Cases Of Amniotic Band Syndrome Research Articles

Gastroschisis prevalence patterns in 27 surveillance programs from 24 countries, International Clearinghouse for Birth Defects Surveillance and Research, 1980-2017.

Gastroschisis is a serious birth defect with midgut prolapse into the amniotic cavity. The objectives of this study were to evaluate the prevalence and time trends of gastroschisis among programs in the International Clearinghouse for Birth Defects Surveillance and Research (ICBDSR), focusing on regional variations and maternal age changes in the population. We analyzed data on births from 1980 to 2017 from 27 ICBDSR member programs, representing 24 countries and three regions (Europe+ (includes Iran) , Latin America, North America). Cases were identified using diagnostic codes (i.e., 756.7, 756.71, or Q79.3). We excluded cases of amniotic band syndrome, limb-body wall defect, and ruptured omphalocele. Programs provided annual counts for gastroschisis cases (live births, stillbirths, and legally permitted pregnancy terminations for fetal anomalies) and source population (live births, stillbirths), by maternal age. Overall, gastroschisis occurred in 1 of every 3268 births (3.06 per 10,000 births; 95% confidence intervals [CI]: 3.01, 3.11), with marked regional variation. European+ prevalence was 1.49 (95%CI: 1.44, 1.55), Latin American 3.80 (95%CI: 3.69, 3.92) and North American 4.32 (95%CI: 4.22, 4.42). A statistically significant increasing time trend was observed among six European+ , four Latin American, and four North American programs. Women <20 years of age had the highest prevalence in all programs except the Slovak Republic. Gastroschisis prevalence increased over time in 61% of participating programs, and the highest increase in prevalence was observed among the youngest women. Additional inquiry will help to assess the impact of the changing maternal age proportions in the birth population on gastroschisis prevalence.

Prevalence of Hand Malformations in Patients With Moebius Syndrome and Their Management.

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. 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. 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. 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.

VP13.05: Prenatal ultrasound diagnosis of fetal limb skeletal dysplasia

To explore the characteristics of fetal limb skeletal deformities by prenatal ultrasound. The prenatal ultrasound results of fetuses with skeletal dysplasia of extremities from October 2016 to January 2019 were retrospectively analysed. A total of 164 cases of abnormal limb skeletal development were detected from 12 weeks to 37 weeks of pregnancy. 15 cases presented with short and angled long bone, short ribs, small thorax, low ossification of skull, short nasal bone or abnormal spine, which were fatal skeletal malformations. There were four cases of fetal limb long bones less than -4SD and 51 cases of club foot. There were 12 cases of overlapping fingers, 4 of which were confirmed to be Trisomy 18. There were seven cases of middle phalangeal bone loss in both hands and 10 cases of dysplasia or deletion of radius with carpal abnormality. In addition, we found three cases of metacarpal and phalangeal abnormalities. Four cases of amniotic band syndrome were characterised by finger shortage, toe shortage, lower extremity abnormality, skull deformation, encephalocele, abdominal fissure, etc. A case of six fingers and six toes with bilateral fetal polycystic kidney and occipital encephalocele was confirmed as Meckel–Gruber syndrome after delivery. Two cases of cleft hand and foot were found. There were four cases of lower extremity rigidity, two cases of which were combined with femur less than -2SD, excessive amniotic fluid, mild neck edema and bilateral small lung, suspected of Pena Shokeir syndrome. One fetus was diagnosed as spinal stenosis, combined with extremely flat face and absent nasal bone. Postnatal x-ray showed that the terminal finger was short and gene chip examination showed no abnormality, which was a rare abnormality of macular achondroplasia. The common missed cases were polydactyly and syndactyly. Three-dimensional ultrasound is intuitive and can provide more information. Prenatal two-dimensional and three-dimensional ultrasound play an important role in the detection and classification of fetal limb skeletal malformations.

