Diagnostic value of fine-needle aspiration biopsy of thyroid nodules in children and adolescents.

From 1971 to 2001, 188 fine-needle aspiration biopsies (FNAB) of the thyroid gland were performed in 169 children and adolescents with thyroid nodules. In 65.4% the results of FNAB were classified as benign. In 13.8% FNAB was considered insufficient for diagnosis, due to the absence or small number of cells. The results of FNAB were classified as suspicious or malignant in 17.6% (n = 33). Surgery was performed in 118 patients (69.8%) and the results of cytological evaluation and histopathology were compared. The accuracy of FNAB was 77.2%, specificity 63.6%, and sensitivity 78.9%, which is less than reported for adults. Histopathological evaluation showed 13 malignant tumors. In two of the 13 malignancies, FNAB was inadequate because of a lack of thyroid cells. Of the remaining 11 malignancies, seven were detected by FNAB but four of these were classified as benign. Because of the lower accuracy of FNAB, we suggest a more aggressive diagnostic and therapeutic approach in children and adolescents than for adults.

Feminizing reconstructive surgery for ambiguous genitalia: the Leipzig experience.

PURPOSE: We report the results of feminizing reconstructive surgery for ambiguous genitalia with regard to the degree of virilization according to Prader and the long-term outcome with special emphasis on sexual intercourse. MATERIALS AND METHODS: Patients 16 to 46 years old with CAH (41) and MPH (17) were followed continuously by an interdisciplinary team that provided standardized hormone substitution and reconstructive surgery. More virilized genitalia were treated using a 2-stage procedure. RESULTS: Of patients with CAH no surgery and clitoris reduction were done in 4 and 2 (Prader I and II), no surgery, a 1-stage and a 2-stage procedure were done in 2, 3 and 4 (Prader III), a 2-stage procedure, a 1-stage procedure and surgery for fistula were done in 16, 4 and 2, respectively, while 1 refused surgery (Prader IV), and a 2-stage procedure was done in 5, including pull-through vaginoplasty in 2 (Prader V). Of patients with MPH no surgery was done in 6 (Prader I and II), a 1-stage procedure and a 2-stage procedure were done in 3 and 1 (Prader III), respectively, and a 2-stage procedure was done in 6, while 1 refused surgery (Prader IV). Revision vaginoplasties were necessary in 7 patients (12.1%) because of scar stenosis in those who underwent 1-stage as well as 2-stage procedures. None of our patients required reconstructive surgery to create a neovagina. Of the 58 patients 32 achieved sexual intercourse and in 17 the postoperative result would allow sexual intercourse, while in 3 the possibility of sexual intercourse is uncertain. For 3 patients sexual intercourse would not be possible since feminizing reconstructive surgery was refused. One patient could not be followed. CONCLUSIONS: Two-stage surgery leads to a favorable outcome in patients with CAH and MPH, and virilized genitalia (Prader IV and V).

Gonadoblastomas in 5 patients with 46,XY gonadal dysgenesis.

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.

[First clinical experience with extended planning and navigation in an interventional MRI unit]. / Erste klinische Erfahrungen mit einer erweiterten Eingriffsplanung und Navigation am interventionellen MRT.

PURPOSE: To present an advanced concept for patient-based navigation and to report on our first clinical experience with interventions in the cranium, of soft-tissue structures (breast, liver) and in the musculoskeletal system. MATERIALS AND METHODS: A PC-based navigation system was integrated into an existing interventional MRI environment. Intraoperatively acquired 3D data were used for interventional planning. The information content of these reference data was increased by integration of additional image modalities (e. g., fMRI, CT) and by color display of areas with early contrast media enhancement. Within 18 months, the system was used in 123 patients undergoing interventions in different anatomic regions (brain: 64, paranasal sinus: 9, breast: 20, liver: 17, bone: 9, muscle: 4). The mean duration of 64 brain interventions was compared with that of 36 procedures using the scanner's standard navigation. RESULTS: In contrast with the continuous scanning mode of the MR system (0.25 fps), the higher quality as well as the real time display (4 fps) of the MR images reconstructed from the 3D reference data allowed adequate hand-eye coordination. With our system, patient movement and tissue shifts could be immediately detected intraoperatively, and, in contrast to the standard procedure, navigation safely resumed after updating the reference data. The navigation system was characterized by good stability, efficient system integration and easy usability. Despite additional working steps still to be optimized, the duration of the image-guided brain tumor resections was not significantly longer. CONCLUSION: The presented system combines the advantage of intraoperative MRI with established visualization, planning, and real time capabilities of neuronavigation and can be efficiently applied in a broad range of non-neurosurgical interventions.

