Near total intestinal aganglionosis with extreme short-bowel syndrome - A difficult surgical dilemma

R. Finaly, Z. Cohen, A. J. Mares

Research output: Contribution to journalArticlepeer-review

9 Scopus citations


Forty cases of total or near-total intestinal aganglionosis (NTIA) were described to date in the English literature. Most cases had a lethal outcome. We describe the 41st case - a Beduin male neonate - who had only 30 cm of proximal hypoganglionic jejunum. He is presently almost one-year-old and thriving on home TPN, receiving one quarter of his caloric requirements orally using pregestamil, an MCT formula. The initial intricate course, diagnosis and several operative procedures, are elaborated. A review of the scant literature is discussed. The elusiveness of the correct diagnosis is pointed out and means to overcome these errors are described. various surgical procedures have been suggested, none of which offer the perfect solution to the severe basic problem of short bowel. Long-term parenteral hyperalimentation is still the main modality of treatment. Based on our modest experience, we suggest saving every possible length of jejunum, even if hypoganglionic, since this bowel, following a few weeks of adaptation, starts to function fairly well, suggesting perhaps some neuro-muscular maturation. The best surgical approach is still pending. We present a report of a child with this disease and discuss the therapeutic dilemma.

Original languageEnglish
Pages (from-to)253-255
Number of pages3
JournalEuropean Journal of Pediatric Surgery
Issue number4
StatePublished - 1 Jan 1999


  • Near-total intestinal aganglionosis
  • Small-bowel syndrome
  • Surgical techniques

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Surgery


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