Amniotic Band Research Articles

P10.04: Amniotic band syndrome: first trimester diagnosis and classification

P10.04: Amniotic band syndrome: first trimester diagnosis and classification

P15.02: Amniotic band syndrome: literature review of prenatal therapy

P15.02: Amniotic band syndrome: literature review of prenatal therapy

Congenital Upper Limb Differences Registry: Development and Challenges of a Prospective Database

There is no financial information to disclose. To improve upon the traditionally retrospective case series of congenital upper limb differences, a prospective cohort multi-center registry for Congenital Upper Limb Differences (CoULD) was created. The hypothesis for the current study is that demographics and clinical presentation of patients in the CoULD registry will be different than other cross-sectional or epidemiological studies. The CoULD registry includes preoperative children from 0 to 18 years of age with a congenital upper extremity difference. Demographic information and prenatal history are collected. Congenital differences are classified using the Oberg-Manske-Tonkin (OMT) classification. For the present study, data from two centers in the CoULD registry was analyzed over a 27- month period. There were a total of 910 differences in 853 patients. Median age at enrollment was 2.9 years (IQR 0.7-8.5). Two-hundred four children (27%) had a positive family history and 241 (32%) had pregnancy-related risk factors. Three-hundred forty-seven (41%) patients had bilateral involvement. Additional orthopedic conditions were registered in 29% (213) and medical comorbidities existed in 30% (221) of subjects. According to OMT group, there were 780 malformations (86%), the majority affecting the hand plate only; 17 deformations (1.8%) and 113 dysplasias (12%). In addition 99 patients (12%) presented with a syndrome, most commonly amniotic constriction bands (Table 33-1). Significant differences were noted in pathologies according to age. Radioulnar synostosis, Madelung deformity, congenital dislocation of the radial head and osteochondromatosis presented almost exclusively in children older than 2 years (P ≤ 0.01); conversely polydactyly, radial longitudinal deficiency and syndactyly presented more commonly in younger patients. Compared with a cross-sectional study (1), CoULD included more pathologies of late presentation and those that do not commonly require surgical care. Similarly, when compared with a retrospective population study (2), CoULD captured more late presenting conditions but did not include the same proportion of pathologies with early surgical treatment (Table 33-2). •Congenital differences present at characteristic ages; the prospective cohort design of the CoULD registry offers the advantage of including a broad spectrum of pathologies including those of late presentation.•This study highlights the differences in prevalence of these conditions found with different study types. CoULD identified more late-presenting and non-operative conditions such as symbrachydactyly, radioulnar synostosis, and transverse deficiency, than other study types.•The CoULD registry will provide a more complete description of the prevalence of congenital upper limb differences presenting in a clinical environment and will offer the opportunity to better characterize this pediatric population.Table 33-1Relative Frequency of Congenital Upper Limb Differences of Patients Enrolled in the CoULD Registry by OMT Classification GroupsOMT GroupsCoULD Total< 2 years> 2 yearsP value(n = 910)%(n = 397)%(n = 525)%I. Malformations A. Malformations Entire Limb3403712131219430.02 B. Malformations Hand Plate440492516318937< 0.001II. Deformations172123510.030III. Dysplasias1131213310019< 0.001(n = 853)%(n = 372)%(n = 481)%IV. Syndromes9912401159120.49 Open table in a new tab Table 33-2Abstract of Key Differences in Relative Frequency of Congenital Upper Limb AnomaliesCoULDGoldfarb et al1P value(n = 897)%(n = 653)%I. Malformation ii. Symbrachydactyly – entire limb597%71%< 0.001 i. Radial longitudinal deficiency374%437%0.01 iv. Radioulnar synostosis597%122%< 0.001 vii. Madelung deformity192%40.7%0.03 iv. Arthrogryposis475%539%0.01 ii. Symbrachydactyly – hand384%417%0.02 iii. Transverse deficiency – hand20.2%224%< 0.001 ii. Camptodactyly556%214%0.03 iv. Distal arthrogryposis202%50.8%0.04 ix. Phalangeal synostosis80.9%00.0%0.02 xi. Cleft hand202%325%0.001II. Deformations 1. Constriction ring syndrome162%295%< 0.001III. Dysplasias i. Osteochondromatosis586%5710%0.03 ii. Enchondromatosis131%20.3%0.03IV. Syndrome9912%10917%< 0.001CoULDEkblom et al2P value(n = 687)%(n = 462)%I. Malformation ii. Symbrachydactyly – entire limb599%153%< 0.001 i. Radial longitudinal deficiency375%112%0.01 ii. Ulnar longitudinal deficiency264%41%0.001 iv. Radioulnar synostosis599%31%< 0.001 ii. Symbrachydactyly386%00%< 0.001 ii. Ulnar deficiency – hand101%163%0.02 iii. Radial polydactyly588%5913%0.02 v. Ulnar polydactyly416%9220%< 0.001 v. Clinodactyly365%398%0.03 ix. Phalangeal synostosis81%00%0.02 x. Synpolydactyly – central71%163%0.001IV. Syndrome9912%142%< 0.001 Open table in a new tab

