Accelerated pubertal development in patients with shunted hydrocephalus

OBJECTIVE: To evaluate pubertal development and peripheral concentrations of gonadotrophins and sex hormones in children with shunted hydrocephalus compared with healthy controls. STUDY DESIGN: 114 patients (52 females, 62 males) and 73 healthy controls (35 females, 38 males) aged 5 to 20 years were analysed for stage of puberty, age at menarche, testicular volume, basal serum follicle stimulating hormone (FSH), luteinising hormone (LH), sex hormone binding globulin (SHBG), testosterone and oestradiol concentrations, and free androgen index. RESULTS: Male gonadal and male and female pubic hair development occurred significantly earlier in the patients than in the controls. The mean age at menarche was significantly lower in the female patients than in their controls (11.7 v 13.2 years; p < 0.001), and lower than it had been for their mothers (v 13.1 years; p < 0.001). Relative testicular volume was higher in the male patients than in their controls (1.2 standard deviation score (SDS) v 0.2 SDS; p < 0.001). The prepubertal patients had higher basal LH (0.13 U/l v 0.08 U/l; p < 0.001) and SHBG (132.3 nmol/l v 109.1 nmol/l; p < 0.01) than the controls. Both the prepubertal and pubertal females had significantly higher basal FSH than their controls (1.57 U/l v 1.03 U/l; p < 0.05, and 4.0 U/l v 2.9 U/l; p < 0.01, respectively). CONCLUSIONS: Hydrocephalic children experience accelerated pubertal maturation, reflected in a younger age at menarche in females and an increased testicular volume in males. This may be because of enhanced gonadotrophin secretion, possibly resulting from unphysiological variations in intracranial pressure.     

The results of a practice survey on the management of patients with shunted hydrocephalus.

The care of patients with shunted hydrocephalus is one of the most common clinical problems facing a pediatric neurosurgeon. Despite the prevalence of this clinical entity, it is our impression that the management strategies for these patients vary significantly amongst pediatric neurosurgeons. In an effort to better understand how varied these practices are, we surveyed 261 neurosurgeons with an expressed interest in pediatric neurosurgery, of whom 149 (57%) responded. The survey contained questions pertaining to preoperative and intraoperative management, choice of shunt valve, treatment philosophies with respect to shunt revision, evaluation process for potential shunt malfunction, treatment strategies for shunt infections and routine follow-up care. The answers to the questions confirmed our expectations that there are a variety of ways to manage these patients.     

Information value of magnetic resonance imaging in shunted hydrocephalus.

OBJECTIVE--To study the role of magnetic resonance imaging (MRI) in evaluating children with shunted hydrocephalus. METHODS--Sixty one asymptomatic children with shunted hydrocephalus or cystic cerebrospinal fluid collections were studied by cranial MRI. The information obtained from the images was classified into three categories: provided (1) a new diagnosis, (2) additional information, or (3) no essential new information. The findings were compared with those of the last follow up computed tomograms. RESULTS--MRI provided a new diagnosis in seven cases (11.5%), and additional information was obtained in 34 (55.7%) cases. In 20 cases (32.8%) no essential new information was obtained. MRI visualised white matter lesions and corpus callosum pathology more often than computed tomograms. CONCLUSIONS--MRI provided new important information in cases of children with shunted hydrocephalus to such an extent that it can be recommended as the primary imaging method for every child with this disorder.     

Low-pressure shunt 'malfunction' following lumbar puncture in children with shunted obstructive hydrocephalus.

Most shunt malfunctions present with signs and symptoms of high intracranial pressure, and computed tomography scans demonstrate ventricular enlargement. However, several authors have described a rare 'low-pressure' hydrocephalic state in which ventricular enlargement can occur in the face of low, or even negative, intracranial pressures. We report 2 children with obstructive hydrocephalus in whom this 'low-pressure state' followed a lumbar puncture; in both children, the shunts were functioning properly despite increased ventricular size on computed tomography scans, and all symptoms resolved (and the ventricles returned to baseline) following a period of enforced recumbency without shunt revision. We hypothesize that subarachnoid cerebrospinal fluid leakage through the puncture site in the lumbar theca decreases the intracranial pressures globally to a point below the opening pressures of the shunt valves. The ventricular cerebrospinal fluid, unable to be drained through either the subarachnoid space or the shunt, accumulates within the ventricular system under low pressure. One consistent feature in our 2 patients has been the postural nature of the headaches. We recommend enforced recumbency and, if necessary, a blood patch to seal the lumbar leakage. Shunt revision or prolonged external ventricular drainage appears to be unnecessary in these patients. Finally, neurosurgeons should be aware of this potential complication.     

