Biomedical subjects
Publications and source records attributed to J Moscovici.
Trough two cases of Sternopagus, study of classification of conjoined twins with precise definition of anatomic characteristic of Sternopagus. Description of our own subject and review of literature. At the end of this study, it is evident that the heart and vascular fusion is always complicated. So, the true Sternopagus are inseparable and no likely to live, in opposition with Xiphopagus and Thoracopagus.
One hundred eleven patients underwent a surgical procedure for correction of intractable gastroesophageal reflux. Twenty children were severely mentally retarded. The range of follow up was 6 months to 15 years. Upper gastro-intestinal series was realised at tenth post-operative day for 111 children, then during the first year for 97 children, between the first and fifth year for 62 children and after the fifth year for 25 children. The first upper gastro-intestinal control (at tenth day) was normal for 111 patients. Next controls revealed, usually during the first post-operative year, an esophago-gastric junction anomaly for 49 patients (44%). Most of them are light (41/111 = 37%): occasional reflux (9 children; more frequent without pyloroplasty) of little paraesophageal hernia (32 children; more frequent with closure of esophageal hiatus by two suture lines, with esophageal fixation on median arcuatum ligament or without esophageal fixation on esophageal hiatus); these light anomalies are symptom free and sometimes transitories. Rarely, anomalies are important (8/111 = 7%): big paraesophageal hernia of recurrence (more frequent with partial posterior fundoplication and in children severely mentally retarded); five patients were symptomatic and had to be reoperated. Also now, we don't use partial posterior fundoplication and in mentally retarded children we prefer a Collis procedure.
The authors report their experience concerning nuclear magnetic resonance imaging (NMR) in 7 children. They emphasize its importance in the post-operative study of portal hypertension. All those children got surgically created porto-systemic shunts. NMR imaging is easy, safe and trustable. It is able to visualise the shunt itself and to demonstrate the patency of this shunt. So, it is interesting when the usual controls have failed, particularly abdominal ultrasonography.
110 arteriovenous fistulas made for hemodialysis, chemotherapy and extended parenteral nutrition are studied. For authors, the side-end radial arteriovenous fistula is the main used, followed by the humero-basilic shunt. The immediately functional fistula's percentage was of 81% with a net progress in the last year since it is now at 92%. Thrombosis prevails in early complications. 32% of delayed complications are minutely studied. They include thrombosis, infections, trophic disturbance of hands, secondary stenosis, hyper-flow with cardiac consequences. Others more unusual delayed complications are described ("Stealing" syndrome, no venous distention, bleeding, pseudo-aneurysm). The authors advice the side-end arteriovenous fistula for hemodialysis and humero-basilic shunt with immediately basilic superficialization for infant's hemodialysis, for chemotherapy and parenteral nutrition.
Two cases of ureteral triplication are reported, on in a child of 4 years the other in a 2-week-old baby. In both cases the triplication was associated with one or two ureteroceles. A literature review is used as a basis for describing the characteristics of this rare malformation.
Attainment of continence after treatment of supra-levatorian ano-rectal malformations is frequently hampered by difficult problems. Cinedefecography and particularly conventional manometry along with myography allow to analyse the different factors involved in incontinence, in connection on the one hand with the type of gut pulled-down, and on the other hand with the sphincteric system. This study allows to propose, under specific circumstances, suitable techniques aiming at modifying either the gut contents, or the properties of the pulled-down bowel, or to reinforce the spincters, particularly with biofeedback rehabilitation techniques.
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Three cases of esophageal stenosis with tracheo-bronchial remnants within the wall of the esophagus, near the cardia, are reported: two girls and one boy; in three, this anomaly contains cartilage remnants. After various procedures, esophagoplasty was necessary when the boy of the first case was fourteen. Two girls was cured by a Thal procedure with follow-up of five and four years respectively. A survey of the references found 43 other cases. This anomaly is more frequent in girls. Tracheo-bronchial remnants are located usually in the esophagus near the cardia and contains cartilage. Rarely the respiratory inclusion is seen in the middle part of the esophagus and no cartilage is then identified. The stenotic segment is lined by squamous cell epithelium and the heterotopic tracheo-bronchial structures are intramural implants. The diagnosis should be suspected in cases of esophageal obstruction presenting in childhood or adult life with a history dating back to infancy or childhood. Radiologically a short stenotic non relaxing segment is the hallmark in the distal esophageal localisation. Resection of the stenotic segment with primary anastomosis is the method of choice in the middle part of the esophagus. That procedure is a good alternative in the distal esophagus.
