PSEUDO AINHUM - A PROSPECTIVE STUDY

Abstract

Background: Amniotic band syndrome also known as ADAM complex, amnioticband sequence, congenital constrictions, bands and Pseudo Ainhum is a congenital disordercaused by entrapment of fetal parts (usually a limb or digits) in fibrous amniotic bands while inutero. 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 & continuesthroughout life if not checked and treated . It is not an uncommon condition in our country butunfortunately data remains scarce. Design: A prospective study. Setting: Pediatric & GeneralSurgical Department of CMH Multan. Period: June 2010 to June 2012. Materials and methods:Characteristic progressive constriction band was undertaken. The patients were examinedclinically 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 byexcision and Z-plasty to prevent or alleviate lymphoedema, separation of distally fused digits andskin grafting of defect. Results: Fifteen patients comprising of ten male & five females wereseen. Age of presentation ranged from second week of life to 12 years with mean age of 14months. A total of twenty one limbs and one penile shaft were effected. These included two rightupper limbs, three left upper limbs, ten right lower limbs and six left lower limbs. Penile shaft ofone of the patients just short of glans was involved. Four main types of lesions were found whichcomprised of constriction rings, intra uterine amputations, acrosyndactyl and simple syndactyl.Conclusions: Pseudo Ainhum is a disease of unknown etiolgy & could cause morbidity in newborn. The syndrome & its complications are amenable to corrective surgery with good results.Early surgery is required for successful outcome.