1/227. Bilateral femoral neck fractures secondary to hypocalcemic seizures in a skeletally immature patient. We report the case of an 11-year-old, skeletally immature boy who sustained bilateral femoral neck fractures during seizure activity. These fractures were due to hypocalcemia. Although this condition is a rare complication of seizures, its early recognition and treatment are essential to minimize complications associated with the delayed treatment of intracapsular hip fractures. ( info) |
2/227. eating disorders in adolescents and young women with spina bifida. This is the first report of the association of spina bifida and eating disorders. Five patients were diagnosed rather late in the course of their illness. They all had been overweight premorbidly and had been urged to lose weight for years in order to improve their mobility. As they dieted, they experienced their weight loss as a source of power that could somehow compensate for their losses and neurologic limitations. They responded to a multidisciplinary intervention. Clinicians taking care of patients with spina bifida need to be cognizant that they may be at risk of developing an eating disorder. Such awareness should influence the quality of nutritional counseling (prevention aspect) and the clinical assessment of sudden weight loss (early intervention). ( info) |
3/227. Labor analgesia with paravertebral lumbar sympathetic block. BACKGROUND AND OBJECTIVES: Provision of labor analgesia continues to be a challenge for parturients with spine pathology or history of back surgery. methods: We report on the use of paravertebral lumbar sympathetic block for first stage labor analgesia in two parturients with spine pathology. ( info) |
4/227. Neuropathic arthropathy: a forgotten diagnosis? Two recent cases involving the hip. Two cases of neuropathic arthropathy of the hip are reported. One was the first manifestation of tabes dorsalis in a 74-year-old man, whereas the second occurred in a 47-year-old woman with a history of spina bifida and L2-L5 epidural lipoma. Radiographic joint destruction occurred within five and three months, respectively. The main clinical and radiological features of neuropathic arthropathy are reviewed, and diagnostic pitfalls are discussed. There are no specific laboratory tests or histologic findings. This now rare condition should be routinely considered in patients with severe joint destruction contrasting with minimal pain. The reasons for the decision to use trochanteric-iliac coaptation in one of our patients and a wait-and-see approach in the other are explained. The literature is reviewed. Ten cases treated by joint replacement have been reported. However, neither joint replacement nor arthrodesis seem capable of restoring satisfactory hip function. ( info) |
5/227. Extrarenal nephroblastic proliferation in spinal dysraphism. A report of 4 cases. Four cases of extrarenal nephrogenic proliferation in the sacrococcygeal region with spinal dysraphism are presented. In two of the cases, features of Wilm's or incipient Wilm's tumor were present. The previous literature on sacrococcygeal nephrogenic tissue is reviewed, and the impact of these findings on the histogenesis of extrarenal sacrococcygeal Wilm's tumor is discussed. ( info) |
6/227. An unusual case of dorsally situated bony spur in a lumbar split cord malformation. A case of lumbar split cord malformation (SCM) with a bony spur situated dorsally is presented. This was associated with a hypertrophied posterior arch. The ventral dura was totally intact, and there was no fibrous septum connecting the bony arch to the dura. To our knowledge, such a case has not been reported earlier. In view of this unique finding, we propose a slight modification in Pang's unified theory of embryogenesis in the development of SCM. ( info) |
7/227. Myelocystocele-cloacal exstrophy in a pedigree with a mitochondrial 12S rRNA mutation, aminoglycoside-induced deafness, pigmentary disturbances, and spinal anomalies. A large Filipino-American family with progressive matrilineal hearing loss, premature graying, depigmented patches, and digital anomalies was ascertained through a survey of a spina bifida clinic for neural crest disorders. deafness followed a matrilineal pattern of inheritance and was associated with the A1555G mutation in the 12S rRNA gene (MTRNR1) in affected individuals as well as unaffected maternal relatives. Several other malformations were found in carriers of the mutation. The proband had a myelocystocele, Arnold-Chiari type I malformation, cloacal exstrophy, and severe early-onset hearing loss. Several family members had premature graying, white forelock, congenital leukoderma with or without telecanthus, somewhat suggestive of a waardenburg syndrome variant. In addition to the patient with myelocystocele, two individuals had scoliosis and one had segmentation defects of spinal vertebrae. The syndromic characteristics reported here are novel for the mitochondrial A1555G substitution, and may result from dysfunction of mitochondrial genes during early development. However, the mitochondrial A1555G mutation is only rarely associated with neural tube defects as it was not found in a screen of 218 additional individuals with spina bifida, four of whom had congenital hearing loss. ( info) |
8/227. Congenital malformations after intracytoplasmic injection of spermatids. Spermatid microinjection into oocytes was applied in cases of intracytoplasmic sperm injection (ICSI)/testicular sperm extraction (TESE) where no spermatozoa could be found in numerous testicular samples. Although several pregnancies were obtained with this procedure, serious concerns remain regarding its safety. Although the relevance of the injection of spermatids is by no means certain, we wish to report that from four pregnancies obtained after injection of elongated spermatids, two cases of major malformation resulted. ( info) |
9/227. Bladder cancer arising in a spina bifida patient. We report the case of a 52-year-old patient with spina bifida, neurologic bladder, and a history of recurrent urinary tract infections (UTIs) in whom a bladder cancer was incidentally discovered. Cytology, cystoscopy, and cystography showed nonspecific, extensive inflammatory lesions. Cystography demonstrated a complex of diverticulae and cellules. Pathologic examination of a diverticulectomy specimen revealed a grade III pT3b transitional and squamous cell carcinoma. Because of the similar disease causation (recurrent UTIs, stones, and indwelling catheterization), we suggest extension of the guidelines proposed for patients with spinal cord injuries (ie, annual serial bladder biopsies) to patients with nontraumatic neurogenic bladder. ( info) |
10/227. Intradural spinal lipoma of the conus medullaris without spinal dysraphism. A 42-year-old man suffering from progressive left radicular sensory motor loss (L4 level) underwent neurosurgical repair. neuroimaging (RMI) had led to the diagnosis of schwannoma of the filum terminale with lipomatous component. Histological examination visualized a true mature lipoma associated with numerous bundles of more or less dystrophic nerve fibers. This histological benign tumor raised the problem of the genesis of intradural lipomas of spinal cord. ( info) |