Amniotic Band Research Articles

SFM Fetal Therapy Practice Guidelines: Fetal Interstitial Laser

AbstractFetal laser therapy was first used in complicated monochorionic pregnancies such as twin-to-twin transfusion syndrome or twin-reversed arterial perfusion sequence. There are some other less common indications such as amniotic band syndrome, chorioangiomas, lower urinary tract obstructions, sacrococcygeal teratomas, and fetal thoracic masses. These practice guidelines discuss the indications, contraindications, and risks of the procedure along with information for patients and draft consent form.

Bovine thoracic Ectopia cordis in southeastern Rio Grande do Sul, Brazil

This study describes and illustrates a case of thoracic ectopia cordis (EC) in a cross-bred calf in the southeastern region of Rio Grande do Sul, Brazil. A cow suffering from dystocia due to fetal static gave birth to a calf with the heart and pericardial sac outside the thoracic cavity and respiratory distress. The animal survived for 40 min after giving birth. EC is defined as the complete or partial displacement of the heart out of the thoracic cavity. It is a rare birth defect in the fusion of the anterior chest wall, which results in the extrathoracic location of the heart. Three possible theories for the development of this condition have been described in animals: the first is related to a primary failure in the descent of the heart and fusion of the midline of the body, the second is associated with rupture of the amnion, and the third is associated with amniotic band syndrome. Toxic plants, inbreeding, and some pharmacological compounds have been associated with the development of the anomaly. However, in most cases, the precise cause of the appearance of the malformation is unknown. This animal was considered a sporadic case of EC. EC has been described more frequently in cattle, although there are reports in other species, such as dogs, cats, and sheep. Treatment can be surgical, but this procedure is rarely an option.

Common Pediatric Hand Anomalies

AbstractUpper 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.

Superior fornix prolapse in a newborn with amniotic band syndrome

Superior fornix prolapse in a newborn with amniotic band syndrome

Keystone Flap in Amniotic Band Syndrome—Innovative Approach of an Established Operative Technique for an Unusual Entity

Amniotic Band Syndrome (ABS) is a complex condition characterized by constricting rings and tissue synechiae, resulting in tissue necrosis and congenital anomalies. In newborns and infants with ABS, tissue necrosis can be profound, requiring a tissue defect reconstruction, realized by a Keystone Perforator Island Flap (KF). Primarily used for reconstruction after skin cancer excisions, KF’s applications expanded to defects of various etiologies and disorders throughout the body. Subsequently, additional KF types adapted to the particular tissue defects were developed. The KF’s preparation is relatively simple to perform leading to shorter operative times, and the postoperative monitoring is less laborious. Individualized surgical approaches and timing are essential for addressing the varied manifestations of ABS, with immediate treatment recommended for vascular compression, all-layered tissue necrosis, and nerve compression cases. To our knowledge, there is no published case in which a KF was used for the reconstruction of tissue defects and release of constriction rings in the context of an amniotic band syndrome. Therefore, the purpose of this article is to introduce the established surgical technique of KFs as an innovative surgical approach with satisfying reconstructive results for tissue defects and constriction ring release in ABS.

Anaesthesia Management of Congenital Alveolar Synechiae Release in a Neonate

Abstract A neonate, with complete fibrous congenital alveolar synechiae and amniotic band syndrome affecting the upper and lower limbs, was posted for surgical release of synechiae under anaesthesia. A neonate with no mouth opening poses many anaesthesia challenges. The surgical and anaesthesia plan was discussed with ear, nose and throat surgeons. Topicalisation of airway, small aliquots of intravenous (IV) ketamine and propofol and sevoflurane inhalation helped achieve adequate depth of anaesthesia to carry out flexible intubating video endoscope-guided nasotracheal intubation successfully. Post-surgery, a check laryngoscopy with D-Blade of C-MACR (Karl Storz Video Macintosh Laryngoscope) video laryngoscope revealed a Cormack Lehane view of grade IV. The trachea was not extubated on table and the baby was shifted back to the neonatal intensive care unit with pressure support ventilation and followed by tracheal extubation next morning. Rest of the course for the baby was uneventful. Anticipation, planning, preparedness, vigilance and team work during anaesthesia management led to a successful outcome in this case.

