No abstract available.
Arnold-Chiari Malformation* ; Meningomyelocele*

A myelomeningocele with spina bifida in the thoracic region is a relatively rare, congenital anomaly. This case, which we have experienced recently, is reported with a review of the literatures.
Meningomyelocele* ; Spinal Dysraphism

The authors report a case of thoracic meningomyelocele. The patient had not have any presenting symptoms until adult age. We confirmed it with radiological(postmyelography CT and MRI) and pathological diagnosis. Pre-, post- and intraoperative SSEP(somatosensory evoked potential) monitoring were performed. Characteristically, the mass contained accessory cord which attached to inner cyst wall with multiple fibrous bands. Meningomyelocele was completely removed without any neurologic sequelae.
Adult ; Diagnosis ; Humans ; Meningomyelocele*

No abstract available.
Biofeedback, Psychology* ; Humans ; Meningomyelocele*

The Currarino triad is a unique complex of congenital anomalies including anorectal malformation, scral bonyabnormality, and presacral mass. The usual symptomatology is constipation due to anorectal stenosis. Threepatients with this triad confirmed by surgical operation during recent two yeas in Seoul National Universitychildren's Hospital were presented with a brief review of clinical features, unique radiologic appearance andpostulated pathophysiology. The presacral mass was a mature teratoma in the first patient, alipomyelomeningocele-epidermoid inclusion cyst in the second patient, and myelomeningocele in the third patient. Acorrect diagnosis of the Currarino triad by radiologists is important, and may help the phsicians to investigatedetailed family history and associated anomalies and to establish adequate surgical plans.
Constipation ; Constriction, Pathologic ; Diagnosis ; Humans ; Meningomyelocele ; Seoul ; Teratoma

PURPOSE: As problem have developed with the right colonic antegrade enema procedure (Malone's procedure/Monti's retubularized ileocolostomy), left colonic antegrade continence enema (LACE) procedure, in which retubularized ileum or sigmoid colon is anastomosed into the sigmoid colon, has gained popularity. The aim of the study was to describe our experience with the LACE procedure. METHODS: We retrospectively reviewed 19 LACE procedures that were performed at the Yonsei University Colllege of Medicine Hospital (Seoul, Korea) from March 2001 to March 2005. RESULTS: Male-to-female ratio was 11 : 8, with median age of 10 years (range, 3~34 years). Most common diagnosis was meningomyelocele (78.9%, 15/19). The median total follow-up period was 23 months (range, 3~37 months); median antegrade continence enema volume used was 600 ml (range, 250~1,500 ml); and median transit time was 30 minutes (range, 15~60 minutes). Patients performed antegrade continence enema with an average of once every 2 days (range, 0.3~3 days). Social continence was achieved in 14 patients (73.7%). Regurgitation of fecal material through stoma was not reported at all in 17 patients (89.5%). CONCLUSION: We recommend LACE as the procedure of choice for patients with congenital malformation or any other condition predisposing to fecal incontinence or constipation intractable to conventional treatment.
Colon* ; Colon, Sigmoid ; Constipation ; Diagnosis ; Enema* ; Fecal Incontinence ; Follow-Up Studies ; Humans ; Ileum ; Meningomyelocele ; Retrospective Studies

OBJECTIVE: To investigate the effect of surgery time on prognosis of newborns with meningomyelocele. METHODS: The records of neonates with meningomyelocele were retrospectively analyzed. Demographic and clinical characteristics as well as information, timing of surgery, and durations of hospital stay and antibiotic therapy were recorded. RESULTS: The records of 30 babies were included in the final analysis. Overall, the mean gestational age was 37.7+/-2.7 weeks, with a mean birth weight of 2967+/-755 g and head circumference of 35.8+/-3.8 cm. In terms of localization, 46.6% of the meningomyeloceles were lumbosacral, 40% were lumbar, 10% were thoracolumbar and 3.3% were thoracal. The mean size of the meningomyelocele sacs was 4.33+/-1.2 cm. Newborns underwent surgery on average of 8.2+/-5.9 days after birth, with an overall mean duration of hospital stay of 30+/-25.1 days. Patients were divided into two groups based on timing of surgery (group 1, < or =5 days; group 2, >5 days), and comparisons between groups revealed that earlier surgery was associated with significantly shorter durations of hospital stay (p<0.001) and antibiotic therapy (p<0.05). CONCLUSION: Early surgical intervention (< or =5 days) was associated with a shorter duration of hospital stay and antibiotic therapy as well as a lower complication rate. We recommend that corrective surgery be undertaken as soon as reasonably possible.
Birth Weight ; Gestational Age ; Head ; Humans ; Infant, Newborn ; Length of Stay ; Meningomyelocele ; Parturition ; Prognosis ; Retrospective Studies