Language differences in young children with myelomeningocele and shunted hydrocephalus

OBJECTIVE: This comparative cohort study examined language differences in young children (preschool to first grade) with myelomeningocele and shunted hydrocephalus (MM/SH). METHOD: A well-validated, standardized language test was administered to 17 children with MM/SH (age range 4-6 years) and 16 age-matched, nonaffected children. RESULTS: Standard scores were obtained within lexical/semantic, syntactic, and pragmatic domains. An analysis of performance on individual subtests within the lexical/semantic domain was striking. Children with MM/SH performed significantly worse (p<0.01) on a test measuring the comprehension of words representing important early learning concepts and on pragmatic tasks that measured their ability to use language functionally in social situations. CONCLUSIONS: Words representing basic precepts and concepts are commonly used in instructions to children in early childhood. Difficulty with functional pragmatic language results in suboptimal communication skills. Academic and community implications are discussed.     

Reduced levels of growth hormone, insulin-like growth factor-I and binding protein-3 in patients with shunted hydrocephalus

OBJECTIVE: Children with hydrocephalus are characterised by slow linear growth in prepuberty, accelerated physical maturation during puberty, and reduced final height. We aimed to study the possible roles of growth hormone, insulin-like growth factor-I (IGF-I), and IGF binding protein-3 (IGFBP-3) in this growth pattern. STUDY DESIGN: One hundred and fourteen patients with shunted hydrocephalus (62 males) aged 5 to 20 years, of whom 17 had spina bifida (six males), and 73 healthy controls (38 males) were studied. Anthropometric measures, body mass index, and body fat mass were assessed and the stage of puberty was determined. Serum growth hormone and plasma IGF-I and IGFBP-3 concentrations were measured. RESULTS: The patients comprised 44 (26 males) who were prepubertal and 70 (36 males) pubertal or postpubertal, while 32 of the controls (19 males) were prepubertal and 41 (19 males) pubertal or postpubertal. The prepubertal children with hydrocephalus had lower IGF-I (p = 0.002) and IGFBP-3 concentrations (p < 0.001) than the controls, and the pubertal children had four times lower basal growth hormone concentrations (p < 0.001). There was a correlation between height SD score and IGF-I levels in the total patient population (r = 0.23; p = 0.01). Peripheral IGF-I concentrations peaked at pubertal stages 2-3 in the female patients and at stage 4 in the controls. The prepubertal patients on antiepileptic treatment, carbamazepine in most cases (73%), had higher IGF-I (p = 0.01) and IGFBP-3 concentrations (p = 0.03) than those who had never been treated with antiepileptic drugs, but still lower IGFBP-3 levels than the controls (p = 0.01). CONCLUSION: Based on these findings, it can be concluded that reduced growth hormone secretion may contribute to the pattern of slow linear growth and reduced final height observed in these patients.     

True precocious puberty: a long-term complication in children with shunted non-tumoral hydrocephalus

In 4/37 (10.8%) children, adolescents and young adults with successfully shunted hydrocephalus, puberty occurred or was occurring precociously, at an age ranging from 7.5 to 8.6 years, with a consequent impairment of their effective or predicted adult height as compared to the familial target height. All four patients had undergone a surgical intervention for the insertion of a ventricular-atrial or a ventricular-peritoneal shunt during the first year of life; since the last surgical shunt revision (at the age of 5 years) no relapse of hydrocephalus had been recorded. The authors conclude that precocious puberty is to be regarded as a not infrequent long-term complication in patients with successfully shunted hydrocephalus.     

