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11/14. Parasitic rachiopagus conjoined twins with herniation of the autosite spinal cord into the parasite: case report.

    A female newborn was found to have a rachiopagus parasite consisting of hypoplastic lower limbs attached to the dorsal thoracolumbar region; at surgery, when the infant was 30 days old, the autosite spinal cord was found to have herniated through a spina bifida into the parasite. ( info)

12/14. Sporadic Larsen syndrome in a preterm female originally diagnosed as positional deformities due to oligohydramnios.

    Few reports describe incidental prenatal diagnosis of sporadic Larsen syndrome by ultrasound, but none of these discuss coincidental oligohydramnios or an association with fetal growth restriction. A 28-year-old woman had prolonged rupture of membranes causing marked oligohydramnios at 32 weeks gestation in her first pregnancy. Labor was induced by vaginal prostaglandins and oxytocin infusion after dexamethasone administration because of concern about placental abruption. She delivered vaginally a live female infant weighing 1960 g (< 10th percentile) with multiple skeletal malformations and characteristic facies originally suspected as positional. Newborn x-rays, however, established the diagnosis of Larsen syndrome with bilateral dislocations of the hips, knees (genu recurvatum), and ankles (club foot). The neonatal karyotype was 46,XX. Parental examination and family history were unremarkable. The newborn did well with bilateral lower extremities cast. Postnatal diagnosis of Larsen syndrome should be suspected in neonates with multiple skeletal abnormalities following oligohydramnios. ( info)

13/14. Surgical treatment of scoliosis in larsen syndrome with bilateral hip dislocation.

    STUDY DESIGN: This is a report of a 12-year-old girl treated surgically for scoliosis associated with bilateral hip dislocation in Larsen syndrome. OBJECTIVE: To describe a rare case of scoliosis associated with Larsen syndrome and bilateral hip dislocation that was treated surgically with follow-up for 15 years. SUMMARY OF BACKGROUND DATA: There are few reports of the long-term follow-up of cases involving surgically treated scoliosis associated with bilaterally dislocated hips. methods: The patient's spine showed a right thoracic curve (T5-T12) with a Cobb angle of 77 degrees and did not show pelvic obliquity on an anterior-posterior radiograph film. On the sagittal alignment of her spine, the thoracic spine showed an abnormal lordosis (T5-T12: 19 degrees), and the lumbar spine had a hyperlordosis (L1-S1: 57 degrees) with a large lumbosacral angle (72 degrees ). We performed a posterior spinal fusion between T4 and L2 using Cotrel-Dubousset Instrumentation, anticipating the restoration of normal lumbar and cervical lordosis, as well as thoracic kyphosis. RESULTS: The Cobb angle of thoracic scoliosis improved from 77 degrees to 28 degrees, and a thoracic kyphosis of 12 degrees (T5-T12) was obtained. Subsequently, on the sagittal plane, the lumbosacral angle (sacral anteflexion) decreased from 72 degrees to 52 degrees, comparable to that of patients with hip dislocation, and the lumbar lordotic angle increased from 57 degrees to 66 degrees. The restoration of thoracic kyphosis resulted in an increase of lumbar lordosis and decrease of sacral anteflexion. At the 15-year follow-up,although the thoracic scoliosis (T5-T12) had increased to 36 degrees, good coronal and sagittal balance had been maintained. The patient is asymptomatic in her spine and hip. CONCLUSIONS: A case of scoliosis associated with dislocated hips in a patient with Larsen syndrome was successfully treated with posterior correction surgery. Fusion surgery between T4 and L2 provided an ideal sagittal balance of the total spine, while preserving 4 lumbar mobile segments. ( info)

14/14. Craniofacial characteristics evidenced in Bartsocas-Papas syndrome from birth to five years. Case report.

    Bartsocas-Papas syndrome (BPS) is a rare congenital disorder, first described in 1972. It is usually characterized by neonatal or intrauterine death, and, as such, only 26 cases have been reported. Physical manifestations such as antecubital and popliteal pterygia (webbing), syndactyly of fingers, toes and talipes are frequently reported. However, oral, facial, cranial and dental anomalies are often overlooked. This case focuses on the latter anomalies and the effect they have on the facial growth of a 5-year-old patient. ( info)
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