A rare case report describing the relation between sweet syndrome and spontaneous recurrent peritonitis

IntroductionSweet syndrome (acute febrile neutrophilic dermatosis) is a subset of rare inflammatory disorders, first described by Dr. Robert Douglas Sweet in 1964 (Sweet, 1964). The co-existence of Sweet syndrome and spontaneous recurrent peritonitis has never been previously mentioned in the medical literature.Presentation of caseWe report a case of a 37-year old female patient with known idiopathic Sweet syndrome, diagnosed on skin biopsy, who presented with multiple episodes of spontaneous peritonitis. Investigation through abdominal laparoscopy showed large amounts of free pus in the abdomen without bacterial isolation.DiscussionDifferential diagnoses, investigations and management of suspected spontaneous peritonitis are discussed. It was suspected that her Sweet syndrome had caused a rare form of previously undescribed recurrent sterile peritonitis.ConclusionThis case illustrates the importance of careful evaluation of patients with known inflammatory disorders, such as Sweet syndrome. It also demonstrates the need to have a multidisciplinary approach, by collaboration between the disciplines of medicine, surgery, microbiology and radiology.     

Bartsocas-Papas syndrome: The first case report of severe autosomal recessive form from Indonesia

IntroductionBartsocas-Papas syndrome (BPS) is an autosomal recessive form of Popliteal Pterygium syndrome (PPS). It is a very rare disease characterized by congenital craniofacial anomalies, popliteal webbing, and genitourinary and musculoskeletal anomalies. Almost all of the cases were reported in dead intrauterine pregnancies.Presentation of caseWe present a 10-month-old boy with bilateral complete cleft lip and palate, abnormal scalp hair, an absence of both upper eyelids, choanal atresia, syndactyly of the third and fourth fingers of the right hand, agenesis fingers on the left hand, bilateral popliteal pterygia, bilateral talipes equinovarus, agenesis of the toes of both lower extremities, intercrural webbing, an absence of testis, and scrotal anomaly. Multistage surgical correction was performed for the multiple congenital malformations.ConclusionWe report the first case of BPS from Indonesia. Gradual management should be performed according to the patient's age and available facilities.     

Laparoscopic treatment of Dunbar syndrome: A case report

BackgroundDunbar syndrome is a rare disorder due to external compression of the celiac trunk by the median arcuate ligament. The symptoms include abdominal pain, nausea and vomiting. Laparoscopic treatment was suggested as a safe procedure. The aim of this study is to present a case of Dunbar syndrome underwent laparoscopic treatment.CasepresentationA 17-year-old female patient presented at emergency room with upper abdominal pain and dyspepsia, related to food intake. A selective arteriography of the celiac trunk revealed stenosis due to compression of the celiac artery. The decompression of the celiac trunk by the median arcuate ligament was performed. Postoperative course was uneventful and the patient was discharged on the 5th postoperativeConclusionsLaparoscopic division of the arcuate ligament in patients with Dunbar syndrome is feasible and safe. This procedure can be performed uneventful in order to restore quality of life of the patient.     

Periodic alternating gaze: a case without any hemispheric lesion

A clinicopathological study of a patient with periodic alternating gaze (PAG) is presented. PAG is usually associated with bilateral cerebral hemispheric ischemia. In our case, the anatomical lesion is very limited, restricted to the crus cerebri. The anatomical pathways involved and the mechanism of PAG are discussed.     

Hemolytic uremic syndrome in small-bowel transplant recipients: the first two case reports

Post-transplant hemolytic uremic syndrome (HUS) is an uncommon but well-described complication in solid organ transplant recipients. Believed to be secondary to immunosuppressive therapy, it has been reported after kidney, liver, pancreas, heart, and lung transplants. In all reported cases, the primary organ affected was the kidney (transplant or native). But until now, no cases after small-bowel transplants and no cases in which the kidney was not the primary organ affected have been reported. We report two cases of HUS in small-bowel transplant recipients. In our first case, clinical presentation was with renal failure; biopsy of the native kidney demonstrated the typical histological changes seen with HUS, namely occlusion of the microcirculation by thrombi and platelet aggregation. Immunosuppression was changed from tacrolimus to cyclosporin, but with no improvement in renal function. In our second case, the transplanted bowel was the primary organ affected. This recipient presented with ulcers in the bowel mucosa, which were believed to be ischemic in origin, secondary to occlusive vascular lesions affecting the small vessels in the transplanted bowel. Her tacrolimus dose was decreased with resolution of ulcers and no evidence of rejection. These two cases represent the first reports of HUS after small-bowel transplants; in addition, our second case represents the first report of an extrarenal graft as the primary organ affected. When caring for small-bowel transplant recipients, physicians must be alert to the possibility of HUS and its various presentations. Received: 29 October 1998 Received after revision: 12 March 1999 Accepted: 3 May 1999     

A case of Foster Kennedy syndrome without frontal lobe or anterior cranial fossa involvement

Foster Kennedy syndrome is a very rare syndrome which includes ipsilateral optic atrophy and central scotoma, anosmia, contralateral papilledema, and, occasionally, ipsilateral proptosis. A large frontal lobe, olfactory groove, or medial third sphenoidal wing tumor, usually a meningioma, creates this syndrome. In this report, the author presents a case of metastastic cerebral tumor with Foster Kennedy syndrome but without frontal lobe or anterior cranial fossa involvement.     

