Multidisciplinary team for the diagnosis and treatment of congenital band syndrome / 中华实用儿科临床杂志

Objective:To explore the significance of multidisciplinary team for the diagnosis and treatment of congenital band syndrome.Methods:Five children with congenital circular band syndrome admitted in the First Affiliated Hospital of the Chinese People′s Liberation Army Air Force Medical University from January 2017 to January 2020 were retrospectively analyzed.They were diagnosed, treated and followed up by a multidisciplinary team including the department of obstetrics and gynecology, ultrasound, and orthopedics from the fetal stage.Results:One case was found with gradually aggravated ring band that affects the blood circulation of distal limbs during fetal examination, who was promptly performed with partial band release under fetoscopy.All cases had Patterson type Ⅰ ring bands at birth, and distal limbs did not have obvious deformity.They were performed with selective annular band resection, and postoperatively followed up for an average of 10.6 (8-13) months.The annular depression of the skin disappeared, and the appearance and function recovered satisfactory.According to the monitoring of side effects scale (Moses), 4 cases were excellent, 1 case was good, and the excellent and good rate was 100%.Conclusions:The multidisciplinary team for the diagnosis and treatment of congenital band syndrome can maximize the professional advantages of physicians with diffe-rent specialties, make early diagnosis and treatment, minimize the compression of the ring band on the limbs, and avoid serious limb deformities.It is worthy of clinical application.

A Newborn With Constriction Ring Syndrome: A Case Report

@#We report a rare case of constriction ring syndrome in a 3515 grams full term female newborn who was born with a constriction ring encircling the fingers of her right hand diagnosed as acrosyndactyly. It is a rare congenital malformation and the risk factors are still unknown. The neonate had severe swelling of her third and fifth fingers, and skin breaches. There were intrauterine amputations in her second and forth fingers. The X-ray images showed deformities in both the size and shape of the bones of her fingers. No additional abnormalities or comorbidities were found. The neonate was referred to a better equipped hospital because of impending necrosis from autoamputation. During anamnesis, the mother recalled that she took haloperidol and trihexyphenidyl until 15 weeks’ gestation. There was neither history of fever, trauma, intake of herbal preparations, nor family history of deformity. Etiology and risk factors of congenital constriction ring syndrome are still under discussion. Early intervention is recommended for a successful outcome.

Secuencia de bandas amnióticas, una actualización / An update on amniotic bands sequence

La secuencia de bandas amnióticas es un desorden congénito caracterizado por anomalías craneofaciales, de la pared corporal y de las extremidades que pueden asociarse con bandas fibrosas fetoplacentarias. Su prevalencia ha sido reportada entre 0,19 y 8,1 por 10 000 nacimientos. Diversas teorías han tratado de explicar su etiología, sin embargo, ninguna, en forma individual, sustenta todas y cada una de las anomalías observadas, por lo que se ha considerado una entidad multifactorial. La identificación de anomalías (pre-yposnatalmente) sugestivas de secuencia de bandas amnióticas permite el abordaje diagnóstico para efectuar intervenciones terapéuticas oportunas que posibiliten la liberación de bandas amnióticas mediante fetoscopia con recuperación de la perfusión de la porción distal de la extremidad involucrada o bien la reparación quirúrgica posnatal y para otorgar asesoramiento genético. Este artículo ofrece una actualización sobre aspectos epidemiológicos, teorías etiológicas, factores de riesgo, características clínicas, diagnóstico (que incluye el diagnóstico prenatal), asesoramiento genético, abordaje terapéutico y pronóstico de esta entidad.

Amniotic bands sequence is a congenital disorder characterized by craniofacial, body wall, and limb anomalies that may be associated with fetal-placental fibrous bands. Its prevalence has been reported to range from 0.19 to 8.1 per 10 000births. Different theories have attempted to explain the etiology of amniotic band sequence; however, none has individually been able to support each and every defect observed, so it has been considered to be a multifactorial condition. The (pre- and post-natal) identification of anomalies suggestive of amniotic band sequence is useful for the diagnostic approach and implementation of timely therapeutic interventions favoring the release of the amniotic bands using fetoscopy with recovery of the involved distal limb perfusion, or else the possibility of performing a post-natal surgical repair. It is also helpful to provide genetic counseling. This article offers an update on the epidemiological aspects, etiological theories, risk factors, clinical characteristics, diagnosis (including antenatal diagnosis), genetic counseling, therapeutic approach, and prognosis of amniotic bands sequence.

Congenital Constriction Ring Syndrome

In general, excision of a constriction ring is not difficult when performed several months after birth, but care should be taken to excise the scar tissue in newborns so as to avoid neurovascular damage. Although Hall et al. and Greene described successful one-stage operations in cases of forearm and leg constriction rings, a two staged Z-plasty around the circumference of the constriction is recommended in neonatal balloon. The authors in this article excised constriction ring through a single operation with Z-plasty, and restored digital contour. In order to preserve neurovascular structures, the authors employed loupe(x3.5) and prevented inadequate sensation and cold intolerance, which may occur after surgery. A one-stage release of congenital constriction ring was performed for 5 constriction rings in four extremities(one patient). No wound problems occurred, even when there had been marked congestion of the extremity distal to the constriction ring. The one-stage release facilitated postoperative care, and there was no need for additional anesthesia or for additional operations, which are necessary when this problem is treated with a release performed in two or three stages.