Oro-palatal dysplasia Bettex-Graf--a new syndrome.

Four cases of an until now undescribed syndrome have been observed in Berne in the last 40 years. All four cases are members of the same family and have occurred in three consecutive generations. They present with a U-shaped palatal cleft, microstomia, hypoplasia of the mandibula and a partial anodontia. An autosomal dominant heredity was demonstrated. Karyograms have been made in three of the patients and in all patients showed an anomaly in the form of a "fragile site" in one chromosome (16 fra 16 [q22]). Surgical and orthopedic treatments were difficult.

Use of cultured keratinocytes in the treatment of severe burns.

A 6 1/2-year-old child was admitted to the emergency ward for third degree burn injuries representing 40% of body surface. Shock therapy was first applied. After débridement and in a series of operating sessions hands were grafted with full-thickness skin and most other wounds were covered with mesh grafts. On the 14th day after admission a piece of approximately 5 cm2 split thickness scalp skin was used to expand the keratinocytes by cell culture techniques according to the method of Rheinwald and Green (7). After 20 days 4 sheets of cells of first subculture, each 10 cm in diameter, representing a surface of approximately 300 cm2, were implanted on the front of the left thigh, which was burnt third-degree deep. Light microscopy of punch biopsies from mesh grafted and keratinocyte implanted sites taken 5 months after grafting showed a well differentiated epidermis overlying scar tissue. The following conclusions were drawn: Autologous keratinocytes did take on a third degree wound. No basic difference was observed neither clinically nor histologically between mesh-grafted and keratinocyte-implanted sites. The epidermis formed by keratinocyte implantation lacked pigmentation but presented an aesthetically better appearance than the mesh-graft treated sites. Given the scarcity of donor sites and the results obtained by implanting keratinocyte cultures, this latter technique may be resorted to in any extended and deep burn injury.

Granular cystitis in girls. Long-term follow-up.

Granular cystitis or 'papular cystitis' is a very common form of cystitis in young girls. In this paper we show the evolution of this affliction in women after puberty. In a series we were able to follow up leukoplakia or follicular cystitis patients to the age of 16 years.

Small bowel esophagoplasty without vascular microanastomosis: a preliminary report.

An intestinal segment through staged augmentation of its blood supply can be well suited to bridge a gap in the esophagus from the abdomen to the neck.

Repair of cloacal malformation using combined posterior sagittal and abdominal perineal approaches.

Cloacal malformations in female infants are not very common and their treatment is most challenging because every case differs from the others. In the light of a well-documented case, the authors describe the complexity of the malformation, the difficulties of making a correct detailed diagnosis, and the chronology of repair. They stress the advantage of the posterior sagittal approach for the first part of the operation, changing for the second part to a supine and later to a lithotomy position. In most cases a satisfactory reconstruction is possible, anatomically as well as functionally.

The clinical applications of demineralised bone powder (DBP)-induced osteoneogenesis.

New bone tissue can be induced anywhere in the animal organism, i.e. even at a site distant from actual bone, by the implantation of demineralised bone powder (DBP). Basic implantation experiments were first carried out and tested in the rat (Bettex-Galland 1985). The results led us to use the experience gained to treat four patients with bone defects with DBP (one bone cyst, and 3 chronic skull defects). The DBP used was prepared aseptically from fresh cadaver bone. The results were assessed by means of x-ray films and/or CT-scan, and the preliminary evaluation is encouraging.

Incidence of gastroesophageal reflux following repair of esophageal atresia.

Gastroesophageal reflux (GER) was looked for retro- and prospectively by various diagnostic methods in 67 patients, subdivided into three groups according to age at time of investigation. Pathological reflux was found in only one-quarter of the patients aged 16-23 years, but in three-quarters of both younger groups followed up for 1.5-14 years so far. Whereas pathological findings were most frequently disclosed by manometry in the older patients, radiological findings were most often decisive in younger patients. Following closure of recurrent tracheoesophageal fistulas and resection of anastomotic stenoses, eight patients developed reflux complications during the first months of life; these were so severe that antireflux plasty became unavoidable. Factors influencing the incidence of GER following esophageal repair were: the definition of GER based on the diagnostic method employed, the patient's age at the time of diagnosis, and anastomotic complications requiring a second operation. The essential time for treatment was the first year of life.

Significance of esophageal manometry and long-term pH monitoring for the evaluation of gastroesophageal reflux in infancy and childhood.

From examinations and clinical observations of 40 infants and a varying number of children from 2 to 11 years who all had characteristic symptoms of gastroesophageal reflux (GER), an attempt was made to establish the diagnostic contribution of esophageal manometry and long-term pH monitoring. A correlation between decreased resting pressure (less than or equal to 12 mmHg), or a sphincter insufficiency, and the degree of reflux symptoms was completely absent in infancy and increased only very slightly in young children. The most influential pH monitoring parameters were reflux during sleep, beyond 2 h postprandial, and during crying. These correlated most with the symptoms in infants. On the other hand, clinical developments were independent of the extent of the pH monitoring findings.

