Presentation and multidisciplinary management of a unique case of lower limb dysmelia resulting from amniotic band syndrome.

A neonate was born with a unique congenital lower limb dysmelia due to an abnormal presentation of amniotic band syndrome. An anomalous soft tissue tether from the plantar surface of the right foot to the right buttock caused extreme knee flexion, tibial rotation and malformation of the developing foot. This complex malformation required a multidisciplinary team (MDT) approach to decide between reconstruction and amputation. The band of tissue was released operatively at 73 days postdelivery, improving knee extension, and the tissue was banked on the thigh as a tube pedicle for future reconstruction. The patient underwent rehabilitation, which has been shown to be vital for synovial joint formation. At 18 months old, the decision was made to proceed with through-knee amputation and a prosthesis. The literature discussed shows the importance of an MDT approach in complex lower limb cases to give the best functional outcome for the patient.

Constrictive amniotic band syndrome, when the embryo gets caught.

Constrictive amniotic band syndrome (CABS) is a rare condition which occurs sporadically and should not be compared with a syndrome as it is known from genetics. However, since the pathogenesis is not yet fully understood, some theories suggest genetics playing a role. Even though CABS is rare it is associated with a high risk of foetal death as seen in 1/70 of these cases. The diagnostics start with ultrasound of the pregnant woman, and if this leads to suspicion of CABS it is possible to supplement with 3D ultrasound or MRI to verify. The treatment involves either in utero or postnatal surgery depending on the case, as argued in this review.

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

LEARNING OBJECTIVES: 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. SUMMARY: 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.

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

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.

Emergency approach in a rare congenital coexistence-ichthyosis and amniotic band syndrome.

Ichthyosis is caused by Mendelian cornification disorders. Hereditary ichthyoses are divided into non-syndromic and syndromic ichthy-oses. Amniotic band syndrome involves congenital anomalies that most frequently cause hand and leg rings. The bands can wrap around the developing body parts. In this study, it is aimed to present an emergency approach to amniotic band syndrome accompanying a case of congenital ichthyosis. We were asked by the neonatal intensive care unit to consult on the case of a 1-day-old baby boy. On physical examination, congenital bands were found to be present on both hands, the toes were rudimentary, skin scaling was present on the entire body, and the consistency of the skin was stiff. The right testicle was not in the scrotum. Other system examinations were normal. However, the blood circulation in the fingers in the distal of the band had become critical. With the help of sedation, the bands on the fingers were excised, and after the procedure, it was observed that the circulation in the fingers was more relaxed than it had been before the procedure. Coexistence of congenital ichthyosis and amniotic band is very rare. Emergency approach to these patients is very important in terms of saving the limb and preventing growth retardation in the limb. As further developments take place in terms of prenatal diagnoses, these cases will be able to be prevented through the early diagnosis and treatment.

[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]. / Akute Weichteilinfektion mit drohendem Fingerverlust bei Schnürfurchensyndrom einer 22-jährigen Patientin mit palmoplantarem Keratoderm-kongenitalem Alopezie-Syndrom Typ 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.

Fetoscopic surgery for amniotic band syndrome: Case series.

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.

Clinical Manifestations of Constriction Band Syndrome.

BACKGROUND: Constriction band syndrome (CBS) is a congenital limb anomaly with varying presentation. We sought to characterize the clinical manifestations of CBS by analyzing a large cohort of patients. Our secondary aim was to evaluate potential risk factors for CBS. METHODS: We retrospectively reviewed the records for all patients with CBS who had presented to our tertiary medical center between 1998 and 2018. Examination by a pediatric orthopaedic surgeon and the presence of pathognomonic features were inclusion criteria. Clinical notes, photographs, and radiographs were reviewed to determine the pattern of limb involvement and associated conditions. Demographic data were compared with population averages to assess for possible risk factors. RESULTS: One hundred and twenty-eight children were included. The most prevalent feature was constriction bands (96%), followed by limb or digit amputations (88%) and syndactyly/acrosyndactyly (69%). Children without constriction bands presented with either acrosyndactyly or growth of an osseous spike from a congenital diaphyseal limb or digit amputation. There was a strong predilection for involvement of central digits of the hands and feet, with sparing of the thumb/great toe and small finger/toe. The average number of involved limbs per child was 2.6; 23% of the children had involvement of only 1 limb. Children with at least 1 additional diagnosis had more limbs affected by CBS than those who were otherwise healthy (2 limbs [interquartile range (IQR), 1-3 limbs] versus 3 limbs [IQR, 2-4 limbs]; p = 0.006), suggesting a more severe phenotype. Children with more limbs involved underwent more surgical procedures (p < 0.001). CBS was associated with gestational trauma, prematurity, low birth weight, young and old maternal age, and higher social deprivation. CONCLUSIONS: Children with CBS can be categorized as having either mild or extensive involvement based on the number of involved limbs and associated conditions. Future investigation of prenatal risk factors is necessary to further elucidate the etiology of this heterogenous condition. LEVEL OF EVIDENCE: Prognostic Level IV. See Instructions for Authors for a complete description of levels of evidence.

