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At least 595 records · Page 33Linked to original sources

Pelvic tilt in neuromuscular disorders.

The pelvic tilt is part of a complex deformity in which both the spine and the hip are involved. By viewing the different planes and analyzing the deforming forces, four different types of pelvic tilt in combination with spinal and hip deformities can be identified. In spina bifida patients congenital deformities of the spine may add to the progression of scoliosis. Analyzing our own patients we found that certain types of pelvic tilt can be related to certain neuromuscular diseases. Moreover our results show that the pelvic tilt can be effectively corrected by spinal surgery, whereas no effect of hip surgery could be demonstrated.

Cerebral Palsy↗

Urinary control after the definitive reconstruction of cloacal anomaly.

PURPOSE: Urinary control after definitive repair of a cloacal anomaly is difficult to achieve. The present report aims to describe the clinical course of urinary control, and the need for the management of bladder dysfunction after reconstruction. METHODS: The present consecutive series consisted of 11 girls who underwent definitive repair of cloacal anomalies over a period of 11 years. Eight patients were associated with hydrocolpos. Radiological examination included a plain X-ray radiograph of the lumbosacral spine and a voiding cystourethrography with or without a urodynamic study. RESULTS: Reconstruction of the cloaca was performed on patients aged between 1 and 3 years using a posterior sagittal approach. Vaginal reconstruction was carried out 13 times in 11 patients using tubularized vaginal flap, distal rectal segment, perineal skin flap, or total urogenital sinus mobilization. Cystostomy or vesicostomy was carried out in four newborns/infants. Another seven patients could void spontaneously but incompletely with residual urine. Occult spinal dysraphism was found in five patients and hemisacrum in two patients. After definitive reconstruction, most patients acquired an adequate to normal bladder volume for 1-year-olds. Normal detrusor-sphincter function was seen in three patients. Detrusor areflexia was seen in two patients who underwent in utero vesico-amniotic shunt. Detrusor underactivity was observed in six patients. Bladder compliance was good in all patients except for one. No patients in the present series showed persistent urinary incontinence from the bladder neck or urethral dysfunction. CONCLUSION: It is postulated that wetting after definitive repair may be the result of overflow incontinence and poor bladder contractility rather than sphincter injury. The main clinical characteristic of bladder dysfunction was a failure to empty. We could not define the exact etiology, but iatrogenic injury from extensive dissection can lead to the higher risks of peripheral nerve damage. Accomplishment of definitive repair involves not only anatomical reconstruction, but also postoperative urinary control, including the initiation of clean intermittent catheterizations under repeated urodynamic evaluations.

Child, Preschool↗

Tethered cord syndrome: an unusual cause of adult bladder dysfunction.

Tethered cord syndrome is a complication of spinal dysraphism. The tethering of the cord does not permit the normal cranial migration of the conus within the vertebral canal and this results in neural dysfunction due to a traction neuropathy. Although this condition commonly presents in childhood, less severe degrees of tethering may remain asymptomatic until adult life. The clinical features, imaging and management of 5 adults with this condition are reviewed. Both urological and neurosurgical intervention are aimed at preservation of function as, unlike the sensorimotor deficit that commonly complicates this condition, improvement in bladder function is uncommon following cord release.

Adolescent↗

Expanded PTFE bladder neck slings for incontinence in children: the long-term outcome.

OBJECTIVE: To assess the long-term outcome of circumferential expanded PTFE (Gore-tex, WL Gore Associates, Scotland) bladder neck slings for achieving urethral continence in children with a neuropathic bladder. PATIENTS AND METHODS: The records were reviewed of 19 children undergoing bladder reconstruction (most with a neuropathic bladder) who had a Gore-tex sling placed circumferentially at the bladder neck, over a 5-year period. Of these, seven had spina bifida; two each spinal dysraphism, surgery for anorectal anomalies and an idiopathic neuropathic bladder; five who developed a neuropathic bladder from other causes, and one born with bladder exstrophy. All children had an uncompliant bladder with a low urethral leak-point pressure on preoperative urodynamics. In all children conventional clean intermittent catheterization and pharmacotherapy had failed. Four had had previous augmentation surgery while 15 had concomitant bladder augmentation and formation of a Mitrofanoff stoma. The main outcome measure was achieving dryness. The original intention of the procedure was also to maintain urethral catheterization. RESULTS: Full details of the follow-up were available in 17 patients. Despite initial good short-term results, at a median follow up of 7 years, in 14 patients the sling had to be removed because of erosion, often with transient urethral leakage before the bladder neck subsequently closed. A bladder calculus was associated with each case of erosion except one. CONCLUSION: Although in the short term this technique had favourable results, it was not a useful technique in the long term.

