Beverly Irwin

Long-term outcome and complications of children born with meningomyelocele

The long-term functional outcome of 101 children born with meningomyelocele between 1971 and 1981 was assessed, by a combination of retrospective chart review and follow-up assessments. The children had been managed at birth using a process ofnonstandardized selection. Eighty-three of the 101 patients survived after a minimum follow-up of 8.6 years, for a mortality rate of 18%. Forty-four of 83 children (53%) were community ambulators, and this correlated well with the presence of intact quadriceps function. Forty-eight children (58%) attended normal school and were grade-appropriate. Sixty-two of 83 patients (75%) were socially continent of urine, and 71/83 (86%) were socially continent of stool. Hydrocephalus was present in 93 of the 101 children in the study, and 85 children were shunted. Half of the shunted children required a shunt revision in the first year of life, and thereafter the rate of revision decreased, so that after 2 years the risk of revision was approximately 10% per year.

Prenatal spinal evaluation and functional outcome of patients born with myelomeningocele : Information for improved prenatal counselling and outcome prediction

OBJECTIVE Prenatal ultrasonography can localize the level of the spinal cord malformation, allowing prediction of the potential postnatal neurological deficit and functional prognosis. METHODS This study has two evaluations: (a) a retrospective prenatal review of 26 fetuses with spinal dysraphism (1987-1991), and (b) a follow-up descriptive study of patients (1971-1981) who underwent closure of the spinal lesion and ventricular shunting in the neonatal period. RESULTS Prenatal ultrasound evaluation enabled the accurate definition of the last intact vertebral level which allows separation of fetuses into three functional groups (last intact level L2, L3-4, L5-sacral). Patterns of ambulation, urinary and bowel continence, and school performance vary according to level of spinal lesion and the neurological deficit. The need for ventricular shunts, the incidence of other spinal malformations and surgical interventions did not vary with the level of the spinal lesion. CONCLUSIONS The functional outcome for patients with myelomeningocele is variable; however, distinct patterns emerge based on the level of spinal dysraphism and the resultant neurological deficit. By relating the level of the fetal spinal lesion to outcome data, more precise functional prognoses can be given to families.

The impact of prenatal diagnosis on neural tube defect (NTD) pregnancy versus birth incidence in British Columbia.

The birth incidence of neural tube defect (NTD) cases in British Columbia (B.C.), and elsewhere in North America, is reported to be declining. This decline is being attributed to folic acid (FA) supplementation and food fortification, but 2nd trimester prenatal screening of pregnancies for NTDs and other congenital anomalies has increased during this timeframe, as well. This descriptive, population-based study evaluates the impact of prenatal screening of NTD-affected pregnancies on (1) pregnancy outcome and (2) reporting of NTD births to the provincial Health Status Registry (B.C.H.S.R.); and it assesses (3) the use of periconceptional FA supplementation. NTD cases were ascertained from medical records of health centres providing care to families with NTD-affected pregnancies and newborns; and from NTD cases reported to the B.C.H.S.R. In 1997–1999, the B.C.H.S.R. published a NTD incidence of 0.77/1000. In this study, 151 NTD-affected pregnancies were identified, with an incidence of 1.16/1000. Partial Reporting of induced abortions in a NTD incidence 45.5% low than the actual incidence. Medical records were available for review on 144/151 pregnancies. Prenatal screening identified 86.1% (124/144) of NTD-affected pregnancies, with 72.6% (90/124) resulting in pregnancy termination, and 27.4% (34/124) continuing to term. Use of FA supplementation in the periconceptional period was recorded in 36.4% of pregnancies (39/107). Thus in B.C. the decline in the NTD incidence is due predominantly to pregnancy terminations following prenatal diagnosis, which reduces the NTD incidence by 60%, from 1.16/1000 to 0.47/1000. Continued efforts for primary and the option of secondary prevention of NTDs are recommended in order to improve newborn health in B.C. and elsewhere. These interventions need to be monitored, however, for optimal health care planning.

Cervical spine instability following cervical laminectomies for Chiari II malformation: a retrospective cohort study

ObjectiveThe treatment of symptomatic Chiari II malformations typically involves multilevel cervical laminectomies in very young children. These patients are at significant risk of cervical instability. The purpose of this study was to determine the incidence and significance of cervical instability after multilevel cervical laminectomies in a cohort of patients decompressed for Chiari II malformation.MethodsPostoperative dynamic lateral cervical spine radiographs were obtained on pediatric patients who had multilevel cervical laminectomies for symptomatic Chiari II malformations. Postoperative cervical spine instability was determined radiographically using published criteria. Clinical instability and need for cervical fusion were also assessed.ResultsNine patients met inclusion criteria for the study. Five of the nine patients (56%) showed evidence of radiographic instability of their cervical spines following surgery for their Chiari II malformations, according to the criteria used. No patient showed evidence of clinical instability or required cervical fusion.ConclusionRadiographic evidence of cervical spine instability following multilevel cervical laminectomies for Chiari II is common but may be of minimal clinical significance. The reason for the lack of clinical instability in what might be considered high-risk patients is not understood.

Evaluating functional and health outcomes for patients with spina bifida 5 and 10 years post-transition into adult care

Background In BC, pediatric patients with spina bifida are followed by a multidisciplinary clinic at BC Children’ sH ospital. Upon graduation, patients transition into the adult healthcare system where they must coordinate their own care. The purpose of this study is to determine the functional and health outcome of graduates transitioning from the spina bifida clinic at BC Children’s Hospital. Materials and methods Graduates of up to 10 years (1999-2008) from the pediatric spina bifida clinic were mailed a questionnaire. It consisted of 11 sections, including two standardized questionnaires on quality of life (QOL), the Medical Outcomes Study 36-item short-form (SF-36) and Spina Bifida-specific Health Related QOL (SBHRQOL). Results

The assessment of management morbidity in children with myelomeningocoele

Address: 1Division of Paediatric Neurosurgery, British Columbias Childrens Hospital, 4480 Oak St, #K3-159, Vancouver, BC, Canada, 2Myelomeningocoele Program, Department of Paediatric Neurosciences, British Columbias Childrens Hospital, 4480 Oak St, #K3-159, Vancouver, BC, Canada and 3Department of Paediatric Neurosciences, British Columbias Childrens Hospital, 4480 Oak St, #K3-159, Vancouver, BC, Canada

OC11.06: Prenatal diagnostic features of closed spinal dysraphism

rostrum height (= thickness), a four-fold increase in the genu height, a two-fold increase in the body height and a three-fold increase in the splenium height. A total number of 18 cases with corpus callosum pathology could be detected. Conclusions: 3-D neurosonography serves as an excellent tool to detect normal growth pattern of fetal corpus callosum. This knowledge will help to detect abnormal growth patterns of the corpus callosum.