VI. Somatic pubertal development.

The pubertal development of 212 randomly selected Swedish urban children has been investigated as part of a prospective longitudinal study of growth and development. The timing and pattern of pubertal changes were in agreement with the findings in other contemporary studies. The good agreement with data on pubertal development reported in other investigations of Swedish children indicates that the present sample was representative of contemporary Swedish children. Two procedures of assessment of secondary sex characters - clinical examination and whole-body photography - have been compared and contrasted. A clinical examination is less laborious and resource-consuming and also has psychological advantages but should be supplemented in boys by the estimation of testicular volume (orchidometry). In girls the two methods have similar precision and reliability. The first pubertal changes may be observed before 9 years in girls (breast development) and before 10 years in boys (genital development). On average the first change takes place about one year earlier in girls than in boys. Peak height velocity (PHV) is an early event during puberty in girls and a relatively late event during puberty in girls and a relatively late one in boys, the sex difference in mean age being about two years. In girls, menarche is a late event, always occurring after PHV. At the age of 13-14 years some boys and girls have not yet begun theri pubertal development, while others have reached the adult stage.     

Reduced levels of growth hormone,insulin-like growth factor-I and binding protein-3 in patients with shunted hydrocephalus

Accepted 25 March 1997
OBJECTIVE—Children with hydrocephalus are characterised by slow linear growth in prepuberty, accelerated physical maturation during puberty, and reduced final height. We aimed to study the possible roles of growth hormone, insulin-like growth factor-I (IGF-I), and IGF binding protein-3 (IGFBP-3) in this growth pattern.
STUDY DESIGN—One hundred and fourteen patients with shunted hydrocephalus (62 males) aged 5 to 20 years, of whom 17 had spina bifida (six males), and 73 healthy controls (38 males) were studied. Anthropometric measures, body mass index, and body fat mass were assessed and the stage of puberty was determined. Serum growth hormone and plasma IGF-I and IGFBP-3 concentrations were measured.
RESULTS—The patients comprised 44 (26 males) who were prepubertal and 70 (36 males) pubertal or postpubertal, while 32 of the controls (19 males) were prepubertal and 41 (19 males) pubertal or postpubertal. The prepubertal children with hydrocephalus had lower IGF-I (p = 0.002) and IGFBP-3 concentrations (p< 0.001) than the controls, and the pubertal children had four times lower basal growth hormone concentrations (p< 0.001). There was a correlation between height SD score and IGF-I levels in the total patientpopulation (r = 0.23; p = 0.01). Peripheral IGF-I concentrations peaked at pubertal stages 2-3 in the female patients and at stage 4 in the controls. The prepubertal patients on antiepileptic treatment, carbamazepine in most cases (73%), had higher IGF-I (p = 0.01) and IGFBP-3 concentrations (p = 0.03) than those who had never been treated with antiepileptic drugs, but still lower IGFBP-3 levels than the controls (p = 0.01).
CONCLUSION— Based on these findings, it can be concluded that reduced growth hormone secretion may contribute to the pattern of slow linear growth and reduced final height observed in these patients.

• Prepubertal children with shunted hydrocephalus have reduced circulating IGF-I and IGFBP-3 concentrations • Pubertal children with shunted hydrocephalus have reduced basal serum growth hormone concentrations • Reduced growth hormone secretion may contribute to slow linear growth and reduced final height in hydrocephalic children • Carbamazepine treatment may increase IGF-I and IGFBP-3 concentrations in the peripheral circulation     

Growth and pubertal development in patients with meningomyelocele: a retrospective analysis

