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BACKGROUND: Amniotic Band Syndrome (ABS) is a rare congenital condition characterized by the formation of fibrous bands within the amniotic sac that can entangle and restrict fetal development, leading to various deformities. In this report, we present an unprecedented case of fetal amniotic band ingestion in a monochorionic diamniotic (MCDA) twin pregnancy. Reporting this case is essential for expanding the understanding of the diverse presentations of amniotic bands, considering this rare entity in the differential diagnosis of prenatal ultrasound findings, and emphasizing the importance of vigilant prenatal monitoring and individualized management strategies. CASE PRESENTATION: A 35-year-old gravida 2, para 1 woman with an MCDA twin pregnancy experienced an uneventful pregnancy until the 33rd week, when routine ultrasonography revealed mild intrauterine growth restriction in both twins. At 35 weeks and 5 days gestation, the patient presented with mild abdominal pain. An emergency ultrasound revealed that Twin A ingested an amniotic band. Despite the absence of overt fetal distress, a comprehensive risk assessment was conducted, taking into account potential complications, such as airway obstruction and gastrointestinal issues due to prolonged amniotic band ingestion. Considering the risks versus the benefits of continued gestation and the near-term status, an emergency cesarean section was performed. The procedure resulted in the successful delivery of both twins with satisfactory Apgar scores. A 9-cm fibrous strand was promptly extracted from Twin A's oral cavity after delivery, confirming prenatal diagnosis. Both neonates underwent thorough examinations, revealing no additional anomalies, and demonstrated normal development during the nine-month follow-up. CONCLUSION: This case highlights the inherent challenges in diagnosing and managing the rare presentations of amniotic band-related complications, particularly in MCDA twin pregnancies. The instance of fetal amniotic band ingestion underscores the vital role of comprehensive prenatal imaging in ensuring accurate diagnosis and tailoring individualized risk assessments throughout the pregnancy in such rare and complex situations.
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Síndrome de Bandas Amnióticas , Cesárea , Gravidez de Gêmeos , Ultrassonografia Pré-Natal , Humanos , Feminino , Gravidez , Ultrassonografia Pré-Natal/métodos , Adulto , Síndrome de Bandas Amnióticas/diagnóstico por imagem , Gêmeos Monozigóticos , Recém-NascidoRESUMO
Introduction: Constriction amniotic band syndrome (CABS) is a rare condition associated with the fibrous amniotic bands that restrict and ensnare the fetus in utero resulting in malformations at birth in one per around 15,000 live births. CABS associated with clubfoot, historically required extensive soft-tissue release due to its propensity for relapse. Case Report: A 2-day-old Caucasian male infant born at 27 weeks gestation through emergency cesarean section due to concern for placental abruption and premature rupture of membranes in the setting of a prenatal history significant for oligohydramnios. The patient presented with non-viable tissue in the right leg requiring amputation with a left-sided clubfoot deformity. Following amputation of the right leg, the clubfoot was corrected with Ponseti method of serial casting and Achilles tenotomy. Three-week post-tenotomy and 6 months of age, a custom fit prosthesis of the right leg allowed for the application of a left abduction foot orthosis which maintained the correction. Conclusion: This case study supports recent literature that CABS-associated clubfoot can be corrected with the Ponseti method. While a contralateral amputation can prevent the use of a traditional bilateral abduction foot orthosis, a custom fitted prosthesis can allow for its use and prevention of a relapse of deformity.
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The purpose of this review is to provide an overview of the perinatal outcomes of fetuses who underwent fetal surgery for the management of Amniotic Band Syndrome (ABS). A systematic review of studies reporting on the perinatal outcome of fetuses undergoing fetoscopic release of amniotic bands according to the (PRISMA) guidelines was performed. The MEDLINE, Embase, Scopus, and Cochrane Library databases were systematically searched. In total, 17 studies reporting 37 cases of ABS that underwent amniotic band release by fetoscopy were included. The median gestational age at which fetal surgery was performed was 22 weeks (range 18-29 weeks). PPROM occurred in 51.3%, while fetal survival reached 89.2%. The success of fetal surgery was 75.7% in preserving and maintaining the functionality of the affected limb. Fetoscopic release of amniotic bands can preserve the affected limb and its function in cases of ABS and prevent fetal death in cases of ABS involving the umbilical cord. Further studies are needed to determine the optimal criteria for selecting patients who can benefit from fetal surgery, considering that it is an intervention that is not free of perinatal complications.
