Four cases of desmosis coli: severe chronic constipation, massive dilatation of the colon, and hypoperistalsis due to of changes in the colonic connective-tissue net.

We present four patients 5, 10, 12, and 17 years of age, each with a long history of severe constipation and hypoperistalsis of the gut. Three had partial or total resection of the colon, all had enterostomies, and only one maintains normal bowel function after reanastomosis. We report the clinical courses, radiologic findings, and operative procedures for all four cases. Preoperative full-thickness biopsies and the resected colon specimens revealed a complete or incomplete lack of the mesh network of collagen. The connective-tissue layer between the circular and longitudinal muscles was missing. Contrary to expectations, the enteric nervous system (ENS) was normal or near-normal in the affected areas. Hypo- and dysganglionosis was found additionally in some proximal segments of colon and/or small bowel. This combination of clinical symptoms and pathological findings is called desmosis of the colon with reference to a working hypothesis in a preliminary report by Meier-Ruge in 1998. Aplastic or hypoplastic desmosis may be the reason for the disturbed gut motility. Histologic examination should thus not only exclude changes of the ENS.

Ultrasound in the diagnosis of fractures in children.

We compared the results of primary ultrasonographic examination of 163 children with 224 suspected fractures with the subsequent radiological findings. The aim was to assess the value of ultrasound in the diagnosis of fractures in children. We found a good correlation for fractures of the long bones of the upper and lower limbs. Ultrasound was most reliable for the detection of simple femoral and humeral diaphyseal fractures and fractures of the forearm. It was less dependable for compound injuries and fractures adjacent to joints, lesions of the small bones of the hand and foot, non-displaced epiphyseal fractures (Salter-Harris type 1) or those with a fracture line of less than 1mm. We were able to distinguish several types of fracture in which the use of ultrasound alone gave reliable information and further radiography was unnecessary. We discuss the advantages and disadvantages of skeletal ultrasonographic studies in children.

Long-term results of treatment of single-system ectopic ureters.

Single-system ureteral ectopia (UE) encompasses a spectrum of malformations involving the bladder trigone, ureter, and kidney. The clinical presentation is variable, and both diagnostic and therapeutic problems are common. Reduced renal function in these patients may result from primary dysplasia, obstruction, vesicoureteral reflux, or recurrent infection. Based on our experience of seven patients, suggestions for diagnostic procedures and criteria for renal saving versus nephrectomy are offered. The relationship between ostium localization, renal function, and long-term results was investigated. From 1972 to 1990, five female and two male patients were studied. During the same period, 31 patients with UE and duplex kidneys were seen. Ages ranged from 1 day to 7 years. A ureteric opening into the bladder neck was associated with dilatation of the ureter and renal pelvis. Two patients had vaginal ectopia and severe renal dysplasia. In one, a cyst of the vaginal wall (Gardner's cyst) was detected at birth. A male newborn had multicystic renal dysplasia on the left and ureteric ectopia to the ductus deferens on the right side. To our knowledge, he is the first patient reported with renal function totally dependent on a kidney with severe UE. Follow-up ranged from 4 to 9 years. One patient died in the postoperative period because of renal failure and sepsis. All the others are well and have normal creatinine values. Improvement of renal function was noted after ureteral reimplantation (URI) in patients with bladder-neck ectopia. The numbers of infections were also drastically reduced. Our observations suggest that the combination of ultrasound, cyst urethrography, and cystoscopy will be diagnostic in most patients. A suspicion of UE should be raised in symptomatic patients with apparently solitary kidneys, enuresis ureterica, or atypical obstructive uropathy. Reduced renal function in some patients with ectopia to the bladder neck will improve after URI. This may be of importance in patients with bilateral anomalies and marginal renal function.

[Omphalocele and gastroschisis. Outcome--complications--follow-up--quality of life]. / Omphalocele und Gastroschisis. Ergebnisse--Komplikationen--Verlauf--Lebensqualität.

From 1970 to 1998, 35 children with omphalocele (OC) and 31 with gastroschisis (GS) were treated at the Department of Paediatric Surgery at Lübeck Medical University. Forty of 43 survivors were examined in 1990, the data of 30 patients were renewed in 1999 and 12 new cases added. Total follow-up was 1-28 years. Primary closure was possible in 25 OCs and 20 GSs. Eighteen children with OC and 8 with GS suffered from additional abnormalities, which were treated simultaneously. Twenty percent of the babies with OC died mostly because of severe congenital anomalies and 12.9% of GS because of infectious complications in combination with other diseases. There were no more deaths in the last decade. Accordingly, there was a reduction in consecutive operations. Improvements were due to better operative and perioperative treatment as well as abortions following improved ultrasound examinations. The results of the literature and our own experience show the benefit of primary closure. A two-stage approach with dura/amnion or a silo procedure prevents high intra-abdominal pressure, therefore, indirect measurements of intra-abdominal pressure can be used exceptionally. Umbilical preservation offers better cosmetic results. Long-term follow-up reveals normal growth and development of the children except for those with severe congenital anomalies. All the others are participating without problems in normal activities and education without reduction in their quality of life. Today an isolated OC or GS is not an indication for abortion. If prenatal OC or GS is diagnosed, paediatric surgeons should be involved in the consultations.

