Growth in precocious puberty

Growth in precocious puberty is a subject of concern to families and clinicians alike. The definition of precocious puberty and the role of obesity in the age of onset have also been areas of debate since the Lawson Wilkins Society recommended a lowering of the age of onset of precocious puberty in US girls. An understanding of growth patterns in normal children with earlier or later onset of puberty and the variable rate of progression between individuals with central precocious puberty as well as the imprecision in available height prediction methods are important in assessing height outcomes in this condition. In the absence of randomised controlled trials in this area, only qualified conclusions about the effectiveness of interventions can be drawn. In general, it appears that height outcome is not compromised in untreated slowly progressive variants of central precocious puberty. In rapidly progressing central precocious puberty in girls, gonadotrophin releasing hormone agonists (GnRH agonists) appear to increase final height by about 5cm in girls treated before the age of eight, but there is no height benefit in those treated after eight years. Scanly data is available to assess treatment effects in boys. GnRH agonists appear to be relatively safe. The decision to treat central precocious puberty should take into account rate of progression of pubertal changes as well as biochemical markers and may need to address other factors (for example psychosocial and behavioural issues) as well as height outcome.     

Central precocious puberty and growth hormone deficiency in a boy with Prader-Willi syndrome

In Prader-Willi syndrome (PWS) hypothalamic dysfunction is the cause of hormonal disturbances, such as growth hormone deficiency (GHD), hypogonadism, and delayed or incomplete puberty. Only a few cases of central precocious puberty (CPP) have been reported. We describe an 8.8-year-old PWS boy, with microdeletion of chromosome 15q, who developed CPP. On admission, height was 131.1 cm (+0.17 SD), BMI 26.2 kg/m², pubic hair (Ph) 2, and testis 4.5 ml. We found increased growth velocity (7 cm/year), high testosterone levels, pubertal response to GnRH test, and advanced bone age (10.6 years). An evaluation of growth hormone (GH) secretion revealed a deficiency. Pituitary MRI was normal. LHRH analogue therapy (Leuproreline 3.75 mg/28 days i.m.) was started at 8.9 years and discontinued at 11.3 years, when the patient had bone age of 13 years. During therapy, growth velocity, testosterone, FSH, and LH peak decreased significantly, with no pubertal progression. Growth hormone therapy (0.24 mg/kg/week) was started at 9.5 years and discontinued at 15.3 years because the patient had bone age of 17 years. After interrupting LHRH therapy the patient demonstrated spontaneous pubertal progression with pubertal gonadotropin and testosterone. At 16.3 years, height was 170 cm (−0.48 SDS), BMI 36.3 kg/m², Ph 4, testis volume 10 ml and there was a combined hypothalamic and peripheral hypogonadism hormonal pattern (normal LH even with low testosterone and undetectable inhibin B with high FSH). To our knowledge this is the fourth male patient with genetically-confirmed PWS demonstrating CPP and GHD and the first with a long follow-up to young adulthood.     

Arachnoid cyst with growth hormone deficiency

On clinical grounds, arachnoid cysts are usually associated with neurological dysfunction. Little is known concerning their involvement in endocrine disorders. A seven-year-old boy was admitted to the hospital for evaluation of an unprovoked afebrile seizure. His neurological examination was normal, however, he had growth retardation. Insulin tolerance and L-dopa growth hormone stimulation tests revealed an inefficient growth hormone response. An MRI of hypophysis and cranium yielded a shift of hypophysis and a large arachnoid cyst.     

Treatment of children with central precocious puberty by a slow-release gonadotropin-releasing hormone agonist