Perinatal outcome after fetoscopic release of amniotic bands: a single‐center experience and review of the literature

To present two successful cases of fetoscopic release of amniotic bands with umbilical cord involvement and provide a review of the literature on fetal intervention for amniotic band syndrome (ABS). Two cases of ABS were considered in conjunction with a review of the literature. A total of 14 fetuses with ABS underwent fetoscopic intervention between 1965 and 2012. Two of the authors independently completed literature searches in PubMed, Ovid and MEDLINE for articles related to ABS. Among 14 cases of ABS (12 published and our own two), 57% and 7% were complicated by preterm premature rupture of membranes and spontaneous preterm birth, respectively. Overall, fetoscopic intervention preserved limb function in 50% (7/14) of cases. Three cases involved intraoperative complications including intra-amniotic bleeding and uterine wall bleeding, and incomplete procedure due to ineffective equipment. Fetoscopic release of amniotic bands with minimally invasive surgery may allow preservation of life and/or limb function in cases of ABS. The acceptable functional outcome in 50% of cases is reassuring, although more experience and further studies are needed to determine the selection criteria that will justify the risk of this invasive in-utero therapy for ABS.

Amniotic band syndrome: a report of two cases and review of the literature

Amniotic band syndrome (ABS), also known as Streeter's dysplasia refers to a continuous spectrum of manifestations due to intra-uterine rupture of amnion. 1 The manifestations range from simple soft tissue constrictive bands to amputation of digits or more severely the whole limb due to dysplastic vasculature. 1 The prevalence is estimated to be about 7.7/10,000 live births 2 and as high as 178/10,000 among abortuses. 3 We herein present two cases of amniotic band syndrome seen at theNiger Delta University Teaching Hospital(NDUTH) with the aim of highlighting its existence in the State as well as throwing light on the prenatal diagnostic challenges and the socio-economic problems faced by families living with physical disabilities.

Amniotic band syndrome associated with orofacial clefts: a report of two cases

AbstractThis article presents two cases of amniotic band syndrome associated with orofacial deformities. There was an uncommon presentation in one of them as Tessier nos. 5, 7 and 30 with irregularly distributed amniotic facial band. The surgical repair of the facial clefts and difficulties in overall management in our environment are further discussed.

3D/4D sonographic evaluation of amniotic band syndrome in early pregnancy: A supplement to 2D ultrasound

We present two cases of amniotic band syndrome diagnosed using two-dimensional (2D) ultrasound with three-dimensional (3D)/four-dimensional (4D) ultrasound in early pregnancy. In Case 1, at 13 weeks' gestation, multiple amniotic bands, acrania, the absence of fingers and amputation of the toes bilaterally were clearly shown using transvaginal 3D/4D ultrasound. In Case 2, at 15 weeks' gestation, several amniotic bands, acrania and a cleft lip were depicted with transabdominal 3D/4D ultrasound. The spatial relationship between the amniotic bands and the fetus was clearly visualized and easily discernible by 3D/4D ultrasound. The parents and families could readily understand the fetal conditions and undergo counseling; they then choose the option of termination of pregnancy. 3D/4D ultrasound has the potential to be a supplement to conventional 2D ultrasound in evaluating amniotic band syndrome.

P22.12: Antenatal diagnosis of amniotic band syndrome-case report

Amniotic band syndrome is a set of congenital birth defects believed to be caused by the entrapment of the fetal parts in fibrous bands in utero. Disunion, deformation, malformation, clefts and constriction bands and amputation of various parts of the body could be seen. It can affect any organ or system and cause a single or multiple defects. We report 6 cases of amniotic band syndrome in the second and trimester of pregnancy. It includes multiple fetal defects. It ranges from hand anomalies, ectopia cordis, congenital transverse deficiency, cleft lip cleft palate, facial cleft. Two cases of ectopia cordis with facial anomalies. 2 cases of isolated limb anomaly includes congenital transverse deficiency, 1 case of multiple limb anomalies and 1 for multiple anomalies including limb, abdominal wall defect, spinal defect, encephalocele and facial anomaly. In our study, the earliest case we diagnose is at 15 weeks. Mean age of the patient is 24 years. Amniotic band syndrome caused a variety of fetal malformations involving limbs, craniofacial region and trunk. It occurs in approximately 1 in 1200 to 1 in 15,000 live births. Occurrence is mostly sporadic. In our case, all are from low risk group. 3D & 4D ultrasound shows multiple amniotic bands floating in the amniotic cavity with 1 of them attached to the fetal part.