Amniotic Band Syndrome with Severe Facial Cleft: A Case Report.

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.

Amniotic Band Sequence: A Rare Case Seen In Birat Medical College

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Amniotic Band Syndrome: Head-to-Toe Manifestations and Clinical Management Guidelines.

After studying this article, the participant should be able to: 1. Understand and describe the basic principles underlying the intrinsic versus extrinsic proposed mechanisms for the development of amniotic band syndrome (ABS). 2. Discuss risk factors and conditions that are associated with the development of ABS. 3. Understand the various presentations and associated clinical implications of ABS by anatomic location. 4. Describe the basic tenets underlying various repair technique options for ABS of the extremities. 5. Discuss the common manifestations of ABS in the face and trunk. 6. Understand clinical implications of fetoscopic surgery as it relates to ABS treatment as a novel method for limb salvage in utero. Amniotic band syndrome (ABS) refers to the development of constrictive bands of fibrotic tissue in utero. It can lead to a variety of clinical manifestations after delivery. There is much debate in the plastic surgery community regarding the exact pathophysiologic mechanism for the development of ABS, and the most appropriate management. This CME article aims to provide an overview of ABS manifestations throughout the body, and to expound on the most recent advances in anticipatory and definitive management of the condition.

Cryptophthalmos: associated syndromes and genetic disorders

ABSTRACT Purpose Cryptophthalmos is a rare congenital condition caused by anomalous eyelid development where the eyelid folds do not develop or fail to separate. Cryptophthalmos can be unilateral or bilateral and can occur in isolation or as part of an underlying syndrome. We aim to identify genetic syndromes associated with cryptophthalmos to facilitate genetic diagnosis. Methods We performed a retrospective medical record review of all patients diagnosed with cryptophthalmos followed at a single center between 2000 and 2020. The analysis included medical history, clinical examination findings, and genetic testing results. Results Thirteen patients were included, 10 (77%) males, mean age of 2.4 years. Eight (61%) had bilateral cryptophthalmos, and 4 (31%) had complete cryptophthalmos. Associated ocular abnormalities included corneal opacities (13/13, 100%), upper eyelid colobomas (12/13, 92%), and microphthalmia/clinical anophthalmia (3/13, 23%). All cases of complete cryptophthalmos had bilateral disease. An underlying clinical or molecular diagnosis was identified in 10/13 (77%) cases, including Fraser syndrome (n = 5), amniotic band syndrome (n = 1), FREM1-related disease (n = 1), Goldenhar versus Schimmelpenning syndrome (n = 1), MOTA syndrome (n = 1), and CELSR2-related disease (n = 1). Conclusion This is the first report of a possible association between cryptophthalmos and biallelic CELSR2 variants. Children with cryptophthalmos, especially those with extra-ocular involvement, should be referred for comprehensive genetic evaluation.

Congenital Nonsyndromic Anonychia of Toes Due to Amniotic Bands

Congenital Nonsyndromic Anonychia of Toes Due to Amniotic Bands

Amniotic Band Syndrome – A Case Report

Amniotic Band Syndrome (ABS) encompasses a wide spectrum of abnormalities, all of which result from the entrapment of various parts of the fetal body in the disrupted amniotic fluid. Because of the randomness of entrapment, each affected individual has the potential to develop a unique deficit. Termination of pregnancy is usually suggested at the time of diagnosis of severe abnormalities, while minor limb defects can be repaired by surgery after birth [1]. Here we report a case of amniotic band syndrome detected during fetal anomaly scan at 19w0d gestational age

Amniotic band syndrome with CNS involvement: a pediatric neurosurgeon's dilemma-a case series and literature review.

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. 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. 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.

CLASSIFICATION OF CONGENITAL AND ACQUIRED SYNDROMES FOUND IN INFANCY

Failure to thrive is the term used to describe the condition in which a young child fails to gain weight satisfactorily. Common reasons for such poor weight gain are parental neglect or lack of food. On the other hand, a large number of important gastrointestinal disorders may be responsible, including those associated with vomiting, such as food intolerance or obstruction of the upper bowel by pyloric stenosis; disorders of digestion and absorption, including celiac disease and cystic fibrosis; and bowel infections. Amniotic band syndrome (ABS) is a set of congenital malformations attributed to amniotic bands that entangle fetal parts during intrauterine life, which results in a broad spectrum of anatomic disturbances - ranging from minor constriction rings and lymphedema of the digits to complex, bizarre multiple congenital anomalies incompatible with life.

