyanotic heart disease.

Autoimmune polyglandular syndrome type 2 also known as Schmidt syndrome. It is a rare disorder involving a combination of Addison’s disease with autoimmune thyroid disease with or without type 1 diabetes mellitus. In this case report one such patient with this rare syndrome is described who presented with hyperpigmentation of knuckles, palms and soles with significant weight loss for 2 months. At presentation she also had severe hypercalcaemia. Severe hypercalcaemia is rare and hypercalcaemia at the initial presentation of Addison’s disease is also unusual. The mechanism of hypercalcaemia in addisons and management of this patient is discussed.

Journal Article; UNLABELLED Carotid-cavernous fistulas are an uncommon diseases characterized by abnormal communications between arteries and veins located in the cavernous sinus. According with Barrow´s classification they could be divided in two groups: direct and indirect. The typical symptoms showed by theses pathologies are: pulsating exophthalmos and orbital blow. The present study describes a case of direct posttraumatic carotid-cavernous fistula in a 26 years old man. Furthermore, we present the images that we used to make the diagnosis. In this light, we decided to treat this case with endovascular approach after considering several therapeutic options. The aim of the present report is twofold. First, we examine the importance of the proper management of the direct posttraumatic carotid-cavernous fistula. Second, we describe this rare syndrome with the goal of proposing suitable treatments. KEY WORDS Carotid cavernous fistulas, pulsating exophthalmos, orbital blow, endovascular approach, Barrow´s classification. Yes

Wall-eyed bilateral internuclear ophthalmoplegia (WEBINO) is a rare syndrome which is characterized by a set of ocular motility abnormalities, especially a large-angle exotropia in primary gaze. One of the management strategies suggested for it is extraocular muscle surgery, particularly transposition surgery. In this 38-year-old male WEBINO patient, we concluded that transposition surgery could effectively alleviate the exodeviation and also the patient's complaints in comparison with resection-recession procedures.

Background Painless legs and moving toes syndrome is a very rare syndrome characterized by continuous and involuntary movement of the toes. The etiology of the disease is not clear though it has been linked to a wide range of neuronal insults including proximal root compression and neuropathy. A previous study has reported bilateral painful legs and moving toes syndrome in a patient with a sacral Tarlov cyst. In this report we present a case of unilateral painless legs and moving toes syndrome in a woman with a sacral Tarlov cyst. Case presentation A 50-year-old Mediterranean woman presented with a 1-year history of involuntary sustained movement of her right toes. Her physical examination and laboratory findings did not show any remarkable abnormality. Her lumbosacral magnetic resonance imaging scan showed a sacral Tarlov cyst. Our patient was given gabapentin, 100 mg per day as a starting dose, and showed modest improvement. Our patient preferred not to continue with the treatment as her symptoms were not disabling and she was only concerned about the cosmetic appearance. Conclusions This report presents a new case of a very rare syndrome called painless legs and moving toes syndrome, which is possibly a variant of painful legs and moving toes syndrome. This is considered to be the first case of unilateral painless legs and moving toes syndrome that is associated with a sacral Tarlov cyst. Although the disease etiology is still unknown and the presence of the cyst can be accidental, neurologists should be aware that Tarlov cyst is a possible cause. In addition, patients with the painless variant who are not disabled by movement of the toes may not require treatment.

Platypnea-orthodeoxia syndrome (POS) is a rare syndrome of severe hypoxemia upon assuming an upright position. It is classically described as shunting from the right atrium to the left atrium usually via a patent foramen ovale (PFO). Alterations in the intrathoracic anatomy after liver resection and regeneration may trigger this condition in patients with clinically silent PFO -a previously unreported cause of POS.

Tolosa Hunt Syndrome (THS) is rare syndrome with an estimated annual incidence of 1 case per million per year. THS is painful ophthalmoplegia caused by nonspecific inflammation of cavernous sinus or superior orbital fissure. It is often unilateral with severe headache and ophthalmoplegia involving third, fourth, fifth and sixth cranial nerves. We present a case admitted in our hospital that came with complaint of severe unilateral headache with loss of vision of left eye. He did not have any other significant history except that he was complaining of partial sensory loss over left upper part of face. Ophthalmologist reference was taken to rule out any other cause involving optic disc and funduscopy which was normal. Further investigations were done which ruled out all possible causes. Patient was started on steroids and on MRI scan and clinical presentation, patient was diagnosed as Tolosa- Hunt Syndrome. Thus we report a rare case of THS which showed gradual recovery with corticosteroids.

The Meigs' syndrome is a rare syndrome defined as the triad of benign solid ovarian tumors, ascites, and pleural effusion, which resolve spontaneously and permanently after tumor resection. Lung collapse due to a large amount of pleural effusion is a common mechanism of death. It always requires surgical treatment. We report an 84-year-old woman with a large ovarian fibroma associated with Meigs' syndrome and short of breath. It is difficult to diagnose preoperatively and is usually misdiagnosed as an ovarian malignancy. Considering the patient's serious clinical condition and assuming that she had Meigs' syndrome with a large ovarian mass and possible lung collapse due to large amount of pleural effusion, we chose the most appropriate surgical treatment after pathologic examination, then enabled definitive diagnosis of the benign tumor and removed the huge ovarian fibroma. This resulted in a timely symptoms resolution, short hospitalization, and relatively low morbidity in elderly.

Fahr's syndrome refers to a rare syndrome characterized by symmetrical and bilateral intracranial calcification. We present a 65-year-old female with Fahr disease, presenting with headache with acute ischaemic stroke with left sided hemiplegia. CT scan of brain reveals irregular variable size hyperdense areas are noted in both basal ganglia regions and in both cerebellar hemisphere.Medicine Today 2017 Vol.29(1): 45-46

Background: “Cocaine-induced agitated delirium with associated hyperthermia” is a rare, almost uniformly fatal syndrome. The incidence of the disease is not known, however, it is believed to have markedly increased since the late 1980s with widespread popularity of crack cocaine. Objective: Recent literature is lacking regarding this rare syndrome. Although almost uniformly fatal, we present a neurologically intact survivor due to a multidisciplinary team approach. Case Report: We are reporting a 41-year-old African-American man who arrived at the trauma center with a rectal temperature of 42.6°C (108.6°F) and a toxicology screen positive for cocaine. The patient manifested many of the known complications of cocaine-induced agitated delirium with associated hyperthermia, including renal failure and coagulation panel abnormalities. With early application of cooling techniques, including ice pack, gastric lavage, and bilateral chest cavity lavage using multiple chest tubes, the patient's core temperature was quickly lowered. Conclusion: This case demonstrated how a multidisciplinary team approach, including emergency medicine and critical care specialists, and aggressive treatment of hyperthermia using bilateral tube thoracostomy and chest cavity lavage enabled our patient's core temperature to be effectively lowered. We were unable to find prior reports of using tube thoracostomy in this manner.

Platypnea-orthodeoxia is a rare syndrome characterized by dyspnea and arterial deoxygenation, which is exacerbated in an upright position and relieved with recumbency. We report a case of platypnea-orthodeoxia in a 56-year-old male who was found to have bilateral lower-lobe pulmonary emboli. He did not have any evidence of an intracardiac shunt. A comprehensive review of the pathophysiology and management of platypnea-orthodeoxia is provided.

Primary hypertrophic osteoarthropathy (PHO) is a rare syndrome with variable clinical and radiological presentations such as joint pains and swelling, clubbing, seborrheic and thickened facial skin and hyperostosis. We present such a case of PHO who was previously diagnosed and treated as inflammatory polyarthritis.