Long-term results of Rehbein's procedure: a retrospective study in German-speaking countries.

AIM: In contrast to other countries, no collective study of Rehbein's procedure in German-speaking nations has been performed. Therefore, our intention was, analogously to Goto and Ikeda's (10) Japanese study in 1984, Kleinhaus's (13) study on Swenson's procedure in 1979, Bourdelat's (2) French-Canadian investigation into Duhamel's technique in 1997 and Martuciello's (11) and Teitelbaum's (16) follow-up in the year 2000, to perform a follow-up study of Rehbein's technique of deep anterior resection. METHODS: The data of 200 patients from 22 German-speaking centers in Switzerland, Austria and Germany were collected. These data were gathered by questionnaire and the children were followed up in the individual participating hospitals for at least 3.5 years after the procedure. The procedure was performed between 1993 and 1997, over a 5-year period. The questionnaire contained 74 items including anamnestic data, diagnostic postoperative treatment and reoperations. RESULTS: Concerning the incidence of anastomotic leaks and resolving anastomotic strictures there was no significant difference between the results in our series and those of the collective analyses made by Hofmann von Kap-herr (7), Holschneider (9) and Sherman (18). In 6.6 % of the 191 patients an anastomotic leak and in 9.9 % a rectal stricture, which had to be dilated, was observed. Concerning late complications, 22.8 % of the children suffered from constipation, 4.3 % from encopresis, 10.6 % from enterocolitis and only 0.5 % from enuresis. The frequency of constipation diminishes over the years. A comparison of the different large series in the literature clearly shows that the incidence of constipation is higher after Rehbein's procedure and the frequency of urinary incontinence and encopresis higher following Swenson's, Soave's and Duhamel's techniques. The incidence of enterocolitis is less after Rehbein's procedure than after Swenson's, Soave's and Duhamel's techniques. CONCLUSIONS: The different results in the literature are due to the individual experience of the author, the very different follow-up methods and the date of follow-up. Therefore, the different results are hard to compare with our study. Nevertheless, Rehbein's anterior resection still could be presented as an adequate and important method to treat Hirschsprung's disease.

Lipoblastoma and lipoblastomatosis: a report of two cases and review of the literature.

Lipoblastoma and lipoblastomatosis are rare benign neoplasms of foetal white fat tissue that occur almost exclusively in infants and children. Two cases are reported and a review of the literature is given. An almost one-year-old girl and a full-term male infant were brought to our hospital with a solid mass in the right thigh. Nine months after total excision of the lipoblastoma, the little girl developed tumour recurrence, with unexpected histopathological maturation. In the case of the little boy, lipoblastomatosis with infiltration of the surrounding muscles, together with involvement of the sciatic and posterior femoral cutaneous nerves was found. Histologically, the tumour showed an unusual inflammatory reaction. The diagnosis of this tumour was made by the pathologist, but the histopathological picture bears a striking similarity to myxoid liposarcoma, and may be indistinguishable. Recent studies describe rearrangements of chromosome 8 q11-q13 region as a new discriminative marker that distinguishes lipoblastoma and lipoblastomatosis from myxoid liposarcoma.

Disseminated choriocarcinoma in infancy is curable by chemotherapy and delayed tumour resection.

Infantile choriocarcinoma has a poor prognosis with only 2 surviving children reported in the literature. 2 additional successfully treated children are presented. 2 infants (age 3 and 4 months at diagnosis) suffering from rapidly progressive choriocarcinoma with widespread haematogenous metastases involving the liver were treated according to the cooperative germ cell tumour treatment protocol (MAKEI 96) of the German Society of Pediatric Oncology and Hematology (GPOH). PEI-chemotherapy (cisplatin, etoposide, ifosfamide; no ifosfamide before the age of 4 months) was combined with delayed tumour resection. Treatment resulted in sustained remission in both children (event-free survival 42 and 40 months). Interphase fluorescent in situ hybridisation (FISH) analysis of the paraffin-embedded tumour sample from case one revealed four to eight copies of chromosomes X, 1 and 17 and two Y chromosomes. Hybridisation with sub-telomere and centromere specific probes for chromosome 1 displayed an imbalance between the short and long arms of chromosome 1. In the tumour cells from case 2, only a polysomy of chromosome X could be proven, other aberrations were not analysed in this case for technical reasons.

