Fetal acalvaria with amniotic band syndrome.
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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. (+info)
Intrauterine sling: a complication of the stuck twin syndrome.
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Stuck twin syndrome usually presents with polyhydramnios in the recipient sac and severe oligohydramnios in the donor sac. The donor is displaced against the uterine wall and remains adherent in that position. We present a case in which the diagnosis was more complicated, owing to the suspension of the stuck twin by a sling within the sac of the recipient. A monochorionic diamnionic twin gestation was complicated by twin-twin transfusion syndrome at 18 weeks of gestation. In our example, the stuck twin was suspended by a sling from the placenta. The sling band represented the intertwin membrane that was folded upon itself. Amniotic fluid from the recipient twin was present in three dimensions around the stuck twin, except for the sling band. The suspension of the stuck twin by a sling within the amniotic fluid of the recipient is an unusual manifestation of the stuck twin syndrome. (+info)
Constriction band of the arm following insertion of a pleuro-amniotic shunt.
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In a 23-week fetus with a large left-sided pleural effusion a double pigtail pleuro-amniotic shunt was inserted uneventfully. A healthy infant was delivered at 38 weeks' gestation. One end of the shunt was in the thoracic cavity but the other end was found wrapped around the upper arm resulting in a constriction band. There was no impairment of the vascular supply to the limb or in its motor or sensory function and by 6 months of age there was only a faint ring still apparent around the upper arm. (+info)
Combined cuboid/cuneiform osteotomy for correction of residual adductus deformity in idiopathic and secondary club feet.
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We used a combined cuboid/cuneiform osteotomy to treat residual adductus deformity in idiopathic and secondary club feet. The mean follow-up for 27 feet (22 idiopathic, four arthrogrypotic and one related to amniotic band syndrome) was 5.0 years (2.0 to 9.8). All healed uneventfully except for one early wound infection. No further surgery was required in the 22 idiopathic club feet but four of five with secondary deformity needed further surgery. At follow-up all patients with idiopathic and two with secondary club feet were free from pain and satisfied with the result. In the idiopathic feet, adductus of the forefoot, as measured by the calcaneal second metatarsal angle, improved on average from 20.7 +/- 2.0 degrees to 8.9 +/- 1.8 degrees (p < 0.05). In four feet, with a follow-up of more than six years, there was complete recurrence of the deformity. In the secondary club feet, there was no improvement of the adductus. We conclude that in most, but not all, idiopathic club feet a cuboid/cuneiform osteotomy can provide satisfactory correction of adductus deformity. Those with secondary deformity require other procedures. (+info)
A malformed fetus in a rudimentary uterine horn pregnancy.
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We present a case of a 13-week pregnancy with a malformed fetus in a ruptured, non-communicating rudimentary horn. The patient, a 21-year-old woman with pelvic and right-sided abdominal pain, was admitted to the gynecology clinic of our institution. A ruptured rudimentary horn pregnancy was diagnosed by ultrasonography. The fetus in the gestation sac showed evisceration of the liver and intestines and an absent left femur. There was an amniotic band extending across the body of the fetus. The ruptured horn was excised by laparotomy. The factors associated with rudimentary horn pregnancy and related fetal abnormalities are discussed. (+info)
Spontaneous resolution of amniotic bands.
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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. (+info)
Prenatal sonographic diagnosis of focal musculoskeletal anomalies.
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Focal musculoskeletal anomalies vary, and can manifest as part of a syndrome or be accompanied by numerous other conditions such as genetic disorders, karyotype abnormalities, central nervous system anomalies and other skeletal anomalies. Isolated focal musculoskeletal anomaly does, however, also occur; its early prenatal diagnosis is important in deciding prenatal care, and also helps in counseling parents about the postnatal effects of numerous possible associated anomalies. We have encountered 50 cases involving focal musculoskeletal anomalies, including focal limb dysplasia [radial ray abnormality (n=3), mesomelic dysplasia (n=1)]; anomalies of the hand [polydactyly (n=8), syndactyly (n=3), ectrodactyly (n=1), clinodactyly (n=6), clenched hand (n=5)]; anomalies of the foot [clubfoot (n=10), rockerbottom foot (n=5), sandal gap deformity (n=1), curly toe (n=2)]; amniotic band syndrome (n=3); and anomalies of the focal spine [block vertebra (n=1), hemivertebra (n=1)]. Among these 50 cases, five [polydactyly (n=1), syndactyly (n=2) and curly toe (n=2)] were confirmed by postnatal physical evaluation, two (focal spine anomalies) were diagnosed after postnatal radiologic examination, and the remaining 43 were proven at autopsy. For each condition, we describe the prenatal sonographic findings, and include a brief review. (+info)
A case of amniotic band syndrome with bilateral epibulbar choristoma.
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An autopsy case of amniotic band syndrome with bilateral epibulbar choristoma is described. The left eye reveals a complex choristoma and the right eye a dermis-like choristoma. Both choristomatous lesions included lenticular tissue suggesting that rupture of the amnion, which is the initial event of amniotic band syndrome, might have occurred at about the fourth week of gestation. Since the other systemic manifestations of amniotic band syndrome are considered to be compression deformities of the fetus caused by oligohydramnios or amniotic band, the occurrence of epibulbar choristomas in both eyes in this case suggests that a compression mechanism may play a role in the pathogenesis of epibulbar choristoma. (+info)