EP15.15: Amniotic band constriction of umbilical cord in third trimester causing stillbirth: a case report

EP15.15: Amniotic band constriction of umbilical cord in third trimester causing stillbirth: a case report

EP20.19: Discordant anomalies in monochorionic monoamniotic (MCMA) twin pregnancy

MCMA twin pregnancy accounts for 1-2% of all twin pregnancies and requires close monitoring due to risk of fetal death and cord entanglement. We report a case of MCMA twin pregnancy with discordant anomalies in both fetuses. A 37 year old G3P2 woman, followed at the fetal medicine clinic with MCMA twin pregnancy since 14 weeks of gestation. The woman had no significant past medical or surgical history and had previous two spontaneous vaginal deliveries at term with no complications. Her anomaly scan at 21 weeks gestation showed multiple amniotic bands with normal parameters for both twins and normal amniotic fluid volume. Twin A was diagnosed with a small VSD, partial amelia of both lower limbs and enlarged bladder but with normal kidneys. Twin B with mildly dilated aortic arch but the rest was normal. Fetal echo was performed confirming the findings. The woman was counselled and offered amniocentesis but she declined. She was followed up closely every 2 weeks until 28 weeks where she was admitted in the antenatal word for preterm premature rupture of membranes. She was planned for an elective Caesarean section at 33 weeks gestation but she went into spontaneous labour and had heavy vaginal bleeding. An emergency Caesarean section was performed and confirmed placental abruption with the presence of cord entanglement. The babies were born alive with good Apgar scores and birth weight of 1.6 and 1.8 kg. Both twins were admitted in the neonatal ICU on CPAP. Examination including echo confirmed the antenatal findings with large PDA and small VSD for twin A and small PDA for twin B. Unfortunately on Day 5 of life twin B was desaturating and showed signs of sepsis. The neonate was intubated but died on the same day due to sepsis and pulmonary hemorrhage. Twin A was discharged in a stable condition after 4 weeks and was given a follow-up with orthopedic for management of the lower limb anomaly.

4D assessment of motoric function in a singleton acephalous fetus: the role of the KANET test

Abstract Acephalous fetus in a singleton pregnancy is an extremely rare case. In twin pregnancy, it could be presumed as one type of twin reverse arterial perfusion sequence (TRAPS). In this particular case report, the situation was different. An acephalous fetus developed in a singleton pregnancy and may have been a complication of an amniotic band in the very early weeks of gestation. Nevertheless proving it is still a constraint. Despite that, motor findings in utero by using four-dimensional (4D) ultrasound were very interesting to study. Movement of the acephalous fetus is challenging thought on fetal behavior theory, as brain development and function play the central role. The Kurjak antenatal neurodevelopmental tests (KANET) was used to measure the fetal behavior of this acephalous fetus. A comparison with post natal movement findings was also done to provide a better understanding.

RETRACTED: Amniotic Band Syndrome With Pseudoarthrosis of Tibia and Fibula: A Case Report