Outcomes of Linear Circumferential Skin Closure for Congenital Constriction Ring Syndrome.

Background: Congenital constriction ring syndrome (CCRS) is a rare condition diagnosed at birth characterised by deformation due to a constriction ring. The usual treatment for CCRS involves excision of the constriction ring and suture of the skin incorporating a Z-plasty to prevent scar contracture. A Z-plasty often results in an unsightly scar. In order to avoid this, we performed linear circumferential skin closure (LCSC). The aim of this paper is to report the outcomes of LCSC for CCRS. Methods: We retrospectively investigated all patients with CCRS who underwent LCSC between 2002 and 2020. Two linear incisions were placed proximal and distal to the constriction ring in parallel, and the constriction ring was excised carefully so as not to damage nerves or vessels. The deep subcutaneous and dermis layers were sutured. The skin was closed using adhesive tape. Two-stage surgery was performed in two patients with severe CCRS of the lower leg to avoid problems with distal circulation. Patients were followed up for at least 1 year and assessed for complications and quality of scar. Results: We performed LCSC for 31 sites in 19 patients, including one forearm, 14 fingers, 10 lower legs and six toes. The median age at the operation was 16 months (range: 4-175). The median follow-up period after surgery was 5.8 years (range: 1.9-16.0). The linear surgical scar had healed well in all patients and there were no complications. There was no recurrence of the constriction ring and no scar hypertrophy, though we did not perform fat mobilisation in all cases. None of the patients required additional surgery and the aesthetic outcome of the linear circumferential surgical scar was maintained at the final observation. Conclusions: Treatment for CCRS using LCSC resulted in no complications, no recurrence of constriction and an excellent aesthetic outcome. Level of Evidence: Level IV (Therapeutic).

Readability of Online Patient Education Materials for Congenital Hand Differences.

The aim of the study is to assess the readability of online patient education materials (PEMs) for congenital hand differences. The top 10 online, English-language PEMs for 10 conditions (polydactyly, syndactyly, trigger finger/thumb, clinodactyly, camptodactyly, symbrachydactyly, thumb hypoplasia, radial dysplasia, reduction defect, and amniotic band syndrome) were compiled and categorized by source and country. Readability was assessed using 5 tools: Flesch Reading Ease Score (FRES), Flesch-Kincaid Grade Level (FKGL), Gunning Fog Index (GFI), Coleman-Liau Index (CLI), and Simple Measure of Gobbledygook Index (SMOG). To account for the potential effect of each condition's name in the aforementioned formulas, the analysis was repeated after replacing the name with a monosyllabic word/s. The mean readability scores of the 100 PEMs were FRES 56.3, where the target was ≥80, FKGL 8.8, GFI 11.5, CLI 10.9, and SMOG 8.6, and the median grade score was 9.8, where the target grade was ≤6.9. Following adjustment, all readability scores improved significantly (P < .001). Postadjustment scores were FRES 63.8, FKGL 7.8, GFI 10.7, CLI 9.1, and SMOG 8.0, and the median grade score was 8.6. Only 1 webpage met the target level using all tools. Two-sample t test for country of publication (the United States and the United Kingdom) demonstrated that PEMs originating from the United Kingdom were easier to read using the preadjustment CLI (P = .009) and median grade metrics (P = .048). A 1-way analysis of variance revealed no influence of condition or source on readability. Most online PEMs for congenital hand differences are written above the recommended reading level of sixth grade, even when adjusted for the effect of the condition's name.

Everything You Need to Know About Amniotic Band Syndrome (ABS)

Amniotic band syndrome (ABS) is a fetal and neonatal condition whereby the lining of the amniotic sac is compromised and may have an impact on neonatal morbidity and mortality. Whilst it is uncommon and is outside the scope of practice for the midwife to treat, the role of the midwife is essential in these circumstances. This article provides a brief overview of cause, diagnosis, care and treatment of these neonates. The inconsistencies in the currently accessible literature are discussed.

