The aim of this retrospective study was to survey the spectrum of oral tumors and tumor-like lesions treated in a pediatric surgical unit. The clinical features and treatment outcome are presented, and guidelines for management discussed. Long-term follow-up was carried out both by re-examination and by means of a questionnaire. A total of 95 patients were encountered over a 30-year period. The age at presentation ranged from 1 day to 16 years, and the male to female ratio was 0.7:1. The lesions were located predominantly on the lips (22%), tongue (21%), and cheek (19%). Patients were divided into five groups based on histological diagnosis. Benign lesions accounted for 83 (87%) of the cases. Of these, 41 (43%) were benign tumors, the most common of which were the hemangiomas (17 cases). Hamartomas accounted for a further 22 benign lesions (23%), among which 12 were lymphangiomas. Furthermore, we saw 14 cases (15%) of mucoceles, ranula and dysontogenetic cysts, and a further 6 cases (6%) were classed as miscellaneous lesions. Simple surgical resection was successful in treating most benign lesions, with occasional re-excision being necessary in lymphangiomas. The long-term effects of treatment include reduction of the red volume of the lips, scarring following resection of parotid hemangiomas, a forked tongue after wedged resection, and partial facial nerve palsy. The group of 12 (13%) malignant tumors consisted of 5 rhabdomyosarcomas, 2 fibrosarcomas, 2 carcinomas of the parotid, 1 osteosarcoma, and 2 metastases. A multimodal approach was used in patients with rhabdomyosarcomas, while fibrosarcomas and parotid carcinomas were normally treated by surgical excision. Six of 12 patients with malignant tumors were alive after a median follow-up of 20.5 years. Re-examination of the malignant tumor group revealed scarring, impaired growth and function of the maxilla associated with local irradiation, and an external salivary fistula. In conclusion, while most oral and maxillofacial tumors of children are benign, malignant tumors of soft tissue, salivary glands and bones must be taken into account. There are specific aspects related to certain developmental and biological characteristics that make a mainly conservative approach preferable in these children.

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The level of fatness at which morbidity increases is determined on an acturial basis. Direct measurements of body fat content, eg hydrodensitometry, bioimpedance or DEXA, are useful tools in scientific studies. However, body mass index (BMI) is easy to calculate and is frequently used to define obesity clinically.An increased risk of death from cardiovascular disease in adults has been found in subjects whose BMI had been greater than the 75th percentile as adolescents. Childhood obesity seems to increase the risk of subsequent morbidity whether or not obesity persists into adulthood.The genetic basis of childhood obesity has been elucidated to some extent through the discovery of leptin, the ob gene product, and the increasing knowledge on the role of neuropeptides such as POMC, neuropeptide Y (NPY) and the melanocyte concentrating hormone receptors (MC4R). Environmental=exogenous factors contribute to the development of a high degree of body fatness early in life. Twin studies suggest that approximately 50% of the tendency toward obesity is inherited. There are numerous disorders including a number of endocrine disorders (Cushing's syndrome, hypothyroidism, etc) and genetic syndromes (Prader -Labhard -Willi syndrome, Bardet -Biedl syndrome etc) that can present with obesity.A simple diagnostic algorithm allows for the differentiation between primary or secondary obesity. Among the most common sequelae of primary childhood obesity are hypertension, dyslipidemia and psychosocial problems.Therapeutic strategies include psychological and family therapy, lifestyle=behavior modification and nutrition education. The role of regular exercise and exercise programs is emphasized. Surgical procedures and drugs used as treatments for adult obesity are still not recommended for children and adolscents with obesity. As obesity is the most common chronic disorder in the industrialized societies, its impact on individual lives as well as on health economics has to be recognized more widely.This review is aimed towards defining the clinical problem of childhood obesity on the basis of current knowledge and towards outlining future research areas in the field of energy homoeostasis and food intake control.

