Surgical therapy of congenital distal urethral stenoses in girls by meatoplasty.

In girls there are three distinct morphological kinds of congenital distal urethral stenoses in the broad sense. Incomplete meatal stenoses are the most frequent of these. Calibration of the urethra appeared to be the most reliable diagnostic tool in revealing distal urethral obstructions. To relieve the urodynamically interfering urethral stenoses we developed a resecting surgical technique. In a retrospective study the follow-up after meatoplasty of 163 girls with recurrent urinary tract infections was analysed. The long-term success rate totals up to 83.5%.

[Technique of open and endoscopic laser treatment in pediatric urology]. / Technik der offenen und endoskopischen Laserbehandlung in der Kinderurologie.

The neodymium:YAG laser is now the most common type of laser applied in pediatric surgery. The bare fibres with diameter of 400 microns and 600 microns can be introduced through the side channel of #8 cystoscope thus the cystoscopic laser can be used even in newborns.--Generally accepted power settings are not yet available for laser therapy in pediatric urology. After 5 years of systemic laser application in children standard parameters have been established. They follow the principle of reaching the desired therapeutic goal using the least energy at the shortest exposure time possible. In the non-contact method 15-25 watts, 0.1-0.3 sec pulses and 0.3 sec intervals are recommended for coagulation, and 100 watts in continuous waves for cutting. The same pulses and intervals are advisable for the contact method using the bare fibre, but 10-12 watts are sufficient for coagulation and 20-25 for vaporization. With these settings all endoscopic procedures in pediatric urology such as the resection of urethral valves and structures, the ablation of ureteroceles and the treatment of hemangiomas can be performed.

Laser treatment of posterior urethral valves in neonates.

The posterior valves of 13 neonates were resected using the neodymium:YAG laser in retrograde transurethral endoscopy. There were no strictures or other complications.

[Is concomitant preventive appendectomy in childhood justified?]. / Ist die präventive Begleitappendektomie im Kindesalter gerechtfertigt?

Appendicitis is an entity without specific symptoms. Multiple false diagnoses result from this, especially in childhood, in pregnancy, and in old age. We discuss whether incidental protective appendectomy is recommended in childhood in small bowel obstructions due to intussusception, comparing the data obtained from 100 children who were appendectomized with those of 32 children who were not.

[The syndrome of the fifth postoperative day after appendicectomy (author's transl)]. / Das Syndrom des 5. postoperativen Tages nach Appendektomie.

41 children were subjected to re-laparotomy because of obstruction after appendectomy. The causes were entero-colitis, occlusive ileus, rupture of the bowel and suture insufficiency. In this paper we report 6 children who had none of these complications. In these 6 children a "5-day syndrome" consisted usually of a fatal outcome with severe general peritonitis without a leakage of the appendix stump. Infection caused the endogenous spread of Bacteroides, following paralytic ileus and septicaemia. Early therapy with antibiotics and eventual re-laparotomy is then necessary.

[Diagnosis and treatment of congenital cysts and fistulae of the neck]. / Diagnostik und Behandlung von angeborenen Zysten und Fisteln am Hals.

The diagnosis and operative treatment of congenital cervical fistulae and cysts are in many cases difficult, especially abnormalities of the first branchial cleft and of the sinus cervicalis. In our hospital 90 congenital cervical fistulae and cysts were treated surgically between April 1972 and August 1979, excluding cysts of the thyroid gland, lymphangiomas etc. Four of these children with a median cyst and two children with fistulae of the first branchial cleft had recurrences due to partial obliteration and fibrosis of the ducts following inflammation. Other causes for recurrence were atypical locations of the fistulae and cysts. Therefore it is necessary to operate the congenital fistulae and cysts as early as possible.

[Disease picture of primary chronic intestinal pseudo-obstruction in childhood]. / Zum Krankheitsbild der primären chronischen intestinalen Pseudoobstruktion (CIPSO) im Kindesalter.

Primary CIPSO involves ileus symptoms without mechanical occlusion; these are caused by absence or ineffectiveness of peristalsis despite normal intestinal wall structure. The neonatal and adult types are differentiated. Ileus is progressive in the neonatal clinical pictures of CIPSO (neonatal CIPSO, MMIHS, congenital short-bowel syndrome) and chronic in the adult type, where it is also ultimately fatal, however. The problems of diagnosis and therapy are discussed and demonstrated in 5 children with primary CIPSO.

