Endocrine evaluation after endoscopic third ventriculostomy (ETV) in children

Objective Endoscopic third ventriculostomy (ETV) is a standard procedure for the treatment of obstructive hydrocephalus in children. Main part of the procedure is the perforation of the third ventricle floor (tuber cinereum). This structure is part of the hypothalamic–pituitary neuronal network of cerebral endocrine regulation. There are no systematic data available about the endocrine status after ETV in children. Materials and methods We examined 20 children who had undergone ETV. Examination included laboratory tests (adrenocorticotropic hormone, prolactin, insulin-like growth factor 1 [IGF-1], IGF-binding protein 3 [IGFBP-3], fT3, fT4, thyroid-stimulating hormone [TSH], serum osmolarity, electrolytes, glucose, urea, follicle-stimulating hormone [FSH] and luteinizing hormone [LH], and testosterone in selected patients), measurement of weight, height, and head circumference, and physical examination. The study was approved by the Ethics Committee of the Medical Faculty of Kiel University. Results In seven patients, prolactin was moderately elevated. One patient demonstrated a significantly increased prolactin (56.3 ng/ml). In all eight patients, this was the only laboratory value that was out of the normal range; all other parameters were normal. Three other patients showed one abnormal parameter (decrease in FSH and LH, increase in TSH, decrease in IGF-1 and IGFBP-3). In nine patients, weight or height was not within the 3rd to 97th centiles for age. Discussion and conclusion More patients than expected demonstrated endocrine laboratory abnormalities. However, there was no clinical relevance in any of the studied patients. It remains inconclusive whether ETV contributes to the abnormalities of prolactin levels or to other endocrine parameters in pediatric patients. Longitudinal studies are necessary to delineate the effect of ETV on endocrine regulation. Presented at the Third World Conference of the International Study Group on Neuroendoscopy (ISGNE), Marburg, Germany, 15–18 June 2005.     

Growth, bone maturation and pubertal development in children with the EMG-syndrome

In 7 patients (5 girls, 2 boys) with the EMG or Wiedemann-Beckwith syndrome, statural growth, bone age (BA), weight and pubertal development were studied longitudinally. Height was above the 90th percentile (%) for chronological age (CA) after age 2 years, reaching an average of 2.5 SD above the mean at or after puberty. Adult or attained height also exceeded significantly (P<0.015) parental (genetic) target height by 13.2 cm on the average. In one girl, adult height prognosis (190 cm) could be reduced to an adult height of 183 cm by high-dose estrogen treatment. In most children, growth velocity remained above the 90th % up to 4–6 years of age and normalized thereafter. In all patients studied, bone age was markedly advanced and particularly so during the first 4 years after birth. Weight was above the 90th–97th % during infancy and early childhood and remained there, appropriate or slightly subnormal for height, until adulthood, except for 3 girls who reached and maintained the 50th % during or after puberty. Spontaneous pubertal development occurred within normal limits for CA and around the 50th % for BA. Except for the marked bone age acceleration, the reason for the increased statural growth and adult height in patients with the EMG syndrome is still unknown.     

The Static Stiffness Index: A Simple Method to Assess the Elastic Property of Compression Material In Vivo

BACKGROUND: The efficacy of compression therapy depends mainly on the exerted pressure and on the stiffness of the material. OBJECTIVE: To propose a simple method by which pressure and stiffness can be assessed in the individual patient. MATERIAL AND METHODS: Using a pressure transducer (Kikuhime small probe, MediTrade, Soro, Denmark) the sub-bandage pressure is measured on the medial aspect of the lower leg at the transition of the gastrocnemius muscle into the Achilles' tendon. The pressure difference between active standing and lying is defined as the static stiffness index (SSI). Results: The accuracy and precision of the probe are good. Unna boot bandages (Lohmann-Rauscher, Vienna, Austria) and multilayer short-stretch bandages show a significantly higher SSI than long-stretch bandages and round-knitted class II stockings. The SSI values are lower than 10 mm Hg for elastic, long-stretch material and higher than 10 mm Hg for inelastic, short-stretch material. CONCLUSION: In future compression trials, pressure and stiffness measured in vivo should be declared.     

Hypothalamic-pituitary-gonadal function in two infants with Smith-Lemli-Opitz syndrome

We report on the hypothalamic-pituitary-gonadal function in 2 male infants with the Smith-Lemli-Opitz (SLO or RSH) syndrome. Both infants had abnormal external genitalia. Basal and LHRH stimulated plasma gonadotropins were normal for age (1 month). Plasma testosterone, androstenedione, and dehydroepiandrosterone sulfate were normal for age and sex. Some forms of congenital adrenal hyperplasia (17,20-desmolase deficiency, 17α-hydroxylase deficiency, and 3β-hydroxysteroid dehydrogenase deficiency) were ruled out by hormonal studies. The endocrinological findings indicate a normal hypothalamic-pituitary-gonadal function and a normal adrenal steroid biosynthesis in these 2 patients. A partial androgen receptor defect causing the genital malformations seems possible in one patient. Whether 5α-reductase deficiency is the cause of the male pseudohermaphroditism in SLO syndrome remains the subject of future studies. © 1992 Wiley-Liss, Inc.     