A total of 82 patients (74 girls, 8 boys) are presently participating in an international multicentre trial for treatment of central precocious puberty (CPP) with a slow release gonadotropin-releasing hormone (GnRH) agonist depot preparation: Decapeptyl-Depot (DD). Of these patients, 53 (3 boys) were previously untreated (group 1) and 29 (5 boys) have been treated before with either a short-acting GnRH analogue or cyproterone acetate (group 2). Fifty-one patients (44 girls, 7 boys) were treated with DD for 12 months or more. Basal plasma luteinizing hormone (LH) levels decreased in both groups after 1 year of therapy. The LH response to intravenous GnRH was reduced in both groups. Basal plasma follicle stimulating hormone (FSH) levels decreased in both groups. Stimulated FSH levels were reduced in both groups after 1 year of DD treatment. Plasma oestradiol levels in the girls decreased to prepubertal levels in both groups. In all patients the clinical signs of precocious gonadarche such as breast development and menstruations (girls) and an increased testis volume (boys), did not further progress and sometimes regressed in several patients. Growth velocity decreased in the girls of group 1 from 9.0±0.72cm/year (mean±SEM) in the last half-year before treatment to 6.3±0.50 in the first half-year of treatment (P<0.01) and to 4.5±0.23 cm/year in the second half-year (P<0.01). After 12 months a stabilization of growth velocity was observed. The BA/CA ratio decreased during treatment in this group of girls, resulting in an improvement of adult height prediction from 161.9±3.3 cm (mean±SEM) at the start to 164.1±3.5cm after 18 months of therapy (P<0.05). No change of height prediction was observed in group 2. At present we consider one i.m. injection of DD every 4 weeks as the treatment of choice in children with CPP.On behalf of the Dutch-German Precocious Puberty Study Group. The clinical investigators participating in the internnational multicentre trial of Decapeptyl-Depot in children with central precocious puberty are: J. Brämswig, Münster; H. Dörr, München; S.L.S. Drop, Rotterdam; M. Gons, Amsterdam; A. Grüters, Berlin; P. Heidemann, Göttingen; U. Heinrich, Heidelberg; U. Irle, Bremen; M. Jansen, Utrecht; K. Kruse, Würzburg; U. Kuhnle, München; R. Mühlenberg, Krefeld; K.E. Mühlendahl von, Osnabrück; R.J.H. Odink, Amsterdam; W. Oostdijk, Leiden; A. Otten, Giessen; B.J. Otten, Nijmegen; W. Petrykowski, Freiburg; C. Rouwe, Groningen; K. von Schnakenburg, Bonn; W. G. Sippell, Kiel; H. Stolecke, Essen; H.U. Tietze, Nürnberg; J.J.J. Waelkens, Eindhoven; W. Weltersbach, Köln; J. Wiebel, Hamburg     

Auxological and endocrinological 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 and pathophysiology have not been well characterized. In this study, 108 patients aged between 3 and 17.8 years were evaluated. Growth was documented on the basis of arm span measurements. Short arm span was found 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 and −1.4 SDS (−5.6 to +1.3) in 65 boys. When growth deficiency (more than −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 followed. Levels of serum insulin-like growth factor and insulin-like growth factor binding protein-3 were low in 22 of 62 (33.8%) patients. In 9 of 62 (14.5%) patients, insulin-like growth factor-1 and insulin-like growth factor binding protein-3 levels were found to be increased. Growth hormone (GH) deficiency was diagnosed by means of two different stimulation tests (clonidine and arginine) in 7 of 62 (11.3%) patients. In another 3 of 62 (4.8%) children, 12 h night time GH sampling showed low maximum peak levels and decreased area under the curve values, suggesting neurosecretory GH dysfunction. Precocious puberty or early onset of puberty associated with elevated luteinising hormone and follicle stimulating hormone concentrations after stimulation with luteinising hormone releasing hormone was found in 13 of 108 (12.0%) patients (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%) tested patients. Conclusions Short arm span in children with hydrocephalus and/or MMC is frequently accompanied by GH deficiency or neurosecretory GH dysfunction. Early onset of puberty is another frequent finding. Both hormonal disorders may be the consequence of damage to the hypothalamus or the pituitary gland caused by increased intracerebral pressure or increased mass of cerebrospinal fluid. Received: 31 May 1996 / Accepted: 21 October 1996     

Treatment of central precocious puberty with an intranasal analogue of GnRH (Buserelin)

