Amniotic constriction bands: a case series and proposed new classification system.

INTRODUCTION: Amniotic constriction bands occur in approximately one in every 15,000 live births and is graded using the Patterson Classification system. METHODS: A case series of all patients with amniotic constriction band that presented to Alder Hey Children's Hospital was compiled between the years 1993 and 2012, data was described and a classification system developed. RESULTS: Thirty seven patients were identified. Of these 17 had amniotic constriction confined to the upper limbs and three of the lower limbs only. Seventeen had both upper and lower limb deformity. Twenty eight children underwent surgical intervention whilst nine to date have not. CONCLUSION: Constriction band of the upper limb was the most common with lower limb bands the least. There is no agreement on the nomenclature of this condition making amalgamation of the literature problematic. A more specific classification of upper limb bands has been suggested which includes anatomical location and depth of band.

Amniotic band sequence: prenatal diagnosis, phenotype descriptions, and a proposal of a new classification based on morphologic findings.

OBJECTIVE: The aim of this study is to describe the phenotype of fetuses affected by amniotic band sequence (ABS) that were diagnosed at the Instituto Nacional de Perinatología Isidro Espinosa de los Reyes and to propose a new classification based on morphologic findings. MATERIAL AND METHODS: Cases with a final diagnosis of amniotic band sequence, diagnosed between January 1993 and July 2010 in the Department of Maternal Fetal Medicine, were reviewed. Demographic, clinical, and periconceptional data were collected, and the defects were described and classified. The association frequencies of the defects were also determined. RESULTS: We included 50 cases with prenatal diagnosis of amniotic band sequence. The mean maternal age was 25.7 ± 6.9 years. Of these patients, 54% (27/50) were primiparous compared to 22% (11/50) who had three or more previous pregnancies. Craniofacial defects were seen in 78% (39/50) of the cases, followed by defects of the extremities 70% (35/50), abdominal wall, spine, and/or thorax 52% (26/50). The most frequent defects were the following: a) Encephalocele and facial clefts in the craniofacial group. b) Shortening at any level in the limb defects group, and c) Alterations of the spinal column curvature in the group of "other" defects. CONCLUSIONS: The amniotic band sequence shows a tendency to affect women who are in their earlier years of reproduction. We observed an inverse relationship between the number of pregnancies and the frequency of presentation of this pathology. The majority of affected fetuses showed a phenotype that fit into one of many groups. Therefore, we propose classifying the amniotic band sequence phenotypes into the following groups: I. Craniofacial defect + limb defect. II. Craniofacial defect + limb defect + abdominal wall, spinal column, and/or thoracic defects. III. Limb defect + abdominal wall, spinal column, and/or thoracic defects; and IV. Isolated defect (craniofacial, limb, or thoraco-abdominal wall). This classification system will be helpful in diagnosing amniotic band sequence. Based on future research studies, we hope that we can use this classification system as a prognosis fetal factor to establish a more accurate fetal prognosis and recurrence probability. Finally, we created a flowchart describing all of the steps that were followed by our Department from the moment an amniotic band was found by ultrasound until the definitive diagnosis was made and the follow up according to the fetal findings.

Fetoscopic release of an amniotic band with risk of amputation: case report and review of the literature.

Amniotic bands can cause limb amputation as well as intrauterine fetal demise. Fetoscopic release of amniotic bands has been shown to rescue limb function. Herein, we describe an interesting case of an amniotic band wrapped around the right forearm as well as the umbilical cord, which was released by fetoscopy. Following the procedure, the blood flow in the forearm was restored and the edema gradually reduced. Full functionality of the arm was confirmed after birth, though the esthetic problem of the imprint of the band on the arm persisted. Furthermore, we review the available literature regarding fetoscopic interventions in amniotic band syndrome.

Constriction band syndrome.

Constriction band syndrome is an uncommon congenital abnormality with multiple disfiguring and disabling manifestations. Early amnion rupture with subsequent entanglement of fetal parts by amniotic strands is currently the primary theory for the development of this syndrome. Management of constriction band syndrome is focused on improving function and development while providing a more acceptable esthetic appearance. The treatment should be tailored to the individual. Timing of repair and surgical planning are important to provide the best functional results for affected hands.

Amniotic band syndrome of the upper extremity: diagnosis and management.

Amniotic band syndrome, a condition involving fetal entrapment in strands of amniotic tissue, causes an array of deletions and deformations. Band formation most frequently affects the distal segments, including the hand. Because of the heterogeneous nature of expression of this disease, treatment is individualized. Timing of repair and surgical planning are important in improving functional outcome. In the patient with distal edema and acrosyndactyly, early repair portends better prognosis. Improvements in prenatal diagnosis and fetoscopic surgical technique may eventually allow treatment of amniotic band syndrome in utero.

Classification of the pattern of intrauterine amputations of the upper limb in constriction ring syndrome.

Twenty patients with congenital upper limb amputations caused by constriction rings were reviewed to classify the pattern of these amputations. In the 20 patients studied, 31 upper limbs had congenital amputations. The pattern of amputation was classified into three types. Proximal upper limb amputation was considered type I and was only seen in one limb. The most common pattern of amputation was digital amputation associated with "coning" or "superimposition" of the digits (type II) and was seen in 20 hands. Type II amputations were subclassified according to the involvement of all, ulnar, radial, or central digits by the constriction ring. In type III amputations (N = 10 limbs), there was no associated coning or superimposition of the digits. This type of amputation was subclassified into type IIIA (multiple-digit amputations within the same hand) and type III B (single-digit amputation). Associated anomalies are reviewed and the pathogenesis of constriction rings is discussed.

