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Birth Defects Res. 2019 Mar 1;111(4):222-228. doi: 10.1002/bdr2.1442. Epub 2018 Dec 27.

Limb body wall complex: Its delineation and relationship with amniotic bands using clustering methods.

Author information

1
ECLAMC (Estudio Colaborativo Latinoamericano de Malformaciones Congénitas) at Hospital Materno Infantil Ramon Sarda, Universidad de Buenos Aires, Buenos Aires, Argentina.
2
ECLAMC at CEMIC (Centro de Educación Médica e Investigaciones Clínicas), Conicet, Buenos Aires, Argentina.
3
CIC (Comisión de Investigaciones Científicas), Buenos Aires, Argentina.
4
INAGEMP (Instituto Nacional de Genética Médica Poblacional), Rio de Janeiro, Brazil.
5
IMBICE (Instituto Multidisciplinario de Biología Celular), Buenos Aires, Argentina.

Abstract

BACKGROUND:

Despite the numerous reports on the limb body wall complex (LBWC), this association has never been adequately defined. Amniotic bands (AB) are frequently present but their role remains unclear. Since most reports were based on clinical and often subjective diagnoses, the aim of this work was to define LBWC and the role of AB, minimizing subjectivity.

METHODS:

Data were obtained from the ECLAMC maternity hospitals network database. A total of 450 live and stillborn infants, born during 1967-2013, with AB or the LBWC were selected. A hierarchical cluster analysis was used to classify cases into homogeneous groups (sharing similar associated defects); robustness of the classification was confirmed with a discriminant analysis. The frequency of associated defects was compared among groups; those whose frequency differed significantly were included in a logistic regression to establish their association within each group.

RESULTS:

The cluster analysis identified two groups: a body wall defect (BWD) predominating in one, AB in the other. These groups were further divided into: BWD (cases with only BWD), AB (with only AB), BWD + AB, and NONE (with neither). Association with caudal defects and lower limb amelia was observed for BWD, with cephalic defects and upper limb amputations for BWD + AB.

CONCLUSIONS:

The results, obtained with the least possible subjectivity, indicated that BWD and BWD + AB are different conditions. Since BWD specifically associates with amelia, we propose that this defect and not any limb deficiency should be considered as inclusion criterium and that it should be included in the BWD acronym as LBWC.

KEYWORDS:

LBWC; amelia; amniotic bands; body wall defects; cluster analysis; limb body wall complex

PMID:
30589520
DOI:
10.1002/bdr2.1442

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