1/23. Fetal acalvaria with amniotic band syndrome.A case of amniotic band syndrome (ABS) presenting with acalvaria is reported. ABS includes a spectrum of non-genetic anomalies, varying from simple digital band constriction to major craniofacial and visceral defects, and even fetal death. Acalvaria is a rare congenital malformation characterised by the absence of the dome-like superior portion of the cranium comprising the frontal, parietal, and occipital bones and dura mater, in the presence of a normal skull base and facial bones with complete cranial contents. No two cases are the same. Acrania or absence of the flat skull bones with disorganised cerebral hemispheres have been reported in the presence of amniotic bands. ABS is an aetiological factor in acalvaria. Appropriate counselling for affected families needs to be given after prenatal diagnosis.- - - - - - - - - - ranking = 1keywords = craniofacial (Clic here for more details about this article) |
2/23. spinal cord tethering associated with amniotic band syndrome.amniotic band syndrome (ABS) comprises fetal morphological abnormalities that may be associated with fibrous amniotic bands that damage developing fetal parts resulting in cutaneous scars, erosions and ulcerations, digital constricting bands, craniofacial and visceral anomalies. Multiple asymmetric encephaloceles and anencephaly are neural-tube-like defects previously reported with ABS. This is the first report of spinal dysraphism with dorsal spinal cord tethering associated with ABS. We examine the pathogenetic theories of ABS in light of this report.- - - - - - - - - - ranking = 1keywords = craniofacial (Clic here for more details about this article) |
3/23. Case of complex craniofacial anomalies, bilateral nasal proboscides, palatal pituitary, upper limbs reduction, and amnion rupture sequence: disorganization phenotype?We report a case of a dizygotic twin with complex abnormalities of head, body, and limbs. The anomalies include the following: lateral and midline cleft upper lip, ectopic palatal pituitary, natal teeth, bilateral nasal proboscides with an absent nose, left microphthalmia with conjunctival-lined cyst, right ocular dysgenesis, bilateral retinal dysplasia, platybasia with skull asymmetry, hydrocephalus secondary to aqueductal atresia, brain hemispheric asymmetry with a parietal-occipital cortical flap, agenesis of posterior corpus callosum, absence of the olfactory nerves and left anterior cerebral artery, leptomeningeal and intraventricular heterotopias, right radial longitudinal terminal meromelia with constriction rings of fingers, partial syndactyly of the third and fourth left fingers, dorsiflexed great toes and pes equinovarus bilaterally, and multiple skin tags with a sacral appendage. Additionally, this twin's placental disc and extraplacental membranes were devoid of amnion. We regard these anomalies as a possible expression of the human homologue of the disorganization phenotype or another gene mutation. Nevertheless, an abnormality of blastogenesis with early damage to organizing tissues of the frontonasal region and limbs, or a vascular disruption, cannot be excluded. Early amnion rupture sequence (possible extraamniotic pregnancy with amniotic bands, limb reduction defects with Streeter bands, and multiple skin tags tapering into amniotic bands) was also present in this case, and may have acted as a contributing factor.- - - - - - - - - - ranking = 4keywords = craniofacial (Clic here for more details about this article) |
4/23. Spontaneous resolution of amniotic bands.The amniotic band syndrome comprises a group of sporadic congenital anomalies characterized by amputations, constriction bands, pseudosyndactylism and multiple craniofacial, visceral and body wall defects. It occurs in approximately 1 in 1200 to 1 in 15 000 live births. Some of the cases present with congenital anomalies that are incompatible with life, but a selected group shows isolated limb constrictions. Repeated sonographic examinations afforded us the opportunity of observing the in utero process of limb strangulation and subsequent spontaneous lysis of an amniotic band attached to a fetal elbow. Surgical fetal therapy with lysis of the constriction ring in utero is an option, but intervention is probably not always warranted. Spontaneous resolution does occur.- - - - - - - - - - ranking = 1keywords = craniofacial (Clic here for more details about this article) |
5/23. amniotic band syndrome with significant orofacial clefts and disruptions and distortions of craniofacial structures.amniotic band syndrome (ABS) is a rare disorder in which bands of mesoderm that emanate from the chorionic side of the amnion and insert on the fetal body can generate a wide variety of disfiguring and disabling malformations. It usually is sporadic, and the incidence is approximately 1 in 15,000 live births, and affected children typically require involvement of several pediatric surgical subspecialties. The authors describe a case of ABS with extensive craniofacial anomalies.- - - - - - - - - - ranking = 5keywords = craniofacial (Clic here for more details about this article) |
6/23. Typical facial clefting and constriction band anomalies: an unusual association in three unrelated patients.The amniotic band sequence is a rare congenital condition in which it is hypothesized that fibrous band of amniotic tissue result in variable soft tissue derangements. Other terms that have been used to describe this condition are amniotic band syndrome and amnion rupture sequence. patients may present with craniofacial, thoracic, abdominal, and/or limb involvement. Previous reports have described the association between the amniotic band sequence and unusual patterns of facial clefting, as described by Tessier, that do not follow normal embryonic lines of fusion [Keller et al., 1978; Bagatin et al., 1997]. We describe three successive cases of the amniotic band sequence, which presented with rather typical facial clefts (cleft lip and palate) in unrelated patients.- - - - - - - - - - ranking = 1keywords = craniofacial (Clic here for more details about this article) |
7/23. 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.- - - - - - - - - - ranking = 1keywords = craniofacial (Clic here for more details about this article) |
8/23. 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.- - - - - - - - - - ranking = 1keywords = craniofacial (Clic here for more details about this article) |
9/23. Endoscopic release of limb constriction rings in utero.amniotic band syndrome is a sporadic condition that may result in constriction bands, amputation and multiple craniofacial, visceral and body wall defects. It occurs in 1/1,200 to 1/15,000 live births. Most cases present with multiple congenital anomalies that are incompatible with life. A small group of fetuses shows isolated limb constrictions that may cause severe limb dysfunction or limb amputation if left untreated. Successful in utero surgical lyses of constriction rings have been reported. We report a case of constriction amniotic bands involving both legs and compromising blood flow to the distal extremity. The constriction ring was successfully released by a minimally invasive endoscopic surgical technique avoiding severe limb dysfunction or foot amputation.- - - - - - - - - - ranking = 1keywords = craniofacial (Clic here for more details about this article) |
10/23. Congenital placental-cerebral adhesion: an unusual case of amniotic band sequence. Case report.Amniotic band sequence is a disruption sequence having a broad spectrum of clinical manifestations ranging from partial amputations to major craniofacial and limb-body wall defects. Most reported cases of placental-cerebral adhesion pertain to patients with severe craniofacial defects who were either stillborn or who died a few hours after birth. The authors present a case of a male infant born with a placental-cerebral adhesion through a cranial defect. This adhesion was separated at birth, and duraplasty and primary scalp closure were performed. A detailed examination of the placenta revealed the presence of multiple amniotic bands. The case demonstrates that survival and normal early post-natal development are possible if the condition is treated promptly.- - - - - - - - - - ranking = 2keywords = craniofacial (Clic here for more details about this article) |
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