The ureterovesical junction was studied by dissection and serial sections in 50 post mortem specimens. Three points are considered in this paper: the structure of the terminal ureter, the anatomical arrangement of the ureteral hiatus and the ureteral sheath. Study of the structure of the intramural segment of the ureter demonstrated on one hand, the perfect continuity of the terminal ureter with the trigone and on the other hand, the abundance of the fibroelastic connective fibers which like the muscle fibers run longitudinally. The compliance of the intravesical ureter is dependent on the balance between these two components. The modification of this balance can lead to the creation of a functional obstacle. The anatomical arrangement of the ureteral hiatus is described. The inner muscle layer of the detrusor extends almost to the ureteral orifice, the truly submucosal part of the ureter thus being very small. The constitution of the ureteral orifice and its relations to the ureter account for the different positions of juxtaureteral diverticula and transhiatal herniae of the bladder mucosa. The many descriptions of the ureteral sheath appearing in the literature are reviewed in light of the findings from the present study. The juxtavesical segment of the ureter is surrounded by a fibroconjunctive sheath which fixes the ureter to the bladder wall. The transparietal segment of the ureter is ensheathed in its adventitia, whereas a fibromuscular sheath cannot be truly individualized over this ureteral segment.
Palliative in utero treatments of certain malformations of the fetal urinary tract are now possible. The most important of these is urethral obstruction by valves. A case of in utero treatment of such a disease is reported, with a six month follow-up of the infant. In utero diversion of urine was performed by means of a catheter placed in the bladder using a technique derived from intra-uterine transfusions. The purpose is to permit drainage of urine from the kidneys into the liquor to prevent permanent damage to the kidneys because of back pressure. The technical problems included deciding the correct size of catheter and the technique of localising the bladder and inserting the catheter under ultrasound control. The ethical problems are raised partly because there is no central ethical committee to make decisions and partly because of the need to evaluate and to inform the patients of the various risks. The patients take the risk on behalf of their children and the mother takes a small risk for her own health.
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The authors reported four cases of mesocaval interposition shunt in children with autogenous vein graft. In three cases, external iliac vein was used as the graft because its appropriate size in diameter and length; for local reasons, jugular graft was used in last case. Post-operative controls by angiography in all patients except the one recent case showed a patent anastomosis; esophago gastric endoscopy three month post operatively in the last case showed regression of varices. External iliac vein is convenient to this shunt. Edema of the lower limb is minimal and well tolerated. The control of hypersplenism and esophogastric varices is progressive with time.
Authors report their experience of the treatment of anterior hypospadias which represent more than 71% of these cases operated all along the eight past years. The different aspects of these malformation are emphasized. For the majority of these cases the correction of the malformation may be obtained by a simple balanoplasty which gives the gland a normal form and height though directing the meatus forward; the redundant foreskin is excised and the penile skin torsion is corrected aligning the median raphe; a deficit of the ventral skin case be filled in with preputial flaps. This technic was used in fifty of the reported cases with very good results both functionally and cosmetically. This procedure is comparable to the technic described by J.W. Duckett and called Magpi for meatal advancement and glanduloplasty incorporated. The results reported here corroborate the good results already published.
The authors present their experience of balanoplasty in the treatment of anterior hypospadias. These forms of hypospadias are defined as those where the meatus is situated at the base of the glans, at the level of the balano-preputial groove or just before, without any real chordee. The treatment involves re-siting the meatus in the penis. These forms represent 70% of all cases of hypospadias. This type of hypospadias is corrected by an extensive meatoplasty towards the tip with reconstitution of the glans and re-centering of the sheath of the penis. This gives good aesthetic and functional results. This technique was designed by J. W. Duckett who named it M.A.G.P.I. (meatal advancement and glanduloplasty incorporated) and has been used in 78 cases; the indications, the method and the results are presented.
