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PubMed2001

Thoracocervical dorsal dermal sinus associated with multiple vertebral body anomalies.

Congenital dermal sinus is a type of closed spinal dysraphism. Cervical and thoracic regions are the rare sites for dorsal dermal sinuses. Dermal sinuses are frequently associated with dermoid or epidermoid tumor and osseous abnormalities such as bifid spinous process. The association of dorsal dermal sinuses with vertebral body anomalies is very rare. We present MR imaging features of a case of thoracocervical dorsal dermal sinus associated with multiple vertebral body anomalies.

Adult↗
PubMed2001

Congenital midline cleft of the posterior arch of atlas: a rare cause of symptomatic cervical canal stenosis.

Developmental symptomatic C1 canal stenosis is very rare. We describe the computed tomography (CT) and magnetic resonance imaging (MRI) appearances in a 8-year-old child who presented with progressive upper and lower limb neurological symptoms and in whom imaging revealed the medial posterior hemiarches of a bifid C1 to be inturned and compressing the cervical cord. This particular configuration of the posterior arch of atlas is frequently associated with other craniocervical bony anomalies and presents with neurological symptoms early in life. Early CT or MRI examination of patients with symptomatic posterior arch of C1 defects is necessary, in order to detect such an appearance, since surgical treatment may prevent neurological deterioration.

Cervical Atlas↗
PubMed2001

Paediatric ultrasound. II. Other applications.

This article focuses on small-parts US in children. The differential diagnosis of cystic neck masses primarily includes lymphangiomas, thyroglossal duct cysts and branchial cleft cysts. Solid masses may be represented from lymphadenopathies, cervical extensions of mediastinal thymus, fibromatosis colli, rhabdomyosarcomas and neuroblastomas. Salivary gland lesions are uncommon in children. Thyroid is best evaluated by US and nuclear scintigraphy. If US shows abnormal thyroid gland, the isotopic scan may be a good complementary method to confirm the diagnosis. Normal parathyroid glands are not visualised routinely by US because of their small size. Parathyroid adenomas are unusual in paediatrics. Ultrasound can be successfully used in the differential diagnosis of the painful scrotum especially with colour flow Doppler. The hallmark of ischaemia is a completely avascular testis. In the paediatric age group, the most common application of US to the musculoskeletal system is the evaluation of the infant hip in the first 6 months of life; however, the refinement of new transducers has further improved the ability of US equipment to evaluate a variety of other musculoskeletal disorders in children, involving tendons, muscles, nerves as well as soft tissue masses. Ultrasound can be a useful screening tool in newborns suspected of having closed spinal dysraphism.

Child↗
PubMed2005

Unusual spinal teratoma with an accessory penis on the back.

INTRODUCTION: Spinal teratomas are extremely rare tumours, and their association with split cord malformation is even rarer. CASE REPORT: This is a case report of an infant with a large teratoma with a lipomyelomeningocele along with a split cord malformation and with an unusual accessory penis and scrotum on the back over the swelling. This, to our knowledge, is the first such case reported in the English literature.

Choristoma↗
PubMed1997

Embryogenesis. Why do we need a new explanation for the emergence of spina bifida with lipoma?

Spina bifida with lipoma is a human malformation that most often affects the lumbosacral area. It is a complex morphological type. Its origin is controversial, and none of the previous hypotheses can be retained in view of the most recent advances in experimental embryogenesis. Contrary to earlier opinions, adipocytes cannot arise from meninges, vessels or glial cells and spina bifida cannot be explained by incarceration of mesodermal tissues during primary neurulation or developmental defect at the level of the tail bud. Spina bifida with lipoma, which actually involves all the derivatives of the so-called dorsal mesoderm, must therefore result from abnormal development of this mesoderm, which is induced by the dorsal neural tube. The location of the primary defect (dorsal mesoderm or neural tube) remains to be established.

Embryonic Induction↗
PubMed1999

Comparison between pediatric and adult neurosurgery: management and future perspectives. Tethered cord syndrome, hydrocephalus, craniosynostosis.

With creation of the many subdivisions within the field of neurosurgery, neurosurgeons have taken on different roles. It is important for neurosurgeons to understand all the different subjects involved to enable them to build up long-term goals in patient management and research. As pediatric neurosurgeons, the authors give accounts of three characteristic diseases encountered in pediatric patients: spinal dysraphism with tethered cord syndrome, hydrocephalus, and craniosynostosis. Drawing on their personal experience of tethered cord syndrome, the authors stress the pathophysiological mechanisms, clinical characteristics and proper management of the pediatric disease. Because pediatric neurosurgeons cover the whole range of neurosurgical diseases of infancy and childhood, the authors emphasize that their actions can be the basis for the management of general neurosurgical patients. A comparison between pediatric and adult neurosurgery is also drawn, with special reference to the interaction between pediatric and adult neurosurgeons.

