Congenital malformations and perinatal morbidity associated with intestinal neuronal dysplasia.

A close relation between different forms of dysganglionosis such as intestinal neuronal dysplasia (IND) type B and aganglionosis has been established. No systematic analysis of other malformations and diseases accompanying IND has been made as yet. Congenital malformations and perinatal morbidity were analyzed in 109 patients with IND seen at the Department of Pediatric Surgery in Mainz from 1977 to 1996. IND was associated with Hirschsprung's disease in 47 cases; 22 children with IND had other abdominal malformations, including anal atresia, rectal stenosis, sigmoidal stenosis, ileal atresia, pyloric stenosis, and esophageal atresia. A cystic bowel duplication, a choledochal cyst, and a persisting urachus were also found. Extra-abdominal malformations such as Down's syndrome, congenital diaphragmatic hernia, aortic stenosis, and malformations of vertebral bodies were seen. Twin siblings of children with IND were either healthy (n=3) or died in utero (n=1). Seventeen children with IND developed severe intra-abdominal complications during the perinatal period such as necrotizing enterocolitis (NEC), meconium ileus, or bowel perforations. NEC was frequently associated with preterm birth. Bowel perforations were seen in mature and preterm newborns with IND. Taken together, IND is found in a variety of obstructive bowel diseases. This may support the hypothesis that IND is a secondary phenomenon or that congenital atresias and stenoses of the digestive tract have a pathogenesis similar to that of intestinal innervation disturbances. IND may also be a part of complex malformation patterns since it occurs with a number of extraintestinal and non-obstructive intestinal malformations.

[Diagnosis and surgical therapy of thyroid gland diseases in childhood]. / Diagnostik und operative Therapie von Schilddrüsenerkrankungen im Kindesalter.

Among children, thyroid disease rarely points to a particular surgical therapy and therefore requires a very specific surgical approach as well as close interdisciplinary cooperation between pediatricians, pediatric surgeons, and nuclear medicine practitioners, in particular regarding the follow-up. As a consequence, excellent functional and long-terms results can be achieved. The extent of the radical surgery pertaining to the small, encapsulated papillary carcinoma is still being discussed and investigated.

[Kirner deformity]. / Die Kirner-Deformität.

A 14 years old boy with Kirner's deformity is demonstrated. He has the typical radial and volar deviation of the terminal phalanges of the 5th finger. Nature and inheritance of the disease are discussed. We assume inborn ossification disturbances. Most authors recommend to avoid any therapy. But we feel that nowadays it is possible to use plaster splints in young children or to do a corrective operation in older ones with good success.

[Diagnostic possibilities and treatment approaches for fracture of the of the intercondylar eminence in the growth period]. / Diagnostische Möglichkeiten und Behandlungsstrategien für die Fraktur der Eminentia intercondylica im Wachstumsalter.

Between 1970 and 1993 29 fractures of the intercondylar eminence were seen in children with almost the same number of cases showing no or minor displacement as those requiring open reduction for major dislocation. Combined knee injury must be suspected the higher the patient's age and the more pronounced the displacement of the fragment. Magnetic resonance imaging (MRI) has gained diagnostic predominance. Arthroscopy remains important for surgical management, especially of associated meniscus and cartilage damage. Early puncture of hemarthrosis is essential. Closed management is the therapy for nondisplaced fragments or those with bone contact or only tuberculum fractures--while displaced fragments require operative reduction. Arthroscopy-aided wire fixation is the preferred technique, followed by open reduction using absorbable suture material (wire in special cases). Traction screws should be used only in older children with large fragments. Our series show good late results because occasional function impairment with knee instability tends to normalize with growth. Corrective orthopedic procedures--if at all--are required only for juveniles or adults.

[New aspects in the treatment of complete and isolated diaphyseal fracture of the forearm in childhood]. / Neue Gesichtspunkte zur Behandlung der kompletten und isolierten diaphysären Unterarmfrakturen im Kindesalter.