Indeterminate cauda equina syndrome: A case report

IntroductionThe presentation of cauda equina syndrome (CES) varies from its classical presentation, especially in its early stages of compression. We present a case of lumbar disc prolapse causing CES in an uncharacteristic way, knowledge of which is essential for orthopaedicians to diagnose this condition early and prevent neurological complications.Case reportA 32-year-old male patient presented to us with complaints of inability to lift his left ankle and numbness over his left leg and ankle for 14 days. Clinical examination showed involvement of left L3, L4, L5 and S1 nerve roots as evidenced by weakness of quadriceps, extensor hallucis longus, extensor digitorum longus muscles and tendo achilles. Knee jerk was absent. The opposite lower limb was normal and there was no evidence of bowel bladder involvement or saddle anaesthesia. The MRI showed L2 L3 posterocentral disc prolapse compressing the cauda equina. The patient underwent laminectomy and discectomy. Post-operatively, the patient showed significant improvement in his sensory symptoms with complete recovery of motor power in 12 weeks.DiscussionIn contrast to the classical presentation of CES, several case series have been reported with varied clinical manifestations like unilateral leg symptomatology, unilateral or bilateral saddle anaesthesia with or without leg symptoms and CES with complete absence of signs and symptoms in the lower limbs. The disc prolapse in our case at L2-L3 level has compressed the left-sided L3, L4, L5 roots with minimal compression of S1. The classical features of CES would have occurred due to the lateral shift of the cauda equina in our case but for our early diagnosis and intervention.     

Not so silent sinus syndrome: A case report

IntroductionSilent sinus syndrome (SSS) is a rare disorder with protean manifestations. An absence of familiarity with ambiguous and atypical presentations may complicate diagnosis and delay management.Case presentationA 28 year old female patient presented with a chronic history of headache, post-nasal discharge and recurrent facial pain refractory to analgesics. Enophthalmos and hypoglobus progressed over a period of 2 months, and a diagnosis of SSS was confirmed via imaging. Definitive treatment was withheld given the patient’s postpartum state and improvement of symptoms.DiscussionSSS typically manifests with painless and progressive, unilateral, enophthalmos and hypoglobus. Since presentation is dominated by ophthalmologic complaints, the ordinary route by which SSS is diagnosed is through ophthalmology review. The predominant complaint in our patient was chronic headaches with facial pain, and mild enophthalmos and hypoglobus were only noted 2 months later at follow-up. This represents an atypical presentation of SSS, and exemplifies the subtle and often ambiguous presenting features of this disorder.ConclusionThe protean manifestations of SSS mean that patients may initially present to specialities other than ophthalmology. To ensure rapid diagnosis and appropriate management, it is important that clinicians, particularly in ophthalmology, maxillofacial surgery, and ears, nose and throat (ENT), are familiar with this obscure condition.     

Maffucci综合征1例

1病例资料患者,男,24岁,因左踝关节、足部多处肿块5年,伴行走不适1年,于2010年1月入院。3年前曾行左踝后方一处结节切除术,诊断为血管瘤。体检：左侧足部皮肤表面多发蓝紫色结节,直径约2 cm;左踝关节前方类圆形包块,大小约5 cm×6 cm,质软,     

Kidney abnormalities in Hajdu-Cheney syndrome

We report a 14-year-old boy from Saudi Arabia with the Hajdu-Cheney syndrome who also had bilateral hypoplastic kidneys and chronic renal failure. This report, along with others in the literature, suggest that renal abnormalities, including cystic disease, vesico-ureteral reflux, glomerulonephritis, chronic renal failure, and hypertension, may be an integral part of this syndrome. Recognizing this fact, may help in the early diagnosis, prevention, and treatment of renal failure in these patients. Received March 19, 1996; accepted April 9, 1996     