Constriction of the lower limb by a congenital amniotic band has been proposed to explain the development of pseudoarthrosis of the tibia and fibula. We report a case of amniotic band syndrome in a preterm female infant with pseudoarthrosis of the tibia and fibula. She was born at 29weeks of gestation with congenital amniotic bands and was noted to have a severely edematous left foot distal to the constricting band with rudimentary digits. The skin was pink and well perfused with palpable pulses. Radiography demonstrated pseudoarthrosis of the tibia and fibula. The limb deformities were managed with splints, positioning, and physiotherapy. She underwent Z-plasty with soft tissue release on the left lower leg on day 7 of life. At 36weeks of postmenstrual age, a repeat radiograph showed markedly improved growth of the tibia and fibula with mature new bone formation, which avoided the need for further surgical intervention. During the follow-up period, she underwent left Syme's amputation at 18months. At 29months of age, the child was able to walk and run without support. The findings from our case confirm the potential for bone growth in patients with amniotic band syndrome, once the constricting band has been released. Simple release of the constriction band with Z-plasty resulted in growth of mature bone, replacing the pseudoarthrosis and, hence, the patient did not require surgical amputation. Thus, one should be cautious when deciding on surgical amputation, even in the presence of pseudoarthrosis, especially in preterm infants. Early limb-preserving surgery with release of the constricting band with an intention to salvage the limb appears appropriate.

Matrix protein organization near the constriction ring of amniotic band syndrome reveals idiosyncrasies of wound repair in infancy

Matrix protein organization near the constriction ring of amniotic band syndrome reveals idiosyncrasies of wound repair in infancy

Abnormal ultrasound appearance of the amniotic membranes - diagnostic and significance: a pictorial essay.

The obstetrical ultrasound may identify the protrusion of the uterine surface or placental area into the amniotic cavity. The differential diagnosis of this pathology with uterine adhesions, septate uterus, circumvallate placenta, amniotic band oramniotic sheet can be sometimes difficult. The purpose of the pictorial essay is to exemplify the presence of the amniotic sheetand circumvallate placenta in routine obstetrics screening of all trimesters of pregnancy.

Discordant malformation in a monozygotic pregnancy: abdominoschisis and sirenomelia

Abstract Congenital malformations occur in up to 10% of monochorionic twins and are usually discordant. Body wall defect (BWD) is a rare entity of very low prevalence, defined as a combination of various malformations, including fetal body mutilations of thoracic and/or abdominal organs that can be caused by amniotic rupture early in embryonic development. Sirenomelia is a form of caudal regression syndrome that presents with lower limb fusion, among other anomalies. In this report, we will present a second trimester monochorionic diamniotic pregnancy, with BWD-abdominoschisis in one fetus and sirenomelia in the other one. Amniotic bands could not be found. Of particular importance in respect to both malformations is the timing of the insult that could have caused both anomalies.

First-trimester screening for fetal structural and chromosomal anomalies by detailed early anomaly scan

Objectives To assess the performance of first trimester ultrasound screening for fetal structural and chromosomal anomalies based on a detailed anomaly and nuchal translucency (NT) scan at 11–13+6weeks' gestation. Methods A prospective cohort study was conducted at Nanjing Drum Tower Hospital. Fetuses with a crown-rump length (CRL) between 45 mm and 84 mm scanned during December 2015 to March 2016 were enrolled in this study. After a detailed first-trimester anomaly scan followed the protocol of systematic standardized scan plans, fetuses with congenital abnormalities were screened out. Second trimester ultrasound screening and postnatal examination were performed for further examination of fetal anomalies. Cytogenetic analysis was performed on the fetuses with informed consent. Results (1) A total of 1 154 fetuses were enrolled in this study and among them, 36 (3.1%) cases of fetal abnormalities were diagnosed through prenatal examination (35 cases) and postnatal examination (one case). (2) Twenty-one (58.3%) out of the 36 cases with structural and chromosomal anomalies were screened out by using the first-trimester scan, including eight cases of congenital cardiac defect (two cases of atrioventricular septal defect, one case of tricuspid atresia, one case of tetralogy of tetralogy, one case of right ventricle aneurysms and one cases of hypoplastic left heart syndrome combined with cystic hygroma with one case combined with polydactyly), four cases of central nervous system anomaly (three cases of exencephaly and one case of anencephaly combined with double outlet right ventricle), two cases of cleft palate/lip with one case combined with double outlet right ventricle, two cases of exomphalos, one case of amniotic band syndrome, one case of spinal bifida combined with megacystis, one case of umbilical cyst, one case of polydactyly and one case of cystic hygroma. One case of twin pregnancy chose selective fetocide to the fetus with exencephaly and 16 cases terminated pregnancy. The other four cases were confirmed by second trimester ultrasound screening and postnatal examination. Fourteen (38.9%, 14/36) new cases of structural and chromosomal anomalies were detected by the second-trimester scan, six of which terminated the pregnancies and the rest were confirmed at term. One (2.8%, 1/36) case of polydactyly was detected postnatally. (3) Chromosomal microarray analysis was performed on 28 cases, seven of which were identified as having chromosomal abnormalities including five cases detected in the first trimester and two cases detected in the second trimester. (4) Out of the 20 fetuses with abnormal NT in early trimester, which accounted for 1.7% of all enrolled fetuses, nine were indentified with major structural or chromosomal abnormalies, a quarter of all abnormal fetus. Conclusions Detailed anomaly scan and NT scan in the first-trimester can increase the detection rate of fetal structural and chromosomal anomalies as compared with the traditional NT scan and provide earlier detection of severe fetal abnormalities as compared with second trimester anomaly scan. Key words: Pregnancy trimester, first; Ultrasonography, penatal; Congenital anormalities; Chromosome aberrations

