| amniotic band syndrome or amniotic disruption complex is a well-known congenital limb abnormality, which occurs in 1 in 1200 to 1 in 15,000 live births. In cases of an isolated band constriction, it has been speculated that the bands lead to decreased blood flow in the constricted limb and subsequent natural amputation. Fetal surgery could be considered in these situations in order to release the constriction band in threatened limb amputation. We present a case of a tight constriction ring secondary to amniotic band syndrome with a favourable outcome, despite the failure of an attempted surgical procedure. ( info) |
42/136. Prenatal sonographic diagnosis of acrania associated with amniotic bands. We describe the prenatal sonographic findings in 2 cases of amniotic band sequence-related acrania. In the first case, sonography demonstrated acrania, asymmetric facial clefts, and digital fusion on the hands and feet. In the second case, sonography demonstrated acrania and amniotic bands adhering to the brain, face, and body. This report highlights the importance of a careful search for amniotic bands and a detailed survey of the fetus' face, digits, and body in the sonographic evaluation of fetal acrania. ( info) |
43/136. Congenital constriction band of the upper arm: the role of three-dimensional ultrasound in diagnosis, counseling and multidisciplinary consultation. constriction band syndrome represents a sporadic condition that may result in amputations, constrictions and other deformities of the fetal limbs and body. prenatal diagnosis by two-dimensional ultrasound has been reported. We present a case of constriction band involving the upper arm in which the assessment by three-dimensional ultrasound significantly contributed to the diagnosis and the multidisciplinary counseling. In fact, multiplanar imaging and surface rendering allowed a clear depiction of the extent of the constriction, the club-hand deformity and the relationship between the amniotic band, the cord and the fetal limb. This case represents a unique and effective application of three-dimensional ultrasound in prenatal diagnosis. ( info) |
44/136. A case study of amnion rupture sequence with acalvaria, blindness, and clefting: clinical and psychological profiles. The purpose of this article is to report the case of a 10-year-old girl born with anophthalmia, bilateral oblique facial clefts, and missing scalp and bones over the temporal and parietal areas of the cranial vault bilaterally. Early amnion rupture seems to be the most probable cause of this rare combination of anomalies. Because no similar case has been reported in the literature so far, we describe here the clinical and psychosocial history of this unusual patient, who has been able to live the intellectually and socially normal life of a blind child in spite of the major craniofacial deformities. The already completed and possible future therapeutic strategies are discussed. ( info) |
45/136. Amniotic band sequence: the use of bone grafting and distraction osteogenesis. Distraction has proven to be a useful technique in cases of severe bone deficiency in the craniofacial skeleton. This is a report of its use in the treatment of a child affected by amniotic band sequence. This 5 year old Hispanic female presented with transverse deficiences of one lower extremity and both upper extremities. Additionally, she was missing the mandible and overlying soft tissue from ramus to ramus. Although free fibula transfer was a possibility, due to the presence of only one lower extremity, it was elected to utilize the technique of distraction. After preliminary bone grafting to unite these two hemimanibles, the bone graft was distracted bilaterally to create a neomandible. Following consolidation and removal of the distraction devices, the child was markedly improved despite what appeared to be a pathologic fracture in the region of the mandibular angle. Repeat distraction was planned. ( info) |
46/136. Congenital skin tube pedicle associated with the constriction band syndrome. A very rare case of congenital skin tube pedicle with congenital constriction band syndrome was presented. Only one report concerning this bizarre abnormality could be found in the available literature, and no consideration of its etiology has previously been published. In our case, the skin tube was accompanied by the congenital constriction band syndrome, the cause of which is generally thought to be external factors. Thus the etiology of the congenital skin tube pedicle might be the peeling and rolling of a strip of the fetal skin by external force. ( info) |
47/136. Congenital scalp and calvarial deficiencies: principles for classification and surgical management. Congenital defects of the scalp and skull present a challenge for care providers because of a combination of their rarity and the magnitude of potential morbidity. Recent advancements in autogenous and alloplastic cranioplasty and scalp reconstruction techniques argue for a comprehensive consideration of this problem. This article (1) reviews the causes of congenital scalp and calvarial defects; (2) proposes a classification system based on defect type, similar to the tumor-node-metastasis classification, in that defect location, defect size, and extent of neuromeningeal involvement are the critical variables; and (3) presents algorithms for care based on the defect classification. A set of management principles on which treatment plans can be based for these unique problems is provided. ( info) |
48/136. Acquired raised bands of infancy: association with amniotic bands. Raised limb bands are a recently described disorder characterized by skin-colored linear plaques typically located on the extremities of affected children. Various etiologies for their development have been proposed, including amniotic bands. We report a child with extensive raised bands of the trunk and extremities and documented amniotic bands in utero. We propose altering the name of this entity to reflect the wider potential distribution of the lesions. The presence of documented amniotic bands and other sequelae typical for the amniotic band sequence in this patient strongly implicates amniotic bands or related pathology in the formation of acquired raised bands of infancy. ( info) |
49/136. Clefting, amniotic bands, and polydactyly: a distinct phenotype that supports an intrinsic mechanism for amniotic band sequence. Amniotic band sequence (ABS) is a well-described condition involving a variety of congenital anomalies in association with fibrous bands. However, many cases are associated with birth defects that are not readily explained by the mechanism of fibrous strings entangling body parts and causing disruption of the fetal structures. The most common of these is typical cleft lip and palate (CLP). Here we describe such a case, with typical ABS limb defects and constriction bands, along with CLP, supernumerary left nipple, polydactyly, and a skin papilla. This case is nearly identical to a child previously described by Guion-Almieda and Richieri-Costa [2000] and may, therefore, represent a previously unrecognized syndrome that overlaps with ABS. Furthermore it may be that cases with ABS-like anomalies associated with CLP represent a different condition, possibly caused by mutations in the genes Disorganization, p63, or IRF6. ( info) |
50/136. The amniotic sheet: a truly benign condition? OBJECTIVE: Amniotic sheets are the result of uterine synechiae that have been encompassed by the expanding chorion and amnion. Radiologically they are seen as 'shelves' in the amniotic cavity. The benign nature of such amniotic sheets has been documented in many case series in the literature. The objective of this study was to determine the characteristics (if any) of amniotic sheets that predict fetal outcome. methods: Between January 2001 and December 2002, detailed scans were performed in 30 476 singleton pregnancies at 18-32 weeks' gestation. Of these, 44 cases of amniotic sheets were detected. The characteristics studied were site of amniotic sheet and whether the amniotic sheet was complete (i.e. no free edge seen on ultrasound) or incomplete (i.e. presence of free edge seen on ultrasound). The primary fetal outcome studied was stillbirth. RESULTS: The incidence of amniotic sheets was 0.14%. Two were complete and 42 were incomplete. Of the 38 cases with known outcomes there were two intrauterine deaths. There was no association between fetal outcome and the uterine location of the amniotic sheet (i.e. upper two-thirds vs. lower third, P = 0.5). There was, however, an association between the completeness of the amniotic sheets and intrauterine death (P = 0.002). Both instances of intrauterine death occurred in the two cases with complete amniotic sheets. Postmortem examination suggested that cord accidents were the cause of intrauterine death in both cases. CONCLUSIONS: This study supports the view that incomplete amniotic sheets are benign. It also suggests for the first time that complete amniotic sheets may be associated with intrauterine death. ( info) |