Bilateral Wilms' tumor--surgical aspects.

Four to eight percent of all Wilms' tumors are bilateral. Achieving curative resection of such tumors by partial nephrectomy or tumor enucleation while maintaining sufficient renal function represents a surgical challenge. Effective preoperative chemotherapy facilitates this aim considerably. Seven patients who were diagnosed to have bilateral synchronous Wilms' tumors between 1990 and 1994 are being reviewed. At the time of initial diagnosis, their mean age was 24.4 months, range 7 to 45 months. In 4 cases, pre-operative imaging did not reveal the full extent of the lesions, and in one of these, involvement of the second kidney was only detected by surgical exploration. Five of the children received at least 4 weeks of neo-adjuvant chemotherapy without primary biopsy, followed by bilateral tumor resection. One child had to be operated on as an emergency without any preliminary treatment, and in one, chemotherapy was interrupted after 3 weeks because of veno-occlusive disease. After a follow-up period of 66 months on the average (range 50 to 81 months), five of the patients are free of recurrence and clinically well--one with a kidney graft. The remaining two patients have died. Discussion is focussed on different management strategies of this rather rare pathology considering SIOP und NWTS protocols.

A pancreatic pseudocyst with acute hemorrhage in a child--secondary to a spherical duodenal duplication? A case report.

Duodenal duplications are rare congenital malformations with variable and non-specific symptoms. A spherical duodenal duplication associated with a pancreatic pseudocyst causing an acute bleeding due to a vascular erosion in a 16-month old child is presented. The possibility of a causal relationship between duodenal duplication and pancreatic pseudocyst is discussed.

Ultrasound assessment of the contralateral groin in infants with unilateral inguinal hernia.

Sonographic and operative findings in both groins of 23 infants presenting with a unilateral inguinal hernia were compared in a prospective study. Ultrasound accurately confirmed the presence of a hernia on the affected side in all infants but provided no useful additional diagnostic information. Ultrasound examination of the unaffected side was less accurate. Surgical exploration revealed the presence of a patent processus vaginalis (PPV) in 18 cases and its absence in five. Ultrasound was found to be accurate in only 15 out of the 23 cases with four false positive and four false negative results. This yielded an accuracy of 65%, a sensitivity of 78% and a specificity of only 20%. It is concluded that ultrasound cannot at present be used alone to plan the management of the contralateral groin, especially as the majority of children who do have a PPV will never develop an inguinal hernia.

Verification of anomalies of the central nervous system detected by prenatal ultrasound.

OBJECTIVE: The accuracy of fetal ultrasound (US) in diagnosing central nervous system (CNS) malformations was assessed with the aim to define in which cases US is reliable enough to assist in decisions on medical indication for abortions without resorting to magnetic resonance imaging (MRI). DESIGN: Retrospective analysis of the course of 69 fetuses with anomalies of the CNS detected on prenatal US in a university hospital. SETTING: General Hospital of Vienna, University of Vienna, Austria. METHOD: Prenatal US diagnosis was verified by postpartal US, MRI or computed tomography (CT) in the live births, and by autopsy of the fetus in cases of pregnancy termination. RESULTS: Abortion was induced in 40 fetuses for anencephaly (n = 4), exencephaly (n = 6), dorsal dysraphism (n = 6), encephalocele (n = 3), pronounced hydrocephaly (n = 11), holoprosencephaly (n = 4), Dandy Walker cyst (n = 5), and 1 complex syndrome - all confirmed on autopsy. In 29 live births, hydrocephaly, meningomyelocele, and microcephaly had always been correctly identified prenatally. Four Chiari malformations had been missed. Agenesis of the corpus callosum had remained unnoticed in 4 out of 14 cases and been erroneously reported in 5. Diagnostic errors were frequent for Dandy-Walker cyst and great cerebellomedullary cistern. CONCLUSION: Transabdominal fetal US did not lead to unjustified interventions. Inaccuracy in diagnosing abnormalities of the posterior fossa and the median telencephalon as well as aetiological clarification of hydrocephalus require additional MRI of the fetal CNS in patients selected accordingly.

Undescended testicles: an epidemiological review.

