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Comparative study of clinical characteristics of amniotic rupture sequence with and without body wall defect: Further evidence for separation

✍ Scribed by Aleksander Jamsheer; Anna Materna-Kiryluk; Magdalena Badura-Stronka; Katarzyna Wiśniewska; Barbara Więckowska; Jan Mejnartowicz; Anna Balcar-Boroń; Maria Borszewska-Kornacka; Mieczysława Czerwionka-Szaflarska; Elżbieta Gajewska; Urszula Godula-Stuglik; Marian Krawczynski; Janusz Limon; Józef Rusin; Henryka Sawulicka-Oleszczuk; Ewa Szwałkiewicz-Warowicka; Janusz Świetliński; Mieczysław Walczak; Anna Latos-Bieleńska


Publisher
John Wiley and Sons
Year
2009
Tongue
English
Weight
75 KB
Volume
85
Category
Article
ISSN
1542-0752

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✦ Synopsis


Abstract

BACKGROUND: Amniotic rupture sequence (ARS) is a disruption sequence presenting with fibrous bands, possibly emerging as a result of amniotic tear in the first trimester of gestation. Our comparative study aims to assess whether there is a difference in the clinical pattern of congenital limb and internal organ anomalies between ARS with body wall defect (ARS‐BWD) and ARS without BWD (ARS‐L). METHODS: Among 1,706,639 births recorded between 1998 and 2006, 50 infants with a diagnosis of ARS were reported to the Polish Registry of Congenital Malformations. The information on 3 infants was incomplete, thus only 47 cases were analyzed. These infants were classified into groups of ARS‐L (38 infants) and ARS‐BWD (9 infants). RESULTS: The ARS‐BWD cases were more frequently affected by various congenital defects (overall p < 0.0001), and in particular by urogenital malformations (p = 0.003). In both groups, limb reduction defects occurred in approximately 80% of cases; however, minor and distal limb defects (phalangeal or digital amputation, pseudosyndactyly, constriction rings) predominated in the ARS‐L group (p = 0.0008). The ARS‐L group also had a higher frequency of hand and upper limb involvement. CONCLUSIONS: This observation suggests that amniotic band adhesion in ARS‐L takes place at a later development stage. Although limited by a small sample size, our study contributes to the growing evidence that both ARS entities represent two nosologically distinct conditions. Birth Defects Research (Part A), 2009. © 2009 Wiley‐Liss, Inc.