Successful Emergent Treatment of Limb-Threatening Amniotic Constriction Band in a Neonate with a 2-year Follow-up: A Case Report.

CASE: A 29-week-old gestational age neonate born by emergent cesarean delivery was found to have a circumferential upper arm amniotic constriction band. Initial removal of the amniotic band at the time of delivery uncovered a circumferential skin band with profound venous obstruction but preserved Doppler arterial flow. Emergent excision of the constricted skin band with fasciotomies of the arm and forearm was performed shortly after birth to preserve the extremity. CONCLUSION: This report outlines a visualized constriction band at the time of delivery causing neurovascular compromise to an extremity of a neonate. Prompt intervention ensued preserving both form and function with an excellent 2-year follow-up.

Disseminated intravascular coagulation complicated by amniotic band syndrome associated with deceased fetal umbilical cord following fetoscopic laser surgery.

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.

Use of an Adipofascial Superficial Circumflex Iliac Artery Perforator Flap to Improve Contour Following Resection of Upper Extremity Amniotic Band Constriction: A Report of Two Patients.

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.

Multiple Continuous Y-to-V-Plasties for Excision and Reconstruction of Constriction Band Syndrome: Case Series and Description of Surgical Technique.

BACKGROUND: Many patients with constriction band syndrome will seek treatment to improve limb function and cosmesis. Although several techniques for excision are reported, cosmetic and functional deficits can persist, including hourglass deformity, lymphedema, and inadequate band excision with resultant neurovascular compromise, which may lead to revision surgery, including amputation. The authors present a novel technique that uses multiple continuous opposing Y-to-V-plasties in a single or staged manner to correct extremity constriction bands. METHODS: Seven patients with 10 involved extremities were included in this retrospective case series. Patient demographics, associated abnormalities, and operative indications were collected from medical records. The primary outcome measures were primary wound healing and release of the constriction band. Secondary outcomes measures were perioperative complications and the need for revision surgery. RESULTS: All patients achieved primary closure with excellent healing in one or two stages. No infections or scar complications occurred, and no revision surgery of the band was required. One patient had mild indentation of scar tissue after their second stage, which improved with scar massage. One patient who had preoperative sciatic compression neuropathy and an insensate foot secondary to band constriction developed chronic calcaneal osteomyelitis requiring a subsequent Syme amputation. Postoperative lymphedema occurred in two patients, which resolved with the use of compression bandages over several weeks. CONCLUSION: Use of multiple continuous opposing Y-to-V-plasties is a safe and effective technique for adequate constriction band excision without residual hourglass deformity and good functional outcomes for patients with partial or circumferential constriction bands. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

[Release of amniotic constricted grooves by hemi-circumferential excision and skin closure without Z-plasty]. / Libérations des sillons de constriction amniotique par excision hémi-circonférentielle et fermeture cutanée sans plastie en Z.

INTRODUCTION: Constriction grooves are typical abnormalities of constriction band syndrome (CBS). Treatment by complete excision and Z-plastic closure is the gold standard for release of these grooves. However, the results of these Z-plasties are often judged to be aesthetically insufficient. The hypothesis was that direct excisional surgery without Z-plasty gave better aesthetic results. The objectives of this study were (1) to evaluate the clinicaal results of the release of amniotic furrows by simple hemi-circumferential excision without resorting to Z-plasties, (2) to study the occurrence of complications, (3) to study the factors leading to complications. MATERIALS AND METHODS: All patients who had release of amniotic grooves by linear hemi-circumferential excision and closure without Z-plasties were included between 2011 and 2017. Release of complete or circumferential grooves was carried out in 2 stages. Clinical evaluation was based on parental satisfaction with the aesthetic appearance and occurrence of any complications or recurrence. RESULTS: Fourteen grooves including 8 circumferential and 6 semi-circumferential were released, in 7 patients. Average age at surgery was 9 months (3-18 months). Some patients had 1, 2 or 3 surgeries. The groove was localized in 7 cases in the leg, in 4 in the fingers and in 1 in the thigh, toes and forearm. At the last average follow-up of 25 months (12-41 months), all parents were very satisfied with the aesthetic appearance. No complications, including scarring, vascular or lymphatic disease, were noted. No recurrence was noted. CONCLUSION: Release of amniotic grooves by simple linear hemi-circumferential excision without Z-plasties is a simple, safe and effective technique. The aesthetic results are very satisfactory. LEVEL OF EVIDENCE: IV - retrospective study.

A new phenotype of amniotic band syndrome with occipital encephalocele-like morphology: a case report.