Congenital malformations and medical conditions associated with orofacial clefts in children in Burkina Faso

BackgroundOrofacial clefts are usually isolated cases but can be associated with other congenital malformations that are either recognised or unrecognised syndromes. The reported prevalence and pattern of such associated malformations, however, vary among studies. Objectives: To assess the frequencies and aetiologies of congenital malformations and associated medical conditions in children with orofacial clefts in Burkina Faso (Western Africa).MethodsA retrospective descriptive study was carried out at the El Fateh-Suka Clinic in Ouagadougou, Burkina Faso. All children who attended surgery for the repair of a cleft lip and/or palate were included in this study.ResultsThe frequency of congenital malformations associated with cleft lip and/or palate was 39/185 (21.1%). In the group with multiple congenital malformations of unknown origin (34 patients; 18.4%), 66.7% had cleft lip and palate, followed by isolated cleft lip (27.4%) and isolated cleft palate (5.9%). The digestive system (35.3%), the musculoskeletal system (19.6%), and eye, ear, face, and neck (15.7%) were the most affected systems. In the group of syndromic malformations (five patients; 2.7%), amniotic band syndrome (one patient), Van der Woode syndrome (one patient), Goltz syndrome (one patient), and holoprosencephaly (two patients) were identified. Medical conditions included anaemia (39.4%), infections (9.2%), malnutrition (7.5%), and haemoglobinopathies (4.3%).ConclusionsCongenital malformations and medical co-morbidities were frequent in children with OFCs. Further studies and a National Malformations Registry are needed to improve the comprehension of OFCs in Burkina Faso.

Effect of Reflex Neuromodulation on an Infant with Severe Amniotic Band Syndrome: A Case Report on the Use of MNRI Techniques for Physical Therapy

Introduction: MNRI offers non-invasive neuromodulating techniques to activate reflex patterns which awaken the body’s natural resources and support functioning of sensory, motor, and cognitive systems. Amniotic band syndrome (ABS) is a rare congenital disorder attributed to the anomalous amniotic bands that entangle fetal parts during intrauterine life leading to a wide range of physical abnormalities in a newborn infant, in this case, located at the superior third portion of the right arm. Due to the severity of the constricting band observed and assessed at birth, the microvascular surgeon opted for immediate post-birth surgery and Occupational and Physical Therapies were respectively initiated at 8 and 10 weeks post-surgery. Objective: This article describes early physical therapy intervention (post-surgery) using the MNRI method and discusses the effectiveness of MNRI techniques in the recovery of arm function. Results: Early intervention using the MNRI neuromodulation techniques with ABS disorder demonstrated unique and faster motor milestones development including voluntary motor and cognitive control of the immobile limb in an infant with the ABS. Summary: The use of the ready patterns of the brain-primary inherent reflexes-can be a key method and an important cornerstone for professionals to build upon for infants with the ABS.

Amniotic constriction band: a report of two cases with unique clinical presentations

Amniotic constriction band is a rare clinical entity with varied manifestations that range from a combination of congenital malformations to isolated malformations that are unique to each patient. The etiology of this entity remains unknown. Herein, we highlight two cases of amniotic constriction band that presented to our unit with unique clinical characteristics. To the best of our knowledge, an isolated circumferential band of scarring on the face with ocular involvement, as demonstrated by the first case, and a combination of bilateral complete cleft lip and palate with bilateral microphthalmia, auto-amputation of the right thumb, and a constriction band on the left thumb, as demonstrated by the second case, are extremely rare presentations of amniotic constriction band that were not previously reported in the literature and therefore necessitate a special mention. We discuss potential etiologies for these cases and review the existing literature on this entity.

