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Management strategies for infants with total intestinal aganglionosis

Osamu Kimura1, Shigeru Ono, Taizo Furukawa

  • 1Department of Pediatric Surgery, Graduate School of Medical Science, Kyoto Prefectural University of Medicine, Kyoto 602-0841, Japan. osamu@koto.kpu-m.ac.jp

Journal of Pediatric Surgery
|July 29, 2009
PubMed

Insights

Management of total intestinal aganglionosis (TIA) in infants requires a combination of surgical and nutritional strategies. Short proximal jejunostomy with extended myectomy-myotomy (EMM) and elemental diet feeding improves survival rates in TIA infants.

Area of Science:

  • Pediatric Surgery
  • Gastroenterology
  • Neonatology

Background:

  • Total intestinal aganglionosis (TIA) presents significant challenges in infant management.
  • Surgical and medical interventions are crucial for improving outcomes in affected infants.

Purpose of the Study:

  • To investigate optimal surgical and medical management strategies for infants diagnosed with total intestinal aganglionosis (TIA).

Main Methods:

  • Retrospective review of six infants with TIA treated between 1980 and 2007.
  • Surgical approaches included jejunostomy at varying distances below the ligament of Treitz (LOT), with and without extended myectomy-myotomy (EMM).

Main Results:

  • Infants receiving jejunostomy without EMM or placed too distally (65-70 cm below LOT) had poor outcomes, including death from sepsis or liver failure.
  • Three infants who underwent short proximal jejunostomy (30-35 cm below LOT) with EMM survived long-term.
  • Parenteral nutrition and elemental diet feeding were associated with reduced complications like enteritis and sepsis.

Conclusions:

  • A combination of short proximal jejunostomy with EMM and nutritional support (parenteral nutrition and elemental diet) is key for TIA infant survival.
  • Growth hormone (GH) administration may be considered for infants with persistent absorptive issues post-EMM.
Abstract

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