[Case of a large intra and extra medullary abscess of the spinal cord due to dermal sinus].

Congenital dermal sinuses, resulting from abnormal neurulation, are relatively uncommon and cause the patients to contract intraspinal infection. Sometimes, this intraspinal infection develops intramedurally abscess with severe neurological deficits. Therefore, a lack of awareness about this problem can result in fatal neurological sequelae. We report the case of a 1-year-old boy who had a lumbo-sacral dimple at birth without further evaluation. He presented repeated fever and rapidly progressive paraplegia. MRI showed a huge intraspinal and intramedullary abscess with dermoid, which was thought to be the result of a dermal sinus. Subsequently, he was transferred to our hospital and received immediate surgery. The patient underwent irrigation of purulent material in the intraspinal abscess including on intramedullary lesion, removal of dermoid tumor, and the resection of the dermal sinus. After that, he was treated with 8 weeks of antibiotic therapy. The patient remained paraplegia but made improvement. This patient illustrates the importance of the recognition and evaluation of skin markers. Prophylactic surgery is indicated to prevent dangerous and recurrent infections of the central nervous system.

Occult spinal dysraphism: evidence-based diagnosis and treatment.

This article reviews the scientific evidence behind the diagnostic tools available for the appropriate workup and management of patients with occult spinal dysraphism (OSD). The diagnostic tools include the use of detailed history and physical examination, plain films, ultrasound, MR imaging, and neurophysiologic tests. In addition, the article discusses the epidemiology of the most common causes of OSD in children, which will allow physicians caring for children to develop a pretest probability of disease and make a more educated decision as to when additional diagnostic testing is required.

Analysis of different treatment modalities of tethered cord syndrome.

INTRODUCTION: Although MRI has improved the diagnosis of tethered cord, many controversies still exist in the treatment of tethered cord syndrome (TCS). Especially the indications for and timing of surgical release have remained topics of discussion. MATERIALS AND METHODS: We retrospectively analysed a group of 41 spina bifida occulta (SBO) patients with a tethered cord to evaluate the results of treatment. Patients were divided into four groups. Twelve asymptomatic tethered cord patients underwent prophylactic surgery (group 1). Ten patients were operated upon because of progressive symptoms (group 2). In the third group 9 patients were treated conservatively at first, but underwent surgery after further progression of symptoms. The 10 patients in group 4 were treated conservatively. RESULTS: The course of symptoms was analysed in the separate groups. Patients in group 1 remained stable during the mean follow-up of 5.7 years. Neurological symptoms appeared to improve most after early surgery (group 2), but progression of symptoms continued in spite of surgical release.

The tethered spinal cord: diagnosis, significance, and management.

Tethered cord is the result of fixation of the spinal cord, which limits motion in the caudal-rostral direction. Most children either have cutaneous markers or a predisposing condition. Prophylactic release of the spinal cord can prevent the long-term disabilities associated with this condition. Although delayed release, following the onset of a neurological deficit, may reverse some lost function, it is unlikely to restore bladder and bowel function.

Successful prenatal therapy of thoracic lesions.

Improvements in prenatal imaging techniques now permit in utero treatment of potentially serious lesions. We describe three cases in which decompression of significant space-occupying thoracic lesions resulted in an excellent clinical outcome. The pregnancies were all carried to term after second or early third trimester intervention and resulted in infants with minimal respiratory distress. Cytologic and chemical analysis of the thoracic fluid aspirated in utero provided diagnostic and physiologic information.

[Closed spinal dysraphism]. / Dysraphisme spinal fermé.

This retrospective review included 133 patients with one or several of the following defects: diastematomyelia, neurenteric cyst, dermal sinus or cyst, meningeal malformation, sacral agenesis, tethering of the spinal cord, and lumbosacral lipoma. Physical evaluation readily identified most lumbosacral lipomas, as well as most cases of diastematomyelia since hypertrichosis over the defect was common. Some patients developed life-threatening clinical manifestations, e.g., meningitis due to a dermal sinus. On the basis of this retrospective review and a review of the literature, the clinical and therapeutic aspects of each defect are discussed.

Percutaneous catheter placement for cyst drainage in the subarachnoid space.