In a retrospective study the records of 447 boys (median age 5 y, age range 2 wk to 12 y) undergoing orchidopexy in a university hospital paediatric surgical department over a 2-y period were analysed for epidemiological factors related to disturbed testicular descent by comparison with the notes of an equal number of otherwise healthy male trauma patients matched for age. There were higher rates of peripartal asphyxia and intrauterine growth retardation (reflected by lower birthweights in combination with equal gestational age distribution), more complicated deliveries, an increased incidence of congenital malformations and more frequent occurrence of a number of chronic diseases in the families of affected boys. First- and second-born boys were over-represented in the study group. A cyclical pattern for the month of birth is suggested by the data, but this did not reach statistical significance. Neither was any statistical difference found for premature delivery, the incidence of extra-uterine fertilization, hormonal treatment of the mother while pregnant, twin pregnancies, threatened or imminent abortions or parental age. The literature dealing with this topic was reviewed. Discrepancies between different studies can be explained, at least in part, by considering different forms of undescended testicles as different nosological entities that should be assessed separately in forthcoming research.

Sertoli-Leydig cell tumor.

Sertoli-Leydig cell tumors belong to the group of sex-cord stromal tumors of the ovary. They account for less than 0,5% of all ovarian neoplasms. The majority of these tumors are benign, and almost all are localised unilaterally. Herewith, we describe an ovarian Sertoli-Leydig cell tumor in an 11-year-old girl. Serum levels of estradiol and progesterone were mildly elevated. while testosterone and androstendion levels were raised to around three- and sevenfold upper normal limit respectively on presentation. A left salpingo-oophorectomy was performed. Microscopically, the tumor was intermediately differentiated. At 6 months follow-up, there was neither clinical nor sonographical evidence of recurrence, and almost all sex hormone levels had returned to within normal range. Treatment of this pathological entity has to be individualized according to patient age, stage of tumor and degree of differentiation.

Bunk beds--a still underestimated risk for accidents in childhood?

UNLABELLED: A retrospective analysis of 218 bunk-bed accidents and a random sample survey with 991 family interviews were performed in order to establish guidelines for bunk-bed accident prevention. Falls from the top bed during sleep (35.1%) or while playing (34.4%) and falling off the ladder (23.2%) are the leading causes of bunk-bed accidents. Of the 218 children, 91 (41.7%) had sustained major injuries, including 3 polytrauma, 7 skull fractures, 44 cerebral concussions, 33 long bone fractures, 2 Lisfranc injuries, and 2 lacerations of the spleen. Of these accidents, 58.3% resulted in minor injuries with 18 fractures in other locations than the long bones or cranial vault, 89 contusions and sprains, 18 skin lacerations and 2 tooth fractures. A total of 23.8% of the accidents occurred in children under 3 years of age. The random sample survey demonstrated that in relation to age groups of children 30.8% (0%-45.8%) of families interviewed had been using bunk beds, with peaks at 3 years (29.8%), 7 years (36.5%) and 11 years (45.8%) of age. Of these bunk beds, 75.3% were equipped with side-rails, 57.3% had placed carpets alongside the bunk bed and 43.0% had used night lights. CONCLUSION: There is only one recommendation: no bunk beds!!!

A novel technique to measure position-dependent resistance changes in the vertebral arteries postmortem: new insights into the aetiology of SIDS?

AIM: A central respiratory regulation disturbance--triggered by impaired oxygen supply to the brainstem--is being discussed as an aetiological factor in sudden infant death syndrome. In this experimental study, further insight was sought into how far cervical spine movements may induce critical narrowing of the vertebral arteries. METHODS: In 10 infant bodies, the vertebral arteries were cannulated close to their origin and perfused with a fluid bolus of 5 ml in 2 s. The intravascular peak pressure created was memorized. Individual resistance indices were computed for every vertebral artery by relating the average pressure maximum from 10 measurements each with maximal rotation of the neck to either side alone and then with additional hyperextension of the cervical spine to the average peak pressure in neutral head position. RESULTS: Alterations of position-induced pressure changes occurred in the vertebral arteries of all children. Considering exclusively the combined movements of rotation plus extension, resistance increased ipsi- and contralaterally--no matter which side the head was turned--in three infants. A further three reacted with resistance surges only contralateral to the direction of rotation, and one only ipsilateral. Double contralateral associated with only one-directional ipsilateral rise was observed twice, and vice versa once. CONCLUSION: The presented method enables crude quantification of postmortem dynamic resistance alterations in the vertebral arteries. How far such measurements reflect authentic circulatory conditions during life remains to be assessed.