Amniotic Band Sequence Syndrome – A Rare Disorder

Amniotic bands sequence is a inherited diseases distinguished by craniofacial, body wall, and limb oddities that may be in relation with foetal-placental fibrous bands. Its pervasiveness has been delineated to range from 0.19 to 8.1 per 10 000 births [1]. It is a customary state prospectively alike with a variety of different confinements’ disability. The deformities arise in the wake of the pompous bodily part have formed usually in early evolution. The required cause of amniotic disruption complex is undisclosed .The three most usual decorative design are constriction ring syndrome signalized by one or more limbs being high-flown; the limb-body- wall complex; and amniotic band syndrome characterized by abnormalities of the head and face (craniofacial abnormalities), inadequacies of the brain and genuine deformation of the appendages [2]. The condition in few cases might be considered prior to nativity (antepartum), unlikely to change upon the sequels of explicit detailed imaging practices, like fetal USG, which may give away the feature inadequacies [3]. Here we present a case of pregnant woman admitted in our Obstetrics and gynecological department with her ultrasonography report indicative of amniotic band and further discussed about antenatal diagnosis, diagnostic and therapeutic approach, prognosis and genetic counselling of amniotic disruption complex.

Bleeding Post Constriction Ring Release Surgery Causing Lower Limb Ischemia in an Infant with Undetected Hemophilia B

Bleeding in the immediate postoperative period causing compromised limb circulation is an alarming complication. It is known to occur in coagulation disorders like hemophilia. When such complications happen in a child with no previous history of bleeding problems, one has to have a low threshold for suspecting a coagulation disorder. Repeated diffuse bleeding in the whole of the surgical wound with no specific bleeders must raise the suspicion and appropriate laboratory tests must immediately be ordered. Bleeding in coagulation disorders can stop only with supplementation of the appropriate missing clotting factor. Early diagnosis is important to avoid excess morbidity. We are reporting a 6-month-old child who underwent surgery for constriction ring syndrome in the limbs with Z-plasty please replace with and developed impending limb ischemia due to bleeding in the immediate postoperative period. The article emphasizes the need to think of the possibility while encountering recurrent bleeding in the postoperative period.

Salvage Reconstruction of a Congenital Hypoplastic Thumb Due to Constriction Ring Syndrome Using a Third Toe Transfer, When the Usual Donor Sites of Index Finger, Great and Second Toes Are Not Available

Isolated third toe-to-thumb transfers have been rarely reported and none in the pediatric population. We describe a third toe-to-thumb transfer for reconstruction of a congenital hypoplastic thumb with excellent functional results and no morbidity in the donor foot.