A technique for percutaneous catheter drainage of cystic masses in the subarachnoid space was developed and shown to be safe and effective in an 18-year-old boy with life-threatening, recurrent neuroenteric cysts that compressed the brain stem, cerebellum, and spinal cord. Percutaneous drainage through a C1-C2 approach was performed on 11 separate occasions. Decompression was always accomplished and no infection or other complication occurred, even with continuous catheter drainage for 9 months. This technique provides opportunities for interventional neuroradiologic therapy in the subarachnoid space. It appears to be suited for percutaneous drainage of intraspinal and intracranial cysts when surgery is not indicated due to intractability or inaccessibility.

Diagnosis and management of the tethered cord syndrome.

Tethering of the spinal cord can occur from a thickened filum terminale, scarring due to myelomeningocele repair, lipomas or diastematomyelia. The typical presentation is a lumbar cutaneous defect, enuresis, unexplained back or leg pain, foot deformities, leg length discrepancies or scoliosis. Spinal ultrasound in neonates and MRI in older children offers ready diagnosis. With laser and microneurosurgical techniques, repair of these defects is safe and effective. The authors recommend that any infant having a mid-line lumbar cutaneous abnormality, such as a hemangioma, lipoma, hair patch or dimple, be evaluated to rule out tethering of the spinal cord.

Cervical spine diastematomyelia in adulthood.

Adult cervical diastematomyelia is a rare malformation and is usually associated with posterior element anomalies. A case of isolated cervical diastematomyelia, which was initially thought to be a herniated cervical disc, is described. Diagnosis and surgical management are discussed.

Diagnosis and management of sacral agenesis.

Herein, the diagnosis and management of 14 cases with sacral agenesis experienced in the last 15 years were reviewed. Age at first presentation tended to be older than that of meningomyelocele patients. All the children displayed partial sacral agenesis or hemisacrum. Occult spinal dysraphism was found in 8. Frequent association of genitourinary and anorectal anomalies required important alterations in the management. Vesicoureteral reflux was found in 8 patients but most of them were treated conservatively. Thirteen children underwent urodynamic evaluation on more than 1 occasion and 12 displayed neurogenic bladder dysfunction (upper motor type 6, lower motor type 5, upper and lower type 1). However, the diagnosis of bladder dysfunction was not final. Clean intermittent catheterization was most frequently assigned but it was difficult in some cases with urethral abnormalities. Overall, 5 children required surgical intervention for their urinary tract, 4 of whom needed multiple procedures. As a result, half the children achieved continence and 1 child, who awaited reconstructive surgery, had slightly elevated serum creatinine. In summary, early diagnosis, complete neurourological evaluation and adequate management with careful follow-up monitoring risk factors to the upper urinary tract are mandatory.

Conservative versus neurosurgical treatment of tethered cord patients.

We compared the outcome of neurosurgical release of a tethered spinal cord in 20 children with the neurological evolution of 21 other children known iwth a tethered spinal cord on nuclear magnetic resonance scan (NMR). Neurosurgery yielded stabilisation of the symptoms without any lasting improvement. Pre-operative conservative medical treatment had to be continued in all operated children. Three of the 20 operated children are showing signs of retethering despite appropriate neurosurgery, indicating that retethering should be considered as a major and frequent complication. In the majority of our children, symptomatic (re)tethering was recognised by the appearance of increased tendon reflexes and a progressive pes cavus, suggestive of an upper motor neuron disease involvement.

Multiple congenital anomalies. An approach to management.

The multiplicity of problems associated with spina bifida and CHARGE syndrome illustrate a management approach for any child with multiple congenital anomalies. Specific etiology should be established whenever possible to accurately predict prognosis, complications, and recurrence risks. A systematic evaluation of individual medical, psychosocial, and developmental issues is necessary to rank priorities within these areas. A management team of specialists to deal with these priorities can then be assembled, and good communication among team members is essential.

The management of children with spinal dysraphism.

Improvements in technology have dramatically increased the survival of children with spinal dysraphism. Because this complex condition affects multiple organ systems as well as the psychosocial functioning of the child and family, these children require care from a host of specialists in order to achieve optimum functioning. This article reviews the pathophysiology and discusses the current management of the medical and psychosocial effects of spinal dysraphism. It also briefly discusses strategies for intervention, long-term outcomes, and some controversies regarding care and screening.