Apart from abdominl muscle deficiency there may be associated narrowing or stenosis of pulmonary artery, mental retardation and deafness. It occurs both in males and females. It results as hypoplasia or aplasia of the abdmoinal muscle, presenting a thin, loose, wrinkled and prune like abdominal with furrow like umbilicus.

Other names

• Eagle - Barrett syndrome
• Fröhlich syndrome
• Obrinsky syndrome
• abdominal muscle deficiency anomalad
• abdominal muscle deficiency syndrome
• abdominal musculature aplasia syndrome
• absence of abdominal muscle syndrome
• aplastic abdominal muscle syndrome
• defective abdominal wall syndrome

Features of this disease

• Nose: Recurrent epistaxis.
• Mouth and oral structure: Cleft lip.
• Thorax: Pigeon chest.
• Abdomen: Hypoplastic or aplasticf abdominal muscles with thina nd lax abdominal wall causing distension and protrusion of abdomen.
• Hand and foot: Talipes planus.
• Extremities: Genu valgum.
• Skin: Multiple telengiectases of skin and mucus membrane.
• Cardiovascular system: Pulmonic stenosis, heart murmur
• Urogenital system: Enlarged bladder, bilateral hydroureter, hydronephrosis, persistent urachus, cryptorchidism.
• Growth and development: Mental retardation.
• ENT: Deafness.

Treatment

Surgical reconstruction of abdominal wall.