Undifferentiated (embryonal) sarcoma of the liver in childhood. Successful combined-modality therapy in four patients.

BACKGROUND: Undifferentiated (embryonal) sarcoma of the liver is a rare, highly malignant, mesenchymal tumor presenting predominantly in late childhood. Four girls, ages 6-13 years, who were treated with combined-modality regimens are reported. METHODS: In the first and second patients, hemihepatectomy resulted in complete removal of the tumor, and multiple-agent chemotherapy was administered postoperatively. In the third patient, only partial resection could be accomplished initially. By synchronous radiation therapy and chemotherapy, the tumor decreased to an extent that it could be resected completely and was totally devitalized on histologic examination. Postoperative chemotherapy was discontinued after 8 weeks. In the fourth patient, the tumor was not resectable at diagnostic biopsy. Polychemotherapy led to a significant reduction of the tumor size, and resection with clear margins could be performed subsequently. Because histologic necrosis amounted to about 95%, postoperative chemotherapy was also discontinued after 6 weeks. RESULTS: All four patients remain well without evidence of tumor recurrence after 79, 41, 36, and 22 months from diagnosis. CONCLUSIONS: The authors suggest that a multimodal therapeutic regimen should be used in patients with undifferentiated hepatic sarcoma.

[Acute exacerbation of chronic recurrent appendicitis in childhood]. / Zur Frage der akuten Exazerbation der chronisch-rezidivierenden Appendikopathie im Kindesalter.

Retrospective analysis of 324 childhood appendectomies revealed microscopic signs of previous inflammation in 66 out of 111 acute cases and in 162 of 182 cases of "chronic" cases presented with a long history of recurrent abdominal pain. Thus a somewhat liberal indication for appendicectomy in childhood seems justified in the appropriate cases.

[Chronic intermittent duodenal obstruction in childhood]. / Der chronisch-intermittierende Duodenalverschluss im Kindesalter.

Chronic intermittent duodenal obstruction caused by stenosis of the distal duodenum is a rare disease. Tight fixation of the ligament of Treitz, compression due to mesenteric lymphomatas or abnormal attachment of the mesocolon can cause intermittent impairment of intestinal passage. It will be necessary to differentiate this against genuine arterio-mesenteric duodenal obstruction as well as nerve motility disorders. History in the appropriate cases reports on postprandial episodes of regurgitation, sensation of fullness, nausea, vomiting and paroxysmal upper abdominal colicky pain. Radiograms always reveal gastroptosis and a varying degree of duodenal obstruction, usually with retroperistalsis. The passage is markedly delayed, with an impairment sometimes at the site of the duodenojejunal flexure. Therapy is always surgical. 8 own cases were cured by leftsided duodenal mobilisation according to Clairmont with additional caudad positioning and fixation of the duodenojejunal flexure.

[Routine gastrostomy in esophageal atresia]. / Zur Frage der Routine-Gastrostomie bei Osophagusatresien.

Contrary to Kiely and Spitz we have been using gastrostomy in almost all cases of oesophageal atresia treated with primary anastomosis. Gastro-oesophageal reflux has never been a major problem and during 25 years of personal paediatric surgical experience we have never performed any type of antireflux surgery. To confirm this we have followed up 71 out of 77 survivors who underwent primary anastomosis and gastrostomy in 3 Departments of Paediatric Surgery. The patients were examined 13 months to 17 years, in average 7 years postoperatively. 50 children were free from symptoms, the remainder complained of increased susceptibility to broncho-pulmonary infections. These children were younger than 4 years. X-ray controls could be performed in 50 patients. The vast majority showed motility disorders of the oesophagus, well known as Yo-Yo-phenomenon with swaying of the dye and delayed clearing of the oesophagus. Distinct gastro-oesophageal reflux was detected in 3 of the 50 cases, but there were no signs of oesophagitis or stricture and the patients were free of symptoms. There were no late strictures and no late mortality. We consider proper conservative management especially with oblique positioning as used in hiatal hernias most important in the treatment of the oesophageal motility disorder as well as gastro-oesophageal reflux in the early postoperative period and advocate routine gastrostomy and insertion of an endless thread for routine bougienage.

[Sonographic diagnosis of hypertrophic pyloric stenosis]. / Die sonographische Diagnose der hypertrophen Pylorusstenose.

50 infants admitted during the years 1981 to 1985 for suspected hypertrophic pyloric stenosis were examined sonographically. A pyloric cockade of 14 mm or more in diameter is diagnostic. A diameter up to 10 mm can be considered normal, whereas values between 11 and 13 mm represent borderline cases, which need consideration of the clinical symptoms and repeated controls. Because of its accuracy sonography can replace X-ray examinations in most of the cases with suspected hypertrophic pyloric stenosis.

[Sonographic diagnosis of hypertrophic pyloric stenosis]. / Sonographische Diagnose der hypertrophen Pylorusstenose.

The sonographic findings of 22 infants suspected of suffering from hypertrophic pyloric stenosis are reported. The central importance of the "cockade-sign" for the sonographic diagnosis of hypertrophic pyloric stenosis is stressed. Because of its accuracy sonography can replace radiological examination of the stomach in most cases.