Adult↗
PubMed2004

Spinal fusion in children with spina bifida: influence on ambulation level and functional abilities.

The aim of this study was to determine the influence of spinal fusion on ambulation and functional abilities in children with spina bifida for whom early mobilization was stimulated. Ten children (three males and seven females) with myelomeningocele were prospectively followed. Their mean age at operation was 9.3 years (standard deviation (SD): 2.4). Spinal curvature was measured according to Cobb. Pelvic obliquity and trunk decompensation were measured as well. The ambulation level was scored according to Hoffer, and functional abilities, as well as the amount of caregiver assistance, were documented using the Pediatric Evaluation of Disability Inventory. All patients were assessed before surgery and three times after surgery, with a total follow-up duration of 18 months after surgery. After spinal fusion, magnitude of primary curvature decreased significantly (p=0.002). Pelvic obliquity and trunk decompensation did not change. In spite of less immobilization as compared with other reported experiences, ambulation became difficult in three out of four patients who had been able to ambulate prior to surgery. Functional abilities and amount of caregiver assistance concerning self-care (especially regarding dressing upper and lower body, and self-catheterization) and mobility (especially regarding transfers) showed a nonsignificant trend to deterioration within the first 6 months after surgery, but recovered afterwards. From pre-surgery to 18 months after surgery, functional skills on self-care showed borderline improvement (p=0.07), whereas mobility did not (p=0.2). Mean scores on caregiver assistance improved significantly on self-care (p=0.03), and borderline on mobility (p=0.06), meaning that less caregiver assistance was needed compared with pre-surgery. The complication rate was high (80%). In conclusion, within the first 6 months after spinal fusion, more caregiver assistance is needed in self-care and mobility. It takes about 12 months to recover to pre-surgery level, while small improvement is seen afterwards. After spinal fusion, ambulation often becomes difficult, especially in exercise walkers. These findings are important for health-care professionals, in order to inform and prepare the patients and their parents properly for a planned spinal fusion.

Activities of Daily Living↗
PubMed2000

Tethered cord syndrome in adults.

BACKGROUND: The tethered cord syndrome (TCS) consists of an abnormally low conus medullaris tethered by a thickened filum terminale or various forms of spinal dysraphism. The adult variant of the syndrome seems not to be as rare as once thought. METHOD: This study includes 11 patients with a TCS. Clinical, radiological findings and outcome were reviewed in these adult patients with TCS. FINDINGS: The patients ranged in age from 18 to 34 years (mean 24.09 years). There were 7 men and 4 women. The presenting symptoms in order of frequency were as follows: low back pain radiating to legs, urinary complaints, weakness in legs and impotence. All patients had magnetic resonance imaging scans. Physical exercise in 8, birth delivery in 1 and carrying heavy objects in 1 patient were determined as the precipitating factors. No precipitating factor could be defined in one of the patients. All patients were operated on, except for one. None of the patients worsened after surgery. INTERPRETATION: The patients presenting with low back pain and sciatica responded to surgery better than those with sphincter problems.

Adolescent↗
PubMed2002

[Congenital anomalies of the spine].

Congenital anomalies of the spine usually originate in (toxic) disturbances during pregnancy. There are occasional hereditary types. These are characterized by the presence of multiple anomalies. Congenital scolioses are often associated with other anomalies like spinal dysraphy (20-30%) and fusion of the ribs. Furthermore heart defects, Sprengel's deformity, cleft palates, hemimelias, clubfeet or congenital vertical talus are frequently associated with congenital scoliosis. We classify in failures of formation, segmentation and combined types. The type of malformation determines the prognosis. One hemivertebra is associated with a risk of progression of 0-2 degrees/year, 2 ipsilateral hemivertebra with 2-3 degrees/year, a unilateral unsegmented bar with 5 degrees/year and a combination of a unilateral unsegmented bar with a contralateral hemivertebra with approximately 10 degrees/year. Conservative treatment is usually ineffective. The indication for operative treatment should be made before the deformity becomes unacceptable, as a correction is difficult and dangerous. The following operations can be carried out: Anterior and/or posterior fusion with or without instrumentation, epiphyseodesis, (hemi)vertebrectomies (with anterior and/or posterior approach), distraction with a halo, osteotomies, separation of the fused ribs and gradual distraction (Campbell's expansion thoracoplasty). The operations carry significant risks and the indication to the various treatment modalities is difficult. Operations should be made under monitoring of sensory and motor evoked potentials. From 498 patients with congenital anomalies of the spine in our observation we have operated on 143. Hemivertebrectomies were the most frequent interventions (56).