[Current status of vaccination and antibiotic prophylaxis in splenectomy. II: Children]. / Standortbestimmung der Impf- und Antibioticaprophylaxe bei Splenektomie. Teil II: Kinder.

Children are exposed to a greater risk than adults for severe late postsplenectomy infection. Therefore, prophylaxis against bacterial infections need to be more extensive. This paper presents a protocol for preventive measures in children. Repeated education of the patient and his/her parents about the consequences of splenic loss is mandatory. Vaccinations against Streptococcus pneumoniae and Hemophilus influenzae are highly recommended and also against Neisseria meningitidis in certain situations. The importance of long-term antibiotic prophylaxis in children is emphasized. Recent advances in vaccine development and the increasing problems with antibiotic resistance are discussed.

[Adrenal cortex carcinoma. A rare cause of a Conn syndrome in childhood]. / Nebennierenrinden-karzinom. Eine seltene Ursache eines Conn-Syndroms im Kindesalter.

A 2 1/3 year old girl presented classical Conn-Syndrome as diagnostic feature of an adrenocortical carcinoma. Sonographic diagnosis was confirmed by CT-scan, typical hormonal pattern and subsequent surgical procedure. Without further therapy there has been no relapse with a follow-up of 3 years.

[Bone tumors and tumor-like changes in the neonatal period and in infancy]. / Knochentumoren und tumorähnliche Veränderungen im Neugeborenen- und Säuglingsalter.

Bone tumours and tumour-like lesions occurring in newborns and infants are described presenting 5 own cases and a literature review. Age specific features in diagnosis, therapy and prognosis are discussed. Due to the rarity of these diseases a centralized registry at a national level is recommended.

Intestinal perforation associated with indomethacin treatment in premature infants.

Within 9 months we observed intestinal perforations in three very low birth weight (VLBW) infants undergoing indomethacin treatment for symptomatic patent ductus arteriosus (sPDA). The three patients exhibited striking similarities in their clinical courses and predisposing factors. Although clinical and histological criteria did not differentiate the perforations from necrotising enterocolitis (NEC), a well-known entity in premature infants, these events were remarkable to us since we had observed no other cases of NEC in recent years. From animal experiments and pathophysiological data, a role for indomethacin in gastrointestinal ischaemic damage must be considered. This communication is not meant to discredit indomethacin treatment. However, awareness of potential complications and careful monitoring during treatment is warranted.

[Arterio-mesenteric duodenal occlusion in children and adolescents--a rare form of intestinal obstruction]. / Der arterio-mesenteriale Duodenalverschluss bei Kindern und Jugendlichen-Eine seltene Form der intestinalen Obstruktion.

The arteriomesenteric occlusion of the duodenum is a rare form of intestinal obstruction in children and adolescents. Precipitating factors are disorders of the spine, loss of weight and kachexia. The angle formed by the mesenteric artery and the aorta determines whether or not an obstruction at this point occurs. With the exception of acute forms of obstruction the treatment is conservative. For patients, in whom conservative treatment is inappropriate, the different surgical approaches are discussed.

[Tumors of the adrenal gland in childhood. Report of 38 patients]. / Tumoren der Nebenniere im Kindesalter. Bericht über 38 Patienten.

During the past 17 years at the Department für Pediatric Surgery, University of Heidelberg, 38 children aged 1.6 to 14 years were adrenalectomized (unilateral 3.3, bilateral 5). Individual diagnoses were: neuroblastoma 23; pheochromocytoma 5; adrenocortical carcinoma 8; adrenocortical adenoma 4; bilateral nodular hyperplasia 2 cases. Patients with histologically benign lesions are alive and without recurrence more than 5 years after surgery, except one patient who developed Nelson's tumor after bilateral adrenalectomy for Cushing's disease. Of the patients with malignant adrenal tumors 21 died within 18 months after therapy was started, a 7 years old girl with an adrenal carcinoma died after a period of 3.6 years of combined treatment. In most cases of adrenocortical tumors virilization was the prominent feature.

Paediatric surgical principles in the management of children with intersex.