One boy and 13 girls with central precocious puberty were treated for 1 year using Buserelin, a GnRH analogue, given intranasally (0.3 mg, four times a day). After 1, 3 and 12 months of therapy, the gonadotropin responses to GnRH were abolished in all the patients whereas mean basal serum concentrations of luteinizing hormone (LH) remained similar to those of pubertal controls. During Buserelin treatment, genital development in the boy and breast development in the girls showed no further progress or some regression. In the boy, serum testosterone levels returned to prepubertal values. In the girls, serum oestradiol levels were variable and, in four of them, vaginal smears showed the persistence of a slight oestrogenic effect during therapy. Pelvic ultrasonography did not show any significant variation in ovarian and uterine lengths. Among the 14 patients, 3 had some progression of pubic hair development, irrespective of serum dehydroepiandrosterone sulphate (DHEAS) levels. In eight patients previously treated with cyproterone, elevated prolactin levels were observed before and during the first month of Buserelin administration. During treatment, mean height velocity was markedly reduced from 11.6 to 6.1 cm/year and mean bone age velocity (±1 SD) was 0.85±0.38 year/year. After 1 year of treatment, the differences in predicted adult height ranged between −0.74 and +1.04 SDS (standard deviation score). These differences were inversely related (r=−0.72) to the prognosis of adult height calculated before treatment. We conclude that, in central precocious puberty, intranasal administration of Buserelin, 1.2 mg/day, may arrest sexual development and reduce height velocity and bone maturation. Improvement of adult height prognosis may occur, especially when it was markedly impaired before treatment.     

儿童颅内蛛网膜囊肿的手术探讨

目的：探讨儿童颅内蛛网膜囊肿的手术适应证和手术方法。方法：回顾我院52例手术患儿的临床资料，对不同手术方式进行总结，结果：采用4种手术方式，其中囊肿切除术17例，囊肿切除并脑池开放手术21例，囊肿－腹腔分流术8例，脑立体定向手术6例，术后随访3个月以上，症状改善率86．5％，CT证实囊肿缩小者76．8％，不同术式比较，囊肿切除并脑池开放手术效果良最好，其症状改善率达90．5％，囊肿缩小者占80．7％，结论：对儿童颅内蛛网膜囊肿应采取积极的手术治疗，手术方式首先囊肿切除并脑池开放手术。     

The effect of treatment with an LH-RH agonist (Buserelin) on gonadal activity growth and bone maturation in children with central precocious puberty

Twenty-five children (23 girls and 2 boys) with central precocious puberty were treated with the LH-RH agonistd-Ser (TBU)⁶-LHRH (1–9) EA (HOE) 766, Buserelin) by daily subcutaneous injection for a period of 11–18 months. Eight girls and 2 boys previously treated with cyproterone acetate (CPA, 100–150 mg/m² body surface per day) and the first seven newly diagnosed patients received 2×10 g Buserelin/kg bodyweight per day for 1 week, followed by a maintenance therapy of 1×10 g/kg per day. After an initial marked increase, oestrogen (E₂) serum levels in girls and testosterone (T) values in boys decreased. After a treatment period of 6–20 weeks the patients received 2×20 g Buserelin/kg per day for 1 week and thereafter a maintenance dosage of 20 g/kg per day to obtain full suppression (i.e. E₂<50 pmol/l; T<1 nmol/l). The remaining eight patients started directly on 2×20 g Buserelin/kg per day followed by 1×20 g/kg per day.All eight girls with menarche before therapy had no further menses. In all girls there was a reduction of palpable breast tissue. Decrease of pubic hair development was observed in 3 girls, an increase was seen in 5 girls, whereas in the remaining 15 girls no change was observed. Both boys had a reduction of testicular volume and of pubic hair. The basal growth rate during the half year preceding Buserelin treatment was elevated in the newly diagnosed patients as compared to the growth rate of the patients previously treated with CPA and to the median growth rate of healthy children.During the first 6 months of treatment the mean growth rate decreased slightly in the patients who had received no previous treatment and increased in the patients previously treated with CPA. During the subsequent year a marked fall of the mean growth rate was observed. Paired comparison of the mean SDS (height/chronological age and height/bone age) and of the mean final height prediction (according to Bayley and Pinneau) of 15 children treated for 18 months revealed no significant change.We conclude that during an observation period of 18 months the daily subcutaneous administration of the LH-RH agonist Buserelin has been a safe and effective method of selectively inhibiting gonadal activity in children with central precocious puberty. With prolonged treatment an improvement of the final height prediction is expected.Abbreviations LH-RH Luteinizing-hormone releasing hormone - CPA cyproterone acetate - SHBG sex hormone-binding globulin - DHEAS di-hydroepiandrosterone sulphate - RUS radius, ulna, short bone - SDS h/ca standard deviation score for chronological age - SDS h/ba SDS for bone age     