Fabrication and multiscale modeling of polycaprolactone/amniotic membrane electrospun nanofiber scaffolds for wound healing.

Enhancing the efficiency of cell-based skin tissue engineering (TE) approaches is possible via designing electrospun scaffolds possessing natural materials like amniotic membrane (AM) with wound healing characteristics. Concentrating on this aim, we fabricated innovative polycaprolactone (PCL)/AM scaffolds through the electrospinning process. The manufactured structures were characterized by employing scanning electron microscope (SEM), attenuated total reflection-Fourier transform infrared (ATR-FTIR) spectroscopy, tensile testing, Bradford protein assay, etc. In addition, the mechanical properties of scaffolds were simulated by the multiscale modeling method. As a result of conducting various tests, it was concluded that the uniformity and distribution of fibers decreased with an increase in the amniotic content. Furthermore, PCL-AM scaffolds contained amniotic and PCL characteristic bands. In the case of protein release, greater content of AM led to the release of higher amounts of collagen. Tensile testing revealed that scaffolds' ultimate strength increased when the AM content augmented. The multiscale modeling demonstrated that the scaffold had elastoplastic behavior. In order to assess cellular attachment, viability, and differentiation, human adipose-derived stem cells (ASCs) were seeded on the scaffolds. In this regard, SEM and 3-(4,5-dimethylthiazol-2-yl)-2,5-diphenyl-2H-tetrazolium bromide (MTT) assays showed significant cellular proliferation and viability on the proposed scaffolds, and these analyses illustrated that higher cell survival and adhesion could be achieved when scaffolds possessed a larger amount of AM. After 21 days of cultivation, particular keratinocyte markers, such as keratin I and involucrin, were identified through utilizing immunofluorescence and real-time polymerase chain reaction (PCR) tests. The markers' expressions were higher in the PCL-AM scaffold with a ratio of 90:10 v v-1 compared with the PCL-epidermal growth factor (EGF) structure. Moreover, the presence of AM in the scaffolds resulted in the keratinogenic differentiation of ASCs even without employing EGF. Consequently, this state-of-the-art experiment suggests that the PCL-AM scaffold can be a promising candidate in skin bioengineering. This study showed that mixing AM with PCL, a widely used polymer, in different concentrations can overcome PCL disadvantages such as high hydrophobicity and low cellular compatibility.

Acute soft Tissue Infection with impending Loss of Finger in amniotic Band Syndrome of a 22-years-old Patient with palmoplantar Keratoderma congenital Alopecia Syndrome Type II

We report on the treatment of a 22-year-old female patient with an acute soft tissue infection in the area of an amniotic band due to palmoplantar keratoderma congenital alopecia syndrome (PPKCA) type II, a very rare genodermatosis with less than 20 cases described in literature. An acute soft tissue infection distal from the pre-existing constriction ring with hyperkeratosis on the right small finger led to a decompensation of the venous and lymphatic drain with imminent loss of the finger. Due to urgent surgical treatment with decompression and debridement of the dorsal soft tissue infection, microsurgical circular resection of the constriction ring and primary wound closure the finger could be preserved. After soft tissue consolidation and hand therapy, the patient achieved free movement of the small finger with subjective freedom of symptoms and good aesthetic results.

Severe Eyelid Malformation With Facial Clefting and Amniotic Bands.

Amniotic bands can lead to eyelid colobomas contiguous with facial clefts, resulting in severe and highly variable eyelid malformations. There is no known underlying genetic cause of amniotic band sequence. Here, the authors review the case of an infant born with large, 4-eyelid colobomatous defects in the setting of facial clefts, amniotic bands and an underlying SMOC1 mutation, which has not previously been linked to amniotic band sequence or eyelid colobomas. Reconstructive technique and the postoperative course are described, and underlying etiologic theories of amniotic band sequence are reviewed and expanded upon. Although amblyopia prevention in this patient with poor visual potential was not a consideration, the goals of improving the patient's ocular surface and maintaining eye contact were achieved.