[Aggressive fibromatosis in childhood]. / Die aggressive Fibromatose im Kindesalter.

Despite its benign microscopic appearance and nonmetastatic behavior, aggressive fibromatosis infiltrates neighboring tissues and has a considerably high recurrence rate (30%-60%). It is a rare lesion (0.03%-0.1% of all tumors) with an incidence of between two and four cases per million residents and per year. There is no agreement regarding the etiology of fibromatosis. Hormonal, traumatic and genetic influences have been described. It is a common manifestation in Gardener syndrome. The treatment of choice is broad surgical resection. When resection is incomplete or impossible, radiotherapy, chemotherapy or chemotherapy in combination with hyperthermia is the second choice of treatment. On the basis of five children observed, we discuss the etiologic factors, classification, clinical aspects and results of surgical and medical therapy.

[Peripheral neuroectodermal tumor of the thoracic wall. Experiences with surgical treatment]. / Der Periphere Neuroektodermale Tumor der Thoraxwand. Erfahrungen mit der operativen Behandlung.

The peripheral neuroectodermal tumor (PNET) is seen nowadays as a more specified form of Ewing's Sarcoma with a worse prognosis. We report our experiences of three children (10-14 years old) with PNET of the right thoracic wall, all treated surgically within 1 year. Complete local resection-the main aspect of PNET therapie-of the initially voluminous tumors was not difficult due to chemotherapy and radiotherapy for 5 months prior to surgery.

[Three-dimensional evaluation of computerized tomography images of pediatric fractures]. / Dreidimensionale Auswertung von Computertomogrammen kindlicher Frakturen.

Three-dimensional reconstruction from CT was performed in pediatric patients after joint, facial, or spinal injury. Three-dimensional reconstruction proved particularly helpful in children with reduced post-trauma joint mobility. Free fragments, joint dislocations, and articulate surface defects were demonstrated. Here, as well as in facial and spinal injury, three-dimensional CT may facilitate and augment the understanding of two-dimensional displays.

[Preventive operations in intestinal abnormalities]. / Prophylaktische Operationen bei Darmanomalien.

In intestinal anomalies, preventive surgical procedures include gastrostomies and enterostomies as well as some special operations such as ligature of the cardia and associated appendectomies. General indications for enterostomies are seen in life-threatening circumstances, i.e. prematurity, surfactant deficiency, major associated malformations and complications. Preventive surgical procedures are necessary in certain cases of esophageal atresia, complicated small bowel atresia, meconium ileus, anorectal malformations, Hirschsprung disease, neuronal intestinal dysplasia and necrotizing enterocolitis. Prophylactic surgery requires detailed knowledge of the definitive therapeutic procedure.

[Long term results and quality of life of children with omphalocele and gastroschisis]. / Langzeitergebnisse und Lebensqualität bei Kindern mit Omphalocele und Gastroschisis.

Thirty-six children who underwent operations for omphalocele or gastroschisis were followed up at an average age of 7.4 years by medical examination. The majority of those who survived the initial treatment showed no signs of physical or psychological disorder linked with their abdominal wall defects. Those children without severe associated malformations were able to conduct a normal life.

[Clinical aspects, classification and prognosis of 7 cases of pediatric fibromatosis]. / Zur Klinik, Klassifikation und Prognose von 7 Fällen kindlicher Fibromatosen.

Seven cases of fibromatoses in infancy and childhood serve as examples to demonstrate this group of rare tumours, taking especially the aggressive forms of juvenile fibromatoses into account. There is a tendency to locally invasive destructive growth without metastasising at any time and a markedly high relapse rate after surgery--a tendency that showed up clearly in our patients, too. Only one child out of four having infantile (desmoid-type) fibromatosis of different localisation did not show any recurrence. The article reports, among other cases, on a desmoid of the stomach and oesophagus in a 15-year-old girl, not described in the literature so far, and on a congenital fibromatosis of the pancreas. Several classifications had to be performed to properly grade the individual cases (1, 6), since no generally valid systematic description of these tumours exists.