Video-assisted gonadectomy in children with Ullrich Turner syndrome or 46,XY gonadal dysgenesis.

Patients with dysgenetic gonads carry a high risk for the development of gonadal neoplasia. The aim of the study is to evaluate indications and feasibility of laparoscopy and video-assisted prophylactic gonadectomy in children with Ullrich Turner syndrome (UTS) or 46,XY gonadal dysgenesis (GoDy). Between 1996 and December 2002 five girls with UTS and nine patients with 46,XY GoDy (female gender role) were explored by laparoscopy. Video-assisted salpingo-oophorectomy or gonadectomy was performed using a three-port technique. Prophylactic salpingo-oophorectomy was exclusively performed in UTS patients with proven presence of translocated parts of the Y chromosome. In three patients with 46,XY GoDy laparoscopy was followed by surgical revision of the groin and open gonadectomy in four patients. In two cases with UTS the removed streak gonads contained small unilateral tumours stage pT1a, and in four cases of 46, XY GoDy histopathological investigation revealed bilateral neoplasms stage pT1b. We found the following tumour types: gonadoblastoma, dysgerminoma, testicular intraepithelial neoplasia, and mature teratoma. In conclusion, investigative laparoscopy gives a good image of the internal genital structures and allows the safe removal of the dysgenetic gonads during the same operation. The high rate of gonadal tumours underlines the indication for early gonadectomy in these patients.

Another case of autosomal dominant exstrophy of the bladder.

OBJECTIVE: Exstrophy of the bladder is a rare malformation due to an anterior midline defect. Most cases of this condition with variable expression occur sporadically, but there are some cases indicative of a strong genetic component apart from environmental factors. This is a report about another rare mother-child pair with bladder exstrophy. METHODS: We present the clinical data of a familial case of bladder exstrophy with an affected mother and her equally affected male fetus. RESULTS: Prenatal diagnosis of bladder exstrophy in the fetus was assessed by ultrasound at the 19th gestational week and was confirmed after termination of pregnancy at the 21st gestational week. CONCLUSION: The present case may be additional evidence for an autosomal dominant inherited variant of this malformation complex with implication for counselling of affected patients.

Idiopathic spontaneous pneumomediastinum: an uncommon emergency in children.

Spontaneous pneumomediastinum (SPM) occurs rarely in children. The diagnosis is based on physical examination and chest radiography. Conservative therapy usually leads to recovery. However, SPM in association with severe hypoxia, tachycardia, metabolic acidosis, and high ventilation pressures indicates clinically significant tension in the mediastinum. A collar mediastinotomy is the treatment of choice in these circumstances.

[MRI-guided pediatric interventions]. / MRT-gestützte Interventionen bei Kindern - Retrospektive Auswertung einer Fallserie.

AIM: There are only a few diagnostic or therapeutic procedures where MR imaging is applied during pediatric interventions. This study will describe indications, complications as well as the advantages and disadvantages of MRI-guided interventions based on a case study in an open MRI scanner. MATERIALS AND METHODS: 14 procedures were performed in an open MRI scanner (Signa SP/i, GEMS) on 13 children (1 - 16 yr) with bone, soft tissue and brain lesions (1 - 4 cm diameter). Localization of the pathology, targeting and final control of the result were based on images acquired with SE-, 3D-, GE-sequences pre- and post-contrast as well as FSE-sequences. Interactive MRI-guided instrument navigation was performed using a multiplanar T1w GE-sequence. RESULTS: Eight biopsies (incl. 1 rebiopsy), two tumor resections, one removal of a free joint mouse and three tumor-markings were performed without complications. Five biopsies provided appropriate sample quality for a histological diagnosis while two samples were too fragmented requiring a second biopsy (one of them MRI-guided). Surgical progress during tumor resection and marking could be controlled during the intervention without repositioning the patient. DISCUSSION: MR image guided pediatric interventions combine the advantage of an imaging system without ionizing radiation and the high soft-tissue contrast. The low number of these kinds of procedures can be explained by the relatively high costs for the intervention, the increased duration for each procedure and the limited availability of open MRI systems. The integrated localization-system helps during planning of the access path when facing complex anatomical structures and provides safe navigation in sensitive regions like the epiphyseal cartilage. Recent and expected developments of the required MR-compatible biopsy instruments could provide higher efficiency for appropriate sample size and quality. Summarizing, MRI-guided pediatric interventions have shown to be a promising method at the beginning of its development.