儿童椎管内硬脊膜外蛛网膜囊肿的外科治疗

目的 探讨儿童椎管内硬脊膜外蛛网膜囊肿的临床特点及其诊治.方法 回顾性分析2001年1月至2009年8月我科收治的4例儿童椎管内硬脊膜外蛛网膜囊肿.其中颈胸段1例,胸腰段3例.临床表现3例患儿出现运动障碍,1例有感觉障碍,1例有小便失禁,1例有根痛表现.4例患儿均采用外科手术治疗,其中2例囊肿节段小于3个椎体的采用椎板切除+囊肿切除术,2例囊肿节段大于3个椎体的采用椎板成形+囊肿切除术.结果 4例患儿术后均恢复良好,除1例患儿术后随访1年下肢仍有轻度乏力外,其余临床症状均消失,恢复学习.随访1～9年均未见囊肿复发,复查脊柱三维CT未见脊柱畸形的发生.结论 儿童椎管内硬脊膜外蛛网膜囊肿是一种少见疾病,外科手术治疗能获得满意的结果,椎板成形术能够减少囊肿节段过长的患儿术后脊柱畸形的发生.

Abstract：

Objective To review the clinical features and surgical treatment of spinal extradural arachnoid cysts (SEACs) in children. Methods Four children with SEAC from were admitted to our department between January,2001 to August,2009. One SEAC was located at the cervicothoracic spine whereas the other three were found in the thoracolumbar spines. All cases were treated by surgery.Two children underwent the laminectomy for resection of the SEAC, which occupied less than 3 segments. SEAC, spreading more than 3 segments in the remaining cases, were treated by laminoplasty and en bloc resection. Results Postoperatively, the children had good recovery and resumed school. Followup of 1 to 9 years revealed no recurrences. The three-dimensional CT spine did not demonstrate any spine deformity in follow-up study. Conclusions SEACs in children are rarely reported in the literature. Excision of the SEAC result in a favorable outcome. The larninoplasty can reduce spinal deformity in patients with the long segment SEAC.     

小儿侧裂池蛛网膜囊肿伴发硬膜下血肿的手术治疗

目的 探讨小儿侧裂池蛛网膜囊肿伴发硬膜下血肿的临床特点、手术适应证和术式选择.方法 回顾性分析2005年7月至2015年8月,中国医科大学附属第一医院神经外科收治的50例小儿侧裂池蛛网膜囊肿伴硬膜下血肿患儿的临床资料,其中亚急性硬膜下血肿者20例,慢性硬膜下血肿者30例,所有患儿均行血肿清除、显微囊肿切除、脑池沟通术,术中尽可能全切囊肿壁,并与蛛网膜下腔、脑池相沟通.结果 所有患儿手术过程均较顺利,术后未出现严重并发症.患儿术后原有症状及影像学表现均有不同程度的改善,平均随访5.7年无复发病例.结论 小儿侧裂池蛛网膜囊肿可引发硬膜下血肿,一旦发生,适合行显微囊肿切除、脑池沟通、血肿清除术.     

Growth and endocrine disorders in optic glioma

Hypothalamo-pituitary function in children with optic glioma may be impaired by the tumour itself and by the high cranial radiation doses used in treatment. This study evaluates the effect of optic glioma and its treatment on patient growth and pubertal development. Twenty-one patients (13 boys, 8 girls), treated for optic glioma by cranial irradiation (45–55 Grays) at a mean age of 5.4 years, were evaluated before (n=10) and/or after (n=21) irradiation. Growth hormone (GH) deficiency was present in only 1 patient tested before irradiation and in all patients after irradiation. Precocious puberty occurred in 7/21 cases, before irradiation in 5 patients and after irradiation in 2 patients. The cumulative height loss during the 2 years after irradiation was 0.2±0.2 SD (m±SEM) in 7 patients with precocious puberty and 1.1±0.2 SD in 14 prepubertal patients (P<0.01). The corresponding bone age advance over chronological age, evaluated 1–3 years after irradiation, was 1.1±0.5 and –0.7±0.3 year in the two groups (P<0.01). The mean height loss between time of irradiation and the final height was 2.3±0.6 SD (n=6). Primary amenorrhoea, associated with low oestradiol levels, occurred in two of the three girls of pubertal age. These data indicate that the high dose of cranial radiation used to treat optic glioma invariably results in GH deficiency within 2 years and that hGH therapy is required when GH deficiency is documented. Precocious puberty, resulting in apparently normal growth velocity in spite of GH deficiency, should be treated with luteinizing hormone-releasing hormone analogues because of the risk of accelerated bone maturation and reduced final height.     