Child↗

Diagnostic value of spinal US: comparative study with MR imaging in pediatric patients.

PURPOSE: To assess the diagnostic value of spinal ultrasonography (US) in pediatric patients. MATERIALS AND METHODS: The findings on 38 spinal US scans in 30 patients (mean age, 5.5 months) were compared with those of corresponding magnetic resonance (MR) images. RESULTS: MR imaging showed a normal spinal canal in 14 of 38 examinations, a congenital anomaly in 22, and a neoplasm in two. In 32 of 38 examinations, US allowed exactly the same diagnosis as MR imaging. In five examinations, US depicted the main abnormality, but MR imaging revealed additional findings. In one examination, no consensus was achieved. Whenever US scans were normal, MR images also did not depict any spinal disorder. In all 24 examinations with abnormal MR findings, US enabled detection of the abnormality. CONCLUSION: Spinal US seems to represent a valuable diagnostic tool for congenital anomalies of the lower spine in infants and is recommended as the primary imaging modality in those patients.

Child↗

Cervical cord tethering mimicking focal muscular atrophy.

Spinal cord tethering rarely occurs in the cervical region. In adults, it usually results from previous operations. However, congenital origin is always diagnosed and treated early in the infant period. We report a 12-year-old boy with cervical spinal dysraphism which was erroneously diagnosed as focal muscular atrophy, a benign form of motor neuron disease. The patient was brought to our hospital because of rapid deterioration of symptoms. Careful evaluation disclosed a hairy dimple at the nuchal area, which led to the correct diagnosis. X-ray of the cervical spine showed spina bifida from C(4) to C(6) levels and fusion of the laminae of C(4) and C(5). Spine MRI studies disclosed that the cervical cord was tethered caudally and dorsally, and the ventral nerve roots were markedly stretched, especially over the left side. Surgical intervention was undertaken and the patient's muscle power improved after untethering. The purpose of this report is to acquaint the reader with a surgically treatable condition that may appear to be benign focal amyotrophy. Skin lesion at the nuchal area should be carefully looked for.

Brachial Plexus↗

An unusual presentation of lumbosacral dermal sinus with CSF leak and meningitis. A case report and review of the literature.

Dermal sinus tracts are common skin manifestations seen with spinal dysraphism. Most of these dermal sinus tracts occur in the lumbosacral region. Though dermal sinus tracts are a common manifestation, their presentation with CSF leak is rare and important as they predispose to recurrent meningitis along with neurological deficits. Here we present an interesting case of a 33-month-old female child with dermal sinus CSF leak, tethered cord and intradural dermoid who presented with meningitis and was treated with excision of the tract, removal of the dermoid and detethering of the cord.

Cerebral Ventricles↗

Prenatal sonographic diagnosis of a rare Cantrell's pentalogy variant with associated open neural tube defect - a case report.

Cantrell's pentalogy is a very rare congenital anomaly. The hallmark of this condition is the presence of ectopia cordis and an abdominal wall defect. Its association with craniorachischisis is even more infrequent with just a few cases reported in the literature. The authors describe a case of a prenatal ultrasound diagnosis of Cantrell's pentalogy with posterior encephalocele and spinal dysraphism associated.

Abdominal Wall↗

Meningocoele manqué.

Meningocoele manqué is a congenital anomaly relating to the spinal cord, cauda equina and the filum terminale commonly found on exploration of the spinal cord in cases of spina bifida occulta. The anomalies consist of fibrous bands, aberrant nerve roots and adhesions within the theca which may tether or press on the neural structures so interfering with nerve conduction and causing increasing neurological deficit. Treatment is to free the neural structures from these bands and adhesions surgically. An account is given of 45 cases with a discussion of their similarity to cases of meningocoele, thus justifying the naming of a previously undescribed clinical entity. Meningocoele manqué demonstrates the relation of spina bifida occulta to spina bifida cystica as having a similar origin differing only in degree of severity. All the cases discussed are drawn from a personal series of 200 cases of spina bifida occulta which have had surgical exploration of the spinal cord.

Diastema↗