Hirschsprung's disease, associated rare congenital anomalies.

OBJECTIVE: Hirschsprung's disease may be associated with a number of congenital anomalies of which Down's syndrome and intestinal atresias are commonly encountered. The study aimed to assess the impact of rare associated anomalies on the diagnosis and management of Hirschsprung's disease. METHODS: A retrospective review of the clinical presentation, diagnosis and outcome of thirty five consecutive newly diagnosed cases of Hirschsprung's disease encountered over two years was performed. RESULTS: Besides Down's syndrome (two), intestinal atresia (one) and pigmentary ocular defects (two), three rare anomalies (Occipital meningocele, Calcific meconium cyst with anal stenosis, Malrotation) were encountered in four of thirty five cases. The clinical features, radiologic anatomy and gross morphology of the bowel were unconventional and the diagnosis was supported by intraoperative acetylcholinesterase staining of biopsies. Though the diagnosis was relatively delayed in these cases, the outcome has been comparable to the rest. The dilemma in their diagnosis and management and their possible pathoembryology is discussed. CONCLUSION: Awareness of such associations and a specific investigative protocol is imperative for timely diagnosis and minimal morbidity in complex presentations of Hirschsprung's disease.

Non familial juvenile polyposis presenting as chronic intestinal obstruction.

Juvenile polyps are the commonest colonic polyps seen in children and present most often with rectal bleed. Intussusception is an extremely rare presentation in juvenile polyposis (JP) syndrome. This case highlights the rare association of ileo-colic intussusception with non-familial Juvenile Polyposis.

Neural hypertrophy in carcinoma stomach.

Neural hypertrophy with hyperplastic Schwann cells in the wall of the stomach along with enterochromaffin cell hyperplasia was incidentally observed at histology in the gastrectomy specimen of a 43-year-old man with carcinoma stomach who had presented with upper abdominal pain of one year duration. The patient had no previous abdominal surgery or evidence of gastrointestinal obstructive pathology. The significance of this neural hypertrophy is not known.

Can we diagnose early Barrett's oesophagus?

OBJECTIVES: The definition of Barrett's oesophagus, has been of late, is stressed on the presence of specialised columnar epithelium with goblet cells and not on other metaplastic epithelia, which however could be the early, immature, undifferentiated cell types such as junctional and gastric types. Hence this study was carried out to find out the ways of detecting the early metaplastic cell forms before the goblet cells appear. METHODS: Among 134 cases of reflux oesophagitis selected in the prospective study of 7 years from January 1992 to December 1998, 31 biopsies were diagnosed as Barrett's oesophagus (BO). These were compared with 20 age matched control biopsies from gastro-oesophageal junction. They were studied morphologically, morphometrically and histochemically for acid, neutral and sulphomucins using alcian blue (AB), alcian blue-periodic acid Schiff (AB-PAS) and high iron diamine-alcin blue (HID-AB) stains. RESULTS: Twelve of the cases were of junctional type, 16 of specialised columnar intestinal metaplastic (IM) type (Type I = 0, Type II = 5, Type III = 11) and three showed a combination of both junctional and Type I IM. Mucins were a mixture of neutral mucin, sialomucin and sulphomucin while the control biopsies showed chiefly neutral mucin. Morphometrically, the epithelial cells in BO were taller (mean 28u in junctional types and 32u in IM) in comparison to the control (mean 24.5 u). CONCLUSION: When the classical specialised columnar epithelium (with goblet cells) in oesophagus is absent, presence of metaplastic junctional type of mucosa, may, for all probabilities be considered as the initial step towards transformation to the classical Barrett's mucosa. In such situations, it is essential to differentiate it from the normal gastro-oesophageal junctional epithelium and is possible with histochemical analysis of the biopsy material aided by morphometry, especially so when the level of biopsy is not indicated.

Colonic carcinomas masquerading as Crohn's colitis.

