Amniotic band syndrome in a monochorionic diamniotic twin pregnancy after rupture of the dividing membrane in the early second trimester: A case report.

BACKGROUND: Amniotic band syndrome is a rare phenomenon, but it can result in serious complications. We report herein our experience of amniotic band syndrome in a monochorionic diamniotic twin pregnancy where rupture of the dividing membrane occurred early in the second trimester. CASE PRESENTATION: A 29-year-old nulliparous woman was referred to us for management of her monochorionic diamniotic twin pregnancy at 10 weeks of gestation. When we were unable to identify a dividing membrane at 15 weeks of gestation using two-dimensional ultrasonography, we used three-dimensional ultrasonography to confirm its absence. Both modalities showed that the left arm of baby B was swollen and attached to a membranous structure originating from the placenta at 18 weeks of gestation. Tangled umbilical cords were noted on magnetic resonance imaging at 18 weeks of gestation. Emergency cesarean delivery was performed at 30 weeks of gestation because of the nonreassuring fetal status of baby A. The left arm of baby B had a constrictive ring with a skin defect. Both neonates had an uncomplicated postnatal course and were discharged around 2 months after delivery. CONCLUSIONS: Attention should be paid to the potential for amniotic band syndrome if rupture of the dividing membrane between twins is noted during early gestation.

Two case reports: Early detection of amniotic band syndrome by adhesion between hand and umbilical cord at 11 to 14 weeks' gestation.

RATIONALE: The significant ultrasonic characteristics of amniotic band syndrome (ABS) are the malformations of fetal affected parts and the band-like echoes in amniotic cavity. This article first suggests that the fetal hand adhered to umbilical cord with restricted movement provides some values in the diagnosis of ABS in early gestational weeks especially when the fetal malformation is not obvious and amniotic band is thin and fine. PATIENT CONCERNS: Two pregnant women had no discomfort and underwent routine ultrasound examination at 11 to 14 gestational weeks. DIAGNOSIS: Only the fetal hand adhered to umbilical cord with restricted movement was detected during the first ultrasound examination at 11∼14 gestational weeks, and the floating band-like echos were detected in the amniotic cavity with follow-up examinations 2 to 3 weeks later. Both of the 2 fetus were diagnosed as ABS by ultrasound INTERVENTIONS:: The two pregnant women underwent the prenatal counseling and were recommended closely follow-up and further examination. OUTCOMES: Two fetuses died in utero between 17 and 19 weeks. After induction of labor, it was found that the hands and umbilical cord of the fetuses were wrapped by amniotic bands, which was proved pathologically as ABS. LESSONS: The adhesion of the fetal hand and umbilical cord is an important ultrasonic sign suggesting ABS with poor prognosis in early pregnancy. We hope that this study can provide some guidance for the early diagnosis of ABS during 11 to 14 week's ultrasound examination.

Osteomyelitis in limb amputated by amniotic band sequence.

A preterm (30+2 week) neonate with below-knee amputation (right lower limb), constriction rings and syndactyly, subsequent to amniotic band sequence, developed pus discharge from the right tibial stump. The neonate did not have clinical features of systemic sepsis. Blood culture was sterile. The pus culture, however, grew methicillin-resistant coagulase-negative Staphylococcus and bone scan was suggestive of osteomyelitis of right proximal tibial stump. Osteomyelitis was likely caused by the contiguous spread of infection from the exposed stump. Neonate was treated with intravenous antibiotics for 4 weeks and discharged on oral feeds.

Severe Intrauterine Amputations in One Dichorionic Twin With Pentalogy of Cantrell: Further Evidence and Consideration for Mechanical Teratogenesis.

Pentalogy of Cantrell (PC) is characterized by midline supraumbilical abdominal wall defect, lower sternum defect, anterior diaphragmatic and pericardial defect, and congenital cardiac anomalies. Several etiological influences have been postulated, however, most of the reported cases are sporadic. In addition, evidence for mechanical teratogenesis in PC is limited. Here, we describe in one dichorionic twin with complete PC, additional severe intrauterine amputations (mainly head and neck) not previously reported resultant from mechanical teratogenesis. This morphologic constellation prompts us to emphasize the consideration of this etiological influence and provides further evidence. In fact, the pattern of anomalies in the affected fetus provides new insight into the severity and presentation of PC due to mechanical teratogenesis, which is a significant etiological consideration in clinical evaluation and implies that the syndrome involves a complex defective fetal development.

Update on embryology of the upper limb.

Current concepts in the steps of upper limb development and the way the limb is patterned along its 3 spatial axes are reviewed. Finally, the embryogenesis of various congenital hand anomalies is delineated with an emphasis on the pathogenetic basis for each anomaly.

