Long-term outcome of neonates with suspected Hirschsprung's disease, but normal rectal biopsy

Eur J Gastroenterol Hepatol. 2016 Aug;28(8):917-22. doi: 10.1097/MEG.0000000000000636.

Abstract

Background and objectives: Hirschsprung's disease (HD) must always be considered in very early-onset constipation. Although HD has a well-described clinical course, little is known about those neonates in whom HD was excluded. We aimed to describe the long-term clinical outcomes of neonates with a clinical suspicion of HD that was excluded by rectal suction biopsy.

Methods: This is a single-center double-cohort comparative study. Neonates who underwent rectal mucosa biopsy for suspected HD were age and sex matched with healthy controls. A survey on clinical outcomes, stooling patterns, and other gastrointestinal (GI)-related conditions was sent to parents. Pathology slides were re-reported by an experienced histopathologist blinded to the clinical data.

Results: A total of 51 neonates were included [25 cases, 26 controls; 41% males, median time of follow-up 4.25 years (interquartile range 2.7-6.9)]. Nine (36%) of patients in the case group required prolonged laxative use for constipation during the first year of life compared with 0 (0%) controls (P<0.001). This difference was maintained at the end of follow-up, with 5 (20%) versus 0 (0%), respectively (P=0.02). Case neonates were significantly more likely to be hospitalized or to be diagnosed with a chronic GI-related condition than the controls (33 vs. 12%, P=0.01; and 19 vs. 8%, P=0.04, respectively).

Conclusion: Neonatal constipation is associated with long-term GI-related disorders and should be considered clinically significant even when the diagnosis of HD is excluded. Neonates with early-onset abnormal stooling patterns should be monitored with adequate pediatrician or pediatric gastroenterologist follow-up.

MeSH terms

  • Biopsy
  • Case-Control Studies
  • Child
  • Child, Preschool
  • Constipation / diagnosis*
  • Constipation / drug therapy
  • Constipation / etiology
  • Constipation / physiopathology
  • Defecation* / drug effects
  • Female
  • Hirschsprung Disease / complications
  • Hirschsprung Disease / diagnosis*
  • Hirschsprung Disease / pathology
  • Hirschsprung Disease / physiopathology
  • Humans
  • Infant
  • Infant, Newborn
  • Intestinal Mucosa / pathology*
  • Israel
  • Laxatives / therapeutic use
  • Male
  • Predictive Value of Tests
  • Prognosis
  • Rectum / pathology*
  • Rectum / physiopathology
  • Risk Factors
  • Time Factors

Substances

  • Laxatives