When colonic carcinomas present with acute abdomen, the operating surgeon and the pathologist face a plethora of diagnostic and therapeutic problems. In this retrospective study of 92 cases of carcinoma colon, 4 presented with acute intestinal obstruction of which three had a turbulent post operative period and died. The resected colonic segment showed on gross examination cobblestone appearance characteristic of Crohn's disease but microscopically was ischemic with the stricture site showing features of an infiltrating poorly differentiated adenocarcinoma. We have made an attempt to study the various pathologic features and analyse their significance with reference to prognosis.

Cytodiagnosis of Kimura's disease.

Kimura's Disease, a rare chronic inflammatory disorder with a benign course affecting the lymphoid tissue is reported hare in an 18 year old South Indian male who presented with bilateral solitary swelling over the mastoid region. Its fine needle aspiration cytology characterised by the Warthin-Finkeldey type giant cells against a background of a bimodal population of lymphocytes and eosinophils prompted the diagnosis of Kimura's disease at cytology itself. This was confirmed at histology. This is the third cytological report of its kind being reported in English literature. Literature with regard to the cytological appearances of Kimura's disease has been reviewed and the various differential diagnoses discussed.

Squamous cell carcinoma of the vulva in association with mixed vulvar dystrophy. A brief report with review of literature.

Of the 74 patients of vulvar lichen sclerosus diagnosed over a period of 29 years from 1966-1995, two cases of mixed dystrophies characterised by lichen sclerosus with squamous cell hyperplasia were studied and one of these on close follow-up, developed an invasive squamous cell carcinoma after three years. The other case has been doing well and has not shown further progression of the lesion. The remaining 72 patients of vulvar lichen sclerosus alone have shown no signification change on close follow-up for a minimum of five years. This paper highlights the fact that all cases of vulvar dystrophy especially, lichen sclerosus must be regularly followed up for development of squamous cell hyperplasia and their progression to overt vulvar carcinoma, emphasizing the need for early diagnosis and treatment of non-neoplastic vulvar dystrophies.

Choriocarcinoma of the ovary in a post-menopausal woman.

A case of a Choriocarcinoma of the Ovary has been reported in a 50 year old post-menopausal woman. At laparotomy, the lesion was seen as a large necrotic ovarian mass. The problems of distinguishing Gestational from Non-gestational ovarian choriocarcinoma are discussed. Cytogenetic studies are indicated to investigate potential reasons for the difference in prognosis.

Post-transplantation epididymitis associated with cytomegalovirus.

Cytomegalovirus (CMV) infection, though usually systemic, has been known to cause localised involvement of organs like lung, liver, testis and gastrointestinal tract. We report a case of cytomegaloviral infection involving the epididymis without systemic manifestations in an young male one month after renal transplantation. The diagnosis was made on histopathologic examination of the epididymo-orchidectomy specimen. Clinical improvement occurred after the emergency epididymo-orchidectomy. To the best of our knowledge, only three cases of CMV epididymitis have been described in the world literature--two in transplant patients and one in an AIDS patient.

Rhinoentomophthoromycosis: a report of three cases.

Three cases of rhinoentomophthoromycosis involving the nasal region are presented diagnosis in the first two cases was made on surgical biopsy and in the third case by fine needle aspiration biopsy. A granulomatous inflammatory reaction was seen in tissue around hollow broad hyphae of the fungus. The hyphal strands were surrounded by eosinophilic deposits Splendore--Hoeppli phenomenon.

Intestinal metaplasia in benign and malignant gastric lesions.

The purpose of this study has been to determine the prevalence of intestinal metaplasia (IM) and its subtypes in normal, benign and malignant states, with special reference to the significance of presence as well as location of sulphomucins in the metaplastic epithelium. Sixty-seven specimens of the normal, benign chronic gastric disease and gastric carcinoma have been studied. The overall prevalence of IM in gastric carcinoma has been found to be much higher (67 percent) than in the normal (nil) and benign lesions of stomach (11 percent). The sulphomucins producing IM also has a higher prevalence in gastric carcinoma (70 percent) but can occur in benign conditions also (40 percent). On the other hand Type III IM, characterized by sulphomucins in the columnar cells is seen only in gastric carcinoma, and not in the benign lesions.