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Amniotic band syndrome (ABS) and clubfoot are distinct congenital musculoskeletal conditions that can occasionally co-occur, creating unique challenges in their management. This paper summarizes the comprehensive discussion on the management of amniotic band syndrome (ABS) and clubfoot, emphasizing the critical role of the Ponseti method and the challenges faced in treatment, thereby providing a basis for further research and improved patient care.
El síndrome de banda amniótica (ABS) y el pie zambo son afecciones musculoesqueléticas congénitas distintas que ocasionalmente pueden coexistir, creando desafíos únicos en su manejo. Este artículo resume la discusión exhaustiva sobre el tratamiento del síndrome de bandas amnióticas (ABS) y el pie zambo, enfatizando el papel fundamental del método Ponseti y los desafíos que enfrenta el tratamiento, proporcionando así una base para futuras investigaciones y una mejor atención al paciente.
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Síndrome de Bandas Amnióticas , Pé Torto Equinovaro , Pé Torto Equinovaro/terapia , Pé Torto Equinovaro/cirurgia , Humanos , Recém-Nascido , Lactente , Moldes CirúrgicosRESUMO
Amniotic band syndrome (ABS) constriction rings in the lower limb are common. Despite this, there is insufficient literature on anatomical abnormalities in the knee joints of children with ABS. There is an increasing incidence of paediatric anterior cruciate ligament (ACL) injuries recently. ACL reconstruction in this population has an extra dilemma of sparing the physis to prevent growth disturbances. Treating both these conditions simultaneously is a challenge that is rarely encountered. In our literature review, we found no case such as this. As such a case is being described for the first time, we also found certain meniscal anatomical variations on diagnostic arthroscopy. A 12-year-old adolescent Indian girl presented with an ACL tear in her left leg after a school sports injury. She had a known case of ABS constriction bands in both her lower limbs. Her distal femoral and proximal femoral physis was fused on radiographs, so we went ahead with a transphyseal ACL soft-tissue graft reconstruction. On the diagnostic round, we found an anatomical variation of the menisci, which was previously not described since arthroscopy of the knee in an adolescent kid with ABS has not been published in the literature as of yet. These kinds of clinical presentations can become common in the future as more and more kids with ABS take part in recreational sports. In such a scenario, having knowledge about common anatomical variations in the knee of such syndromic patients is essential. While performing ACL reconstructions in this population, we have to be aware of the risk of growth deformities along with vascular and neurological complications, which are added risks with constriction bands around the lower limb.
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Upper extremity congenital anomalies in the newborn are second only to congenital heart anomalies. Some of the more commonly encountered upper extremity anomalies are trigger thumb, thumb hypoplasia, polydactyly, syndactyly, and amniotic band syndrome. While some conditions occur in isolation, others are known to commonly occur in association with syndromes. Familiarity with these conditions is important not only to provide adequate evaluation and workup of these patients but also to deliver appropriate surgical intervention and prepare parents with appropriate expectations. In this article, we outline the etiology, classification, surgical management, and outcomes of these five commonly encountered upper extremity congenital anomalies.
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Cases of severe bilateral Tessier 4 cleft with unilateral Tessier 3 cleft and additional involvement of the amniotic band syndrome are rarer. This case reports a very rare case of ABS with severe facial cleft. Postoperative progress was satisfactory and the patient achieved functional recovery. The patient underwent several miner reconstructive surgeries and appropriate surgical treatment is necessary to restore the patient's social life.
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Key Clinical Message: Amniotic band sequence (ABS) should be on the differential for newborns with limb defects. ABS is diagnosable prenatally with prenatal ultrasound; however, there are cases where the diagnosis of ABS is made only after delivery of the newborn. Abstract: Amniotic band sequence (ABS) is an uncommon congenital disorder where strands of amniotic tissue cause entrapment of the limbs, body wall, and viscera leading to an array of congenital malformations. We report a case of a newborn with prenatally undiagnosed amniotic band sequence.
