AUXOLOGICAL AND ENDOCRINOLOGIC EVALUATION OF CHILDREN WITH HYDROCEPHALUS AND OR MENINGOMYELOCELE/

Short stature and precocious puberty are frequently found in children with hydrocephalus and/or meningomyelocele (MMC). However, aetiology a nd pathophysiology have not been well characterized. In this study, 10 8 patients aged between 3 and 17.8 years were evaluated. Growth was do cumented on the basis of arm span measurements. Short arm span was fou nd in 47 (43.5%) children with hydrocephalus and/or MMC. Mean arm span was -2.0 standard deviation score (SDS) (-6.4 to +0.8) in 43 girls an d -1.4 SDS (-5.6 to +1.3) in 65 boys. When growth deficiency (more tha n -2.0 SDS) was diagnosed by arm span measurement (24 F, 23 M) or when early sexual maturation was found (6 F: 9 M), endocrine evaluation fo llowed. Levels of serum insulin-like growth factor and insulin-like gr owth factor binding protein-3 were low in 22 of 62 (33.8%) patients. I n 9 of 62 (14.5%) patients, insulin-like growth factor-1 and insulin-l ike growth factor binding protein-3 levels were found to be increased. Growth hormone (GH) deficiency was diagnosed by means of two differen t stimulation tests (clonidine and arginine) in 7 of 62 (11.3%) patien ts. In another 3 of 62 (4.8%) children, 12 h night time GK sampling sh owed low maximum peak levels and decreased area under the curve values , suggesting neurosecretory GH dysfunction. Precocious puberty or earl y onset of puberty associated with elevated luteinising hormone and fo llicle stimulating hormone concentrations after stimulation with lutei nising hormone releasing hormone was found in 13 of 108 (12.0%) patien ts (age 7-9 years). Free thyroxine was abnormally low in 2 of 62 (3.2% ) patients. Cortisol was within the normal range in all 62 (100%) test ed patients. Conclusions Short arm span in children with hydrocephalus and/or MMC is frequently accompanied by GH deficiency or neurosecreto ry GH dysfunction. Early onset of puberty is another frequent finding. Both hormonal disorders map be the consequence of damage to the hypot halamus or the pituitary gland caused by increased intracerebral press ure or increased mass of cerebrospinal fluid.