Cystic rectal duplication: a rare cause of neonatal intestinal obstruction.

A case of cystic rectal duplication revealed on day 2 of life by a low intestinal occluding syndrome is reported. Radiologic imaging (ultrasonography, cystography, rectography) showed a large, retrorectal liquid formation in the pelvis and abdomen, with pelvic compression of the terminal alimentary canal and lower urinary tract. Magnetic resonance imaging demonstrated a liquid formation with clearly defined edges and no medullary involvement, thus ruling out the possibility of a previous meningeal hernia. Biological markers were within normal limits. On day 4, a 9 x 6-cm cystic rectal duplication was removed, followed by a temporary colostomy. Pathologic examination demonstrated typical rectal architecture with ciliated cells. Radiologic and clinical findings at 2-month follow-up were reassuring. This case report is exceptional for the following reasons: (1) As a rule, rectal duplications are relatively rare (70 cases reported in the literature); (2) The means of disclosing a neonatal rectal duplication is unusual (4 cases reported in the literature); (3) The volume of the malformation was considerable; and (4) Heterotopic ciliated epithelium was present.

Localized esophageal leiomyoma and hypertrophic osteoarthropathy.

The authors report the case study of a 13-year-old girl, suffering from epigastric pain related to an important infectious syndrome and from marked hypertrophic pneumosteopathy (clubbed fingers, diffuse cortical hyperostosis). Radiological examination (chest x-ray and computed tomography scan) showed a partly calcified right posterior mediastinal mass. The esophagoscopy showed a fistular emanation from the esophagus to the tumor. The biopsy results were of no diagnostic significance, and the samples demonstrated a bacterial and fungal colonization. An endoscopic sonogram showed an inhomogeneous mass with hyperechoic structures developed between the mucosa and the outer muscular coat of the esophagus. The patient underwent surgery, which showed a large intraparietal tumor of the esophagus, which, through pathological examination, was confirmed to be a benign leiomyoma. Postoperative follow-up was simple and the pneumosteopathic symptoms regressed rapidly. This disease is rare in children under 15 years of age. It is only exceptionally associated with a hypertrophic pneumosteopathy. This pathology is diagnostically challenging to the surgeon. The endoscopic ultrasonography is the best procedure in the assessment of subepithelial tumor and extrinsic compressions of the oesophagus.

Antenatal finding of intestinal obstruction caused by isolated segmental jejunal dilatation: a case report.

The authors report a case of segmental jejunal dilatation that was discovered antenatally and manifested clinically as lower obstruction in a newborn boy. The antenatal ultrasound findings at 29, 30, and 31 weeks' gestation showed a constant hypoechogenic image (6 x 3 cm in diameter) localized to the right flank of the fetal abdomen, thought to be situated in the right colon. A prenatal diagnosis of incomplete right colonic stenosis was considered. In the immediate postnatal period, there were clinical manifestations of intestinal obstruction; however, results of a contrast enema and rectal biopsies were normal. An upper gastrointestinal contrast study showed a dilated jejunal loop situated approximately 15 cm from the ligament of Treitz. During surgery, a large dilated jejunal loop (7 cm in diameter, 15 cm in length) was found and resected. Histopathologic examination confirmed the diagnosis of segmental jejunal dilatation. This is the first antenatal illustration of this rare pathology, and it supports the hypothesis of a congenital origin for this anomaly.

Systemic cat scratch disease: hepatic and splenic involvement about 3 pediatric cases.

Numerous diseases can lead to multinodular lesions of liver and spleen; surgical biopsy can be required for the etiologic diagnosis. Among these diseases, systemic cat scratch disease has been recently described. Macroscopical appearance of the lesions is evocative and must be known by surgeons. Three children with systemic cat scratch disease involving liver and spleen are reported.

Congenital extra-hepatic portocaval shunt. Concerning a case of antenatal diagnosis.

The authors report a case of congenital extra-hepatic portocaval shunt in a two-year-old girl. While pointed out antenatally by ultrasound and suspected by a follow-up ultrasound at the age of two years, this anomaly could be confirmed only by coupled ultrasound scanning and color Doppler, which allows for dynamic anatomic and morphologic evaluation. Comments concern mainly the embryologic hypothesis and the clinical tolerance of this malformation (because no therapeutic measures were adopted).

[A little known cause of persistent fever and granulomatous hepatitis in children: cat scratch disease]. / Une cause peu connue de fièvre prolongée et d'hépatite granulomateuse chez l'enfant: la maladie des griffes du chat.

BACKGROUND: Extensive hepatic and splenic involvement in cat-scratch disease has rarely been reported. CASE REPORT: A 2 1/2 year-old boy suffered for 2 weeks from high-grade fever, abdominal pain and alteration of his general condition. Ultrasonography revealed multiple hypoechogenic nodules in liver and spleen. The CT scan also showed hypodense lesions. An open liver biopsy was performed 5 weeks after the onset of illness. Histopathology of a resected nodule demonstrated neutrophilic granulomatous inflammation with central abscess formation. Recent cat exposure, suppurated epitrochlear lymph node 15 days before admission were also consistent with cat-scratch disease which was confirmed by elevated anti-Rochalimaea antibody titers. Follow-up showed complete resolution of all hepatic and splenic lesions within 6 months and emergence of splenic calcifications. CONCLUSION: Cat-scratch disease should be considered in the diagnosis of fever of unknown origin and hepatosplenic abscesses in children.

[Prenatal diagnosis of a small intestinal volvulus]. / Diagnostic anténatal d'un volvulus du grêle.