[Acute abdomen in immunoreaction mediated diseases]. / Akutes Abdomen bei den durch Immunreaktionen vermittelten Erkrankungen.

Assessment of the acute abdomen as a real or simulated complication in immunovasculitis is extremely difficult. Despite modern laboratory and imaging procedures, the number of false diagnoses is considerable, and a superfluous, falsely indicated emergency laparotomy can be just as fatal as the failure to perform a necessary surgical intervention. It is important to do a careful follow-up, including sonography and possibly laparoscopy, and to bear in mind the experience of H. Bailey with PSH that complications are often suspected but only seldom found.

Incidence of malformations of the urinary tract in association with clefts of lip, alveolus, and palate.

Malformation syndromes accompanied by cleft development in the lip-alveolus-palate (LAP) region are sometimes associated with anomalies of the efferent urinary tract. In the present clinical study, 68 children with an LAP-cleft were routinely subjected to radiological examination of the efferent urinary tract. These revealed pathological alterations, in varying degrees, in 26 children. The relationships between cleft type and urinary-tract malformation are analysed. The results show the significance of early interdisciplinary investigations.

[Advantages and limits of primary final correction of congenital abnormalities]. / Vorteile und Grenzen der primären Endversorgung angeborener Fehlbildungen.

Complete correction of malformations in the first hour or days of life has always been the target of pediatric surgeons. The possible halt of palliative operations involving the application of stomata in the trachea, esophagus, stomach, bowels and at the diverting urinary tract is to lighten the situation for parents and neonatologists. Requirements for a definitive primary surgical correction are an accurate diagnosis and careful selection of the child. Criteria include not only age and bodyweight, but also the presence of co-existing malformations, diseases and complications. Thus it is also possible to correct complex malformations primarily in one operation and to withdraw the use of stomata.

[Frequency of urinary tract abnormalities in cleft lip-maxilla-palate patients]. / Die Häufigkeit von Harnwegsanomalien bei Lippen-Kiefer-Gaumenspalten.

Malformations of the urinary tract favour urinary tract infection; on the other hand, a urinary tract infection is often the first indication of a malformation of the urinary tract. Further signs are externally visible malformations, such as cheilognathopalatoschisis. In two years we examined 53 children with schistases urologically and found urinary tract anomalies in 39,6%.

[Megacalycosis in childhood]. / Die Megakaliose im Kindesalter.

Congenital megacalycosis is a renal dysplasia characterised by hypoplasia of the medullary pyramids and associated non-obstructive dilatation of the calyces. Calyceal dilatation promotes via disturbed urodynamics the manifestation of chronic interstitial nephritis and calculus formation. Therapy is primarily not necessary, but in the presence of stone development and chronic infections surgical procedures may become indispensable. Pathogenesis, diagnosis, differential diagnosis and therapy of megacalycosis in two children are discussed.

Bilateral Wilms tumor in a boy with severe hypospadias and cryptochidism due to a heterozygous mutation in the WT1 gene.

Mutations in the WT1 gene causing Wilms tumors were first reported in WAGR syndrome (Wilms tumor, Aniridia, Genitourinary malformation, mental Retardation) and Denys Drash syndrome (pseudohermaphroditism, Wilms tumor, nephropathy), but only in a few patients with hypospadias and cryptorchidism without other signs of Denys Drash (DDS) or WAGR syndrome WT1 mutations were identified. We report a boy, who was born in 1989 with hypospadias and bilateral cryptorchidism. Previous karyotyping and endocrine studies had ruled out any known cause of male pseudohermaphroditism. Subsequently, he developed a bilateral Wilms tumor, which was detected by palpation at the age of 15 months during a routine visit by the general pediatrician. Because of its extensive size, surgery and chemotherapy were needed for treatment. Analysis of the WT1 gene was performed 5 y after diagnosis and revealed a C to T transition in one allele generating a stop codon at codon 362 and subsequently leading to a truncated protein with loss of its ability to bind to DNA. No signs of DDS or WAGR syndrome are present in the boy. The work up of this patient and the so far known few comparable cases from the literature lead to the conclusion that in newborns with severe urogenital malformations not due to known chromosomal or endocrine disorders mutational screening of the WT1 gene should be performed, to evaluate the high risk of developing a Wilms tumor. We favor mutational screening in these patients as an easy tool for investigation, because in the future it will probably decrease the necessity of frequent control visits in patients without a WT1 mutation.