Prediction of independence and intelligence at birth in meningomyelocele.

OBJECTIVE: The aim of this research is to compute a prediction of the future intelligence of every neonate with a meningomyelocele (MMC) on the day of birth to inform the parents. Earlier research revealed that the variables ventricle size, open arch above vertebra lumbar 3, a very small or a very large head can be attended by an IQ below 85 points: the turning point between dependence and independence. METHODS: Until now no research has been done combining the values of influencing variables in one equation. Our study used the equation of multiple longitudinal linear regression. For reference, data from the files of 148 neonates born in the years 1960-1980 were compared with those of Dutch children sampled by Verger. The working hypothesis that the IQ level is directly related to congenital deformities of the nervous system needs to be tested. As standard the values of variables at birth, the head circumference and length, the size of the ventricles and the highest open vertebral arch, are to be used. RESULTS: The Wisc-R of the MMC cohort had a mean of 84 points and 55% had an IQ above 85. The correlation computation of the values of all available variables with the values of the measured Wisc-R revealed the significance of the neuroanatomical variables, size of ventricle and level of the highest open vertebral arch and for the anthropomorphic variables Length and Length/Circumference. To establish the relationship between the Wisc-R value and the values of the significant variables an equation with the multiple linear regression method was used. The measured IQ is made dependent on the significant variables size of ventricle, level of highest open arch and quotient Length by Circumference. This equation produces a value called predicted IQ. The predicted IQ was 92%, the same or nearly the same as the measured Wisc-R IQ. CONCLUSION: This conformity of the measured IQ and the predicted IQ proves the possibility of using these birth data for prediction.

Sexual functioning of adult patients born with meningomyelocele. A pilot study.

OBJECTIVES: This pilot study was started to get an impression of the sexual activity and sexual difficulties of adult patients born with meningomyelocele in control at Utrecht University Hospital, and to find out whether these problems deserve more attention from the Spina Bifida Team. METHODS: Seventeen patients (9 men, 8 women; aged 19-44) were asked to participate; 11 (8 men, 3 women) decided to take part in this study. All these patients were interviewed by their physician about their present sexual life; 6 of them also completed the 'Questionnaire for screening Sexual Dysfunctions' (QSD). RESULTS: With 1 exception, all patients expressed a desire for sexual contact. Five of them masturbated and 6 patients sometimes had sexual contact. Eight patients had problems in the field of sexuality, but only 3 were dissatisfied with their present sexual life. CONCLUSIONS: It is advantageous to invite adult patients born with meningomyelocele for an interview about their sexual life. When raising this subject, however, one has to act with caution: for quite a few patients (in this study mostly women) sexuality still is a delicate subject.

Treatment of spinal deformity in myelomeningocele: a retrospective study in four hospitals.

Correction of spinal deformities in patients with myelomeningoceles (mmc) is notoriously complicated. To identify the deformity, frequency, gravity and complications of treatment a retrospective study was carried out on 61 patients from four hospitals. 45 patients had neurological defect above L3 and were wheelchair-bound. The indications for surgery were progressive disturbance in sitting balance, pain and pressure sores. Two types of spinal deformity were identified; scoliosis (often thoraco-lumbar or double major curves) and kyphosis (usually angular and lumbar). The mean age at operation was 12 years 8 months (2y 6m-19y 7m). Several operative procedures were used; posterior, anterior and circumferential fusion, and resection of the kyphosis. 52 solid fusions were achieved with variable correction at the cost of many complications such as excessive blood loss (2), post-operative pressure sores (15), failure of instrumentation (15), deep infection (11), and death following a CSF leak (1). 4 patients died from unrelated causes. Only 16 patients had no complications. The type and severity of scoliosis and kyphosis, operative technique, results and complications were correlated to identify the risks and define the optimal surgical technique for each type of spinal deformity in mmc.

Spina bifida: feelings, opinions and expectations of parents.

Interviews with sixty-seven parents of spina bifida children show that a common problem is "shared parenthood" which, in turn, contributes to the child's difficulty in finding its own identity. The history of the parents after the birth of an affected child follows a pattern typified by "breaking points." At these points the parents reformulate the aim of management. When the physicians are unaware of these new aims and continue their regular treatment, they make it impossible for the parents to face the incurability of their child. This gives the physicians a continued responsibility, not unlike the decision to treat after birth.