Our retrospective analysis of growth and pubertal development includes 109 children and adults with meningomyelocele (MMC) (52 M, 57 F) aged 3.2-21.0 years (median 8.9 years). Anthropometric data, growth-retarding factors and data on pubertal development were analysed in comparison to the normal population using standards from Prader et al. (1). The results (mean ± SD) were as follows. Fifty patients (46.8%) had short stature (height SDS for chronological age (SDS CA)< -2). The supine length was influenced by the level of the lesion (height SDS CA: ≥ L2 -3.13 ± 1.62, ≤ S2 -0.46 ± 1.27), ambulatory status, skeletal deformities and pubertal stage. The mean adult height ( n = 15, age 16.1–21.0 years) measured 141.3cm for women (height SDS CA -3.83 ± 1.79) and 159.2 cm for men (height SDS CA -2.27 ± 1.81). In 82.6% of the subjects ( n = 90), arm spans were within the normal range. Reduced arm spans (SDS < -2) as found in 19 patients (17.4%) with short stature (mean height SDS CA -3.29 ± 1.29) may be caused by factors other than neurological lesions and skeletal deformities, and require further endocrinological studies. Out of 27 pubertal patients, central precocious puberty was diagnosed in five girls. The stages of puberty in MMC girls developed earlier than expected for the age-related group.     

Growth hormone secretion in patients with constitutional delay of growth and pubertal development

Growth hormone levels were measured every 30 minutes during sleep over 9 hours in 20 prepubertal patients with constitutional delay of growth and puberty (CGD) and in 10 age-matched controls, all of whom had had normal GH responses to an orally administered dose of clonidine. We found no significant difference in the mean 9-hour overnight GH concentration between groups (4.5 +/- 1.8 ng/ml (mean +/- SD) in the CGD group, 4.4 +/- 2.8 ng/ml in the control group). Total GH output (258 +/- 99 U vs 222 +/- 135 U), total number of nocturnal GH pulses (3.6 +/- 0.8 vs 3.3 +/- 1.3), mean peak GH response during nocturnal sampling (13 +/- 1.2 ng/ml vs 13.2 +/- 1.3 ng/ml), and basal somatomedin C concentrations were not different in the children with growth delay and controls. We conclude that prepubertal patients with constitutional delay of growth and puberty secrete GH normally and do not seem to have any abnormality in GH regulation.     

Normal pubertal development in a female with carbohydrate deficient glycoprotein syndrome.

A girl is reported who presented with many of the clinical and biochemical characteristics of type I carbohydrate deficient glycoprotein syndrome. Unusually, however, she experienced a normal pubertal development.     

Normal standard of pubertal development in Singapore school children.

A cross sectional study of the pubertal status of normal school children in Singapore was performed in 1987. The results of the data analysis are presented as an update from the last survey performed in 1977. The norms of pubertal development in boys and girls are thus defined.     

Growth and pubertal development in patients with congenital adrenal hyperplasia due to 11-beta-hydroxylase deficiency

To evaluate growth and pubertal development in patients with congenital adrenal hyperplasia due to 11-beta-hydroxylase deficiency, data were collected retrospectively from the charts of 24 patients, 15 girls and nine boys. Growth before diagnosis was characterized by a rapid acceleration in the second half of the first year of life, with rapid advancement of bone age. Final height was severely compromised in all the patients, regardless of age at diagnosis and quality of therapeutic control. Onset of puberty was precocious in male patients and normal in female patients. In this respect, treatment with hydrocortisone acetate proved to be superior to cortisone acetate or prednisone.     

Outcome in shunted hydrocephalic children.

The purpose of the present study was to identify the main risk factors for poor outcome in children with shunted hydrocephalus, including the timing of the operation. The medical records of patients born between 1984 and 1992, who had undergone shunt operation for hydrocephalus before 1994, were analysed retrospectively. The following data were collected: aetiology of hydrocephalus, other pathology in addition to hydrocephalus, timing of drain implantation, number and indication of revisions and several outcome parameters such as school type, seizures and morbidity parameters. Children with a post-haemorrhagic hydrocephalus fared poorly. Children with hydrocephalus due to spina bifida or another congenital malformation had considerably better outcome scores. Pathology in addition to hydrocephalus negatively influenced the prognosis. Regarding the timing of drain implantation, we found a poorer mental and linguistic development in children operated more than 1 month after diagnosis. Patients with more than two revisions seem to have a higher chance of developing seizures. In conclusion, this study confirms earlier reports that peri- and postnatal haemorrhage is an important risk factor for poor outcome. More importantly, we found that delay in surgical treatment might also be a risk factor for poor outcome in hydrocephalic children.