Long-distance resection of the trachea with primary anastomosis in small children.

While tracheal resection with primary anastomosis has been accepted as the therapy of choice for tracheal stenoses in adults since the 1960s, only 26 case reports are available on continuity resections of the trachea in children. The advantages of continuity resection include the one-stage procedure and the preservation of the natural wall structures. Anastomosis should be accomplished with pericartilaginous sutures and absorbable suture material. The increased tension on anastomoses inserted following resection of long tracheal segments does not necessarily have a negative influence on wound healing. The case histories of three small children are given in this report: they all underwent tracheal continuity resections involving four or five rings because of postintubation stenoses. The results of resection after up to 5 years' follow-up are very good, so that this method can be recommended in preference to the reconstructive tracheoplasty in children, which takes longer. If the cricoid cartilage is also involved a combination of tracheal resection and cricoid resection/dilatation is recommended.

[Results of treatment following 474 Leadbetter-Politano ureterocystoneostomies in vesicorenal reflux]. / Behandlungsergebnisse nach 474 Ureterozystoneostomien nach Leadbetter-Politano beim vesiko-renalen Reflux.

This is a report of our experience with 474 operations for vesico-renal reflux according to the Leadbetter-Politano technique at the Department of Paediatric Surgery, Medical University of Lübeck. Diagnosis of vesico-renal reflux is mostly based on urinary tract infection or enuresis. Indication for primary surgery was low pressure reflux or anomalies and ectopies of the ureteric orifice, found on cystoscopy. Ureterocystoneostomy using a modification of the Leadbetter-Politano technique was the method of surgery used, resecting the mostly fibrous distal ureteric segment. In our patients, the relapses of reflux or ureteric stenoses were found very rarely (only 1.1% each). Freedom of complaints could be gained by surgery in 80% of the cases. According to the results in the group followed up by us, primary surgical management without long-term antibiotic treatment is indicated in patients with anomalies or ectopies of the ureteric orifices.

Esophagus replacement by free, autologous jejunal mucosa transplantation in long-gap esophageal atresia.

There is apparently no ideal operative technique in the treatment of long-gap esophageal atresia, as is shown by the plurality of operative procedures described in the literature. Our own technique is presented, based on vaginal replacement by free, deserosized jejunal segments according to Wilfingseder. In addition, the muscularis propria layer was removed to improve trophic supply of the transplant which occurs initially only by diffusion. The free transplanted jejunal mucosa segments prevented transmural contamination of the mediastinum within the esophageal defect. Former animal experiments on beagles, as well as clinical experience with two newborn babies with long-gap esophageal atresia, showed that splinting for 4-6 weeks--beyond the time of the most intense wound contraction--and subsequent dilatation treatment could prevent circular and longitudinal shrinking of the transplants, thus avoiding stenosis. Histological findings revealed that on the average free-transplanted jejunal mucosa/submucosa tubes 8 cm long in dogs and 5 cm long in babies formed tubes of granulation tissue with the surrounding mediastinum, lined by persistent jejunal epithelium and partly by ingrowing esophageal epithelium. The two babies died 1/4 and 1 year following the operation, from their severe associated malformations. This, however, enabled us to do a thorough morphological examination of the interposed transplants and to document the complete healing. The advantages of the method described are primarily definitive bridging of the defect, less problems and risks as compared with elongation methods, and possibly shorter hospitalization.

[Esophageal replacement in long stretch atresias by free transplanted small intestine mucosa]. / Oesophagusersatz bei langstreckigen Atresien durch frei transplantierte Dünndarmmucosa.

The treatment of long gap oesophageal atresia is still a problem. In a self-developed method oesophageal defects were repaired by interposition of free jejunal mucosal/submucosal transplants. Two children, in whom long gap oesophageal-atresia was surgically repaired by inserting such transplants of 5 cm each, subsequently showed total histological healing, environmental adaptation with a surrounding fibrous scar. Functionally, there was free oesophageal passage to food. The children died of associated congenital anomalies three versus 12 months postoperatively. The advantages of this method are being emphasized.

Hereditary alpha1--antitrypsin deficiency associated with congenital extrahepatic bile duct hypoplasia.

A child, two months of age, suffering from cholestasis and biliary cirrhosis, was found to be homozygous for alpha1-antitrypsin deficiency Pi type ZZ associated with high degree extrahepatic bile duct obstruction. The clinical, protein-chemical, genetic, histological and immuno-histochemical findings in the patient are reported and the relationships between these two anomalies interpreted. An interaction between the very rare defects rather than random association is suggested.