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Acro-pectoro-renal dysplasia

Synonym(s) -
Prevalence <1 / 1 000 000
Inheritance Not applicable
Age of onset Neonatal
  • Q87.8
MeSH -
MedDRA -


Acro-pectoro-renal field defect is an association of a genito-urinary anomaly with Poland anomaly. Poland anomaly is characterized by congenital absence of the pectoralis major muscle with ipsilateral digital and shoulder girdle anomalies. Genito-urinary anomalies may include renal agenesis, renal hypoplasia, duplication of the collecting system, ureterocele, ureteral reflux, hypospadias, undescended testicles or ureteopelvic junction obstruction. In the majority of cases, the genito-urinary anomaly is also ipsilateral. Acro-pectoro-renal field defect is extremely rare; only a dozen cases have been described so far. As in Poland syndrome, boys are more often affected than girls. The etiology is unknown. Intelligence is normal; the prognosis is good with a severity of disability varying from case to case. Multidisciplinary management (urologic, nephrologic, orthopedic, pediatric etc.) must be adapted to each child. Orthopedical management may consist of various methods of surgery and/or prosthetic restoration.

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