A Case Report of Active Amniotic Band Syndrome with Progressive Lymphedema Causing Vascular Insufficiency: Radical Excision of the Overgrown Tissue

Introduction: Amniotic band syndrome (ABS) is an uncommon congenital anomaly characterized by multiple incapacitating manifestations. It may affect the bone, muscle, nerves, and vascular bundles according to the depth of the constriction band. Material and methods: Here we present a 2-month-old infant patient with active ABS in the lower limb at risk for amputation, in which the constriction bands were released surgically using the extensive approach. A two-step surgery was performed. In the first step, a Z-plasty of the anterior portion of the proximal constriction band was performed. In a second step, the bag on the foot was removed and subsequently the posterior part of the constrictive band was resected. Result: A follow-up of five years after surgery, respectively, revealed fully functional foot and restoration of blood supply. Conclusion: This procedure allowed to establish normal circulation in the limb with active ABS after birth. Removal of the constriction band improves the distal tissue and avoids progressive deformity. Surgery of the SBA may be performed in one or two steps.

いわゆる amniotic band syndrome の1例と発生論的考察

いわゆる amniotic band syndrome の1例と発生論的考察

Amniotic band syndrome: A clinical brief

Amniotic band syndrome (ABS) results from bands of amnion entangling fetal parts. They may manifest as constriction rings or complex congenital anomalies resulting in stillbirth. Karyotyping is important for exclusion of inherited disorders and proper counseling. Two case reports one stillbirth and the other with constriction ring of fingers and mild hydronephrosis are presented. The aim of this paper is to make awareness and stress the need for doing thorough work-up in all cases of constriction bands.

Streeter Dysplasia, from Pelvic to Digits: A Case Report

Background: Streeter dysplasia is a term to describe fetal congenital syndrome which mainly characterized by constriction band on appendages, prenatal amputations of extremities, and acrosyndactyly. This syndrome has wide range of clinical manifestation between patients, as reflected by many other terms to describe this syndrome. Case: The author reported five cases of Streeter dysplasia with constriction band on different locations of the body, with a patient having a constriction band around pelvic and other multiple anomalies, patient with constriction around leg and caused acute limb ischemic, and several cases of acrosyndactyly around hand and foot. Result and Conclusion: Constriction band release surgery, as well as correction surgery for other abnormality was performed, either by direct closure or Z-plasty with satisfactory result in functional and aesthetic.

The prevalence of amnion rupture sequence, limb body wall defects and body wall defects in Alberta 1980-2012 with a review of risk factors and familial cases.

Prevalence rates of amnion rupture sequence, limb body wall defect, and body wall defects vary widely. Comparisons are difficult due to small case numbers and the lack of agreement of definition, classification, and pathogenesis. This study reports the prevalence of cases classified in five distinct categories. The Alberta Congenital Anomalies Surveillance System data on live births, stillbirths, and terminations of pregnancy (<20 weeks gestation) occurring between 1980 through 2012 with the ICD-10 Royal College of Paediatrics and Child Health Adaptation codes used for congenital constriction bands (Q79.80) and body wall complex (Q89.7) were reviewed. During the 33-year-study period, there were 153 eligible cases ascertained from 1,411,652 live births and stillbirths, giving a prevalence of 1.08/10,000 total births. There were more males (52%) than females (45%) and 3% were of unknown sex. The average maternal age, birth weight, and gestation was 27 years, 2,701 g, and 35 weeks, respectively. Limb deficiencies occurred in 78% of cases. Amniotic bands with limb deficiency was the most common phenotype (48%). Digital limb deficiency was the most frequent type (56%); however, cases with body wall defects had more severe types of limb deficiencies. The upper limbs only were affected more times (44%), and there was no side preference. Most cases are sporadic but a number of familial occurrences have been reported although some have insufficient documentation and others misdiagnosed. A review of putative risk factors gives conflicting results. © 2016 Wiley Periodicals, Inc.

Neonatal leg fracture and constriction ring syndrome: A case report and literature review

Congenital constriction of the limbs is usually due to amniotic band syndrome, which often causes damage to the skin and soft tissues. We report an unusual case in which a neonate had recent fractures of both leg bones with an amniotic band encircling the limb. Non-operative treatment was successful. Challenges to the management of neonatal limb constriction include the absence of a consensus about the best treatment and the high frequency of damage to vessels and nerves.