A retrospective analysis of 332 children with osteomyelitis (OM), managed from 1966 to 1996, was undertaken to evaluate etiology, clinical course and treatment results. In 64% of all patients positive bacterial cultures were obtained, Staphylococcus aureus, streptococci, pneumococci, and Haemophilus influenzae were the most frequently cultured pathogens. In two-thirds of the cases long bones (femur, tibia, humerus) were affected. Osteoarthritis or suppurative arthritis was evident in 27%; 32 of 170 (19%) re-evaluated patients had moderate or severe sequelae. Risk factors for an unfavorable course were the onset of disease in early infancy, suppurative arthritis, and an affected epiphysis. Suppurative arthritis, in particular, needs early evacuation to prevent sequelae. In recent years we observed an increasing number of patients presenting with atypical forms of OM. Since 1989 10 patients were considered to have chronic recurrent multifocal OM (CRMO). In 6 of them the clavicle was involved; their ages ranged from 3 to 14 years. The erythrocyte sedimentation rate was elevated (median 48, range 9-110 mm), while other inflammatory parameters like C-reactive protein (median 9, range <5-85 mg/l) or leucocyte count were slightly elevated or normal. Histopathology was stage-dependent, with a predominance of lymphoplasmacellular infiltration. A nonbacterial origin of CRMO is probable but not proven. Histopathology is not suitable for differentiation between bacterial and nonbacterial forms of bone inflammation.

Uteroplacental insufficiency leads to fetal growth retardation which is a major cause of perinatal and postnatal morbidity. In the present study we investigated the relationship between prenatal hemodynamic disturbances and postnatal intestinal perfusion and gastrointestinal function in small for gestational age neonates. Prospectively, 124 preterm neonates with a birth weight below 1500 g were assigned to one of two groups according to the prenatal Doppler sonographic measurements: neonates with or without prenatal hemodynamic disturbances. We defined a pathological fetal perfusion using a pulsatility index of uterine arteries, umbilical artery and fetal thoracic aorta above the 90th percentile and a pulsatility index of the middle cerebral artery below the 10th percentile of a normal group. We compared intestinal adaptation in both groups as well as the blood flow velocity wave forms of the superior mesenteric artery in all neonates. Postnatally, all 42 neonates with prenatal hemodynamic disturbances were classified to be small for gestational age. Thirty-seven of these neonates developed abdominal problems with delayed meconium passage, abdominal distension, bilious vomiting and a delay in tolerating enteral feeding within the first days of life. Five of them needed surgical intervention, but none of these infants revealed typical signs of necrotizing enterocolitis. In contrast, all neonates born after normal prenatal perfusion were classified as appropriate for gestational age. Only 19 of 82 neonates of this group showed signs of intestinal disturbances postnatally. Doppler sonography demonstrated significantly lower systolic, mean and END-diastolic flow velocities, and higher pulsatility indices of the superior mesenteric artery in neonates with prenatal hemodynamic disturbances. This may occur as a result of a postnatally persistent redistribution of regional blood flow and results in gastrointestinal problems and may adversely affect gut motility.

Two-stage surgery leads to a favorable outcome in patients with CAH and MPH, and virilized genitalia (Prader IV and V).

We describe five patients with 46,XY gonadal dysgenesis who developed gonadoblastomas, dysgerminomas, a mature teratoma, and a testicular intraepithelial neoplasia. The age of the patients was between 12.2 and 18.5 years. The external genitalia were normal female in two cases, in three they were intersexual. Four of our patients presented with slight retardation of puberty followed by stagnation. Most importantly the development of the breast (Tanner stage 2-4) did not correspond with pubic hair stage (Tanner stage 4). The patients can be classified as virilized phenotypical females. Serum testosterone was detectable in three, estradiol in two patients. None of the gonadoblastomas showed immunoreactivity with antibodies against steroid hormone receptors and against testosterone and estradiol, respectively. Probably the immature cells are able to produce steroid hormones. Only steroid-like cells with Leydig cell appearance showed positive cytoplasmatic immunostaining for testosterone in three patients. The findings in our patients underline that dysgenetic gonads must be removed as early as possible to prevent development of malignant tumors.

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