[The function of the esophagus following cardiomyotomy in childhood achalasia]. / Die Funktion des Osophagus nach Kardiomyotomie bei der kindlichen Achalasie.

Cardiomyotomy and fundoplication were performed in 7 children with achalasia of the oesophagus. They were followed up clinically, radiologically and manometrically from 7 to 18 years. Virtually all clinical symptoms and complaints disappeared immediately postoperatively and the patients remained free from symptoms well into adolescence except for very mild intermittent dysphagia in two of them. The residual mega-oesophagus and the disturbances of motility were least apparent in those operated on in early infancy. It may, therefore, be assumed that the motility pattern is more likely to return to normal in the oesophagus of the infant due to the relatively short period of dilatation. Fundoplication after Nissen did not alter the existing motility but was able to prevent gastro-oesophageal reflux and its possible grave consequences in all cases.

Renal tumours other than Wilms' tumour in the paediatric age group.

The non-Wilms' renal tumours in the paediatric age group may be classified according to age of onset, clinical-biological behaviour and morphology. This distinction is of practical importance because treatment and prognosis are different from those of Wilms' tumours. We report on clinical features, histological patterns, treatment and outcome of 9 cases seen between 1959 and 1981: - renal cell carcinoma (3 patients) - congenital mesoblastic nephroma (2 patients) - bone metastasizing renal tumour (3 patients) - angioleiomyolipoma (1 patient) Our experience is compared with the few reports of the same conditions in the literature.

[Kidney transplantation in childhood: 10 years' experience]. / Nierentransplantation im Kindesalter: Bilanz nach 10 Jahren.

We report our experiences with 35 renal transplantations in 31 patients in childhood and adolescence. Kidneys were obtained exclusively from deceased donors. At the present time 23 patients are still alive, 19 with a functioning graft whereas 4 are again on haemodialysis. Three-year patient survival rates are 80% and three-year graft survival 62%. Chronic rejection was the main reason for loss of graft or graft function in 14 of 19 patients. Our experiences confirm that renal transplantation is the optimal available therapy for terminal renal insufficiency in childhood.

[Double kidney with ureterocele]. / Die Doppelniere mit Ureterozele.

Twenty-six children with ureteroceles--twenty-three with unilateral double collecting system and three with bilateral double kidney--are analyzed. The clinical and radiological features of these ureteroceles are presented. The different possibilities of surgical management and their results are discussed. In 14 of these children we performed resection of the ureterocele with en bloc reimplantation of both ureters. Eleven cases were treated by resection of the ureterocele and upper pole nephroureterectomy and four cases by nephroureterectomy. Vesicoureteral reflux in both renal pelvic moieties was found in five cases after ureteral reimplantation en bloc. Because of frequent bilateral disease (10%) and the common association of other urological malformation it is preferable to try to perform ureteric reimplantation rather than primary nephroureterectomy even when the possibility of postoperative reflux is considered.

Endocrinologic aspects and problems in neuroblastoma. Pediatric surgical considerations.

In addition to chemotherapy and radiotherapy, surgery still has a place in the treatment of neuroblastoma, even though this place is no longer a central one. As has been obvious for some time, the treatment of malignancies is no longer the task of one physician. The teamwork of the chemotherapist, radiotherapist and surgeon has produced unforeseen successes, far superior to those obtained by any single modality. The immunologists are also at work in the laboratory; we surmise that they will provide us with additional surprises sooner or later.

[Surgical indications in cardial insufficiency in infants]. / Zur Operationsindikation bei der Kardiainsuffizienz des Säuglings.

Indications for fundoplication in 70 infants with cardiac sphincter insufficiency are analysed and subdivided into absolute and relative indications in accordance with complications conditioned by reflux. Endoscopy has proved to be the most important diagnostic method for deciding whether antireflux surgery is necessary. It enables proof or exclusion of oesophagitis as the most frequent and most severe consequence of reflux. Functional tests, such as manometry and long-term measurement of pH, are important especially in cases where severity and pattern of the disease have not been clarified. Such tests enable assessment of the function of the cardia and tubular oesophagus, and hence the hazards to which the oesophagus is exposed by the reflux of the gastrointestinal contents. Hence, they represent a contribution to a reduction of the incidence rate of surgery to the absolute minimum which is a "must" at this age, without exposing the young patient to the risk of a serious complication.

[Ultrasonographic antenatal detection of obstructed bladder (author's transl)]. / Die Ultraschall-Untersuchung der fetalen Blasenobstruktion.

The antenatal diagnosis of bladder obstruction may be possible by ultrasonography of the mother. We report herein four cases of prenatally detected megacystis: two were Prune Belly syndromes, one of which was aborted. The two others were female infants, one had a megacystis-microcolon-hypoperistalsis syndrome, in the other hypoperistalsis was present along with megacystis. Transuterine-transfetal bladder taps were used to relieve bladder pressure in utero.