Growth hormone secretion, puberty and adult height after cranial irradiation with 18 Gy for leukaemia

The dose of prophylactic cranial irradiation given to patients for acute lymphoblastic leukaemia has been decreased from 24 to 18 Gy, but the beneficial effect of this decrease on growth is controversial. This study compares the growth hormone (GH) secretion and growth of 35 patients (20 boys) given 18 Gy at 3.7 ± 0.3 (SE) years, and routinely evaluated 5.4 ± 0.4 years after irradiation to define the indications for GH treatment in these patients. Of these, 63% had a low GH peak (<10 μg/l) after one (22 cases) or two (17 cases) stimulation tests. The plasma concentrations of insulin-like growth factor I and its GH-dependent binding protein were normal for age in all but two cases. The height changes between irradiation and evaluation were correlated with the GH peaks (P < 0.03) and were concordant, except in patients with early puberty. This occurred in 16 patients including all 12 girls irradiated before 4 years of age. A significant (P < 0.03) reduction in height (SD) between irradiation and adult height occurred in untreated GH-deficient patients (−1 ± 0.3, n = 6), but not in GH-deficient patients given GH (−0.6 ± 0.3, n = 8) or in those with normal GH peak (−0.4 ± 0.3, n = 7). Conclusion In children irradiated for acute lymphoblastic leukaemia, GH deficiency is frequent after 18 Gy but its impact on adult height is smaller than after higher doses. We suggest that the indications for gonadotropin releasing hormone analogue therapy should be broad in patients with early or rapidly progressing puberty and those for GH therapy in those patients with a below average constitutional height before irradiation. Received: 17 November 1997 / Accepted: 9 February 1998     

The effect of cyproterone acetate on adrenal cortical function in children with precocious puberty

8 children with precocious puberty were treated with cyproterone acetate (CPA). During treatment there were no definite clinical signs of depressed adrenocortical function. The plasma cortisol concentrations were grossly depressed and the diurnal cortisol rhythm was abolished. Two months after discontinuation of CPA treatment the adrenocortical function had greatly improved. The lysin-vasopressin stimulation test revealed in one child a normal, in another child an exaggerated ACTH response during CPA therapy. Fasting plasma ACTH concentrations were elevated compared with normal controls, but they were very low compared with patients with Addison's disease. The results suggest that CPA has a twofold effect leading to adrenocortical insufficiency: i.e., inhibition of cortisol secretion by the adrenals themselves and inhibition of ACTH secretion at the hypothalamo-pituitary level.Dedicated to Professor Dr. Horst Bickel, Heidelberg, on the occasion of his 60th birthday anniversaryPresented in part at the International Symposium of Androgens and Antiandrogens, Milan, April 26–28, 1976     

促性腺激素释放激素类似物联合重组人生长激素对中枢性性早熟女童身高的影响

目的 研究促性腺激素释放激素类似物（GnRHa）与重组人生长激素（rhGh）联合治疗以及GnRHa单用对骨龄≥10岁的特发性中枢性性早熟（ICPP）女童成年身高的改善情况。方法 将6个医学中心确诊为ICPP符合研究条件的80例女童（年龄9.0±0.7岁,骨龄≥10岁）根据治疗方法分为GnRHa与rhGh联合治疗组（31例）及GnRHa单用组（49例）。观察治疗前后的预测成年身高、接近成年身高和身高净获等各项指标的变化。结果 两组在治疗后按骨龄的身高标准差分值均较治疗前有显著改善（P<0.01）,其中GnRHa与rhGh联合治疗组明显优于GnRHa单用组（P<0.01）。联合用药组接近成年身高（157±6 cm vs 157±4 cm）、身高净获（4.68 cm vs 3.89 cm）、停药时预测成年身高（161±5 cm vs 158±5 cm）、接近成年身高与遗传靶身高差值等指标均略高于GnRHa单用组,但差异无统计学意义（均P >0.05）。结论 GnRHa与rhGh联合治疗或GnRHa单用组均能改善骨龄≥10岁ICCP女童的成年身高,但两药联用优势不明显。对ICPP患儿预测成年身高的评估需要慎重,停药时的预测值偏高。     

Recent advances in the diagnosis and treatment of precocious puberty

In the last two decades, the diagnosis and treatment of precocious puberty has undergone important changes. The use of supersensitive assays to determine gonadotropins and gonadal hormones has increased the sensitivity and decreased the number of blood samples required to assess the diagnosis. The introduction of gonadotropin-releasing hormone (GnRH) agonists produced a revolution in the diagnosis and treatment of this disorder. Recently, the use of long acting GnRH agonists improved the adherence of patients to medical treatment and decreased the need for uncomfortable repeated doses. The medications in the treatment of the GnRH independent causes of precocious puberty, and the important revelations in the pathophysiology of these disorders, have advanced our knowledge and management of the affected children.     