Development of a fetal rabbit model to study amniotic band syndrome.

Amniotic band syndrome (ABS) is a group of fetal malformations caused by fibrous adherences. Species such as sheep, rats, and mice have been used to study this syndrome. We developed a fetal rabbit model using 24 fetuses from punctured uteri. We found one case of syndactyly, one case of amniotic banding, two cases of extremity deformities, one case of a tail deformity, one case of head compression, and one case of open eyelids. Other malformations have been described in an amnion rupture sequence model (exencephaly and cleft palate). The rabbit fetus is an adequate model in which to develop this syndrome.

Síndrome de bridas amnióticas: caso clínico y revisión del tema / Amniotic band syndrome: case report and review of the literatura

El síndrome de bridas amnióticas (SBA) es un conjunto de anomalías congénitas, que asocia lesiones por constricción o amputación de miembros o dedos, asociado a la presencia de bridas amnióticas. Es una entidad con baja incidencia y suele ser de aparición esporádica. Suele cursar con anillos de constricción en la parte distal de los miembros o en los dedos o en casos más graves presentar amputación completa de miembros u asociación con otras malformaciones. El diagnóstico prenatal se produce sólo en el 29-50% de los casos. Presentamos el caso de una paciente con diagnóstico ecográfico de brida amniótica en la semana 12 de gestación (AU)

Amniotic band syndrome is a set of congenital birth defects consisting of constriction rings and limb or digit amputations, associated with the presence of amniotic bands. The incidence of this complication is low and its occurrence is sporadic. This syndrome usually causes constriction rings in the distal end of limbs or digits, which, in severe cases, can lead to complete amputation of the limbs or other malformations. Only 29-50% of cases are diagnosed prenatally. We report the case of a patient with an ultrasonographic diagnosis of amniotic band syndrome in week 12 of pregnancy (AU)

When is fetoscopic release of amniotic bands indicated? Review of outcome of cases treated in utero and selection criteria for fetal surgery.

OBJECTIVES: The objectives are to analyze the outcomes of fetal interventions for fetal limb abnormalities associated with amniotic band syndrome (ABS), to compare the outcome with the known natural history, and to establish selection criteria for fetal intervention. MATERIAL AND METHODS: In a Medline search, six cases of prenatal fetoscopic interventions for ABS were found. An unpublished case was added. RESULTS: Review of the seven cases of treated ABS in utero suggests that abnormal, but present blood flow at Doppler distal to the area constricted by the band may optimally identify cases suitable for fetal surgery. We propose a prenatal classification in stages of cases of ABS based on ultrasound and Doppler findings. Premature rupture of membranes (PROM) occurred in five patients (71%). The median gestational age (GA) at delivery was 34.8 weeks (range 32 to 39). The median time between procedure and PROM was 6 weeks (range 4 days to 14.3 weeks). The median time between procedure and delivery was 11.8 weeks (range 5-17). CONCLUSION: The use of a uniform prenatal classification of cases of ABS may allow a more precise correlation of prenatal findings and postnatal outcome.

Circumferential abdominal skin defect possibly due to umbilical cord encirclement.

We report on a newborn black male twin with a distinctive circumferential abdominal skin defect who was identified through the Active Malformation Surveillance Program at the Brigham and Women's Hospital. There were no other malformations, and amniotic disruption was not present. Although it cannot be proven, we believe that this skin defect may have been caused by in utero encirclement of the abdomen by an umbilical cord.

Amniotic band facies.

Craniofacial deformities of 14 patients with amniotic band syndrome at one institution were reviewed for morphologic similarities. In addition to associated cleft lip and palate, vertical and oblique facial clefts, which were not associated with embryologic lines of fusion, were seen. It is hypothesized that the prominence of the nasal processes combined with the adjacent stomodeal orifice results in utero surfaces, which can lead to free band attachment and adherence, resulting in a spectrum of similarly oriented facial defects.

Chorioamniotic membrane separation: a potentially lethal finding.