From 1984 to 1992 153 children were treated at the pediatric surgical department of the University of Mainz, of whom 19 had fractures of the radius, 9 of the ulna and 125 had a combination of both in the diaphysis of the forearm. Factors like patient's age, type of fracture and therapeutic methods were analyzed. One hundred and forty-one children were treated conservatively, 12 by operation. One hundred and one of these patients were healthy, when discharged (about 8 weeks after accident). Follow-up was possible in 69 cases. We found that occasional a remaining dislocation angle does not impair function: in 65 cases the result was "good" and "very good", but only "moderate" in 4 cases. These 4 children had been treated conservatively by reposition and plaster cast; 2 of these 4 children showed bone reconstruction without dislocation. Twenty-two of the 56 X-ray follow-up's showed persisting dislocation. To prevent functional problems (2 of our cases) it is essential to obtain a very exact reposition of diaphyseal forearm fractures. Therefore we would recommend a more generous indication for operation, preferably using elastic-stable Nancy pins.

[Surgical indications of funnel chest]. / Zur Operationsindikation der Trichterbrust.

Pathological findings in ECG represent indications for operation only if they are accompanied by subjective complaints and psychogenic problems. The extent of the abnormality usually does not correlate with the patient's complaints. Disappearance of such complaints after surgery does not be considered as a sign of successful surgery. Even a deep funnel chest is not necessarily an indication for operation if the patient has no complaints and can manage the situation. A flat funnel chest should not be operated on. The operation can be performed in patients between 4 and 24 years of age. If severe associated anomalies require operation as well, the choice is not for correction of the funnel chest first.

Acute multilocular osteomyelitis in infancy and childhood.

38 cases of acute multilocular osteomyelitis out of a total of 165 osteomyelitis patients were treated between 1974 and 1986 at the Department of Paediatric Surgery and Paediatric Diseases at the University of Mainz. Osteomyelitis in infants was separated from juvenile osteomyelitis because of the different problems inherent to this disease depending upon the age; likewise, multilocular remote involvement was separated from regional multiple involvement. It was found that distant involvement is of special importance in infancy. 6 of the 8 cases in this group presented with symmetrical involvement. This interesting phenomenon is described in this article for the first time in acute multilocular osteomyelitis; so far, it was known to occur only in chronic recurrent multifocal osteomyelitis (CRMO). The infants in this group were predominantly newborn who were particularly severely handicapped by predisposing factors such as necessary intensive-care measures or surgical interventions. Our findings showed that in this age group most of the problems occurred, namely: 3 deaths and 2 healing defects in 8 children. For this reason it is imperative to effect particularly close monitoring of multilocular acute osteomyelitis in infancy. This must be considered as a paediatric and paediatric-surgical emergency requiring not only accurate diagnostic measures but also early internistic and surgical treatment to improve the prognosis of this specific disease pattern.

[The concept of the acute abdomen]. / Zum Begriff des akuten Abdomens.

The term "acute abdomen" stands for a group of abdominal symptoms which rapidly get worse and therefore require immediate treatment--especially conditions associated with peritonitis, ileus or massive bleeding. No time should be wasted on lengthy diagnosis or organizational problems. In the majority of cases a simple clinical diagnosis gives sufficient indication for surgery. Various manifestations simulating acute abdomen in children are discussed in order to prevent unnecessary laparotomy.

[Surgical therapy of Crohn disease and ulcerative colitis in childhood]. / Chirurgische Therapie des Morbus Crohn und der Colitis ulcerosa im Kindesalter.

Surgery in children with Crohn's disease--like in adults--most frequently consists in the repair of stenoses, abscesses and fistulae. However, if growth and development are retarded, surgery must also be used to prevent irreversible impairment. Seventeen (of 32) patients were considerably retarded before surgery; 13 of these recovered to normal after operation. As regards ulcerative colitis, surgery is rarely needed in children, but if so, the indication and procedure is the same as for adults.

[Surgical strategies in thyroid gland diseases in childhood and adolescence]. / Chirurgische Strategien bei Schilddrüsenerkrankungen im Kindes- und Jugendalter.

