Constipation in children with neurofibromatosis type 1
CE Pedersen, K Krogh, C Siggaard… - Journal of pediatric …, 2013 - journals.lww.com
CE Pedersen, K Krogh, C Siggaard, IM Joensson, A Haagerup
Journal of pediatric gastroenterology and nutrition, 2013•journals.lww.comMethods: A total of 20 children with NF1 (age 8.2±2.4 years) were evaluated with medical
history; clinical examination; digital rectal examination; bowel and dietary diaries; Rome III
criteria; measurement of rectal diameter by transabdominal ultrasound; and radiographic
estimation of CTT. The control group for assessment of rectal diameter comprised 23 healthy
children (mean age 9.1±2.7 years). Results: A total of 6 children with NF1 (30%) were
constipated according to Rome III criteria. Average rectal diameter was significantly larger …
history; clinical examination; digital rectal examination; bowel and dietary diaries; Rome III
criteria; measurement of rectal diameter by transabdominal ultrasound; and radiographic
estimation of CTT. The control group for assessment of rectal diameter comprised 23 healthy
children (mean age 9.1±2.7 years). Results: A total of 6 children with NF1 (30%) were
constipated according to Rome III criteria. Average rectal diameter was significantly larger …
Methods:
A total of 20 children with NF1 (age 8.2±2.4 years) were evaluated with medical history; clinical examination; digital rectal examination; bowel and dietary diaries; Rome III criteria; measurement of rectal diameter by transabdominal ultrasound; and radiographic estimation of CTT. The control group for assessment of rectal diameter comprised 23 healthy children (mean age 9.1±2.7 years).
Results:
A total of 6 children with NF1 (30%) were constipated according to Rome III criteria. Average rectal diameter was significantly larger than for healthy children (32.9±7.2 mm vs 21.4±5.9 mm, P< 0.0001). Median CTT in NF1 children was 53 hours (range 26–101). Compared with existing normative data, CTT was prolonged (> 84 hours) in 3 (19%).
Conclusions:
Symptoms of constipation were surprisingly common in children with NF1. Correspondingly, rectal diameters were abnormally large and a higher proportion than expected had prolonged CTT. The underlying pathophysiology remains obscure, but we hypothesise that abnormalities of the enteric nervous system or disturbed cellular growth could be present.
Lippincott Williams & Wilkins