Magnetic resonance features of supravalvular aortic stenosis in William''s syndrome. Report of 2 cases

A wandering spleen and splenic cysts are uncommon conditions in children. A combination of both entities has only rarely been reported in the literature. Another case of this complex pathology in 12-year-old girl is presented. She was initially referred for evaluation of a large abdominal mass. Diagnostic studies raised the suspicion of a hematoma in an abnormally located spleen, but the definitive diagnosis was established at laparotomy. Successful surgical management consisted of total cystectomy and splenopexy.     

Clinical features of 201 cases with Duane''s retraction syndrome

OBJECTIVE: To summarize the clinical features of 201 cases with Duane's retraction syndrome (DRS) and discuss its differential diagnosis. METHODS: We retrospectively summarized the 201 cases from 1979 to 1996. The clinical features including chief complaints, sexual distribution, age at first visit, laterality, type of presentation, ocular deviation in the primary position, refractive errors, amblyopia, globe retraction, change of the palpebral fissure, upshoot and downshoot in adduction, binocular single vision, and its associated ocular and non-ocular anomalies were analysed. RESULTS: There were 99 males and 102 females with a female-to-male ratio 1:1. The 65.88% of DRS cases had left eye involvement with two-to-one predilection for the left eye. The most common form of the syndrome was type I (184 patients, 91.54%). Exotropia was the most common deviation in the primary gaze (72 patients, 35.8%). Among 118 patients, most had abduction deficits, globe retraction in adduction, and faceturn as to maintaining single binocular vision. Crocodial tears (26 patients, 11.93%) was the most frequently encounted ocular abnormalities. CONCLUSIONS: Diagnosis of DRS in a typical case is not difficult, however, children with bilateral abduction deficits which may mimic DRS must be differentiated from the following four motility disorders, namely, abducens nerve palsy, Moebius syndrome, congenital oculomotor apraxia, and congenital or infantile esotropia.     

Felodipine use in pregnancy. Report of three cases

BACKGROUND: Calcium channel blockers are effective agents for the management of chronic hypertension and are being used with increasing frequency. If their safety and efficacy during pregnancy can be documented, women can be counseled to continue their antihypertensive agent during pregnancy. To our knowledge, the use of felodipine, a calcium channel blocker of the dihydropyridine group, during pregnancy has not been described. CASES: We report three cases of felodipine use in pregnancy by women with chronic hypertension. CONCLUSION: In women with severe hypertension (diastolic blood pressure > 100 mm Hg) who require pharmacologic treatment of it during pregnancy, felodipine appears to be an acceptable option.     

Neuroleptic malignant syndrome associated with multiple joint dislocations in a trauma patient

The structure of the O-antigenic polysaccharide from Escherichia coli O141 has been determined. NMR spectroscopy and sugar and methylation analyses were the principal methods used. The sequence of the sugar residues could be determined by NOESY and heteronuclear multiple-bond connectivity (HMBC-) NMR experiments. The polysaccharide is composed of pentasaccharide repeating units with 1 O-acetyl group/repeating unit. The following structure, where Rha is 6-deoxymannose is concluded: carbohydrate sequence [see text].     

Actual asymptomatic epidural hematomas in childhood. Report of three cases

It is well known that the mortality of epidural hematoma (EDH) cases is directly related to the patient's level of consciousness at the time of surgery. The authors report three actually asymptomatic patients with mild head injury, in whom the diagnosis of EDH was possible because of quite broad indications for computed tomographic (CT) scanning, which allowed diagnosis and treatment in an early phase. These cases illustrate the possibility of an EDH in a totally asymptomatic patient.     

Moyamoya and Down syndrome. Clinical and radiological features

BACKGROUND AND PURPOSE: Moyamoya disease is a chronic occlusive cerebrovascular disorder characterized by progressive stenosis of the supraclinoid internal carotid artery, with the secondary development of enlarged basal collateral vessels. It may occur as a primary disease or as a syndrome in association with a variety of conditions, and its pathogenesis remains unexplained. There are relatively few reports describing the occurrence of moyamoya in Down syndrome. The aim of this study is to describe the clinical and radiological features of moyamoya syndrome associated with Down syndrome (MM-DS) and to explore theories of moyamoya pathogenesis in these patients. METHODS: Seven children with MM-DS underwent brain imaging, transfemoral angiography, and serial neurological exams. Neurological deficits, poststroke recovery, radiographic infarct characteristics, and angiographic abnormalities were reviewed. RESULTS: The clinical and radiological features of primary moyamoya disease overlap with those of MM-DS. Hemiplegia and aphasia were the most common presentations. Motor recovery was excellent in five of seven cases. Cerebral infarcts were superficial or deep and can occur in a watershed distribution. Angiography demonstrated involvement of the internal carotid artery and its branches bilaterally in all seven cases and the posterior cerebral arteries in four cases. CONCLUSIONS: The clinical and radiological features of MM-DS overlap with primary moyamoya disease. We postulate that a protein encoded on chromosome 21 may be related to the pathogenesis of moyamoya disease. Although the neuronal substrate is abnormal in Down syndrome patients, recovery from hemiplegic stroke in patients with MM-DS is comparable to recovery in patients with primary moyamoya.     