Abnormalities, Multiple↗
PubMed1994

Neuroblastoma and lumbar hernia: a causal relationship?

Lumbar hernia is a rare occurrence in infants and children. Congenital variants have been described, frequently associated with musculofascial and skeletal abnormalities, specifically "lumbocostovertebral syndrome" (LCV) and/or meningomyelocele. In LCV syndrome, lumbar herniation results from a single somatic defect occurring during the third to fifth week of embryonal development. Meningomyelocele may predispose to lumbar herniation secondary to abnormalities in muscular innervation related to nerve entrapment in the spinal dysraphism. Acquired lumbar hernia generally can be attributed to surgery, infection, or trauma. Localized neuropraxis, temporary or permanent, may be the underlying factor common to all these defects. The author presents two pediatric cases of lumbar hernia associated with intrathoracic neuroblastoma. The first patient, a 15 month old, had a lumbar hernia after excision of a thoracic ganglioneuroblastoma. This resolved within 1 year, without specific therapy. The second patient, a 4 month old with a lumbar hernia and a large intrathoracic neuroblastoma, had "resolution" of the hernia within 4 months of excision of the paravertebral tumor. In these cases it appears that the lumbar hernia resulted from neuropraxis secondary to intrathoracic paravertebral tumor and its management. In both cases, this deficiency was temporary and resolved without specific therapy. The association of lumbar hernia and intrathoracic neuroblastoma has not been reported previously. These cases suggest the advisability of a screening chest x-ray in children presenting with this diagnosis. Similarly, the cases suggest a role for conservative treatment for the hernia itself, when the neural impairment resulting in the defect is of a temporary nature.

Female↗
PubMed1972

Gas myelography.

Explore the source record for details and available documents.

Cervical Vertebrae↗
PubMed1995

[Percutaneous vertebroplasty].

Vertebroplasty is a new therapeutic method which by way of filling with acrylic cement in the vertebral body gives a stabilization of the vertebra and an antalgic effect in painful lesions involving the spine. Main indications consist of spine angiomas, metastases and osteoporotic fractures involving the vertebral body. In most patients, vertebroplasty gives a very good and durable antalgic effect as in cases of angiomas or metastases as in post-therapeutic sequellae in malignant hematosarcomas. In osteoporotic fractures, antalgic effect is obtained very rapidly, but the follow-up is actually insufficient to evaluate the long term benefit.

Hemangioma↗
PubMed1991

Prenatal diagnosis of fetal diastematomyelia in a pregnancy exposed to acyclovir.

A rare form of spinal dysraphism, diastematomyelia, was diagnosed in a pregnancy exposed to acyclovir at the time of implantation. The prenatal diagnosis was based on the ultrasound examination of the fetus. Pathologic evaluation of the postabortion fetus confirmed the diagnosis. The use of acyclovir in pregnancy and associated anomalies are reviewed.

Acyclovir↗
PubMed2007

Pediatric urinary incontinence: evaluation of bladder wall wraparound sling procedure.

PURPOSE: In 1996 a surgical procedure for sphincter deficiency was reported in patients with neurogenic urinary incontinence. To our knowledge we report the first series that assesses this procedure since it was initially described. MATERIALS AND METHODS: Seven female and 7 male patients with spinal dysraphism and neurogenic intrinsic sphincter deficiency underwent surgery between 1997 and 2005. All patients had an associated poorly compliant or small capacity bladder. Patient age ranged from 8 to 22 years (mean 14) and all patients presented with persistent urinary incontinence despite intermittent catheterization and pharmacotherapy. Evaluation was based on clinical examination, as well as preoperative and postoperative urodynamic assessments. The surgical procedure involved wrapping a pedicle strip of anterior bladder wall around the bladder neck and fixing it on the pubic symphysis. Bladder augmentation cystoplasty was also routinely performed during the same procedure. RESULTS: Of 14 patients 13 were completely dry and 1 had stress incontinence. Followup was 2 to 8 years (mean 5). Postoperative urodynamic evaluation showed a maximum urethral pressure increase of 40%. We also observed an increase in bladder capacity of 105% and improved bladder compliance (7 ml/cm H(2)O preoperatively vs 35 ml/cm H(2)O postoperatively). CONCLUSIONS: The bladder wall wraparound sling procedure provides excellent results for continence in association with bladder augmentation.

Adolescent↗