Biomedical subjects

S Hofmann-v Kap-herr

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

7 recordsLinked to original sources

PubMed2002

The clinical relevance of non-classified dysganglionoses and implications for a new grading system.

In addition to the classified types of dysganglionosis, certain non-classified dysganglionoses (NCD) (types 1-4) were introduced by Meier-Ruge in 1992. Clinical data on these conditions are limited. Among 134 children with intestinal dysganglionoses (ID) treated from 1979 to 1999, 12 were identified to have a NCD. Their clinical course is presented. The existence of mild ID (type 1) is difficult to demonstrate. Current definitions and data on clinical relevance are not convincing. An indication for surgical treatment is not present. Isolated hypogenesis of the submucous plexus (SMP) (type 2, n = 8) is clinically a more severe kind of intestinal neuronal dysplasia type B and often requires early surgical intervention, but not resection. When associated with aganglionosis, its recognition is important for surgical strategy, to avoid complicated clinical courses, which are frequent if total or nearly-total resection is not performed. Hypogenesis of the myenteric plexus (MP) (type 5, n = 1) has received little attention so far. The sporadic appearance of heterotopic nerve cells of the SMP in the mucosa (type 3, n = 1) is physiologic; clusters of such cells, however, are probably of pathologic value, especially in combination with other types of ID in the same patient. Heterotopic nerve cells of the MP (type 4, n = 3) in the circular and longitudinal muscle layers are highly pathologic. This clearly-defined type is of major clinical relevance and requires complete resection. A severe disturbance of the migration process is the underlying cause. To simplify the terminology of IDs, a grading system based on the anatomic structures and clinical findings is proposed: innervation disturbances of the mucosa (grade I) are of limited clinical significance. Isolated malformations of the SMP (grade II) may require an enterostomy, but do not require resection except in certain cases associated with distal aganglionosis. Dysganglionosis of the MP (grade III) usually exhibits more severe symptoms and resection is indicated, especially with associated hypo- or aganglionosis. In aganglionic bowel (grade IV) resection is mandatory.

Child↗

PubMed2000

Complex long-segment intestinal dysganglionosis.

A case is reported with aganglionosis of the rectum, sigma, and descending colon; dysganglionosis with heterotopic ganglionic cells in the muscularis propria of the hypoganglionic transverse colon; and extreme hypoganglionosis (without detection of ganglionic cells) of the ascending colon and distal ileum. The ileum showed a transition zone with hypoganglionosis and intestinal neuronal dysplasia (IND) type B. As to the etiology of such complex intestinal innervation defects, pre- and perinatal perfusion deficits must be considered because their localization seems to be linked to the vascular anatomy of the colon. Early diagnosis may be difficult, causing a delay in operative treatment and multiple operations. Different manifestations of dysganglionosis may be found in the same patient. The classical continuum of distal aganglionosis followed by proximal hypo- or dysganglionosis and then normally innervated bowel may not always be present. Therefore, in children with recurrent (sub-)ileus after resection of an aganglionic bowel segment, additional dysganglionosis such as IND or hypoganglionosis or even complex intestinal dysganglionosis should be excluded by full-thickness colon and small bowel biopsies.

Hirschsprung Disease↗

PubMed1989

[Traumatic hip dislocation in childhood].

The article reports on eight cases of traumatic dislocation of the hip in children. Six of these were genuine dislocations and two dislocation fractures. The children were between 5 and 13 years of age at the time of injury. Seven of these 8 children could be followed up one to 21 years after the accident. All 7 children were free from complaints at the time of follow-up examination; in one case only we found a moderate loss of function in the injured hip joint. In this patient the x-ray film showed deformation of the head of the femur after partial necrosis of the femoral head, as well as initial signs of coxarthrosis. Prognosis of this rare injury in children is favourable if repositioning is performed in time and if relief of the hip is effected for the proper period of time, depending on the individual case.

Adolescent↗

PubMed1987

Endotracheal and endobronchial tumours in childhood.

Following a review of the literature on the most common types of endobronchial tumours (carcinoid cylindromas and mucoepidermoid tumours), four cases of such bronchial tumours in children are reported. Two of them underwent isolated bronchial resection, whereas one of the others had to undergo bilobectomy in addition and the other, pneumonectomy. The patients remained free of recurrences after a follow-up of 5-20 years. The advantage of circumferential resection and some technical aspects are discussed.

Adenoma↗

PubMed1986

[Post-traumatic radio-ulnar synostoses in childhood].

This article reports on five children treated surgically during 1973-1975 for posttraumatic radioulnar synostosis. The synostoses had developed as a complication after a proximal radial fracture. Lesion of the proximal ulna plays an important part in creating such an osseous union. Another factor of equal importance is the traumatization of the tissues during surgical treatment of the fracture, especially if debris of the periosteum and fine bone fragments additionally enter into the space between radius and ulna. Formation of excess callus, and hence synostosis, is also promoted if repositioning or re-repositioning of a proximal radial fracture is effected too late. The article presents the technique of surgical treatment of proximal radioulnar synostosis with Lyodura sheathing. Follow-up examination of the five children during an observation period of five years showed good results in two children, a moderate result in one, and poor results of surgery in two cases. Improved results may be expected from further improvement of the surgical method, such as resection of the bicipital tuberosity (tuberositas radii) or from additional partial sheathing of the ulna at the side facing the radius.

Adolescent↗

PubMed1985

Pulmonary arteriovenous fistula in childhood.

We report two cases of children with pulmonary arteriovenous fistula treated at the University Clinic of Mainz. This angiodysplasia is relatively more diffuse and the clinical signs are more marked in childhood. The non-invasive techniques should be preferred for diagnosis. Complications occur frequently even in asymptomatic children so it is recommended to operate them. The local excision without extirpation of lung tissue should be preferred to segmentectomy or lobectomy for peripheral lesions. Therapeutic embolisation should be tried in inoperable cases.

Arteriovenous Malformations↗

PubMed1981

[Indication of appendectomy in chronic appendicitis in children. (author's transl)].

The results of the study presented in this paper are based on a comparison of two equally large groups of 33 children each with chronic appendicitis on whom appendectomy had been performed, as well as 33 children with inguinal hernia and relevant surgery. The preoperative and postoperative signs and symptoms were compared in both groups. It was found that reduction of symptoms was significantly greater following appendectomy than after herniotomy. Hence, the question whether after diagnosis based on a more liberal interpretation of the indication for appendectomy in chronic appendicitis in children, the overall well-being of the children can be improve significantly, should be given an affirmative answer.

Adolescent↗