Self-Mutilation in Neurodegeneration with Brain Iron Accumulation

Neurodegeneration with brain iron accumulation (NBIA) is the term applied to a heterogeneous group of disorders resulting in iron deposition in the basal ganglia. Well-known phenotypic features are progressive regression with extra pyramidal involvement and a variable course. A 10-year-old child born to consanguineous parents presented with progressive generalized opisthotonic dystonia, retrocollis, oromandibular dyskinesias, apraxia for swallowing, optic atrophy and severe self-mutilation of lips. MR imaging showed brain iron accumulation. Other causes of self-mutilation were excluded. Early infantile onset, ophisthotonic dystonia with oromandibular dyskinesias and characteristic MR images are suggestive of NBIA. There is only one case reported in the literature of self-mutilation in this condition.     

Peritonitis – the Eastern experience

Peritonitis is a common emergency encountered by surgeons the world over. This paper aims to provide an overview of the spectrum of peritonitis seen in the East. Studies dealing with the overall spectrum of secondary peritonitis in various countries of this region were identified using Pubmed and Google. These were analyzed for the site and cause of perforation and the mortality. It was observed that perforation of duodenal ulcers was the most the commonly encountered perforations. These are followed by small bowel and appendicular perforations. Colonic perforations were uncommon. The overall mortality ranges between 6–27%.     

Duodenogastric reflux after choledochoduodenostomy

Duodenogastric reflux (DGR) has been implicated in several disease processes. The present study was carried out to document the incidence and evaluate the clinical significance of DGR after choledochoduodenostomy (CDD). A total of 13 patients who had undergone cholecystectomy with a standard side-to-side CDD for choledocholithiasis or chronic pancreatitis were studied by symptom evaluation, scintigraphy, endoscopy, and gastric mucosal histology at least 6 months after surgery. The scintigraphic findings were then compared with those of 10 patients who had undergone cholecystectomy alone. Only two patients (15%) had mild dyspeptic symptoms. The incidence of DGR after CDD was 69% compared to 20% in the cholecystectomy alone group (P < 0.05). In the majority of patients the DGR was only mild to moderate and the severity correlated well with the degree of endoscopic gastritis, but not with the clinical symptoms or histological findings. These results indicate that while CDD is associated with a high incidence of DGR, its occurrence does not produce significant clinical symptoms.     

Pancreas-sparing duodenectomy for trauma.

The application of pancreas sparing duodenectomy (PSD) in extensive duodenal trauma has not been fully explored. We report 3 caes of duodenal trauma in whom PSD was performed successfully and with good results.     

APPRAISAL OF SURGICAL AND ENDOSCOPIC MANAGEMENT OF CHOLEDOCHOLITHIASIS

One hundred and ten patients with common bile duct (CBD) stones were treated in the Department of Surgical Gastroenterology at SGPGIMS, Lucknow, India between January 1989 and December 1992. The primary modality of treatment was surgery in 62 patients (group I) and endoscopic sphincterotomy (ES) in 48 (group II). The two groups were well matched with respect to clinical features and presence of medical risk factors. Surgical clearance of CBD stones was achieved in 58 patients (93.5%; group Ia). Four patients (7%) had retained stones following surgery (group Ib). In group II, the CBD was cleared by endoscopic means in 20 out of 48 patients (42%) and was categorized into group IIa. In the remaining patients ES was followed by CBD exploration (group IIb). Significantly higher morbidity was seen in patients needing CBD surgery following attempted endoscopic clearance, because of ES-related complications, such as bleeding, cholangitis, septicaemia and numerous others. Use of ES to treat CBD stones on a routine basis was therefore not found to be any better than one-time surgical exploration.     

An autopsy case of sudden death in a patient with sickle cell disease

An autopsy finding of sudden death due to disseminated intra-vascular sickling of RBCs in a young adult male from Madhya Pradesh while undergoing army recruitment rally, is reported because of its rarity in this part of the country.     

AgNORs in endometrial lesions

Silver nucleolar organizer region (AgNOR) staining was employed in one hundred specimens of endometrium. These included fifteen normal controls (Proliferative + Secretory endometrium) and eighty five lesions. Endometrial lesions comprised of endometritis (15), endometrial hyperplasia (25) and endometrial carcinoma (45). Three micron thick sections of paraffin embedded tissue were subjected to AgNOR staining as described by Crocker and Smith with a little modification of 0.01% safranin counterstain--The mean AgNOR scores were found to increase steadily from normal to endometritis to endometrial hyperplasia and carcinoma--The observations revealed statistically significant differences in values between atypical hyperplasia and carcinoma also. AgNOR staining and scoring is simple, inexpensive and a useful adjunct to routine histopathology to evaluate endometrial lesions especially to differentiate borderline lesions. Though scores cannot be standardized and fixed for a particular lesion as there are intralaboratory variations.