[Surgical diseases of the efferent gall ducts in newborn infants and children].
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Biomedical subjects
Publications and source records attributed to C Janneck.
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Report on 4 cases of a rare syndrome known as Currarino triad. The features of this triad consist of constipation, anorectal malformations, presacral masses and a curved defect of the os sacrum (scimitar defect). Currarino was the first to detect autosomal-dominant hereditary transmission in about 50 per cent of the patients. Each congenital or chronic constipation should prompt an early radiological examination including x-ray films of the os sacrum and the anorectum to exclude or to find a Currarino triad. The finding of a "scimitar sacrum" makes it essential to perform a contrast enema of the anorectum and a CT of the pelvic structures in the patient and his family. The possibilities of management depending on the nature of the presacral masses and their communication to the rectum and/or to the spinal channel are described. It is emphasised that a subtitle division of the rectal and spinal tissues has to be the first aim of operation to prevent a dangerous and life-threatening infection of the meningeal sac.
Case report of an extremely rare retrorectal duplication, containing gastric mucosa folds with a fistula to the rectum in a 10-year-old boy with myelodysplasia syndrome, anal stenosis and a sacral defect. Persistent tar-like stools prompted detailed investigations, followed by extirpation of the doubling. The combination of anorectal malformations, presacral masses and sacrococcygeal defect may point to a Currarino triad, an autosomal dominant hereditary syndrome which can be simply detected or identified by x-ray examination of the os sacrum.
Report on a pelvic hernia in a female neonate caused by longitudinal rectal duplication. Additional malformations were: Anal atresia with rectovaginal fistula, dysplastic kidney with partial doubling of the lower urinary tract and left foot. Pre-operative radiological examination should include: Opacification of the rectum--CT and excretory urography. The attachment of the totality of pathologic findings to partial caudal twinning syndrome is discussed.
Nine cases of a syndrome are described, which is known as the "Currarino triad" and which belongs to the group of malformations in which there is a persistent neurenteric communication. The features of the triad consist of ano-rectal anomalies (particularly ano-rectal stenoses), a curved, but limited, sacro-coccygeal defect ("scimitar sacrum") and a presacral tumour, which may be an anterior sacral meningocoele, a teratoma, a cyst (dermoid or neurenteric a cyst (dermoid or neurenteric cyst) or a mixture of these. In at least 50% of cases, the triad is familial and autosomal dominant. Of our nine cases, seven were familial. A dangerous complication is meningitis due to infection of the cystic component, or fistula formation between the colon and spinal canal (one of our cases). Other complications are a fixed filum terminale ("tethered cord") and malformations of the urogenital tract. The risk of malignancy in a teratoma is low, but exists. When considering the indications for surgery, this must be kept in mind, as must the risk of infection, and damage to neural structures during operation. In all cases of early obstipation the sacrum should be x-rayed in order to exclude a Currarino triad. The finding of a sacral defect and a presacral mass makes it essential to obtain CT of the pelvis with contrast in the distal gut and in the meningeal sac. The same is true for screening of the family (including views of the sacrum).
Intrapericardial diaphragmatic hernia is characterised by a displacement of abdominal organs covered by a peritoneal diaphragm, into the pericardium. Eventration of the septum transversum can be defined morphologically but not clinically or roentgenologically. Basing on the development of the diaphragm, it would be possible to determine intrapericardial hernia as a very early inhibition malformation.--The article describes the successful outcome of treatment of a male infant, 9 months of age, who had an intrapericardial hernia. Frequent occurrence of rupture of the pericardium with the ventral defect syndrome may allow the conclusion that isolated intrapericardial hernia and eventration of the septum transversum can be considered as minor forms of the ventral defect syndrome.
The management of 12 children is reported in relation to the use of the synthetic adhesive Butyl-Cyanoacrylate in the prevention and treatment of ileus in children. This method is discussed and contrasted to standard procedures.
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A new operative procedure is described for bat ear abnormality, resulting from isolated hypertrophy of the concha or by a ventriposition of the whole ear. The procedure is described in detail and its advantages are stressed. It is a short operation which does not leave a visible scar and achieves reliable results. The post-operative findings in 11 patients are reported.
The use of ventriculo-atrial shunts for the various forms of hydrocephalus can lead to an excessive drainage of cerebro-spinal fluid, resulting in a collapse or increasing obliteration of the ventricular system. This finding was confirmed in 76 children with congenital or acquired hydrocephalus who were examined by computerised axio-tomography (CAT scan). If raised intracranial pressure develops there will be symptoms of raised pressure which are discussed. The acute as well as the prophylactic management is described and the dehydrating effect of dexamethazone in children with hydrocephalus and a collapsed ventricular system is discussed.
Three rare cases of spherical duplication of the alimentary tract and a duplication with a pedicle associated with the ileum are reported. The treatment advocated for the duplication of the cervical oesophagus and of the ano-rectal region is the removal of the mucosa with suturing of the partially removed cyst edges. The rare intraluminous duplication of the pylorus can be removed by a duodenoantrostomy which can be performed easily in the new-born.
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The value of the ileoanal pull-through operation in the surgical treatment of children with ulcerative colitis is shown by this 10-year postoperative follow-up study of 6 patients. The results are based on a questionnaire and the performance of ileoscopic and histological follow-up examinations. In 5 of the 6 children (now adults), normal continence was present and there were no complaints. Postoperative difficulties in a girl now 17 years of age are attributed to an incorrect initial diagnosis.