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BACKGROUND: Amniotic band syndrome (ABS) is a rare congenital disease characterized by a broad spectrum of congenital anomalies resulting from the strangulated developing organ(s) by the detached fibrous amniotic band. The prevalence of CNS involvement in ABS is rare, but the mortality rate in these cases is high, while morbidity among the surviving patients is inevitable. CASE REPORT: Three-month-old male, 9-month-old female, and newborn female babies were presented with head lump(s), severe facial cleft, syndactyly, and finger amputation. The patient's head imaging confirmed meningoencephalocele as the cause of the head lump in 2 patients; meanwhile, a porencephalic cyst was identified as the origin of head lumps in the other patient. VP shunt placement surgery was performed as the initial management in 2 patients, while one patient directly underwent meningoencephalocele resection surgery. Craniofacial and limb reconstructions were planned as the follow-up management in all cases. Unfortunately, one patient died of complications from suspected aspiration, while another never returned for follow-up treatment. CONCLUSION: Here, we report 3 ABS cases with CNS involvement. Despite the severe disfigurement and disability, the inexistence of fatal malformation might lead to long-term survival. The treatment of malformation(s) that might predispose to another fatal condition and surgery(-ies) to improve functional outcomes and patient's social acceptability should be prioritized in managing the surviving ABS patients.
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Síndrome de Bandas Amnióticas , Neoplasias do Sistema Nervoso Central , Fissura Palatina , Meningocele , Segunda Neoplasia Primária , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Síndrome de Bandas Amnióticas/cirurgia , Síndrome de Bandas Amnióticas/complicações , Neoplasias do Sistema Nervoso Central/complicações , Fissura Palatina/complicações , Encefalocele/diagnóstico por imagem , Encefalocele/cirurgia , Ossos Faciais , Meningocele/complicações , NeurocirurgiõesRESUMO
Amniotic band syndrome (ABS) refers to fibrous bands that appear to entangle various fetal parts in utero, leading to deformation, malformation, or disruption. To alleviate implementation of this diverse malformation, an early diagnosis on ultrasound is necessary to explain to the patient, thus, avoiding psychological shock and requiring timely intervention. Case Presentation: In the present case report, the authors describe a case of ABS that was diagnosed at the time of delivery at full term. Although the male newborn was alive, the infant underwent the distal deformity of amputated limbs and clubfoot. He has currently been followed up for the reconstruction treatment. Clinical Discussion: ABS remains a challenging diagnosis for obstetricians following the onset timepoint. A prenatal ultrasound scan is carefully required to detect the morphologic abnormalities of the fetus. Postnatal management should be integrated by a multidisciplinary team in order to improve the infant's outcome. Conclusion: ABS is an extremely dangerous entity during pregnancy, which leads to poor outcomes for the infant. An early detection on ultrasound helps in preparing better for the acceptance of the mother and the family as well as the prognosis afterwards.
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We aimed to evaluate the fetoscopic procedure indications, procedure-related complications, and neonatal outcomes in cases diagnosed with amniotic band syndrome (ABS). Stage II and III cases according to Hüsler classification were included for fetoscopic surgery. Scissors were used to release the amniotic band in six cases, and a diode laser was used in one case. A single entry was made in all cases. The majority of the children acquired a functional limb (71.4%). Fetal morbidity was mainly linked to the consequences of preterm premature rupture of the membranes (57.1%) and preterm birth (28.5%). Excluding complicated cases, fetoscopic band release is encouraging in cases of ABS in the limbs.
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Síndrome de Bandas Amnióticas , Nascimento Prematuro , Gravidez , Feminino , Criança , Recém-Nascido , Humanos , Fetoscopia/métodos , Síndrome de Bandas Amnióticas/diagnóstico , Síndrome de Bandas Amnióticas/cirurgia , Síndrome de Bandas Amnióticas/complicações , Nascimento Prematuro/cirurgia , Endoscopia , FetoRESUMO
This is a case of a fetus affected by an amniotic band detected at 20 weeks of gestation. A presumptive diagnosis was made based on the ultrasound features. The ultrasound showed an abnormally developed right lower limb and no other associated fetal abnormalities. The unilaterality of the defect decreases the chances of genetic abnormality or an early vascular insult. The postnatal examination of the newborn concluded that the prenatal diagnosis was right.
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A 1-day-old late preterm, small-for-gestational-age female presented with a caudal appendage-a rare finding-and abnormalities in all 4 limbs most consistent with amniotic band syndrome. The caudal appendage was lateral to midline, measured 3 cm × 0.5 cm, and had no bony abnormalities or spinal cord tethering. Limb abnormalities consisted of brachydactyly, oligodactyly, and syndactyly. Renal and head ultrasounds and an echocardiogram were normal. Chromosomal microarray showed deletion of EPHA3, which is not associated with a known phenotype. The multidisciplinary approach of managing this infant with the rare finding of a caudal appendage and limb abnormalities is presented.