[Early and late complications after implantation of central venous catheters]. / Früh- und Spätkomplikationen nach Anlage implantierbarer zentralvenöser Kathetersysteme.

The use of implantable central venous catheters by the puncture-technique of Nagy is a safe method performed by trained surgeons. Due to the high mobility and then reduction of painful blood samples this method contributes favourably to the improvement of the quality of life of children with chronic diseases. The analysis of 140 catheters implanted in the Department of Pediatric Surgery of the University of Leipzig between 1995 and 2000 showed 11 cases with early complications. As the most frequent late complications were infection and thrombosis in 51 children. Neutropenia is a particular risk factor during polychemotherapy of malignant tumors. Staphylococcus epidermidis was the most frequently isolated bacillus. Catheter associated infections are only partly treatable by antibiotic therapy. In 29 of 44 cases the explantation of the catheter was necessary. Only a strict hygienic regimen would minimize the risk of infection

Treatment of transient posttraumatic bile-duct stenosis by laparoscopic-assisted cholecystotomy.

A 10-year-old boy developed severe obstructive jaundice following blunt abdominal trauma. Endoscopic retrograde cholangiography and magnetic resonance cholangiography revealed a stricture of the common bile duct. A cholecystostomy tube was inserted under laparoscopic guidance. After temporary bile drainage and a cholecystoenteric bypass the patient recovered.

Wilms' tumour metastasis to the testis: long-term survival.

We report the case of a 3-year-old boy with a Wilms' tumour of unfavourable histology with metastatic spread to the right testis. Orchiectomy and chemotherapy led to disease-free survival for more than 8 years.

[Temporary changes of personality traits in children suffering from solid tumor--a prospective study]. / Vorübergehende Persönlichkeitsveränderungen von Kindern mit soliden malignen Tumoren - eine prospektive Studie - Persönlichkeitsveränderungen von Kindern mit malignen Tumoren -

BACKGROUND: The objective of this prospective study was to assess the changes of personality traits in children suffering from solid tumors. PATIENTS AND METHODS: For evaluation we used a self-rating personality questionnaire for children between 9 and 14 years. 39 children with solid tumor compared to 67 healthy children of the general community were examined. For the assessment of children we choose three points in time: 1. Immediately after diagnosis, 2. at the end of therapy and 3. one to three years after. RESULTS: Initially, the children had significantly more behavioral and emotional problems than children of the community. At the end of therapy the patients showed unambiguous changes in personality traits. In the patients population we noticed to have a higher tendency to behave aggressively and egocentric. Patients tended to isolate themselves socially compared to healthy children in the community. No significant differences between children suffering from cancer and the control group were found one to three years after finishing the therapy. CONCLUSION: The distress of cancer patients during therapy seems to influence the personality development. In the longer term, the prevalence of psychological problems experienced by children treated for cancer does not differ from that found in children of the general community.

Prenatal hemodynamic disturbances -- pathophysiological background of intestinal motility disturbances in small for gestational age infants.

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.

Outcome of paratesticular involvement in infants with neuroblastoma.

The authors report on 3 infants suffering from disseminated neuroblastoma (NB) involving the testes or paratesticular structures. INSS stage 4 in 2 cases, and "biological" INSS stage 4S were considered, respectively. One patient with a stage 4 NB died of tumor progression; one patient is under therapy. The patient with NB 4S was cured with preservation of both testes after antineoplastic chemotherapy and reduction of the retroperitoneal primary. J Pediatr Surg 36:E23.

[External femorotibial transfixation in femoral fracture with joint involvement in a child]. / Die externe femurotibiale Transfixation bei Femurfraktur mit Gelenkbeteiligung beim Kind.

Long bone fractures combined with joint injuries run a high risk of destabilising the articulations. Remaining joints incongruence can lead to early arthosis especially in cases of severe injuries or not achieved anatomical reduction. A number of osteosynthesis methods are available for anatomical repair of the articular facet. This report presents a seven years old boy with an open comminuted fracture of the distal femur and consecutive joint instability, treated with a Transfixation (Orthofix) of the knee joint. The functional results suggest this method as an alternative treatment.

Growth-arrest and inhomogenous echotexture of the affected testis after tumor enucleation for unilateral Leydig cell tumor.

The authors report on an 8-year-old boy with unilateral left-sided Leydig cell tumor. After enucleation of the tumor, endocrine disturbances resolved. Long-time follow-up for more than 7 years was characterized by growth-arrest of the affected gonad and the unchanged appearance of a circumscribed hypoechogenic residual lesion within the testis.

Clinical aspects of obesity in childhood and adolescence--diagnosis, treatment and prevention.

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 adolescents 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.