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Biomedical subjects

S Hofmann-von Kap-herr

Publications and source records attributed to S Hofmann-von Kap-herr.

At least 19 recordsLinked to original sources

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.

Abnormalities, Multiple↗

[Diagnosis and surgical therapy of thyroid gland diseases in childhood].

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.

Adolescent↗

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.

Acute Disease↗

[The concept of the acute abdomen].

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.

Abdomen, Acute↗

[Surgical therapy of Crohn disease and ulcerative colitis in childhood].

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.

Abdomen, Acute↗

[Surgical strategies in thyroid gland diseases in childhood and adolescence].

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)

Adolescent↗

[Importance of thoracic trauma in the multi-traumatized child].

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.

Child↗

[Comparison of surgical and conservative treatment methods as exemplified in the juvenile femur].

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.

Bone Plates↗

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.

Catheterization↗

[Modern diagnosis and therapy of traumatic hemobilia in childhood].

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.

Aneurysm↗

Anal sphincter substitute using autologous smooth muscle in a fold-over, half-cylinder, double plasty (SMFD-plasty): a new method of treatment of anorectal incontinence.

A new operative procedure for improving continence is presented. It is used in patients with intermediate and high types of anorectal anomalies. A smooth muscle fold-over half-cylinder double plasty (SMFD-Plasty) is performed in infants immediately following the pull-through which must be done exactly through the puborectal sling. Results in six neonates and 4 older children so far are excellent. This method is a suitable replacement, particularly for the function of the internal sphincter. Further modifications of this new method are presented. They are recommended for secondary surgery (second pull-through, levator plasty, secondary megacolon, and post pull-through anal prolapse) as well as a primary procedure for continence improvement in patients treated according to the posterior method of Pena and de Vries.

Anal Canal↗

[Mesenteric cysts in childhood].

5 children with mesenteric cysts, 3 boys and 2 girls, aged 2 to 10 years, were admitted with acute abdominal pain. In adults the most common symptom is chronic abdominal pain whereas in children the onset of symptoms is acute due to intestinal obstruction. Sonography, performed in two patients, is the diagnostic method of choice. Excision of the cyst required resection of the small intestine in 2 patients, while simple enucleation was possible in 3. All 5 patients survived, and there have been neither complications nor recurrences. Early recognition and appropriate resection of these occasionally life-threatening malformations are associated with a good prognosis.

Abdomen, Acute↗

[Appendicitis in childhood].

Appendectomy was performed on 1,059 children at the University Clinic of Paediatric Surgery in Mainz from 1. 1. 1975 to 31. 6. 1983. For the retrospective examination of the indication for appendectomy histopathological and intraoperative findings were analysed and evaluated. Histologically five types of appendicitis were differentiated: 1. acute appendicitis (two forms: acute ulcero-phlegmonous appendicitis with or without perforation and acute superficial appendicitis), 2. chronic appendicitis, 3. lymphatic hyperplasia, 4. submucosal fibrosis, 5. rare diseases. In 618 cases (= 58.3%) acute appendicitis was diagnosed histologically. In another 203 cases (= 19.2%) intraoperative findings (e.g. Lymphadenitis mesenterialis, Meckel's diverticulum) were retrospectively collected; they caused symptoms similar to those of appendicitis. However, there remain retrospectively 22% of all appendectomised children with no indication for laparotomy. The statistical analysis of postoperative complications showed a significant dependance from the histopathological findings. The highest rate of complications was seen in cases with perforated (34%) or non-perforated (10%) ulcero-phlegmonous appendicitis. Children with acute superficial appendicitis had a complication-rate of 5%; those with lymphatic hyperplasia and submucosal fibrosis of 6% each. Relaparotomies were almost exclusively necessary in cases with acute appendicitis; septic and pulmonary complications were mostly seen either in infants with malformations or other perinatal risks, or in children with additional severe diseases. Therefore non-acute appendicitis justifies a wide indication for appendectomy because of a low complication-rate; this, however, is not valid for high-risk children (e.g. malformations). In these cases sonography might be useful for preoperative diagnosis.

Adolescent↗

[Functional colon sonography in neuronal intestinal dysplasia. Report of 3 cases].

For the first time morphology and function of the colon were analysed by sonography in three children with M. Hirschsprung and neuronal-intestinal dysplasia. After one year two children attained almost normalisation of colon-peristalsis in the nonaganglionic segments. A resection of the neuronal-dysplastic segments was not necessary. In one child there was seen no change of the distal colon motility. The limit of resection at operation was determined by sonographic diagnosis following observation of stimulated motility. Sonographic long-time examination in connection with medicamental stimulation of the colon allows determination on timing and kind of therapy in neuronal-intestinal dysplasia.

Age Factors↗

[Importance of infection following laparotomy in childhood].

Laparotomy was performed on 579 children at the University Clinic of Paediatric Surgery in Mainz from 1.1.1975 to 31.12.1982. The children were up to 15 years of age; appendicitis or inguinal and umbilical hernia cases were not included. Postoperative sepsis occurred in 74 patients (12.8% of all children with laparotomy); in 51 cases positive bacteriological findings were seen besides the clinical and clinicochemical ones. Sepsis morbidity was particularly high in children who had not yet completed their first year of life (postoperative sepsis occurring in approximately every fourth infant); among the disease patterns, the following were particularly prominent: Defects of the abdominal wall (23 out of 50 children developed postoperative sepsis); intestinal atresia (18 out of 59 children); intestinal perforation (11 out of 39 children). In addition, sepsis morbidity was enhanced after relaparotomies. Gram-negative bacteria were most frequent among the 51 patients with bacteriologically positive findings; these bacteria consisted mostly of representatives of the group of enterogenous pathogens. These groups of bacteria were also the most frequently occurring pathogens in mixed and secondary infections. 33 out of 74 children with postoperative sepsis died. The mortality rate was 68% in prematurely born infants compared with mature newborns. Lethality was highest among children with congenital defects of the abdominal wall and intestinal perforations. Among the patients with bacteriologically positive findings the lethality was particularly high with multiple attacks of sepsis, in case of septitides caused by multiple pathogens, by Candida albicans and after relaparotomy.

Adolescent↗

[Optical-acoustical analogous biofeedback conditioning in the treatment of fecal incontinence in childhood].

This is a report on the treatment of anal incontinence by biofeedback conditioning. Since August 1982 9 children with incontinence (8 rectoanal agenesia, one Morbus Hirschsprung) were treated by simultaneous optical and acoustical analogous biofeedback conditioning. Training of sensitivity and voluntary contractions were performed and controlled electromanometrically. According to the pressure waves electrical alterations were transformed and feedback visually and acoustically. Results of the training of the maximal voluntary contraction are presented and the superiority of this method for the coordination of the anorectal sphincter function is stressed.

Adolescent↗