Pseudo ainhum - a prospective study

ABSTRACT

Amniotic band syndrome also known as ADAM complex, amniotic band sequence, congenital constrictions, bands and Pseudo Ainhum is a congenital disorder caused by entrapment of fetal parts [usually a limb or digits] in fibrous amniotic bands while in utero. Syndrome which is featured by one or more characteristic fibrous bands that encircle, deepen, strangulate and amputate parts of the human body. It start in a fetus and continues throughout life if not checked and treated. It is not an uncommon condition in our country but unfortunately data remains scarce. A prospective study. Pediatric and General Surgical Department of CMH Multan. June 2010 to June 2012. Characteristic progressive constriction band was undertaken. The patients were examined clinically and findings recorded. An X-ray to rule out underlying bone anomaly was done. Treatment required multiple staged procedures. Correction of the constriction ring was done by excision and Z-plasty to prevent or alleviate lymphoedema, separation of distally fused digits and skin grafting of defect. Fifteen patients comprising of ten male and five females were seen. Age of presentation ranged from second week of life to 12 years with mean age of 14 months. A total of twenty one limbs and one penile shaft were effected. These included two right upper limbs, three left upper limbs, ten right lower limbs and six left lower limbs. Penile shaft of one of the patients just short of glans was involved. Four main types of lesions were found which comprised of constriction rings, intra uterine amputations, acrosyndactyl and simple syndactyl. Pseudo Ainhum is a disease of unknown etiolgy and could cause morbidity in new born. The syndrome and its complications are amenable to corrective surgery with good results. Early surgery is required for successful outcome: