Umbilical cord ulcer - intestinal atresia

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Classification according to ICD-10
Q41.- Congenital absence (s), atresia and stenosis of the small intestine
Q42.- Congenital absence, atresia and stenosis of the large intestine
P02.6 Damage to the fetus and newborns due to other and unspecified conditions of the umbilical cord
ICD-10 online (WHO version 2019)

Umbilical Cord Ulcers - Intestinal atresia is a very rare congenital syndrome with a combination of umbilical cord ulcers and intestinal atresia . The main clinical feature is severe intrauterine bleeding .

The first description comes from 1991 by the American pathologists Robert W. Bendon, R. Wes Tyson, Virginia J. Baldwin and coworkers.

distribution

The frequency is given as less than 1 in 1,000,000, so far about 66 affected people have been reported. The cause is so far unknown. Umbilical cord ulceration develops in 6–14% of unborn babies with intestinal atresia of the upper gastrointestinal tract . It is believed that these with in utero success forming regurgitation of bile related. Vascular overreactions and ischemia are also discussed .

Clinical manifestations

Clinical criteria are:

diagnosis

The suspected diagnosis can be made by fine ultrasound , a polyhydramnios is the first indication. Evidence of blood in the amniotic fluid or visible umbilical cord bleeding in the color Doppler are alarm signals.

Differential diagnostics

The following are to be distinguished:

Healing prospects

The prognosis is considered unfavorable with a high mortality intrauterine or as a newborn.

literature

  • M. Ohyama, Y. Itani, M. Yamanaka, K. Imaizumi, T. Nishi, R. Ijiri, Y. Tanaka: Umbilical cord ulcer: a serious in utero complication of intestinal atresia. In: Placenta. Volume 21, Number 4, May 2000, pp. 432-435, doi: 10.1053 / plac.1999.0480 , PMID 10833382 .
  • M. Yamanaka, M. Ohyama, M. Koresawa, M. Kawataki, I. Ohsaki, Y. Tanaka: Umbilical cord ulceration and intestinal atresia. In: European journal of obstetrics, gynecology, and reproductive biology. Volume 70, Number 2, December 1996, pp. 209-212, doi: 10.1016 / s0301-2115 (95) 02559-6 , PMID 9119106 .

Individual evidence

  1. ↑ a b c d e f Umbilical cord ulcer - intestinal atresia. In: Orphanet (Rare Disease Database).
  2. ↑ RW Bendon, RW Tyson, VJ Baldwin, KA Cashner, F. Mimouni, M. Miodovnik: Umbilical cord ulceration and intestinal atresia: a new association? In: American Journal of Obstetrics & Gynecology . Volume 164, Number 2, February 1991, pp. 582-586, doi: 10.1016 / s0002-9378 (11) 80026-8 , PMID 1992705 .

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