Biomedical subjects
Publications and source records attributed to M Bolkenius.
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.
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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.
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.
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.
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.
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.
477 patients with soft tissue sarcomas, including 87 children (18.2%) were treated at the Surgical Center of the University of Heidelberg between 1950 and 1980. 22.8% of the patients with solid tumors (excluding brain tumors) in childhood were soft tissue sarcomas. In the pediatric age group there were 30% rhabdomyosarcoma and 20% angioblastic sarcoma. Since introduction of combined tumor therapy (surgery, radiation, and chemotherapy) the survival rate increased impressively from (29% to 58%).
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.
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).
In recent years a multidisciplinary therapy contribution has brought about a remarkable change from a purely morphological to a markedly functional type of surgery in corrective operations of intersexual genitals. Methods of surgery generally in use today are described groupwise according to functional aspects. Their development is subject to cultural influences just as it depends on general medical progress and recent psychological knowledge.
The authors report on a consecutive series of 123 children (46 males, 54 females) with congenital duodenal obstruction who were operated on between 1962 and 1980 at the Department of Paediatric Surgery, University of Heidelberg, Surgical Centre. 78 patients (64%) had an extrinsic obstruction; in 36% of the children a membraneous stenosis or complete atresia was observed. 74 children (60%) were mature at birth and had no additional malformations. Out of the remaining 49 children, 25 were mature but had additional malformations. 13 prematurely born children without and 11 prematurely born children with additional malformations were seen. 17 (14%) out of 123 children died. In 74 cases with uncomplicated duodenal obstruction only 5 children (5%) died, as compared to 13 children (27%) out of 49 cases with complicated obstruction. A division of our patients into two time spans (1962 to 1971, 1972 to 1980) shows a total lethality of 25% for the first series, which included 39% cases with complications. In a consecutive series of 54 children operated between 1972 and 1980 no child died.
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.
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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.