Immunohistochemical evaluations of ultrashort-segment Hirschsprung's disease. Report of three cases

PURPOSE: Unlike classic Hirschsprung's disease, short-segment and ultrashort-segment varieties are usually found to be latent and milder. Ultrashort-segment Hirschsprung's disease may present as intractable chronic constipation in children over one year of age, adolescents, and adults. Anorectal myectomy has been shown in many instances to provide effective long-term treatment for certain patients with ultrashort-segment Hirschsprung's disease. Histologically, the affected segment in Hirschsprung's disease has been shown to have increased cholinergic nerves, lack of nitric oxide synthase-containing neuronal elements, and show moderate to severe loss of myenteric neurons. METHODS: Here, we report three cases that showed clinical and manometric evidence of ultrashort-segment Hirschsprung's disease. Two of the three patients responded well to myectomy. RESULTS: Detailed histologic and immunohistochemical evaluation of the internal anal sphincter and a comparison with three normal controls revealed absence of nitric oxide synthase-containing neurons in both cases that responded well to surgery and continued presence of these neurons in the patient who did not respond. A review of the current literature on various treatment modalities is included. CONCLUSIONS: Anorectal myectomy provides long-term relief of this chronic problem in a subgroup of patients with ultrashort-segment Hirschsprung's disease who lack nitrinergic neurons at the internal anal sphincter.     

Disseminated Blastoschizomyces capitatus infection in acute myeloblastic leukaemia. Report of three cases

Three new cases of Blastoschizomyces capitatus infection occurring in neutropenic patients with acute myeloblastic leukaemia are reported. B. capitatus was isolated from blood cultures in all patients. All three patients were treated with amphotericin B, but only one was cured from the infection. Our study confirms the emergence of B. capitatus as an opportunistic agent of disseminated fungal infection in leukaemic patients.     

Clinical and demographic features of Meige's syndrome

Fifty five patients with Meige's syndrome were examined for clinical and demographic features. The mean age of onset was 52.3 years. The peak age of onset was in the sixth decade with a male to female ratio of 1.11:1. The mean duration of illness was 3.7 years. Commonest initial symptom was increased blinking, seen in 30 cases (54.5%). Twenty five patients (45.4%) had complete syndrome of blepharospasm with oromandibular dystonia, whereas 24 patients (43.6%) had blepharospasm alone and the rest (6 patients, 10.9%) had oromandibular dystonia. The extension of spasm beyond cranial muscles was observed in 10 patients (18.1%). Eleven patients had family history of dystonia or other extrapyramidal disorders. Incidence of depression was high in these cases.     

Gas in the temporomandibular joint: computed tomography findings. Report of 3 cases

BACKGROUND: The advantages of pancreatogastrostomy over pancreatojejunostomy after pancreaticoduodenectomy are still debated. This study analyses the results of pancreatogastrostomy to identify factors that could influence immediate outcome. METHODS: During a 10-year period, 160 consecutive patients underwent a pancreatogastrostomy. There were 109 men (68 per cent) and 51 women (32 per cent) with a mean(s.d.) age of 59(10) (range 22-82) years; 27 patients were older than 70 years. The following parameters were assessed: mortality rate, morbidity, reasons for reoperation, length of hospital stay, duration of nasogastric tube and drainage. RESULTS: Hospital mortality rate was 3 per cent; overall morbidity rate was 30 per cent. The reoperation rate was 12 per cent, mainly because of bleeding at the pancreatic margin. Delayed gastric emptying occurred in 36 patients. The overall rate of pancreatic fistula was 2.5 per cent. Age, sex, indications for pancreatoduodenectomy, and the texture of the pancreatic remnant did not influence the occurrence of pancreatic fistula or delayed gastric emptying. CONCLUSION: This study confirmed that pancreatogastrostomy is a safe procedure with low mortality and morbidity rates.     

Delayed internal capsule infarctions following radiofrequency pallidotomy. Report of three cases

The authors report on a series of patients with idiopathic Parkinson's disease (IPD) who underwent stereotactic radiofrequency (RF) pallidotomies, three of whom suffered delayed postoperative strokes. These three belonged to a group consisting of 42 patients with medically intractable IPD in whom 50 pallidotomies were performed. All three patients had significant previous vascular disease and were in a high-risk group for cerebral infarction. A postoperative magnetic resonance (MR) image was obtained immediately after the pallidotomy was performed to document the placement of the RF lesion and to rule out any hematoma. The delayed strokes occurred on postoperative Days 10, 51, and 117 in patients with previous vascular disease (Group 1, 11 patients). No strokes occurred in the group with the vascular disease risk factor (Group 2, 11 patients) or in the group with no risk factors for vascular disease (Group 3, 20 patients). This observation is statistically significant (p < 0.05). The T2-weighted MR images showed the lesions as high-intensity signals extending to the posterior limb of the internal capsule ipsilateral to the pallidotomy site. The poststroke T1-weighted images obtained in two patients showed persistent contrast enhancement of the RF lesion and no enhancement around the stroke lesion. Clinically and radiographically, these discrete new lesions represent delayed infarctions, suggesting that RF lesioning can induce delayed injury in adjacent tissue. Patients with previously identified vasculopathy may be at risk for delayed capsular infarction following RF pallidotomy.