Bilateral cleft lip and palate and limb deformities: a presentation of amniotic band sequence?

We report a case of a child born with bilateral complete cleft lip and palate, absence of the left lower leg, complete absence of the right arm, and absence of the second and third distal phalanges of the right foot. The child had no other associated abnormalities. Prenatal ultrasonography recognized the cleft lip and palate but failed to appreciate the limb deformities. Although this specific constellation of abnormalities has not been previously reported in the English literature, it may represent an amniotic band sequence or two unrelated anomalies. However, unlike the vast majority of patients with amniotic band sequence described, there were no associated bizarre facial clefts or cranial defects. The case history and a review of the literature will be presented, with emphasis placed on categorization of these specific anomalies.

Amniotic band syndrome: the association between rare facial clefts and limb ring constrictions.

The pathologic cause of both rare craniofacial clefts and congenital limb ring constrictions is the subject of some debate. Uncommon though these two conditions are, they have often been anecdotally reported in association. This study sought to determine the frequency of congenital limb anomalies in general and limb ring constrictions in particular amongst a population of patients with rare craniofacial clefts. Eighty-five cases of nonsyndromal, rare, craniofacial cleft were identified from a population of 280 patients assessed at the Women's and Children's Hospital, North Adelaide, with a diagnosis of rare craniofacial cleft classifiable by the Tessier system. Twenty-two patients (25.9 percent) displayed congenital limb anomalies. Eleven of these (12.9 percent) showed evidence of limb ring constrictions, a frequency much greater than in the general population. The group with limb ring constrictions demonstrated a significantly greater complexity of involvement with craniofacial clefting than the non-limb ring constriction group (4.27 clefts/patient versus 2.3 clefts/patient, p < 0.01). The distribution of craniofacial cleft locations in patients with evidence of limb ring constrictions was found to differ significantly from those with other or no limb anomalies (p < 0.01). The clefts in cases in which limb ring constrictions coexist are largely restricted to the paramedian 2-12, 3-11, 412 axes. This study confirms an association between rare craniofacial clefts and limb ring constrictions. The two conditions may therefore possess common etiology.

Application of the IFSSH(3)-classification for congenital anomalies of the hand; results and problems.

The extended classification proposed by the IFSSH was used to classify 1013 hand anomalies in 925 hands of 650 patients. We found associated anomalies in 26.7%. The classification was straightforward in 86%, difficult in 6.6% and not possible in 7.8%. In group I the radial and ulnar deficiencies, limited to the hand and without forearm deficiencies should be included. Group II was the most important group including 513 anomalies. We propose to include in this group the Madelung deformity, the Kirner deformity and congenital trigger fingers and trigger thumbs. Triphalangeal thumbs are a problem, we suggest to list this anomaly in group III and to consider it as a duplication in length. It is not always possible to evaluate the (transverse) absence of the fingers or hand. Longitudinal deficiencies (group IIB), symbrachydactyly group (V) and amniotic bands (group VI) occasionally develop a phenotype similar to the genuine transverse deficiency (group IA).

Síndrome de limb-body wall complex en embarazo gemelar a propósito de un caso y revisión de la literatura / Syndrome of Limb-body wall complex in twin Dizygotic pregnancy topurpose a case and review of the literature

Presentamos un caso de Limb-Body Wall Complex en embarazo gemelar dicigótico. El gemelar acompañante no tenía malformaciones y solo era pequeño para la edad gestacional. El primer feto se obtiene por vía vaginal normal. Al intentar extraer en poládico al dismórfico no fue posible. Se extrajo por cesárea y muere a los pocos minutos de nacido. El feto malformado presentaba amelia de brazo y pierna derechos, escoliosis severa, toraco-abdominosquisis y cordón con tres vasos. Se apreciaba magma en cara anterior de tronco donde había órganos torácicos externos, rodeados de membrana amniótica. Pensamos que al haber ruptura del amnios en etapa temprana embrionaria, se producen adherencias toraco-parietales que mantienen el corazón fuera del tórax e impiden el cierre esternal para siempre junto a un fenómeno de disrupción vascular que ocasionaria las malformaciones de los miembros y cabeza

Constriction band-induced vascular compromise of the foot: classification and management of the "intermediate" stage of constriction-ring syndrome.

Gangrene of an extremity secondary to a congenital constriction band may result from in utero or postnatal vascular compromise. Often ths process is completed in utero following spontaneous resolution of the vascular insufficiency, resulting in a healed wound by fetal repair and regeneration or in amputation of the distal part. When this process is progressive as a result of worsening lymphaticovenous and/or arterial obstruction with associated soft-tissue necrosis, salvage of the distal part can be accomplished by immediate decompression to evacuate the lymphedema fluid, staged band excision, Z-plasty closure, and topical antimicrobial therapy of the open wound. A revised classification of constriction-ring syndrome incorporating the "intermediate" stage (3B) of severe lymphaticovenous compromise with soft-tissue loss is introduced.