Long-term outcome of congenital cystic adenomatoid malformation.

To determine the long-term outcome of congenital cystic adenomatoid malformation (CCAM), the records of all patients with CCAM treated in three Hungarian paediatric surgical centers between 1977 and 1996 were reviewed. Patients were followed for up to 20 years following diagnosis and treatment. In 20 patients CCAM was diagnosed postnatally and in 3 prenatally. Biodata including the operative procedures are presented. Follow-up findings between 18 months and 20 years after diagnosis showed better height and weight growth in patients operated upon in later childhood compared with those operated upon in infancy. The older the patient at the time of diagnosis, the better was the long-term outcome.

Giant ureteral stone in a 4-year-old boy.

A 4-year-old boy presented with sudden onset of fever, pyuria, and bacteriuria. Ultrasound revealed left hydronephrosis and hydroureter. A plain abdominal radiography and excretory urogram showed a giant ureteral stone measuring 9 cm causing ureteral obstruction. Underlying anatomic or metabolic abnormalities were not detected. Extraction of the stone resulted in complete disappearance of the hydronephrosis and hydroureter.

[Duplication of the alimentary canal in infants and children]. / A tápcsatorna kettözetei csecsemökben és gyermekekben.

This is a review of 30 duplications of the alimentary tract in 28 patients treated at the Surgical Unit of the Children's Department of the Medical University of Pécs, Hungary and at the Department of Pediatric Surgery of the Medical Academy of Dresden, Germany, from 1964 to 1989. The ages of patients ranged from 1 day to 13 years, 80 per cent were less than 2 years of age at initial presentation. There were 6 thoracic, 20 abdominal and 2 thoracoabdominal duplications. Distended abdomen, vomiting, bowel obstruction and palpable abdominal mass were most frequently encountered. Plain thoracic and abdominal X-rays, ultrasonography, barium esophagogram, barium meal and enema were the most common diagnostic procedures. Emergency operative intervention was required in 18 patients. One infant died of an unrelated disease. Twenty-three duplications were cystic and 3 tubular. One patient had an appendiceal duplication, and another patient a flat lumenless duplication located on the perineum close to the anal opening. The surgical procedure--removal of the duplication--should not be more radical than necessary to eliminate the potential complaints and prevent recurrence. During surgery the common blood supply shared between the duplication and the native bowel must be carefully protected to avoid undue sacrifice of normal bowel.

Alimentary tract duplications in infants and children.

This is a review of 30 duplications of the alimentary tract in 28 patients treated at the Surgical Unit of the Children's Department of the Medical University of Pécs, Hungary, and at the Department of Pediatric Surgery of the Medical Academy of Dresden, Germany, from 1964 to 1989. The ages of patients ranged from 1 day to 13 years, 80 percent were less than 2 years of age at initial presentation. There were 6 thoracic, 20 abdominal and 2 thoraco-abdominal duplications. Distended abdomen, vomiting, bowel obstruction and palpable abdominal mass were most frequently encountered. Plain thoracic and abdominal x-rays, ultrasonography, barium esophagogram, barium meal and enema were the most common diagnostic procedures. Emergency operative intervention was required in 18 patients. One infant died of an unrelated disease. Twenty-three duplications were cystic and 3 tubular. One patient had an appendiceal duplication, and another patient a flat lumenless duplication located on the perineum close to the anal opening. The surgical procedure--removal of the duplication--should not be more radical than necessary to eliminate the potential complaints and prevent recurrence. During surgery the common blood supply shared between the duplication and the native bowel must be carefully protected to avoid undue sacrifice of normal bowel.

[Management of intussusception in infancy by hydrostatic desinvagination under ultrasonic monitoring]. / Csecsemökori bélbetüremkedés kezelése ultrahanggal ellenörzött hidrosztatikus dezinvaginációval.

The ultrasonographic diagnosis of intussusception may be a recent complementation of X-ray fluoroscopy controlled hydrostatic desinvagination. Ultrasound is also useful in follow-up of the effectiveness of conservative management. By presenting their experience gathered by successful treatment of one case, the authors' aim is to contribute to the spread of this method.

Abdominal calcifications in infants and children.