Zinner综合征一例报告并文献复习

目的 报告1例Zinner综合征病例,分析其诊治经过,并复习相关文献,提高对于本病的认识。方法 回顾性分析1例Zinner综合征病例,患者为18岁男性,因“反复尿频、尿痛、会阴区疼痛不适1年,加重伴发热1 d”入院。经影像学及实验室检查诊断为Zinner综合征。结果 入院后给予抗感染治疗7 d后临床症状消失,血常规、降钙素原、C反应蛋白感染指标正常,继续抗感染治疗1周出院。3个月后再次入院复查有轻度尿频,无尿痛症状,检查血常规无异常。行腹腔镜下左侧精囊囊肿切除术,术后病理明确精囊囊肿壁炎症浸润。术后5 d顺利出院。术后6个月内随访尿频、尿痛、会阴区疼痛等不适症状未再发。结论 彩超、CT、MRI检查是诊断Zinner综合征的重要手段,病理检查是明确精囊囊肿炎症浸润的依据。急性精囊囊肿感染伴同侧肾缺如、输尿管发育畸形是Zinner综合征的表现形式之一,感染控制后行腹腔镜精囊囊肿切除术安全有效。     

Closed posterior subtalar dislocation without any associated fracture: A case report and review of the literature

Pure posterior subtalar dislocation without any medial or lateral displacement of foot is extremely rare and hardly reported in the literature. Such an injury is more likely to be open and associated with fractures of the surrounding bones of the foot. Here we report a rare case of closed pure posterior subtalar dislocation without any associated fracture. We discuss in detail the mechanism of such an injury and highlight the importance of prompt closed reduction and early mobilization to ensure a satisfactory long term outcome.     

Hemifacial spasm associated with Marfan's syndrome: A case report

The authors describe a case of hemifacial spasm associated with Marfan's syndrome. A 24-year-old-woman suffered from left hemifacial spasm for 6 years. She had undergone surgical treatments for bilateral ectopia lentis and kyphoscoliosis in the second decade of life. She also had unusually long and slim limbs with arachnodactyly. Three-dimensional CT angiography revealed bilateral tortuous and elongated vertebral arteries. Microvascular decompression was performed following a left lateral suboccipital craniotomy. The root exit zone of the left seventh nerve was directly compressed by the proximal segment of the anterior inferior cerebellar artery (AICA) and the left vertebral artery. These arteries were decompressed with pieces of Teflon cotton. The patient's symptoms completely resolved following surgery. We review cerebrovascular disorders in Marfan's syndrome and discuss the pathogenesis and possible mechanisms of vascular compression in these patients.     

Trichobezoar with and without Rapunzel syndrome in paediatric population: A case series from a tertiary care centre of Northern India

IntroductionTrichobezoars are concretions formed by accumulation of hair in stomach. Usually, trichobezoar is confined to the stomach, but rarely it may extend from the stomach to the small intestine and even colon. This is an unusual form called Rapunzel syndrome. Our experience with this rare entity of Rapunzel syndrome and interesting entity of trichobezoar is being presented with review of literature.Presentation of casesWe, at our institute, encountered four cases of trichobezoar in last five years, out of which two were found to be of Rapunzel syndrome. All of these cases were managed successfully by open surgical intervention in view of the very large size of the mass in all the cases.DiscussionThe clinical presentation is highly variable ranging from asymptomatic cases diagnosed incidently to serious gastrointestinal symptoms and complications. Cases of trichobezoar have been reported in literature very infrequently but Rapunzel syndrome is extremely rare and less than 50 cases have been reported in medical literature till date.ConclusionTrichobezoar leading to Rapunzel syndrome is an extremely rare entity. The clinical presentation is usually vague and non-specific. Treatment is mainly surgical because of delayed presentation in majority of the cases. Psychiatric illness is the usual association.     

Misdiagnosed coronary artery diseaseeacute aortic syndrome: A case report

It is estimated that 15% of traffic accidents cause trauma to large vessels. In about 70%–95% of cases, aortic rupture took place in the distal ligament of the left subclavian artery, whereas in the remaining cases rupture occurred in the ascending aorta above the aortic valve. Trauma to the aortic sinus and coronary arteries is rarely found in traffic accident victims. Therefore, coronary artery trauma is often misdiagnosed as coronary atherosclerotic heart disease. The present case is a 42 years old male who survived from a traffic accident. He presented with aortic sinus and left coronary artery trauma. He was misdiagnosed as having coronary atherosclerotic heart disease, and therefore wrongly given antiplatelet medicine, such as aspirin, in another hospital. Definite diagnosis was achieved in our hospital, and the patient underwent Bentall and mitral valve replacement, as well as tricuspid valvuloplasty. The aortic occlusion time during surgery was 47 min, and the total cardiopulmonary bypass time was 63 min. After surgery, transthoracic echocardiography confirmed that all the artificial valves worked sufficiently. The patient felt good and symptoms such as asthma and decreased exercise tolerance disappeared. This case taught us that acute aortic syndrome cannot be ignored when patients present with pectoralgia; antiplatelet medication should not be given before definite diagnosis.