Primary empty sella: differences and similarities between children and adults

To identify possible differences between empty sella in children and adults we studied 43 subjects (age 13.6 ±5.4 years, range 4.1–27 years) with hypothalamic-pituitary disorders and empty sella at magnetic resonance imaging. Pituitary function, presence of non-endocrine symptoms, perinatal history, sellar volume, pituitary height, midline or intrasellar anatomical abnormalities were evaluated. Twenty subjects had isolated growth hormone deficiency, 17 multiple pituitary hormone deficiency and 6 puberty disorders (3 precocious puberty, 2 idiopathic delayed puberty, 1 Kallmann syndrome). The group with multiple pituitary hormone deficiency had a higher percentage of subjects with complete empty sella, i.e pituitary height < 2 mm (p = 0.016), or intrasellar anatomical abnormalities (p = 0.0002) than the other groups. The subjects with puberty disorders had a mean sellar volume higher than the other groups (p < 0.05). Apart from pituitary dysfunction, symptoms of the empty sella syndrome were infrequent (9.3% of cases) in our subjects. The age of our subjects, the frequent association between empty sella and pituitary dwarfism and the non-enlarged sellae suggest a different aetiology, perhaps congenital, for empty sella in our subjects. As in adults, empty sella may be associated with both pituitary hypo- and hyperfunction.     

空蝶鞍患儿的垂体激素改变及治疗剂量观察

目的探讨空蝶鞍（ES）患儿垂体激素的改变及激素替代治疗（HRT）的剂量。方法测定1999年9月～2006年3月以生长迟缓就诊的20例ES患儿（经下丘脑-垂体MRI确诊）垂体激素,并与年龄匹配的正常对照组进行比较;同时观察HRT的合适剂量。结果20例中18例有难产史,所有患儿均伴有多种垂体激素缺乏症（MPHD）：ES患儿FT4和F显著低于对照组,其中12例〉10岁的男性患儿FSH、LH、T均显著低于对照组（P〈0.001）,E2和PRL均显著高于对照组,6例禁水加压素试验和MRI检查证实为垂体性尿崩症。相应HRT剂量：重组人生长激素为（0.11±0.01）U/（kg·d）,左旋甲状腺素钠（1.6±0.7）μg/（kg·d）,氢化可的松（10.2±2.4）mg/m^2,弥凝（0.11±0.03）mg/m^2。中枢性甲减患儿甲状腺素用量小于先天性甲减患儿。结论ES患儿常伴MPHD,需全面激素替代治疗才能使其安全生长。有难产史或MPHD表现者应常规做下丘脑垂体MRI,以了解其形态结构改变,协助治疗及改善预后。     

Primary empty sella turcica in children

The authors describe cranio-facial deformities found in two sisters and associated with spinal anomalies, short stature and delayed skeletal maturation. The principal radiological features were an enlarged J-shaped sella turcica and intrasellar cisternal herniation. Enlargement of optic foramina and internal acoustic canals were also present. These asymptomatic cases of empty sella seem to be part of a general dysplastic syndrome rather than a local disease.     

Empty sella syndrome, panhypopituitarism, and diabetes insipidus

We present an 18-month-old girl with short stature, obesity, panhypopituitarism, diabetes insipidus, and visual defects. Postmortem examination revealed brain atrophy due to a diffuse encephalopathy, numerous calcified neurons in cerebral cortex, deep telencephalic and diencephalic nuclei, diffuse neuronal necrosis in hypothalamic nuclei, moderate atrophy of optic nerves, very thin hypophyseal stalk, and empty sella with the hypophysis compressed to the dorsal aspect of the concavity. Our hypothesis is that the presence of an empty sella in a child with hypophyseal-hypothalamic abnormalities should alert physicians to the existence of hypothalamic lesions secondary to a perinatal insult. We discuss the possible pathogenesis of these findings as well as lines of evidence available in the literature.     

The "empty sella" in childhood

The "empty sella" (ES), a common entity in adults, is so named because a cerebrospinal fluid-filled arachnoid hernia fills the pituitary fossa and compresses the pituitary gland, creating the illusion of an "empty" sella. We report our experience of ES in childhood. Our four cases represent associations with ES that have not been previously described in childhood: case 1 was associated with central precocious puberty; case 2 with secondary ES resulting from shrinkage of a prolactinoma during bromocriptine therapy; case 3 with pseudotumor cerebri; and case 4 with no significant pathology. Together, they account for approximately 1 percent of cases of radiographically enlarged sella tursica investigated here. This brings the number of cases of ES reported in children to 27. Review of these 27 cases reveals seven with local skull dysplasia and/or a familial inheritance. The remainder lacked clinical homogeneity or similarity to the common adult variety of ES. Children with ES did not exhibit the higher female preponderance reported in adults with ES. They showed a higher frequency of secondary cases (4/27), and of associated endocrine (10/15) and visual (8/17) abnormalities than is seen in adults with the "empty sella."     