In the Department of Paediatric Surgery of the University Hospital of Mainz and the Department of Paediatric Surgery of the University Hospital of Frankfurt/M 90 children with thyroid gland lesions were operated on between 1970 and 1988. 78 patients had benign findings and 12 had malignant tumours. The most frequent operative indication for benign lesions was the euthyroid goitre (57 patients). We found nodular goitre in 45 patients, cystic goitre in 11 patients, and diffuse goitre in only 1 patient. 20 children were suffering from hyperthyroidism (11 with Basedow's disease, 9 with autonomy) and 1 from Hashimoto's thyroiditis. Among the malignant tumours, papillary carcinomas were the most frequent (5 patients), followed by follicular carcinomas (2 patients) and C-cell carcinomas (2 patients), and 1 case each of C-cell hyperplasia, retothelial sarcoma and malignant lymphoma. The following operative interventions were necessary: Among the patients with the benign findings, 28 unilateral subtotal resections were done (17 right side and 11 left side). 24 lesions were enucleated, 24 goitres were subjected to subtotal bilateral resection; in 1 case subtotal resection on the right side and hemithyroidectomy on the left side was performed, and in one patient polresection was necessary. All the 12 patients with malignant tumours underwent thyroidectomy: In 4 cases one-step operations were done and in other 8 cases multistep operations were performed.(ABSTRACT TRUNCATED AT 250 WORDS)

[Importance of thoracic trauma in the multi-traumatized child]. / Die Bedeutung der Thoraxverletzung beim multitraumatisierten Kind.

The rate of thoracic trauma in multitraumatized children was assessed on the basis of two series of investigations: thoracic injuries increased from 8% to 34%. Severe injury to the thorax is seen in spite of a decrease in multitraumata. Thoracic trauma is a central lesion with only occasional involvement of the peripheral extremities. To assess such injuries it is essential to pay attention to factors like age-related blood volume, specific fluid metabolism, different periods of thoracic elasticity, and the relation of lung volume to the child's age.

[Comparison of surgical and conservative treatment methods as exemplified in the juvenile femur]. / Vergleich operativer und konservativer Behandlungsmethoden am Beispiel kindlicher Oberschenkel.

In a series of 329 children treated for fractures of the femur between 1968 and 1982 a comparison was made between the results of mere extension treatment against operative compression plate treatment. The follow-up shows that conservative methods suffer from high rate of axial deviation, particularly antecurvature, while operative treatment has the negative effect of a higher, incidence of persistent leg lengthening and of re-fracture. The advantages of operative management, namely precise positioning of fracture, low damage to soft tissue and - above all - unimpaired motility of the young patient justify its preferred use in the treatment of femur fractures in children - except for long oblique fractures.

[Improving continence by reoperation]. / Kontinenzverbesserung durch Reoperation.

Between 1969 and 1988, 191 children underwent operations for anorectal anomalies in the Pediatric Surgical Department of Mainz University Hospital. Ninety-six had deep and 84 had intermediate or high malformations; the type was unknown in 11. Of these patients 53 had impaired continence and 8 still have a protective colostoma. 37 of the remaining 45 children had check-ups and reoperations (5 were deep types). After reoperation there was full continence in 14 patients, partial continence in 11, persisting incontinence in 11 and one case could not be assessed. Our results recommend reoperation using modern surgical methods of patients with continence.

[Modern diagnosis and therapy of traumatic hemobilia in childhood]. / Moderne Diagnostik und Therapie der traumatischen Hämobilie im Kindesalter.

The authors report on the first case of haematobilia diagnosed in childhood by means of sonography. Massive haematemesis occurred in a six-year-old boy on the 57th postoperative day after an oversewn rupture of the liver. Sonography revealed an atonic gallbladder with stratification phenomena and free floating highly reflecting coagulates, as well as pulsations and flow phenomena in an area of the size of a bean at the floor of a necrotic cavity of the liver (aneurysm). Relaparotomy was done by locating the aneurysm via sonography and performing a purse-string ligature. The high mortality rate of 10 to 20% can be reduced if the gradually evolving condition is identified at an early stage, which means that regular sonographic examinations should be conducted in the postoperative control of liver rupture. Intraoperative sonography with sterilisable sound heads enables accurate location of the source of bleeding.

Pressure-induced growth (PIG) of atretic esophagus: a contigent management for high-risk esophageal atresia.

A method is described by which a balloon catheter is inserted into the lower pouch, avoiding aspiration pneumonia due to overflow of gastric contents via a lower tracheoesophageal fistula in high-risk cases of esophageal atresia. As a side-effect there is an enlargement of the lower pouch, termed by the authors "pressure-induced growth". The prevention of aspiration pneumonia, induction of growth of the atretic segment, and the application of this method as the first part of a staged management of esophageal atresia in high-risk cases are the advantages.