Migration and maturation pattern of fetal enteric ganglia: A study of 16 cases.

Aims: To study the migration and developmental pattern of ganglion cells in fetuses aged 9-21 weeks, and to document whether the migration was occurring circumferentially equally in the entire axis or if there were discrepancies in different portions at the same level. Settings and Design: The hypothesis regarding the pathogenesis of Hirschsprung's disease mainly revolves around two schools. One is the single gradient migration of ganglia and the other is a dual gradient migration theory. Understanding the embryological development of enteric ganglia is necessary to study the pathogenesis of intestinal innervation disorders. Materials and Methods: We studied the development of intestinal ganglia in fetuses aged 9-21 weeks. Serial longitudinal sections from the colon were studied, the first one including the squamo-columnar junction, for the presence and the nature of ganglion cells with Hematoxylin and Eosin, and neurone-specific enolase immunostaining. Transverse sections from proximal gut were studied in a similar fashion. Thus, we evaluated the migration pattern as well as the nature of ganglia in the fetuses. We also measured the length of distal aganglionic segment in these growing fetuses. Results: We noted that ganglion cells appear first in the myenteric plexus followed by deep and superficial submucous plexus. We also found evidences in favor of dual migration theory, and the distal aganglionic segment varies around the circumference of the rectal wall. Conclusions: We got evidences in support of a dual migration pattern of intestinal ganglion cells. The level of distal aganglionic segments when measured from squamo-columnar junction varied with the age of gestation and the length was incongruous. The description of distal aganglionic segment may help surgeons while taking biopsies or during operative procedures.

Sickle cell hepatopathy.

Sickle cell hepatopathy is a well-documented entity that ranges from the self-limiting hepatic right upper quadrant syndrome to the potentially lethal intrahepatic cholestasis and acute hepatic sequestration syndromes. We describe a 26-year-male with homozygous sickle cell disease who had this unique hepatic presentation and was documented to have characteristic findings of cholestasis, portal inflammation and sinusoidal dilatation on histopathology.

Primary amyloidosis presenting as intrahepatic cholestasis.

Hepatic involvement in primary amyloidosis is not rare but is often clinically silent. A mild elevation of the serum alkaline phosphatase level and hepatomegaly are the most common findings. We report a case of primary amyloidosis in an adult male presenting with intrahepatic cholestasis where viral, drug, alcohol, and autoimmune etiologies were all excluded and the definite diagnosis was established by liver biopsy with Congo red staining. Subsequently the patient was found to have monoclonal light chain disorder.

Retinal vasculitis--an initial presentation of systemic lupus erythematosus.

Retinal vasculitis is an intra-ocular inflammatory condition with diverse aetiology and a rare manifestation of systemic lupus erythematosus, often associated with antiphospholipid antibodies. A 13 years male presented with fever for 20 days associated with photosensitive skin rashes. Three years back he had suffered painless, progressive dimness of vision. Then a clinical diagnosis of retinal vascular occlusion was made. Examination revealed his visual acuity was reduced to finger counting at 3 metres distance and extensive retinal vasculitis in the right eye. His serum antinuclear factor was positive in high titre. A final diagnosis was made as systemic lupus erythematosus. He was treated with methylprednisolone. His fever subsided, skin rashes healed and vision did not deteriorate further.

Panniculitis-like T-cell lymphoma with fatal termination.

A 58-year-old male presented with three weeks history of multiple subcutaneous nodules and fever. Histopathological feature of the nodule was characteristic of panniculitis. Three months later, the patient presented with carvical lymphadenopathy and compressive thoracic myelopathy and a diagnosis of diffuse mixed- cell lymphoma was established. Immunohistochemical study of subcutaneous lesions confirmed their T-cell origin. Chemotherapy was started but patient succumbed to his disease.