Association of Bartter's syndrome and empty sella

Bartter's syndrome is characterized by hypochloremia, hypokalemia, metabolic alkalosis associated with renal potassium leakage, and normal blood pressure despite increased plasma renin activity. Although association of empty sella with Gitelman syndrome has been reported, no association has been reported with Bartter's syndrome. Here we report a patient with Bartter's syndrome and empty sella. A 12 month-old male patient presented with a history of nausea, vomiting, abdominal distension, constipation, and edema in the lower extremities that had begun in the early postnatal period. The patient was born at 32 weeks gestation by operative delivery for polyhydramnios. Blood pressure was normal. Serum sodium, potassium, calcium, phosphate, chloride, albumin and alkaline phosphatase levels were 129 mEq/l, 2.5 mEq/l, 9 mg/dl, 3.8 mg/dl, 72 mg/dl, 4.2 g/dl and 1285 IU/l, respectively. Serum magnesium level was normal. Arterial blood gas levels revealed pH 7.55 (normal, 7.35-7.45), PCO2 33.6 mm/Hg (36-46), base excess +7.1 (+/- 2.3), and total CO2 33.6 mmol/l (23-27). Renin and aldosterone levels were elevated. Urine had pH 8.0 and specific gravity 1.010. Urinary calcium excretion was 22.8 kg/day (urine calcium/creatinine ratio 0.46). Urinary potassium and chloride levels were elevated. MRI of the brain was normal except for partially empty sella. We present the first pediatric patient with the association of Bartter's syndrome and empty sella.     

Hypothalamic-pituitary dysfunction in primary empty sella syndrome in childhood

In a series of 37 consecutive CT scans performed in children referred to our pediatric endocrine unit, an empty (eight) or partially empty (one) sella turcica was found in nine (24%) patients with short stature or delay in sexual maturation, precocious puberty, or hypoparathyroidism. The size and contour of the sella were abnormal in only three patients. Five of the nine children had evidence of decreased growth hormone secretion as determined by subnormal GH secretory responses to provocative tests (peak GH concentration less than 7 ng/ml) or assessment of endogenous 24-hour GH secretion (mean 24-hour GH concentration less than 3 ng/ml). Two children had multiple pituitary hormone deficiencies. Although primary empty sella syndrome was often associated with hypothalamic-pituitary dysfunction in this series, the prevalence of an empty sella in normal children is unknown. Further identification and evaluation of children with empty sella may provide new information regarding the cause of pituitary dysfunction in childhood.     

Primary empty sella syndrome in childhood: association with precocious puberty

Primary empty sella syndrome has been considered an infrequent finding in childhood. One hundred and twelve cases have been reported in children ages 0.7 to 18 years. The frequency of primary empty sella ranged from 1% to 58%. Endocrine abnormalities were described in nearly all children while visual abnormalities were noted in only 6%. Growth hormone deficiency was the single most common hormonal dysfunction noted in children with primary empty sella. Precocious puberty has been rarely reported in association with primary empty sella. We report the case of a 7-1/2 year old girl with gonadotropin dependent central precocious puberty and a partially empty sella who had no other hypothalamic-pituitary dysfunction. We suggest that pubertal abnormalities be included among the endocrine disorders potentially associated with the primary empty sella syndrome.     

Empty sella in short children with and without hypothalamic-pituitary abnormalities