The effect of gonadotrophin releasing hormone analogue on height prognosis in growth hormone deficiency and normal puberty

We have treated seven pubertal children, five (three female, two male) with growth hormone deficiency and two (one female, one male) with constitutional short stature with intranasal (D-Serine⁶) gonadotrophin releasing hormone (GnRH) analogue (Buserelin) for a mean of 0.84 years (range, 0.5–1.3). Treatment was successful in arresting pubertal development but there was no improvement in final height prognosis.Abbreviations GnRH gonadotrophin releasing hormone - GH growth hormone     

Final height after combined growth hormone and GnRH analogue treatment in adopted girls with early puberty

Background: Girls adopted from developing countries often have early or precocious puberty, requiring treatment with gonadotrophin-releasing hormone (GnRH) analogues. During such treatment, decreased growth velocity is frequent. Aim: To study whether the addition of growth hormone (GH) to GnRH analogue treatment improves final height in girls with early or precocious puberty. Methods: Forty-six girls with early or precocious puberty (age ≤9.5 y) adopted from developing countries were randomized for treatment for 2-4 y with GnRH analogue, or with a combination of GH and GnRH analogue. Results: During treatment, the mean growth velocity in the GH/GnRH analogue group was significantly higher compared to the control group. Combined GH/GnRH analogue treatment resulted in a higher final height: 158.9 cm compared to 155.8 cm in the GnRH analogue-treated group. Three out of 24 girls (13%) in the combined group and nine of the 22 girls (41%) treated with GnRH analogue alone attained a final height below -2 standard deviation scores (SDS).

Conclusion: The difference between the two groups is statistically significant, and possibly of clinical importance. A future challenge is to identify a subgroup with clinically significant advantage of GH addition to GnRH analogue treatment. Being very short on arrival in Sweden and being short and young at start of treatment are possible indicators.     

Effectiveness of growth hormone (GH) therapy in GH-deficient children and non-GH-deficient short children

The growth response during short-term growth hormone (GH) treatment was evaluated in eight prepubertal non-GH-deficient (non-GHD) children and compared with six prepubertal GH-deficient (GHD) patients. Standard doses of GH can improve growth rate in GHD and in some non-GHD patients. In neither group the growth response can be predicted by the acute increase in Thymidine Activity or Somatomedin-C levels. A diagnostic trial of GH treatment may be the only certain method of selecting the short non-GHD patients who may benefit from long-term GH therapy.Abbreviations GH growth hormone - GHD growth hormone deficient - Sm-C somatomedin C - TA thymidine activity     

Growth and sexual development in children with meningomyleocoele

Forty-five children (25 girls; 20 boys) with meningomylcocoele (MMC) were assessed for growth, skeletal maturation and pubertal development. The spinal defects were operated on shortly after birth and all children required cerebral drainage for hydrocephalus.Standard deviation scores for height, sitting height, subischial leg length, head circumference, weight and bone age were compared with aged-matched data from a normal Swiss population. The children with MMC were shorter (height SDS boys -2.34±1.57; girls -2.01±1.57, mean±SD, P<0.0005), secondary to a decrease in trunk and lower limb length: Arm length was normal. The head circumference was increased, significantly in the girls (SDS+1.49±1.21, P<0.005). Weight was decreased in both sexes, this being significant in the boys (SDS -1.11±1.23, P<0.005); however, comparison of weight with height suggested that children with MMC were relatively obese. Bone age was significantly advance in both boys (SDS+1.07±2.13, P<0.025) and girls (SDS+1.36±1.77, P<0.0001). Secondary sex characteristics appeared early in both boys and girls and one girl and one boy presented with puberty advanced by several years. Cryptorchidism occurred in 25% of boys with MMC.Abbreviations MMC meningomyleocoele - H height - SH sitting height - AL arm length - W weight - SILH subischial leg height - BA bone age - SDS standard deviation scores