Hepatoblastoma in two cousins in a family with adenomatous polyposis. Report of two cases.

Two cases of hepatoblastoma in cousins in a family with familial adenomatous polyposis (FAP) are reported. Twenty-five cases of hepatoblastoma with family history of FAP have been documented in the literature, but there has never been a report of two cases of hepatoblastoma in the same polyposis family.

Anal tonometry in the neonatal period for the diagnosis of Hirschsprung's disease.

A double balloon technique was used for anal tonometry in 22 infants. The weight was less than 2,500 g in 10 of the infants. Ileus or delayed passage of meconium was seen in 9 infants. Thirteen infants had not revealed any symptoms of intestinal obstruction during the first week of life. In all cases, except one, it was possible to demonstrate reflexes from the internal anal sphincter. In 18 infants normal curves were registered from the internal anal sphincter. None of these developed Hirschsprung's disease. In 3 infants a pathological pattern of contractions of the internal anal sphincter was demonstrated. Two of them proved to have Hirschsprung's disease. In the third case normal reflexes were registered after one year. Possibly the innervation of rectum and the internal anal sphincter was disrupted during the neonatal period in connection with enterocolitis and ileus, thus representing a reversible case of achalasia.

Intranasal LH-RH treatment of cryptorchidism. A clinical trial and 5 years follow-up.

The effect of intranasal LH-RH on cryptorchidism was investigated in 45 prepubertal boys with 68 undescended testes. A daily dose of 1.2 mg LH-RH was given for 4 weeks. A total of 16 testes (24%) descended. Follow-up examination 5 years later showed that relapse had occurred in two cases. Fifty-two testes did not descend during the LH-RH treatment. However, seven of these testes descended spontaneously during puberty. So far surgical treatment has been carried out in 39 of the remaining 45 testes. Anatomical anomalies (ectopic position of the testis, open processus vaginalis, abnormal epididymis) explained the failure of LH-RH to cause descent in the majority of the surgically treated cases.

Balloon-dilatation of esophageal strictures in children.

Dilatation of benign esophageal strictures by means of a balloon-catheter is a well-established procedure in adults. This method is also useful in children. The technique is described and the results of treatment in 10 children are reported. In all our patients the stricture developed after an operation for esophageal atresia with an end-to-end anastomosis. Dilatation with a balloon-catheter seems to have the same or an even better effect than the traditional dilatation with bougies. Complications following balloon-dilatations have been few and negligible in this series and dilatations can be performed using sedation instead of general anaesthesia. Therefore, this method is recommended.

Prenatal obstruction of the ileum diagnosed by ultrasound.

A case of low atresia of the ileum, diagnosed prenatally by real-time ultrasound scanning, is presented. The ultrasound examination showed progressive distension of intestinal loops, with strong peristaltic movements. The stomach was also distended, presenting as a large cystic area in the upper left abdomen. Real-time ultrasound technique is most advantageous in the diagnosis of fetal ileus. Prenatal diagnosis of fetal intestinal obstruction is of great importance, making early and safe treatment of the newborn possible.

Clinical results of early and late operative correction of undescended testes.

Operative treatment of undescended testes was performed in 556 boys (695 testes). The histopathological changes in the testicular tissue were more severe in older patients. However, post-operative clinical results were found to be significantly better in boys treated immediately before puberty than in boys treated at an earlier age.

CT in the staging of children with malignant tumours.

The application of CT whole-body scanning as an adjunct in the staging investigations was evaluated in 56 children with various types of extra-cranial solid malignant tumours. CT proved to be superior to conventional films in the detection of pulmonary or pleural dissemination. The abdominal lymph nodes were difficult to evaluate by CT, and abnormal nodes could only be diagnosed when they were unequivocally enlarged. The role of CT in the detection of liver metastases could not be established in this study due to the rare occurrence of liver involvement. CT seemed to be a valuable method in the prediction of the operability of the primary tumour, but seemed to have an inability to show local invasiveness of the tumour. CT is an important supplement to conventional, noninvasive methods in the staging of these patients because CT is able to detect otherwise undetectable dissemination and this results in an improved therapeutic approach in individual cases.

Electrophysiological investigation of hydronephrosis in children.

The hydrostatics and dynamics of the upper urinary tract were studied in 12 children with congenital hydronephrosis by means of intrapelvic pressure and bipolar electromyography leads from the pelvis and ureter. Studies were performed intraoperatively with the child under general anesthesia. Measurements were obtained during a baseline period of normal diuresis and during diuresis induced by furosemide. The baseline pressure in the hydronephrotic pelvis was within normal limits in most cases but a tendency toward an increased mean baseline pressure was observed in severe hydronephrosis when the cases were grouped according to the excretory urogram. During diuresis the average pressure increase in severe hydronephrosis was parallel to that in moderate hydronephrosis. The peristaltic pattern showed discoordination in pelvis conus transmission but usually preserved anterograde transmission from the conus down the ureter. The results seem to indicate a slight functional obstruction at the pelvioureteral junction. This obstruction leads to the observed alterations in peristalsis and to a slight elevation of the intrapelvic pressure in more severe cases.

High incidence of undetected neoplasia in maldescended testes.

Testicular biopsy specimens from 4 (8%) of 50 men previously treated for maldescended testes had a carcinoma-in-situ pattern. 2 of these men had adjacent invasive carcinoma (seminoma and embryonal carcinoma). The patient with embryonal carcinoma had an enlarged testis but the 3 other patients with neoplasia had no clinical signs or symptoms of malignancy. Routine follow-up including testicular biopsy in young men with maldescended testes is essential because of the increased risk of malignancy and may detect testicular neoplasia at a stage when orchidectomy alone is curative.