Sonographic detection of chorioamniotic membrane separation (CMS) has been considered a benign incidental finding. We now report 6 cases of CMS identified by prenatal ultrasound; 1 in an otherwise normal pregnancy and 5 following fetal surgery. Following membrane separation, amniotic bands formed and compromised the umbilical cord in 4 cases leading to 2 fetal deaths. In the first case, CMS was detected by ultrasound at 22 weeks' gestation in an otherwise uncomplicated pregnancy. Because CMS was considered benign and umbilical cord blood flow was ample, the mother was followed by intermittent sonographic examinations. Fetal demise occurred 2 weeks later, clearly due to umbilical cord strangulation by an amniotic band. Surprised by this unexpected outcome, we reviewed our experience with CMS after hysterotomy for fetal surgery. Out of more than 40 fetal surgical cases, we have 5 cases in which CMS was recognized after hysterotomy. Three of these fetuses had umbilical cord compromise by a band of amniotic membrane leading to 1 fetal death. This experience demonstrates that membrane separation may be associated with amniotic band formation which can lead to cord strangulation and fetal compromise. Following fetal surgery, serial ultrasound evaluation and close fetal monitoring are indicated. In otherwise unremarkable pregnancies, clinician awareness of the possibility of amniotic band formation following CMS should be heightened. In either situation, knowledge of this potential life-threatening complication may identify cases in which cord compromise requires emergent delivery or fetoscopic release of the strangulating amniotic band.

A new in utero model for lateral facial clefts.

The etiopathogenesis behind the formation of atypical craniofacial facial clefts remains unknown. To test the hypothesis that physical restricting forces such as amniotic bands can lead to the formation of these unusual clefts in the postorganogenesis period, we have modified a previously reported fetal lamb model of amniotic band syndrome to examine the effects of these bands on craniofacial development. Five 70-day gestation fetal lambs (term, 140 days) were exposed via a maternal hysterotomy. In each animal, an attempt was made to create a lateral craniofacial cleft by applying a 2-0 nylon suture as a constriction band to the growing face. The sutures were attached to either the zygomatic arch or the infraorbital rim externally and then looped circumferentially into the oral commissure. Each suture was positioned so as to create either a Tessier type 5 or a Tessier type 7 cleft. Four of five fetal lambs survived to term. Both types of lateral facial clefts were effectively produced using this model. In each group, the presence of an intraoral constriction band led to the formation of macrostomia, with an average 7.4-mm lateral displacement of the oral commissure. In addition to these soft tissue changes, each animal also had partial bony clefting (i.e., a bony groove) induced by the pressure of the restriction band across the growing facial skeleton. In the two lambs with the Tessier type 7 cleft, incomplete bony clefts developed across the zygomatic arch. In three animals with bands placed across the medial infraorbital rim, significant infraorbital and malar bony clefts formed similar to a classic Tessier type 5 facial cleft. No evidence of tissue necrosis, maceration, or ulceration was noted in any animal. These data present, for the first time, evidence that the constriction of craniofacial growth by external forces such as a swallowed amnionic band can lead to the development of lateral facial clefting involving both soft tissue and bony elements. These malformations are likely due to a combination of directly tethering normal tissue migration and an increase in local pressure, which produces cellular ischemia and apoptosis. Furthermore, our data demonstrate that these clefts can occur later in fetal development during a period of facial growth rather than during the period of primary facial morphogenesis.

Human prenatal craniofacial development related to brain development under normal and pathologic conditions.

A survey is given of current knowledge of the interrelationship between facial, cranial and brain development in humans. First, normal facial, cranial (mandible, maxilla, palatine bone, cranial base, theca cranii, dentition), and brain development are described separately. Then, developmental interrelationships are illustrated under normal and pathologic conditions (cleft lip and palate, holoprosencephaly, anencephaly, amniotic band sequence). New observations are described in detail, and references are given to previously published articles. A close interconnection exists between the development of the face, the craniofacial skeleton, and the brain. This is illustrated by new observations in cleft palate fetuses and new theories about the etiology of holoprosencephaly and tooth agenesis. The survey focuses, moreover, on the importance of the face and the cranial base in endocrine development. Borderlines between face regions and cranial regions with different developmental origin are set up for future elucidation of the etiology behind syndromes involving the craniofacial regions.

Bridas amnioticas: tratamento cirurgico / Amniotic bands: surgical treatment

Neste trabalho, os autores propoe a realizacao, num unico estagio operatorio de multiplos Z, apos a resseccao total de bridas amnioticas congenitas ou bandas constrictivas de membros. Apresentam aspectos embriologicos da deformidade e discutem, ainda que, superficialmente, fatores etiopatogenicos. Os resultados em dez doentes operados foram considerados bons. A tecnica e aplicada por outros autores e tem apoio na morfologia da brida e na circulacao cutanea do segmento distal afetado. A cirurgia deve ser realizada mais precocemente possivel, dada as possibilidades de afeccoes cutaneas, edemas linfaticos e ate gangrena do segmento distal. Acrescem-se a estes fatores aspectos socios economicos, porquanto, reduz o numero de internacoes.