Two children, one with dysgenesis of the gonads and chromosomes 46XY and one with true hermaphroditism, are described. The principles of therapy with special reference to the operative correction of the external genitalia are discussed. One child was made a male and the other a female. The diagnostic procedure, heredity, hormonal status, chromosome analysis, histology of the gonads, radiological investigations, and skeletal age will partly determine the future sex, as will the decision of the parents and sociological aspects. The sex must be determined as early as possible. In girls, operative corrections should be performed at the time when their own oestrogen and gestagen production is starting. In boys enlargement of a micropenis can be produced by careful administration of testosterone.

Adrenal tumours.

A 15-year review of 34 children with suprarenal tumours is presented. With the exception of one adrenal carcinoma and 4 neuroblastoma, all 12 cortical and 22 medullary tumours (neuroblastomas predominated in the latter group with 19 cases) showed hormonal hyperactivity. The symptomatology, the fundamental steps in diagnosis and therapy and the operative results are discussed.

[Various aspects of pediatric surgery in surgery of the spleen]. / Kinderchirurgische Aspekte zur Chirurgie der Milz.

The method of partial splenectomy by applying fibrin glueing has become a safe surgical procedure thus opening new aspects of pediatric surgery of the spleen. Indications for partial splenectomy are discussed with special regard to Hodgkin's disease. The autotransplantation of splenic tissue--a localized form of iatrogenically induced splenosis--is considered as secondary to partial splenectomy since the arterial perfusion and the filter function of the spleen are not maintained. In cases, where partial splenectomy cannot be performed, splenic tissue should be placed in a retroperitoneal pouch of the splenic bed. The vulnerable infant omentum is rejected as a possible site of implantation since there is danger of spreading splenic tissue into the whole abdominal cavity inducing further complications by infections or neoplasma with splenic participation.

[Supportive testosterone treatment in surgical repair of hypospadias]. / Unterstützende Testosteronbehandlung bei der operativen Korrektur von Hypospadien.

Hypoplastic development of the phallus renders repair of hypospadias more difficult and very often limits functional and cosmetic results. We therefore tried to simplify surgery and to improve results by perioperative low-dose intramuscular testosterone treatment. 9 children with hypospadias of varying degree were treated with testosterone enantate 25-250 mg x 3 (2 injections at 3 weeks intervals preop. and 1 injection postop). Bone age, growth and phallic development were followed up to 2 years. While there was a reliable degree of phallic growth in most of the boys, in only 3 of the children we observed a slight transitory acceleration of skeletal development, in 7 a transitory increase of growth rate. No signs of virilisation were noted. We believe that in selected cases of hypospadias a short-term low-dose testosterone treatment may be an acceptable means to simplify and improve repair.

[Problems of intramural haematomas in childhood. A report of 5 cases]. / Beitrag zur Problematik der intramuralen Darmhämatome im Kindesalter-Bericht über 5 Fälle.

5 cases of intramural haematomas are discussed. The haematomas were of different origin, were localized at different sites and treated differently. In one case an obstructive duodenal haematoma developed after a liver and spleen rupture. This hematoma was treated by gastro-enterostomy. In the second case the cause could not be determined. The third haematoma was induced by an intestinal clamp. In the last two cases there was a tendency to haemorrhages (Morbus Schönlein-Henoch and haemophilia A).

[Physiopathology of portal hypertension (author's transl)]. / Physiopathologie de l'hypertension portale.

The physiopathology of portal hypertension is discussed. The pathogenesis of this affection may be of a congenital, or acquired infectious or iatrogenic nature, and the percentage frequency of each of these causes is presented. The different morphological forms of portal hypertension in children are outlined, emphasis being placed on the fact that the blood obstruction is usually pre-hepatic in location. A study of the modifications in blood circulation due to the obstruction demonstrates the importance of the collateral network, both interior and exterior to the liver. Complications of portal vein thrombi include splenomegaly, ascites, and alterations in hepatic parenchymatous tissue, producing alteration in liver oxygen perfusion. The effect on the systemic circulation and brain function are discussed.

[Segmental resection of the spleen and glueing with human fibrinogen - an alternative to splenectomy and autotransplantation (author's transl)]. / Partielle Milzresektion mit Fibrinklebung - Eine Alternative zur Splenektomie und Autotransplantation.

Basing on exemplary case histories - trauma, benign tumour and staging of lymphogranulomatosis - the possibility of maintaining the function of splenic tissue by means of partial splenic resection is discussed. Indication, surgical approach including fibrin glueing and postoperative follow-up are described, taking recent literature into account, and are discussed as an alternative to splenectomy and autotransplantation.