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.

Early Surgical Intervention in Amniotic Band Sequence for Upper Extremity Motor Nerve Palsies: A Case Report.

Urgent surgical intervention for amniotic band sequence (ABS) is currently indicated for concerns of vascular compromise and progressive lymphedema. Peripheral motor nerve palsies are rare, and reports of surgical intervention in these cases describe persistent motor dysfunction. We report band release and ulnar, median, and radial nerve decompression in a 1-week-old with a severe upper extremity constriction band and signs of ulnar nerve motor dysfunction. A literature review on nerve exploration and outcomes of patients with motor nerve palsy from ABS was performed. Early evidence of ulnar motor function was observed at 5.5-month follow-up. Previous reports of nerve decompression for upper extremity constriction bands with motor nerve palsy document poor recovery after interventions beginning at 3 months of age. In this case, band release and nerve decompression were undertaken at 7 days of age, and we observed early motor recovery. This finding suggests that very early surgical intervention in the neonate may facilitate nerve recovery in appropriate candidates.

Clinical assessment of the results of one-stage circular incision techniques for limb ring constriction due to amniotic band syndrome.

BACKGROUND: Amniotic band syndrome (ABS) is a congenital malformation that results in abnormalities in many parts of the body. Most surgical treatments for ABS used multi-stage Z-plasties. The purpose of this study was to assess the clinical results of one-stage circular incision techniques for limb ring constriction due to amniotic band syndrome. METHODS: We reviewed 27 patients with limb ring constriction in ABS from 2010 to 2020. The mean ages of the patients were 11.7 months (range, 0-72 months). The complete circular incision release the ABS. All patients' operations were used one-stage circular incision surgical techniques, including patients with multiple bands. All the patients were followed up with a period ranges from 2 years to 10 years. Patient-reported visual analog scale (VAS) scar ranking on a scale of 0 (minimum satisfaction) to 10 (maximum satisfaction) were used to evaluate esthetic outcomes. RESULTS: After our surgery, all the limbs, toes, and fingers were rescued, and the lymphedema reduced remarkably. The VAS scores (mean ±â€ŠSD) for patient satisfaction were 7.55 ±â€Š1.89. The surgical treatment of amniotic band syndrome in a one-stage circular incision is safe and effective. CONCLUSION: The one-stage circular incision surgical techniques have many advantages, including reduced surgical invasiveness, scar formation, and the cost of treatment. LEVEL OF EVIDENCE: Level IV-retrospective case series.

Delayed Onset of Osteomyelitis in Fetal Limb Amputation Secondary to Amniotic Band Syndrome: A Case Report.

CASE: We present the case of an infant with an amputation from amniotic band syndrome and no other complications. During the seventh week of life, the infant developed severe pain in the affected limb that was ultimately found to be indicative of osteomyelitis. CONCLUSION: The infant displayed minimal systemic response, and cultures that grew methicillin-sensitive Staphylococcus aureus were needed to confirm the diagnosis. Surgical debridement, stump revision, and oral antibiotic therapy provided definitive treatment for our patient. The reader is encouraged to consider the possibility of osteomyelitis in similar circumstances.

Fetoscopic Release of Amniotic Band Syndrome: An Update.

Amniotic band syndrome is a rare condition. There have been few cases reported of fetoscopic band dissection. The aim of this case series is to report 3 cases of fetoscopic treatment for amniotic band syndrome, including indication for surgery, technical aspects, complications and outcomes. Fetoscopic treatment was performed respectively at 23 5/7 , 26 5/7 and 18 3/7 weeks' gestation. Two procedures were performed with a laser fiber through a single trocar whereas one surgery was performed with scissors. In conclusion, fetoscopic release of the amniotic bands in case of amniotic band syndrome is feasible with encouraging results in order to prevent amputation and dysfunction of the extremities.

Sinus pericranii-unusual anatomic obstacle to posterior decompression on an amniotic band sequence.

Abnormal venous drainage in patients with craniofacial anomalies is relativity uncommon. Sinus pericranii is a rare vascular malformation characterized by communication between intracranial dural sinuses and extracranial venous drainage systems. The association between sinus pericranii and amniotic band syndrome has not been described. We report on a 7-month-old girl diagnosed with amniotic band sequence who underwent posterior fossa decompression due to intracranial hypertension and Chiari malformation type I. The computed tomography and magnetic resonance identified the connection between the sagittal sinus and the scalp. During the operation, the presence of sinus pericranii was a complicating factor limiting the proper exposure because of the risk of bleeding. Patients with craniofacial anomalies and sinus pericranii present an increased risk of serious surgical complications and consequences from craniofacial surgery, especially from cranioplasty and posterior decompression. Special attention must be paid to abnormal venous drainage, and vascular imaging studies are sometimes required.