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Fryns syndrome in children with congenital diaphragmatic hernia.

Abstract：

PURPOSE:Fryns syndrome is characterized by multiple congenital anomalies including Congenital Diaphragmatic Hernia (CDH), and has a reported poor prognosis with a survival rate during the neonatal period of approximately 15%. This report details the management and outcome of patients with Fryns syndrome and CDH. METHODS:Records of all liveborn patients with CDH between 1995 and 2001 in 83 hospitals were entered into the CDH database. Those with Fryns syndrome were reviewed retrospectively. RESULTS:A total of 1,833 patients were entered in the database, 23 of these had Fryns (1.3%). All patients experienced early distress requiring intubation. Ten patients (43%) were found to have other major anomalies. Seven patients underwent surgical repair at an average age of 7.5 days (range, 6 hours to 14 days). Mortality rate was 83% compared with 33% of patients with unilateral CDH (P =.01). Ten patients died within the first 24 hours. The parents of 6 patients withdrew support. Of the 4 survivors, 3 have marked developmental delay, whereas the fourth has not yet undergone formal assessment. CONCLUSIONS:The prognosis of infants with Fryns syndrome and congenital diaphragmatic hernia remains grim. Early genetic counseling and recognition of lethal anomalies may assist in determining which patients may survive.

journal_name

J Pediatr Surg

journal_title

Journal of pediatric surgery

authors

Neville HL,Jaksic T,Wilson JM,Lally PA,Hardin WD Jr,Hirschl RB,Langham MR Jr,Lally KP,Congenital Diaphragmatic Hernia Study Group.

doi

10.1053/jpsu.2002.36695

subject

Has Abstract

pub_date

2002-12-01 00:00:00

pages

1685-7

issue

12

eissn

0022-3468

issn

1531-5037

pii

S0022346802002968

journal_volume

37

pub_type

杂志文章

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