Abdominal and pelvic calcifications are usually incidental findings and require further measures to determine their origin. Most laboratory investigations are of little help. Plain anteroposterior and lateral x-rays are essential. The time of appearance and localisation of a calcification is of diagnostic importance. Amorphous, granular and irregular calcification can be an early sign of malignancy. Mobility of a calcification also helps to clarify its origin. Over the past 15 years abdominal and pelvic calcifications, excluding urological radiodensities, have been found in 63 patients up to fourteen years of age at our institute. A migrating deposit in the omentum, a spontaneously amputated calcified ovary mimicking a vesical calculus and a congenital retroperitoneal xanthofibroma caused the greatest difficulty in establishing a preoperative diagnosis.

[Ethical considerations in the management of hand abnormalities associated with multiple malformations (author's transl)]. / Ethische Uberlegunaen in der Behandlung der Handanomalien bei multiplen Missbildungen.

Of 300 assorted patients with various congenital abnormalities of the hand, there were 12 with mutiple malformations. It can be concluded that: 1. An accurate diagnosis requires genetic and chromsomal investigations. 2. If there is a slight organic or mental abnormality or damage, the treatment of the hand abnormality is justifiable and should be treated just as an isolated abnormality. 3. If the associated somatic or mental abnormality is severe, the correction of the hand abnormality should be delayed in accordance with the overall status of the child.

[Rare disorders in neck surgery]. / Seltene halschirurgische Erkrankungen.

The diagnosis and treatment of unusual surgical conditions of the neck in the newborn, infancy, and paediatric age-group are discussed. The median cervical cleft, the scalenus cervical rib syndrome, the cervical teratoma, changes of the thymus, tumour-like abnormalities of the large veins, calculus formation of the sub-mandibular salivary gland, severe traumatic and iatrogenic injury may all present difficult diagnostic and therapeutic problems.

Changes in connective tissue metabolism due to bone fractures in children aged 10--14 years. III. Urinary hydroxyproline and glycosaminoglycan excretion following bone fractures treated in different ways.

Urinary hydroxyproline and glycosaminoglycan excretion was studied in the course of fracture healing in twenty 10--14-year-old children with fracture of the femur or of the tibia and fibula. In 11 children reduction and plaster cast were used, in 5 osteosynthesis, and in 4 extension of 6--21 days duration combined with immobilization by plaster cast. In the state of resorption, an increase was observed in the urinary excretion of hydroxyproline and total glycosaminoglycans and in the six glycosaminoglycan fractions studied. As a result of the soft tissue damage accompanying the fracture, the excretion of hyaluronic acid, heparan sulphate, and dermatan sulphate showed the most marked increase. No statistically significant relationship could be demonstrated between the mode of treatment and the amounts of urinary hydroxyproline and glycosaminoglycan, but the stage of resorption was prolonged in the case of treatment with traction. At the beginning of regeneration, a decrease in hydroxyproline excretion and then an increase corresponding to the intensive synthesis of collagen were demonstrated. Glycosaminoglycan excretion during the development of fibrous callus was considerably below the control value. Excretion of the metabolites decreased markedly in the five operated patients. in the case of extension, the stage of regeneration prolonged by 4 to 10 days. In the stage of calcification, urinary hydroxyproline excretion was increased while the excretion of glycosaminoglycan and its fractions was the same as in the controls except in the patients treated with traction. No statistically significant relationship was found between the urinary amounts of hydroxyproline and glycosaminoglycan and the mode of treatment. In the case of traction, fracture repair and the formation of connective tissue callus were prolonged.

Remnants of vitelline duct: analysis of 66 cases.

In twenty years, 66 infants and children with remnants of vitelline duct requiring surgery have been admitted. The patients were classified into three groups: patient vitelline duct (20 cases); Meckel's diverticulum as the primary surgical diseases (19 patients); and Meckel's diverticulum found incidentally at surgery (27 patients). The male preponderance in the groups of patent vitelline duct and symptomatic Meckel's diverticulum was 9:1. In contrast with other data, Meckel's diverticulum requiring surgery occurred with nearly equal frequency up to fourteen years. The gravest complication in the cases of patent vitelline duct were a T-shaped protrusion of ileum and a small bowel volvulus around the fibrous cord or the patent duct; and in the cases of Meckel's diverticulum causing symptoms, intestinal obstruction, bleeding peptic ulceration or inflammation. Three deaths occurred in newborn age in connection with patent vitelline duct, and one patient died who belonged to the group of asymptomatic Meckel's diverticulum.