A study was conducted on growth hormone (GH) response to oral clonidine (0.15 mg/m²), GH and cortisol responses to i.m. glucagon (0.1 mg/kg), and glucose response to an oral load of glucose (1.75 g/kg). Measurements were made on the circulating concentrations of free thyroxine (FT4), thyroid stimulating hormone (TSH) and different growth parameters and CT sellar images in 25 GH deficient children (Peak GH response to clonidine and glucagon<7 ug/ml), 15 growth retarded children (Ht<5th percentile for age and gender) with sickle cell disease (SCD) and GH deficiency, 30 randomly selected children with normal variant short stature (NVSS) (HtSDS 2SD below the mean for age and gender with normal GH response to stimulation (>10 ug/ml) and 20 age-matched normal children were evaluated. Out of the 25 children with GH deficiency, five had multiple pituitary hormonal deficiency (GH<TSH and/or ACTH. deficiencies), and 20 had isolated GH deficiency. Empty sella, either complete or partial, was detected in 9 out the 20 children with isolated GH deficiency (45%), 4 out of the 5 children with multiple pituitary deficiency (80%), all the children with SCD and GH deficiency (100%), 3 out of the 30 children with NVSS (10%) and in none of the normal children. The insulin-like growth factor-1 (IGF-I) concentrations were significantly lower in the two groups of children with GH deficiency compared to those with NVSS. The height standard deviation scores (HTSDS) were significantly lower and the annual growth velocity was slower in children with idiopathic GH deficiency and empty sella compared to those with NVSS and those with empty sella associated with SCD. The bone age delay (yr) did not differ among the 3 groups of children with short stature. All children with isolated GH deficiency associated with empty sella had normal body mass indices (BMI), while all the children with SCD and empty sella had BMI below the 5th percentile for the corresponding age and gender. None of the children had glucose intolerance. In conclusion, children with growth retardation and abnormal hypothalamic pituitary functions have high incidence of empty sella. However, empty sella is detected in considerable number (10%) of short children with normal hypothalamic pituitary function.     

The primary empty-sella syndrome and diabetes insipidus in a child

The empty-sella syndrome is uncommon in pediatric patients. Hypothalamic-pituitary dysfunction is common in these patients but involvement of the posterior pituitary gland is very rare. We report a seven-year-old girl with empty-sella syndrome who first developed arginine-vasopressin deficient diabetes insipidus and then anterior pituitary gland deficiency. The empty-sella syndrome should be included among the causes of arginine-vasopressin deficient diabetes insipidus in pediatric patients.     

Primary nephrotic syndrome during childhood in Turkey

BACKGROUND: Minimal change nephrotic syndrome (MCNS) is the most common cause of primary nephrotic syndrome (NS) during childhood. However, recent studies from different countries have reported an increasing incidence of focal segmental glomerulosclerosis (FSGS) in children. METHODS: This is a retrospective study in which 392 Turkish children who were diagnosed with NS during the last 10 years and were followed for at least 2 years, were evaluated. Mean age of the study group was 4.6 +/- 3.4 years (range 0.9-16 years) and 232 were male and 160 were female. RESULTS: In total, 280 patients were diagnosed as MCNS with their initial presentations, laboratory features, and clinical course. Kidney biopsy was performed in the remaining 112 children according to current recommendations. The results showed that membranoproliferative glomerulonephritis (MPGN) was the most common histopathologic diagnosis, 38 (34%) of the 112 patients were found to have MPGN. The number diagnosed as FSGS was 26 (23%). A significant difference was found between the age groups for both MPGN and FSGS, the former being more common in children >6 years of age and the latter more frequent in children 相似文献   

The sella in childhood hypothyroidism

Two sellar configurations have been noted in primary childhood hypothyroidism. In the older child the sella appears unusually round and slightly enlarged, and the term cherry sella has been used to describe its appearance. Enlargement is due to rebound hypertrophy of the pituitary gland and is reversible with adequate, early treatment. If reversal of these changes is not accomplished one should consider the possibility of an adenoma having developed. In the young child and infant the sella appears more immature and bowl-like. Enlargement is not a prominent feature in this age group, and indeed was difficult to detect with certainty. Both configurations, but especially the cherry sella of the older child, are characteristic enough to enable one to suggest the diagnosis of primary hypothyroidism from lateral skull films.     

Psychocutaneous disorders in childhood and adolescence

The adverse impact of skin diseases on an individual's physical and emotional well-being is well recognised, particularly in children as it is reflected in their growth and development. Conversely, the psychological and psychosocial issues that a child or young person may face, can manifest as a skin problem. Cutaneous manifestations of an underlying primary psychiatric problem are a poorly recognised entity amongst paediatricians. Limited knowledge and experience in this realm increases diagnostic and management challenges. The discipline of Psychodermatology attempts to address these problems by relating skin disease to intrinsic and extrinsic mental health factors. This review summarises the current literature on Psychocutaneous conditions with expert opinion from the authors own experience. The importance of a holistic approach in a multidisciplinary setting to manage this vulnerable group of children and young people cannot be over-emphasised.