Results of the modified Hartl gracilis plasty.

Follow-up results of patients who had a gracilis plasty for incontinence after rectal atresia showed that in 8 of 11 patients there was marked improvement. 6 children had good continence and 3 sufficient continence. Similar results have been reported by others and are encouraging. The high percentage of infected wounds did not seem to influence the results. The results in operations for incontinence with myelomeningoceles were different. Only 5 times was there sufficient continence and in only 2 patients was subjective improvement recorded. It must be pointed out that the function of the gracilis muscle was tested preoperatively by electromyographic examination. These unsatisfactory results can undoubtedly also be achieved with conservative therapy. Incontinence with myelomeningoceles is therefore no indication for a gracilis plasty. These varying results appear to indicate that success can only be obtained with a gracilis plasty if there are still functioning parts of the pelvic musculature present and if parts of the anorectum are able to mature. If this is true, then there is a direct connection between the results we obtained and those achieved by free muscle transplantation or transposition of a denervated muscle. No doubt, the gracilis plasty is not the only available method to achieve continence, but it will often improve the function of the sphincter in patients with incontinence following rectal atresia operations.

[Children hospitalized for surgery: a cognitive program for the prevention of behavioral disorders]. / Das chirurgisch kranke Kind im Krankenhaus: Ein kognitives Programm zur Reduktion von Verhaltensstörungen während des Aufenthaltes und nach der Entlassung.

To compare the conditions of behavioral disorders of hospitalized children in pediatric and surgical wards an investigation was made on 60 children aged 3 to 6 years who had to undergo surgery. The results were compared with earlier findings on children hospitalized in pediatric wards (Wanschura and Löschenkohl 1979, Schandl and Löschenkohl 1980). Most of the conditions of behavioral disorders could be confirmed. Conditions which inhibit behavioral disorders are: opportunity to play freely, contact to other children, positive contact to medical staff, play organized by parents who visited their children, higher frequency of visits, higher age of the child, active searching for contact by the child, confidence in the medical staff. Conditions which reinforce behavioral disorders are: Higher frequency of earlier hospitalizations, previous psychic traumas, anxiety of the mother related to illness. A psychological intervention program based on a cognitive theory of anxiety combined with the transfer of positive affects by a photographed model who had shown no fear in different situations of hospitalization and surgery significantly reduced behavioral disorders of hospitalized children in the hospital and after release from the hospital in the home environment.

[The chronically altered appendix in children. Cooperative work of the Austrian Society for Pediatric Surgery]. / Die "chronisch veränderte" appendix beim kind. Gemeinschaftsarbeit der Osterreichischen Gesellschaft für Kinderchirurgie.

In this multicentric study the anamnestic data and parents questionaires of 2-29 children were evaluated, whose appendix vermiformis at the time of operation showed no signs of inflammation. 31,1% macroscopically showed cicatrisation or fasciation, in 13,7% other enteral or gynaecological (1%) findings were present. In 1194 of the cases of patho-histological examination of the appendix had been made of which 36.4% were negative, 37.7% showed cicatrisation, 15,0% oxyuriasis and 10,4% coproliths. In the discussion of the significance of the chronically altered non-inflamed appendix we compared patients with and without macroscopical or microscopical alterations. We came to the conclusion that the probability-index as to sex, duration of symptoms, complexity of symptoms, incidence of postoperative well-being, proved the chronically altered appendix not to be an illness per se, but the result of spontaneously arrested inflammation. Since the rate of children admitted with a perforated appendix is high (15-20%) in comparison with the rate of complications after removal of non-inflamed appendices (2,9%), we believe that according to the diagnostical problems the principle can be maintained: in dubio pro operatione.

[Treatment of Wilms' tumor (author's transl)]. / Therapie des Wilms-Tumors.

Uniform treatment based on the therapeutic approach of the 1st and 2nd US National Wilms' Tumor Study was decided on in March 1976 by paediatricians, surgeons, urologists and radiotherapists in Austria. Wilms' tumour was diagnosed in 34 children between 1 january 1976 an 29 february 1980 (stage I: n = 11, stage II: n = 8, stage III: n = 8, stage IV: n = 7). Parents of two children refused treatments; both children have since died of metastases. Of the remaining 32 children 29 (90.6%) are alive, 10 for more than 4, 15 for more than 3 and 19 for more than 2 years after diagnosis. 21 children are without need of treatment. Three children have died, one due to postoperative complications, one due to haemorrhagic chickenpox, but free of tumour, and one after insufficient treatment. Two of the five children with a recurrence between 2 1/4 to 15 months after diagnosis had been treated inadequately in the initial phase. The tumour free survival rate in 74.2%. Two children with early occurring or recurrent lung metastases have survived for 53 1/2 and 54 months up to now.

[Traumatic herniae in childhood (author's transl)]. / Traumatische Hernien im Kindesalter.

Traumatic herniae in childhood are discussed on the basis of four cases, one of diaphragmatic rupture, two of abdominal wall herniae and one of intercostal hernia. The increase in such injuries is attributed to the rise in road accidents, especially involving bicycles. The typical mode of injury in the cases of abdominal and thoracic wall herniae was by falling onto the handle bars.

