Paediatric mesenteric lipoma, an unusual cause of repeated abdominal pain. A case report.

Fatty masses, especially solid lipomas, in the paediatric abdomen are very rare. We present such a case, that of an 11-year-old boy who was admitted with abdominal pain and distension. The pre-operative diagnosis of lipoma was suggested by US and CT. The diagnosis of simple lipoma arising in the leaves of the small bowel mesentery, without immature cells, was verified microscopically after the operation. The tumour was enucleated from the mesenterium leaving the intestine intact. We underline the importance of US and CT as pre-operative diagnostic tools.

Sacrococcygeal teratoma in Sweden between 1978 and 1989: long-term functional results.

Thirty-four patients operated with sacrococcygeal teratoma in Sweden between 1978 and 1989 were reviewed. Twenty-five children were available for functional follow-up. The patients and their parents were interviewed for bowel and micturition habits. Fourteen patients from this number were subjected to anorectal manometry (56%). Fifteen children reported normal bowel habits (60%). In 10 patients (40%) soiling was observed. However, four of them were under 3 years of age and wearing diapers, which made the functional evaluation difficult. The manometries showed normal and resting tone and squeezing pressures in 10 patients and subnormal values in four patients who also had soiling problems. All investigated children showed normal rectoanal inhibition reflex. Twenty-one patients reported normal micturition, in four (16%) urinary incontinence was recorded. Two of the latter children required intermittent clean catheterization, one was on medication and the last one lives without any treatment. No difference in functional outcome was noted between patients with intrapelvic or extrapelvic tumor location. Retrospectively, it is not possible to know whether the observed functional outcome is due to tumor growth or the result of surgery. Preoperative clinical investigation and in some cases anorectal manometry and cystometry could theoretically resolve this problem.

Tissue expansion facilitates operation of large myelomeningoceles.

Two children had delayed operations for large myelomeningoceles. As a preparatory operation one silicon tissue expander was inserted on each side of the lesion and successively inflated by weekly injections of saline. After an expansion time of 5 to 6 weeks the definitive procedure was performed. The newly formed fibrous capsule greatly facilitated the reconstruction of the spinal canal. The surplus of skin and subcutaneous tissue allowed the large defects to be easily close in the midline. Both children managed quite well postoperatively.

Neuron-specific enolase as a marker for intestinal neurons. An immunocytochemical study of the human intestinal tract.

Surgical specimens from various parts of the human intestinal tract as well as suction biopsy specimens, including mucosa and submucosa of the rectum, were fixed in formalin and embedded in paraffin by routine procedures. The distribution of immunoreactive areas indicating the presence of neuron-specific enolase (NSE) was then determined by using a sheep anti-human-NSE antiserum prepared in our laboratory. The immunocytochemical method revealed, in distinct contrast to other tissue components, the cell bodies of ganglion cells in the submucosa (Meissner's plexus) and in the muscle layers (Auerbach's plexus). The nerve bundles of the submucosa, of the muscle layers, and of the subserosal connective tissue were also stained, whereas the thin nerve processes of the mucosa were identified only rarely. The smooth muscle cells were stained weakly, but this reaction did not interfere with the identification of the neurons and their processes. Immunocytochemical demonstration of NSE is obviously a valuable additional method for visualization of the intrinsic intestinal innervation. It might well be that this technique will be of advantage in the diagnosis of pathologic processes, such as those occurring in Hirschsprung's disease and allied conditions.

Infection as a cause of peritoneal catheter dysfunction in ventriculo-peritoneal shunting in children.

Peritoneal catheter dysfunction is a significant complication of VP shunting in children. Improvement in technique and material should reduce complications such as detachment and breakage. Shunt infection, even if subclinical, should be suspected as the cause of early peritoneal catheter obstruction, especially if recurrent, as well as in cases of organ perforation. Cultures, prolonged if necessary, should be taken and infection should be treated prior to shunt revision. We have had no recurrence of infection or peritoneal catheter dysfunction after treatment with exteriorization of the infected shunt, antibiotic therapy and insertion of a new shunt on the contralateral side. In infected cases antibiotic therapy, both pre- and intra-operatively, should be a matter of course. With these precautions a better result with the ventriculo-peritoneal shunt should be achieved.

Complications in the treatment of hydrocephalus in children. A comparison of ventriculoatrial and ventriculoperitoneal shunts in a 20-year material.

Two homogenous materials of hydrocephalic children operated upon during a 20-year period (1961-1980) are compared. During the first half of the period the ventriculoatrial shunt was the only type used in 103 patients. After that there was a change to the ventriculoperitoneal shunt which was used in 104 patients, whereof 35 were converted from ventriculoatrial to ventriculoperitoneal shunts. Thus the material consists of 172 patients. The complications were less serious and the mortality rate much lower with the ventriculoperitoneal method. The revision rate, however, was about the same for both types of operation, but the revisions and the operative procedure for peritoneal shunts were generally much more simple and more rapidly performed. Therefore it is our opinion that in children the ventriculoperitoneal route is preferable to the ventriculoatrial route. With better knowledge of the underlying cause of distal obstructions in the former route we think that it will be possible to reduce the complication and revision rates even further.

A boy with true hermaphroditism and sex chromosome mosaicism and a fertile woman with Turner mosaicism in a family with a translocation 8p:19P.

In a family with a balanced translocation t(8;19)(p21p13), there was a boy with true hermaphroditism and a karyotype 46,XX/46XY, t(8p;19p), and a woman with Turner mosaicism 46,XX, t(8p;19p). Both of them had whole body chimerism, which in the boy and possibly also in woman was due to the occurrence of double fertilization followed by fusion of the zygotes. The pathogenetic importance of the translocation for the development of these aberrations, and the clinical picture in the two patients are discussed.