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.

Further insights into unusual acrania-exencephaly-anencephaly sequence caused by amniotic band – first trimester fetoscopic correlation with two- and three-dimensional ultrasound

Objectives Antenatal detection of acrania-exencephaly-anencephaly (AEA) sequence beyond 10 completed weeks of gestation is usually straight-forward. An earlier detection and classification of the causative conditions prior to disaggregation of exposed dysplastic brain tissue remains challenging. Case presentation We present two- and three-dimensional ultrasound correlated with fetoscopic findings of an unusual type of neural tube defect at 11 gestational weeks caused by an amniotic constriction ring resulting in cystic degeneration of the developing skull. Conclusions By giving further illustrative insights into early defective brain development, this report confirms recent findings of an unusual subtype of acrania-exencephaly-anencephaly sequence suggesting early disruption of the developing brain, following an amniotic entrapment of the skull.

SURGICAL TREATMENT OUTCOME OF AMNIOTIC BAND SYNDROME (ABS) INVOLVING THE FINGER AND LEG WITH INFECTION OF A FOUR-MONTHS MALE CHILD: A CASE REPORT

ABSTRACTAmniotic band syndrome is an uncommon congenital disorder without any genetic or hereditary predisposition factor. It involves fetal entrapment in strands of amniotic tissue and causes an array of deletions and deformations. The aim of this article was to report surgical treatment outcome of amniotic band syndrome finger and infected intrauterine leg amputation. A case of four months male child with complaint of incomplete formation and constricting band on his left leg was reported. On the stump of left leg initially there was a small lump. Over time the size of the stump grew bigger and infected wound appeared. Other deformities were constricting band on proximal phalanx of the middle finger of left hand, acrosyndactyly of first and second toe of right foot and congenital scrotalis hernia. We performed a release surgery of constriction ring band of proximal phalanx of the middle finger of left hand with z-plasty incision and below knee amputation for left leg. We followed up patient in one year after operation. The patient complained no pain and no sign of infection. Patient could walk normally and independently with good activity daily living. We concluded that procedure with z-plasty incision had good result and avoided morbidity. Below knee amputation procedure and application of suitable prosthesis provided satisfying outcome on patient activity daily living and ambulation. Key words: Amniotic band syndrome, intrauterine amputation, z-plasty, infectioABSTRAKSindrom amniotic band merupakan kelainan bawaan yang jarang terjadi dan tanpa adanya kecenderungan faktor genetik atau keturunan. Kelainan ini diakibatkan terlilitnya janin dalam untaian jaringan amnion dan menyebabkan berbagai jenis kehilangan dan kecacatan. Tujuan dari artikel ini adalah melaporkan luaran tatalaksana operasi sindrom amniotic band pada jari tangan dan amputasi pada tungkai yang terinfeksi intrauterine. Pasien adalah seorang anak laki-laki empat bulan dengan amputasi tungkai bawah kiri disertai constriction band pada bagian proksimal. Pada ujung tungkai bawah kiri didapatkan benjolan dengan ukuran minimal yang makin lama ukuran sisi distal menjadi lebih besar dan tampak luka dengan nanah. Kelainan bentuk lainnya adalah constricting band pada phalanx proksimal jari tengah tangan kiri, acrosyndactyly jari kaki pertama dan kedua kaki kanan serta congenital hernia skrotalis. Kami melakukan operasi release constriction ring band phalanx proximal jari tengah tangan kiri dengan irisan z-plasty dan below knee amputation tungkai kiri. Follow up pasien setelah operasi satu tahun. Tidak ada keluhan nyeri, tidak ada tanda infeksi, pasien dapat berjalan normal dan aktivitas sehari-hari dengan mandiri. Kesimpulan bahwa prosedur dengan irisan z-plasty memberikan hasil yang baik dan menghidari morbiditas pasien. Prosedur below knee amputation dan penggunaan prosthesis yang tepat memberikan hasil yang memuaskan pada aktivitas sehari-hari dan ambulasi pasien. Kata kunci: Sindrome amniotic band, amputasi intrauterine, z-plasty, infeksi

The Constriction Ring of Amniotic Band Syndrome Reveals Idiosyncrasies of Wound Repair in Infancy

ABSTRACTWe hypothesized that the constriction rings in Amniotic Band Syndrome (ABS) are the sequelae of localized mechanical injury. Typical scarring pattern was examined in skin tissue of ABS origin, containing an amniotic band constriction, for collagen and elastin distribution, and the ratio of collagen I to collagen III (CI:CIII). A skin sample from an extra finger was the control. In the ABS specimens, sub-epidermal structures were intact and present throughout, and collagen I exhibited a normal basket-weave pattern. At the site of constriction in both ABS samples, reticular dermis elastin fibers were fragmented and papillary dermis elastin fibers were absent. In the control tissue, the reticular dermis contained relatively thick, branching fibers of elastin, and papillary dermis elastin was present.The elastin fragmentation at the constriction ring indicates localized elastin remodeling in response to injury. The absence of elastin in the papillary dermis of the constriction likely indicates a localized disruption in elastin formation. The formation and deposition of collagen and the presence of sub-epidermal structures favor a non-scarring phenotype, while the organization of elastin favors that of localized scarring.Summary StatementA rare, fetal model of healing after constrictive injury of skin, in which elastin fragmentation at the constriction injury indicates remodeling explained by the differential expression of elastin during gestation.