PURPOSE: The aim of this study was to investigate the efficacy and tolerability of extended-release oxybutynin(oxybutynin ER) in children with a neurogenic bladder. MATERIALS AND METHODS: Fifty-four patients(21 myelomeningocele and 33 lipomyelomeningocele) with a neurogenic bladder were enrolled in the study. We reviewed the medical records and performed a telephone interview. The treatments were changed from immediate-release oxybutynin (oxybutynin IR) or other anticholinergics to oxybutynin ER from August to December 2006. The mean age of the study patients was 11.1 years (range 4 to 18 years) and the mean body weight was 37.9kg(range 16.2 to 72.0kg). All patients were asked about the effectiveness, side effects and compliance with the medication. The number of voids, volume of urine per void or clean intermittent catheterization(CIC) and number of incontinence episodes were also evaluated. RESULTS: The mean duration of oxybutynin ER treatment was 16.3 weeks (range 7-25 weeks). Twenty-six patients(48.1%) responded they had improvement in voiding symptoms. Among the patients, there was a significant reduction in the number of incontinence episodes(from 3.3 to 1.3, p<0.001) with the change in medications. The number of voids or CIC per 24 hours and the maximum volume of urine per void or CIC did not show a significant change. Another twenty-eight patients(51.9%) responded that the improvements were maintained. Among these patients, there were no significant changes of the medications. Only five patients (9.3%) changed their medication because of the side effects. CONCLUSIONS: The results of this study showed that the extended-release oxybutynin was effective and well tolerated in children with a neurogenic bladder.
Body Weight ; Child* ; Cholinergic Antagonists ; Compliance ; Humans ; Interviews as Topic ; Medical Records ; Meningomyelocele ; Urinary Bladder, Neurogenic*

PURPOSE: Using proteomic analysis, this study was performed to see the characteristics of proteins expression in the muscles of spastic cerebral palsy patients. MATERIALS AND METHODS: We studied twelve specimens from six patients with spastic cerebral palsy, three patients with myelomeningocele, and three normal people who underwent orthopaedic surgeries due to trauma. We studied the extracted proteins showing differences in the two-dimensional electrophoresis, and the prominent thirteen proteins were re-evaluated by proteomics and the reverse transcriptional polymerase chain reaction, which was to clarify the relationship between gene and protein expression. RESULTS: Among fifteen proteins, six proteins were found to be higher in normal people, and nine were found to be higher in the groups of patients by spot histogram. The results of proteomic analysis with MALDI-TOF for fifteen proteins showed that the expression of DJ-1 was related to cerebral palsy. CONCLUSION: This study shows that strong expression of DJ-1 is related to spasticity and cerebral palsy. We showed for the first time the possibility of any relationship between spastic condition and DJ-1 expression.
Cerebral Palsy ; Electrophoresis ; Humans ; Meningomyelocele ; Muscle Spasticity ; Muscles ; Polymerase Chain Reaction ; Proteins ; Proteomics

PURPOSE: This study was undertaken to ascertain if the Churchill's classification of the bladder, in children with spina bifida, reflects the characteristic clinical manifestation of the patients, and can therefore help to determine the best corrective surgical procedure in patients with spina bifida. MATERIALS AND METHODS: The patient population consisted in 148 lipomeningomyelocele and 92 meningomyelocele patients. Patient bladders were classified, according to Churchill, based on the filling, holding and voiding abnormalities(types I-V). The renal damage was expressed as grades A-D, according to the severity of the defect. RESULTS: Ninety patients were classified as type I and 5(6%) of them had renal damage. Thirty-three patients were classified as having type II and 7(21%) of them had renal damage. Five patients were classified as having type III, all with normal kidneys. Of these 5 patients, 2 received ileal augmentation and anti-incontinence surgery. 55 patients were classified as having type IV bladders and renal damage was found in 11(20%). Fifteen patients received augmentation and anti-incontinence surgery simultaneously. Two patients underwent ileal augmentation only, but incontinence continued postoperatively. Fifty-seven patients were classified as having type V bladder, and renal damage was found in 25(44%), and 15 patients received augmentation. All the type V patients that had augmentation cystoplasty maintained their continence state postoperatively. CONCLUSIONS: Neurogenic bladders in pediatric patients with spina bifida can be classified by Churchill's classification. The individual types showed characteristics of the clinical state. This classification seems to help in determining the best corrective surgical procedure in individual neurogenic bladder types of pediatric patients with spina bifida.
Child ; Classification* ; Humans ; Kidney ; Meningomyelocele ; Spinal Dysraphism* ; Urinary Bladder ; Urinary Bladder, Neurogenic*