Fetoscopic release of umbilical cord amniotic band in a human fetus

Umbilical cord amniotic bands occur in approximately 10% of cases of amniotic band syndrome and are a well-known cause of fetal death. An unexpected amniotic band encircling the umbilical cord was diagnosed during a fetoscopic procedure to release a leg constriction. Both bands were released fetoscopically using a YAG laser. We report the first case of an amniotic band involving the umbilical cord diagnosed and released prenatally.

Craniofacial anomalies of the amniotic band syndrome in serial clinical cases.

Amniotic band syndrome has been proposed as a sequela of intrauterine rupture of the amnion, resulting in oligohydramnios and passage of the fetus into the chorionic cavity. Amnion disruption with loss of amniotic fluid, causing fetal compression and localized fetal ischemia, possibly results in a pathogenic mechanism of extremely variable malformations. The prominence of the nasal processes and the adjacent stomodeal orifice facilitates free band attachment and adherence, resulting in a spectrum of similarly oriented facial defects. The authors present six consecutive cases of amniotic band syndrome with cleft lip and palate (facial cleft 3, 5, 7, and 10, isolated or combined) that were associated with other craniofacial anomalies, such as craniosynostosis and hypertelorbitism. They also present limb malformations and discuss the proposed pathogenesis and the surgical challenges in functional and aesthetic restoration.

Amniotic band syndrome: a population-based study in two Australian states.

A search for cases of amniotic band syndrome was made in two population-based Australian birth defects registries, using defined selection criteria. Over a period of 4 years in Western Australia and 5 years in South Australia, 25 cases of amniotic band syndrome had been identified as such by the two registries, and an additional 15 new cases were identified by the study selection process, giving an annual prevalence of amniotic band syndrome over the study period of 2.03 per 10,000 births. Similar proportions of male and female infants were affected, although the syndrome was more common in mothers younger than 25 years of age, and in first births. Limb defects only (upper and/or lower) were found in 24 cases, limb-body-wall defects in four cases, and complex craniofacial and other malformations in 12 cases. A heightened awareness of the syndrome should enhance the identification of amniotic band syndrome, which has implications for genetic counselling, and our understanding of the aetiology and pathogenesis of this condition.

The amniotic band syndrome: single-stage correction

Two cases of amniotic band syndrome treated by single-stage excision of the ring and multiple Z-plasty repair are presented. In both cases the author found the procedure technically easy and clinically safe. No complications were encountered in either case.

Amniotic bands ? A report of two unusual cases

Two cases of amniotic band syndrome with unusual localization are presented. In one of them the amniogenic band was localized to the head and forehead, in continuity from the eyebrows to the occiput. In the other the bands were localized to the sacrum and prevented body flexion by the creation of a contracture.

The amniotic band syndrome: Antenatal sonographic diagnosis and potential pitfalls

Amniotic band syndrome causes a variety of fetal malformations involving the limbs, craniofacial region, and trunk. Six prenatally diagnosed cases of amniotic band syndrome are discussed. The diagnosis was based on sonographic visualization of either amniotic sheets or bands associated with fetal deformation or deformities in nonembryologic distributions known to characterize the amniotic band syndrome. Seven additional cases are considered in which an aberrant sheet of tissue with a free edge was visualized within the amniotic cavity but no restriction of fetal motion or subsequent deformity was demonstrated.