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

Abstract

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: > or = L2 -3.13 +/- 1.62, < or = 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 141.3 cm 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.

Putative height acceleration following tethered cord release in children

“…Disorders that damage the spinal cord have been associated with reduced height attainment in children, and a common clinical observation among children with myelomeningocele is disproportionately shorter lower extremities. 19,29,33 Two other important findings in this study are consistent with the hypothesis that spinal cord tethering may correlate with growth restriction. Consider the group of children who experienced positive changes in height percentiles after surgery.…”

Evaluation of femur length during the second and third trimesters in fetuses with myelomeningocele

“…We are unaware of prior reports describing lower limb biometry in fetuses with myelomeningocele, although it is known that children with myelomeningocele have leg growth defects that are demonstrable before 8 years of age 1,13 . Several etiologies have been proposed for such lower extremity foreshortening.…”

Wachstumshormontherapie bei Patienten mit Meningomyelozele

“…Patienten mit Meningomyelozele, deren Lebenserwartung und -qualität in den letzten Jahren durch eine interdisziplinär konzipierte Langzeitbetreuung zunehmend verbessert werden konnte, sind häufig von Störungen des Wachstums und der Pubertät betroffen [2,7,11,13,15,20,21,27,32]. Eine Reihe von wachstumslimitierenden Faktoren, bedingt durch die neurologische Läsion, wie motorische, sensible und trophische Störungen der unteren Extremitä-ten, Skelettdeformitäten, Immobilität, rezidivierende Infektionen, Ernährungs-störungen etc., tragen zum Wachstumsdefizit der Betroffenen bei.…”