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El síndrome de banda amniótica (SBA) o complejo de disrupción de banda amniótica es aquella malformación congénita que ocurre como consecuencia de bridas amnióticas de etiología heterogénea, patogénesis que involucra una serie de manifestaciones clínicas fetales, tales como constricción, amputación y múltiples defectos craneofaciales, viscerales y de la pared del cuerpo. La prevalencia estimada de SBA oscila entre 1:15,000 y 1:1,200 nacidos vivos. Afecta a ambos sexos por igual. El diagnóstico prenatal puede sospecharse tan pronto como el primer trimestre tardío, cuando las imágenes por ultrasonido detectan anillos de constricción, amputaciones de extremidades y/o defectos craneofaciales. La terapia prenatal puede ofrecer una alternativa de tratamiento con la liberación de anillos de constricción bajo fetoscopia en aquellos fetos que se verían beneficiados con el procedimiento.
Amniotic band syndrome (ABS) or amniotic band disruption complex is a congenital malformation that occurs because of amniotic flanges of heterogeneous etiology, a pathogenesis that involves a series of fetal clinical manifestations, such as constriction, amputation, and multiple craniofacial, visceral and wall defects. The estimated prevalence of ABS ranges from 1:15.000 to 1:1.200 liveborn. It affects both sexes equally. Prenatal diagnosis may be suspected as early as the late first trimester when ultrasound imaging detects constriction rings, limb amputations and/or craniofacial defects. Prenatal therapy may offer an alternative treatment with release of constriction rings through fetoscopy in those fetuses that would benefit from the procedure.
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Fetoscopic laser surgery occasionally causes amniotic band syndrome, in which the disrupted amniotic membrane constricts fetal body parts, leading to functional or morphological loss. We report a case of fetal distress at 31 weeks of gestation in the larger surviving twin after fetoscopic laser surgery for selective intrauterine growth restriction, necessitating emergent cesarean section. Physical examination of the infant showed constriction rings caused by a disrupted amniotic membrane on the digits, and the distal part of the right index finger was necrotic because of tight strangulation by an amniotic band with the umbilical cord of the deceased smaller twin. Laboratory data showed severe coagulopathy, and the infant was diagnosed with disseminated intravascular coagulation (DIC). Immediate treatment improved his condition. DIC may have been associated with the necrotic finger, which was strangulated by the umbilical cord of the deceased fetus, because neither maternal coagulopathy nor an underlying neonatal disorder was detected.
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Síndrome de Bandas Amnióticas , Coagulação Intravascular Disseminada , Transfusão Feto-Fetal , Terapia a Laser , Síndrome de Bandas Amnióticas/complicações , Síndrome de Bandas Amnióticas/cirurgia , Cesárea/efeitos adversos , Coagulação Intravascular Disseminada/complicações , Feminino , Transfusão Feto-Fetal/complicações , Transfusão Feto-Fetal/cirurgia , Fetoscopia/efeitos adversos , Humanos , Recém-Nascido , Fotocoagulação a Laser/efeitos adversos , Gravidez , Cordão UmbilicalRESUMO
Amniotic band syndrome is a rare disorder, which consists of a combination of malformations in which the main feature is the existence of an amniotic band that can envelop the limbs, the body wall, and/or the viscera. We report a case of an antenatal diagnosis of amniotic band syndrome in a 21-year-old female patient at 20 SA + 1 day, who had a medical abortion.
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Amniotic band syndrome (ABS) is a congenital disease that causes a variable degree of deformity and tissue disruption. Surgical excision of fibrotic tissue and advancement of local flaps is the gold standard for constriction bands of the upper limb. We report the use of an adipofascial Superficial Circumflex Iliac Artery perforator (SCIP) flap to improve contour following amniotic band resection in the upper extremity in two patients with ABS. The use of this microsurgical technique can be considered in the occasional patient when there is a deficit of soft tissues after release of the contracture band that cannot be addressed with locally available tissue.