Head circumference of children with Williams-Beuren syndrome

Head circumference is considered an important parameter of brain growth and development. Syndrome-specific standards for head circumference in Williams-Beuren syndrome (WBS) are not available to date, although mental retardation is a leading manifestation in the syndrome. Therefore, we investigated head growth in 63 girls (251 measurements) and 88 boys (298 measurements) with WBS between birth and adulthood. Most measurements in both sexes were from the first 4 years of life (n = 162 in girls and n = 189 in boys). Mean (±SD) head circumference at birth was 33.39 ± 1.38 cm and 34.02 ± 1.44 cm for term girls and boys, respectively. Although head growth in WBS girls and boys was at a slower velocity, the pattern of head circumference was similar to that in the normal population. After the age of 3 months, head circumference started to fall below the normal mean in girls (0.5–2 cm). In boys, mean head circumference was below the normal mean already at 1 month of age (2 cm). The deficit increased to 3 cm from 6 months to 4 years. Adult OFC was 52.85 ± 1.75 cm (n = 16) compared to 55.70 ± 1.83 cm (n = 46; P < 0.00001) in WBS women and 55.51 ± 1.68 cm (n = 30) compared to 57.87 ± 1.29 cm (n = 31; P < 0.00001) in WBS men. During development, microcephaly is only seen in about one third of WBS patients. © 1994 Wiley-Liss, Inc.     

Compression and walking versus bed rest in the treatment of proximal deep venous thrombosis with low molecular weight heparin

OBJECTIVE: The purpose of this randomized controlled trial was to evaluate the benefits of compression and walking exercises in comparison with bed rest in the acute stage of proximal deep venous thrombosis (DVT). METHODS: Forty-five patients with proximal DVT that was proved with compression ultrasound scan or phlebography were randomized into three groups. Group A consisted of 15 patients who received inelastic compression bandages (Unna boots on the lower leg, adhesive bandages on the thigh), and group B consisted of 15 patients who received thigh-length compression stockings, class II. Group C consisted of 15 patients who underwent bed rest and no compression. All patients received dalteparin, 200 IU/kg per body weight, subcutaneously every 24 hours. The clinical characteristics of the three groups were comparable. Primary end points were the reduction of pain assessed daily with the Visual Analogue Scale and the Lowenberg test, the reduction of leg circumference at the ankle and calf levels, and the improvement of clinical scores. The daily walking distance was measured with a pedometer. Safety parameters were ventilation-perfusion scans and duplex ultrasound scans performed on days 0 and 9. RESULTS: The daily walking distance was between 600 and 12,000 m in the compression groups and averaged 66 m in the bed rest group. The pain level showed a statistically significant reduction starting after the second day in the compression groups (A and B) and after 9 days in the bed rest group C (P <.05). The same was true for the measurement of leg circumference. Improvement of the clinical scores was significantly better in the compression groups compared with the bed rest group (P <.01). There was no significant difference concerning the occurrence of new pulmonary emboli and regression of thrombus diameter. Progression of thrombi in the femoral vein was greater and occurred more frequently in the bed rest group than in the other two groups (P = not significant). CONCLUSION: Mobile patients with acute proximal DVT treated with low molecular weight heparin should be encouraged to walk with compression bandages or medical compression stockings. The rate of resolution of pain and swelling is significantly faster when the patient ambulates with compression. The risk of pulmonary embolism is not significantly increased by this approach.     

Adventitial stripping of the radial and ulnar arteries in Raynaud's disease

Adventitial stripping of the palmar arch, the palmar common digital arteries, or the proper digital arteries is a last resort in the treatment of refractory primary or secondary Raynaud's phenomenon. Seven patients who had adventitial stripping of the ulnar and radial arteries proximal to the wrist and resection of the nerve of Henle, if identifiable, are presented. All of them were evaluated by telethermography, acral rheography, and a questionnaire before and after surgery. All were asymptomatic after surgery with satisfactory healing of the ulcers at the fingertips. None of them relapsed during the follow-up time of 1.5 years.     

Repyloromyotomy for recurrent infantile hypertrophic pyloric stenosis after successful first pyloromyotomy

The authors report on a male infant aged 4[frac12] weeks who underwent a pyloromyotomy for hypertrophic pyloric stenosis. After an uncomplicated postoperative course with normal feeding and weight gain, projectile vomiting reoccurred. The boy underwent a repyloromyotomy for recurrent hypertrophic pyloric stenosis. The underlying cause of a recurrent hypertrophic pyloric stenosis after a successful pyloromyotomy may be explained by the natural history of the disease.