Segmental aganglionosis in Hirschsprung s disease in newborns - a case report

Vol. 56 No. 2 Suppl., 2015
This supplement was not sponsored by Outside Organizations.


Radu Ninel Balanescu, Laura Balanescu, Andreea Alecsandra Moga, Gabriel Cristian Dragan, Florin Bogdan Djendov

Segmental aganglionosis Hirschsprung s is an extremely rare condition. Described as a segment of normally ganglionated bowel surrounded proximally and distally by aganglionosis, zonal aganglionosis is of interest because it may cause confusion in interpreting surgical margins. Diagnosis of segmental lesions in Hirschsprung s disease may be missed as it is rarely suspected at initial surgery. We report the case of a 2-week-old baby girl admitted to our clinic for abdominal distension and vomiting. Considering the family history (near total colonic aganglionosis in a 2-month-old sister with unfavorable outcome), the suspicion of Hirschsprung s is raised and serial large intestine biopsies are taken. Intraoperatively, a transverse colon stenosis caused by an incomplete web is noticed and segmental colectomy with anastomosis is performed at this level. Histopathological and immunohistochemical results established the diagnosis of segmental transverse colon aganglionosis, with the presence of ganglia cells in the ascending and descending colon. Subtotal colectomy with ascending colon pull-through was performed with favorable postop evolution. In our patient, the association with a transverse colon stenosis raised suspicion concerning the diagnosis of Hirschsprung s disease, but considering the family history, extended biopsies were taken and the correct diagnosis of zonal aganglionosis was established. Although zonal aganglionosis lesions are extremely rare, this case illustrates the point that the presence of ganglia cells at the resection line is not sufficient to guarantee postoperative function. Extended intestinal biopsies should be included in the algorithm for management of long segment Hirschsprung s disease and will enable the surgeon to correctly detect zonal aganglionosis.

Corresponding author: Laura Balanescu, MD; e-mail:

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Vlad Andrei Budu, Ioan Alexandru Bulescu, Alexandra Schnaider, Cristiana Gabriela Popp, Claudia Lucia Toma, Carmen Aurelia Mogoanta, Gheorghe Muhlfay

Extrapulmonary tuberculosis is a rare condition determined by Mycobacterium tuberculosis. It can affect any organ, and has a higher incidence with the increase of HIV infection, or in countries with high pulmonary tuberculosis. Diagnosis is difficult, mostly because of non-specific symptoms and a low rate of presentation for medical consult when symptoms do occur. Complete diagnosis is usually set by histological, immunohistochemical examinations, and also with Polymerase Chain Reaction (PCR) in selected cases. The authors present a case of concomitant tuberculosis of the nose, paranasal sinuses and subglottic larynx, without primary involvement of the lungs. The diagnosis was imposed by histological examination and immunostaining of probes obtained in surgery. The treatment was surgical debridement followed by specific antituberculosis medication.

Corresponding author: Ioan Alexandru Bulescu, MD, PhD candidate; e-mail:

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