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Síndrome de Bandas Amnióticas , Retalho Perfurante , Procedimentos de Cirurgia Plástica , Síndrome de Bandas Amnióticas/cirurgia , Constrição , Humanos , Artéria Ilíaca/cirurgia , Recém-Nascido , Retalho Perfurante/irrigação sanguínea , Retalho Perfurante/cirurgia , Procedimentos de Cirurgia Plástica/métodos , Extremidade Superior/cirurgiaRESUMO
Introduction: The term Streeter's syndrome is a term used to describe rare congenital malformations that includes a variety of clinical presentations usually consisting of a constriction band around a part of the body which can be as superficial as involving just the skin which can be only cosmetic and asymptomatic or can be as deep as causing restricted circulation distally which may be in incompatible with life. Such conditions are remarkably rare accounting for an incidence range from 1:1.2 k to 1: 15 k live births and 178:10 k spontaneous abortions [1]. Males and females are uniformly affected. Almost all cases are sporadic; extremely rare evidence of familial transmission. The entity has been described in the literature in 34 different terms, (such as amniotic rupture sequence, ADAM complex, constriction band syndrome, Streeter's dysplasia, etc.) due to its extremely variable clinical features and lack of understanding of the etiology. This results in a lack of understanding and creates unnecessary stress for the surgeon/physician as well as the parents. Case Report: We discuss case reports of two such cases with their simple nature of treatment with their outcomes. Conclusion: There is a significant deficit in the education of cases with low incidence, such is the case with pediatric patients presenting with amniotic bands, which usually present and are associated with Congenital Talipes Equino Varus deformity. In such cases, improper or incorrect treatment and/or neglect of the constriction may lead to the vascular deficit and eventually auto-amputation of the segment distal to the amniotic band.
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BACKGROUND: Moebius syndrome is a disorder characterized by facial and abducens nerve paralysis. Patients can present a wide range of upper extremity malformations. Literature focused on orthopedic manifestations of Moebius syndrome shows variability in the prevalence and clinical presentation of upper extremity anomalies. The aim of this work is to evaluate the prevalence of upper extremity malformations in patients with Moebius syndrome, clarify its various clinical presentations, and present treatment strategies for their management. METHODS: This is a retrospective, cross-sectional study including patients with Moebius syndrome and upper extremity malformations between 2012 and 2019. Data include demographic characteristics, Moebius syndrome subtype, type of malformation, affected extremity, and surgical procedures underwent. Quantitative data were recorded as mean (standard deviation [SD]), and qualitative data were expressed in terms of totals and percentages. Statistical association between Moebius syndrome subtype and development of upper extremity anomalies was evaluated using binary logistic regression. RESULTS: Twenty-five out of 153 patients (16.3%) presented upper extremity malformations (48% male). Mean age of presentation was 9.08 ± 9.43 years. Sixty-eight percent of the malformations were unilateral. The most common presentations included Poland syndrome and simple syndactyly with 8 cases each (32%), followed by 5 cases of brachysyndactyly (20%), 3 cases of amniotic band syndrome (12%), and 1 case of cleft hand (4%). No statistical association was found between Moebius syndrome subtype and odds ratio for development of upper extremity anomalies. Thirteen patients (52%) underwent reconstructive procedures. CONCLUSION: Poland syndrome and syndactyly are the most common anomalies in patients with Moebius syndrome. Patients may present with a wide range of hand malformations, each patient should be carefully evaluated in order to determine whether surgical treatment is needed and to optimize rehabilitation protocols.
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Deformidades da Mão , Síndrome de Möbius , Síndrome de Poland , Recém-Nascido , Humanos , Masculino , Criança , Adolescente , Feminino , Síndrome de Möbius/epidemiologia , Síndrome de Möbius/cirurgia , Síndrome de Möbius/complicações , Estudos Retrospectivos , Prevalência , Estudos TransversaisRESUMO
Amniotic band syndrome (ABS) is a congenital abnormality that can cause a variety of deformities. Here, we report a case of ABS in which the amniotic band adhered to the skull, causing a partial cranial defect that was difficult to differentiate from an occipital encephalocele. The mother was a 24-year-old with an unremarkable medical and family history. Ultrasonography performed at 16 weeks showed that the fetus had a membranous structure in the occipital region. Occipital encephalocele was suspected, and she underwent cesarean section at 38 weeks. A male newborn was delivered, and his left occipital skin had a defect measuring 2 cm, from which a cystic structure had prolapsed. Cranial magnetic resonance imaging showed that the cystic structure had homogeneous high signal intensity on T2-weighted images. The neonate then underwent repair of the occipital encephalocele. During the operation, the membranous structures and dura were not continuous. Histological examination revealed that the membranous structures were composed of amnion, suggesting that this was a case of ABS. ABS may present with an encephalocele-like morphology without affecting the brain tissue and meninges. If an atypical encephalocele is found after birth, examination of the placenta may be helpful for a definitive diagnosis.