[Immobilisation hypercalciuria (author's transl)]. / Immobilisationshyperkalziurie.

The calcium excretion was investigated in 31 children who were immobilised because of fracture of the femur. Children over the age of 5 years treated with Kirschner wire extension showed a statistically significant rise of calcium excretion as compared with a control group but this reverted rapidly after mobilisation. Younger patients treated by overhead traction showed a decreased calcium excretion. The Calcium Excretion Quotient (CEQ) based on the calcium excretion in the early morning specimens of urine and the specific gravity were used to determine hypercalcemia. Dietary management or drug treatment is not necessary in this situation.

Long-term results of pulmonary resections in childhood.

We have found that following lung resection in childhood, one-third of the patients followed-up at least 10 years postoperatively presented mainly minor complaints. Compared with follow-up examinations about 8 years age [4], findings had changed only in two bronchiectasis patients. In both cases a moderate exertion dyspnea had occured. Although pulmonary function tests still showed normal values, the radiologic findings according to residual bronchiectasis had increased. Altogether, pulmonary function tests, however, sometimes show a considerable interference with lung ventilation, which seems to be astonishingly well compensated in childhood. They should not be neglected, however, with regard to strains and physiological changes in later life. Therefore, the indications for pulmonary resection in childhood have to be evaluated very carefully and the extent of the resection should be kept as small as possible.

[Radiological assessment of diaphragmatic function in operated cases of congenital defects of the diaphragm]. / Zur radiologischen Funktionsdiagnostik des operierten Zwerchfells

An investigation was undertaken in a series of 10 children operated upon for congenital diaphragmatic defects in regard to the value of simple thoracic X-ray pictures for functional assessment of the repaired diaphragm. The radiographs were taken in forced inspiration as well as in forced expiration and indices were calculated for the mobility of the diaphragm and the costophrenic angle. Moreover, an attempt was made to estimate diaphragmatic contraction by comparing the length of the hemidiaphragm during inspiration and during expiration. The obtained results gave sufficient information for routine investigations of diaphragmatic function and compared well with those of radiocinescopy. An impairment of the mobility of the diaphragm and the costophrenic angle was statistically demonstrated in our follow-up series as compared with a control group of healthy children.

[Experimental investigations of muscle transplants in correction of congenital diaphragmatic defects (author's transl)]. / Experimentelle Untersuchungen zur Funktion transplantierter Muskellappen bei angeborenen Zwerchfelldefekten

Rives muscle plasty using a pediculate flap of the latissimus dorsi muscle is an approved method for correction of large congenital diaphragmatic defects. To study morphology and function of the transplanted muscle flap 10 beagle dogs were operated upon bilaterally in two stages. Thus a total of 20 muscle transplants was evaluated. A standard Rives plasty was performed, emg electrodes were inserted into the diaphragm as well as into the muscle transplant. The surgical procedure was tolerated very well, there was no operative mortality. The period of observation varied between 18 and 48 weeks, the average being 30 weeks. Postoperatively there was no electric activity found in the muscle transplants, whereas the diaphragm showed its characteristic interference pattern during inspiration. In 5 cases, however, action potentials appeared in the muscle flap after 12 to 16 weeks. They had the semblance of fibrillation potentials and short sharp waves and were considered to be preceding reinnervation potentials, which occured 24 to 32 weeks postoperatively in these cases. In 2 cases the emg findings were inconstant, in the residual 13 cases there was no electrical activity in the muscle transplant during all the observation time. The emg findings corresponded to the histological postmortem findings. These showed in altogether 6 cases numerous intact muscle fibres as well as nerve trunks with neurites, especially in the neighbourhood of the diaphragm. In the middle of the flap and even more near the thoracic wall the number of intact muscle fibres decreased. Thus a partial reinnervation due to muscular neurotization starting from the diaphragmatic margin was assumed in these cases. In the rest of the flaps the muscles were largely atrophic and showed considerable lipomatosis, only minor fibrosis and never a distinct scar formation. Therefore, the flaps represented living tissue with intact vascularization and functionally good adaptability. Reinnervation, observed in some cases, is not the main factor for the good clinical results obtained with Rives muscle plasty, but can improve adaptability and elasticity of the transplant considerably. According to the lack of scar formation and the preponderance of fatty tissue an increase of the transplant considerably. According to the lack of scar formation and the preponderance of fatty tissue an increase of the transplants size during growth seems possible. Thus Rives muscle plasty using a flap of the latissimus dorsi muscle to cover large congenital diaphragmatic defects seems morphologically as well as functionally superior to other procedures especially those using plastic material.

Operations for the improvement of faecal incontinence.

Experiences and results are reported of Pickrell's gracilis sling transplantation in 30 cases. 24 cases could be followed up. In 21 of these there was definite improvement of continence. The favourable results are attributed to the contractibility of the muscle after transplantation. After high anal and rectal atresiae, a maturation of anorectal structures and a belated rectal adaption reaction may be observed, which can be attributed to the gracilis sling operation. It seems, therefore to be sensible to perform the operation earlier than has been practised hitherto.