The authors review 178 cases of anomalies of the pyelo-ureteric junction, with a total of 189 anomalies. Almost one half of the cases were less than 5 years old (47.2%) and about 1/4 were less than 1 year old (24.7%). In girls, the incident which reveals the diagnosis is most often an infection and in boys, it is pain or haematuria. 26% of the cases had an associated urological lesion (23 cases of vesico-ureteric reflux, 8 cases of renal stones, 5 cases of mega-ureter and 4 cases of horseshoe kidney). 133 children were operated, essentially by resection of the pyelo-ureteric junction with an upstream by-pass. From the 126 cases with sufficient follow-up, 48 (38.1%) obtained great improvement, 63 (50%) a moderate improvement and 15 (11.9%), a stabilisation of the dilatation. In all cases except 3, the pyelo-ureteric junction was perfectly catheterizable. The histological lesions of the junction were variable and difficult to systematise, but usually extended beyond the junction to involve a large part of the pelvis. The variability of the caliceal dilatation makes any attempt at classification difficult. The authors recommend cystography in every case, as associated reflux was discovered in 16% of cases. Retrograde uretero-pyelography is practically no longer indicated. The isotope kidney scan with DTPA technetium, with a hyper-diuresis test, is often used, as it gives an idea about the clearance of filtration and about the degree of obstruction. Quantitative DMSA kidney scan can reveal the effects on the renal parenchyma. Conservation of the isthmus of a horseshoe kidney never interferes with the uretero-pyelic re-implantation. After correction of the anomaly of the uretero-pyelic junction, 5 cases of unilateral reflux out of 8 and 3 cases of bilateral reflux out of 10 resolved spontaneously.
Thirty six patients with Hirschsprung's disease were treated with a Duhamel pull-through procedure. Of the patients 32 were boys, 31 were diagnosed in the first three months of life, 30 had aganglionosis restricted to the rectum and to the sigmoid colon. The absence of mortality and the low complication rate confirmed that the Duhamel operation is a benign procedure. Highly satisfactory clinical and radiological results were observed during the follow-up period. Some patients presented a clear pressure fall of anal tonus with inflation of the pelvic digestive tract balloon. The sensing capabilities and the efficacy of this reflex were not so high than in normals controls. The relationship between the existence of this reflex and the clinical story of the patients might be interesting to follow-up in the future.
Four cases of xanthogranulomatous pyelonephritis in children are reported. In three cases, the disease took a diffuse form and in the fourth it was of a localised form. Around these cases, the authors review 39 case histories published between 1963 and 1978 and which involve only children. An over-all assessment of the 43 cases thus gathered together allows the general picture of xanthogranulomatous pyelonepn 9 cases, it was of the localised type; and in 4 cases, the pyelonephritis only involved one or another pyelon in patients with re-duplication. The diffuse type is more common in boys and the localised type in girls. Twenty-nine cases were found in children of less than five years. In 23 cases, there was a proteus infection. The etio-pathogenesis of xanthogranulomatous pyelonephritis remains obscure. The main clinical and therapeutic problems are dealt with.
The authors reported seven cases of megaesophagus by achalasia in children treated by transabdominal esophagomyotomy. There were five girls and two boys. Nor infantile neither familial forms were seen. Five children were cured clinically and radiologically at the third post operative month. Two failures were successfully treated by Thal's procedure. We concluded: (1) Infantile achalasia is not rare in recent literature; recurrent respiratory troubles and familial forms are frequent; prognosis is serious in this age groups. (2) Esophagus's width divided by thoracic vertebral corpus's height is a more objective criteria to appreciate megaesophagus's importance than esophagus's width alone. (3) Endoscopic biopsy is inadequate to detect tracheo bronchial remnants. (4) Appropriate treatment in children is surgical esophagomyotomy with fundoplication.