Ninety-five percent of the neonatal cases of bowel atresia result from jéjuno-ileal obstructions. Frequency is estimated from 1/3,000 to 1/5,000. We observed a case of small bowel volvulus secondary to jejunal atresia diagnosed at 35 weeks gestation. The ultrasound examination was performed due to decreased perception of active fetal movements. Fetal extraction was successful before perforation and meconial peritonitis.

[Posterior epispadias with unknown complete duplicated urethra]. / Epispadias postérieur avec duplicité uréthrale complète méconnue.

The authors report a case of posterior epispadias associated with complete ventral urethra only discovered during the urethroplasty phase of the operation. This malformation is a rare form of urethral duplication and should be considered in any case of apparently isolated posterior epispadias.

Renal oncocytoma in a child.

Oncocytoma is an exceptionally rare renal tumor in children. We report on one case of a twelve-year-old girl treated by nephrectomy.

[Chronic intestinal pseudo-obstruction syndrome in infants and associated anomalies]. / Le syndrome de pseudo-obstruction intestinale chronique du nourrisson et les anomalies associées.

The authors report 2 cases of functional intestinal pseudo-obstruction in infancy associated with intestinal and urologic anomalies. In the first case many intestinal obstructions occurred from the age of 3 weeks and the boy was operated on at 14 months of age. A short small bowel and an intestinal malrotation were found at surgery; the alimentary canal was completely aperistaltic, and an ileostomy was performed. Further operations were carried out, for obstruction due to adhesions, and lastly to perform another ileostomy. An antenatal diagnosis of megacystis had been made with ultrasonography. In the second case, the pseudo-obstruction syndrome occurred at the age of 1 month, due to a volvulus of the small bowel with malrotation. A second operation, one month later because of lack of intestinal transit showed an aperistaltic bowel and a colostomy was performed. The intestinal continuity was set up again at the age of 9 months and a fractional feeding was started. A megacystis was found during urologic investigations. Growth is correct for both children at 3 years of age. A review of the literature allowed to list the most frequent digestive or extradigestive anomalies associated with this syndrome.

[The possibilities of endoscopy in the treatment of various childhood ureteroceles. Apropos of a case]. / Possibilités de la voie endoscopique dans le traitement de certaines urétérocèles infantiles. A propos d'une observation.

A case is reported of a large left ureterocele in a duplicated system at the expense of the upper renal pelvis. This ureterocele caused acute pyelonephritis and dilatation of both left systems. It was treated by a classic endoscopic incision of its roof but 18 months later, infecting vesico-renal, grade 2, reflux was developed. This secondary reflux was treated successfully by submeatal polytetrafluoroethylene's (Teflon) paste injection. This new combination of endoscopic techniques proved to be available in this case.

'Sting' procedure in the treatment of secondary reflux in children.

The authors present their experience (24 children) with the endoscopic subureteral injection of Teflon paste in the correction of secondary vesicoureteral reflux: reimplantation failure; ureterocele; obstructive bladder; exstrophy; postchemocystitis, and Hutch's diverticulum. Of these 24 cases (31 refluxing ureters), 18 were treated with success. Each cause justifies a comment on the particularities of its endoscopic treatment. The 'sting' can be particularly attractive to cure secondary reflux, avoiding the difficulties and complications of open surgical reimplantation.

[Carpal tunnel syndrome in children. Apropos of 4 personal cases]. / Le syndrome du canal carpien chez l'enfant. A propos de 4 observations personnelles.

Four cases of Carpal Tunnel syndrome during childhood are reported. Two of them are post traumatic cases; for one child, the syndrome was caused by hypertrophic cal six months after trauma. The other child has been operated because of a median and ulnar nerve deficit syndrome, which appeared one month after fracture. In the third observation, the authors have seen an abnormal muscle just in front of annular anterior ligament of the carpus during surgery. This muscle was assimilated to hypertrophic Palmaris Brevis muscle. In the last observation, the adolescent had a Poland syndrome with Sprengel malformation. In this paper, the authors point out clinical particularities due to musculo-tendinous malformation which can lead to Carpal Tunnel syndrome.

[Urachal pathology]. / La pathologie de l'ouraque.

Reviewing 5 recent cases, different anatomical and clinical forms of partial or complete urachal persistency are described: patent urachus, urachal cyst, urachal sinus and urachal diverticulum. The risk of late malignant evolution of this embryonic vestige justifies radical surgical removal at time of diagnosis.

[Comparative characteristics of the course of upper obstructive uropathies diagnosed during the perinatal period]. / Caractères évolutifs comparés des uropathies obstructives hautes dépistées en période périnatale.

This study compares two groups of 30 infants each, less than 1 month old. The first group includes neonates with ureteropelvic junction obstruction (UPJO) and the second with primary obstructed megaureter without reflux (POM). Diagnosis was made prenatally in 80% of UPJO and 64% of POM. 19 infants with UPJO and 24 with POM no required immediate surgery but have been just supervised. Contrary to UPJO, even grade III POM can present spontaneous resolution. Radiographic improvement is seen generally from 3d to 4th++ months. Tc-DTPA renal diuretic scan (10 cases) and percutaneous pyelomanometry (4 cases) are more accurate tests for UPJO than for POM.

[Treatment of vesico-renal reflux in children with endoscopic injection of polytetrafluoroethylene (teflon). Apropos of a series of 100 cases of reflux in 72 children]. / Le traitement du reflux vésico-rénal de l'enfant par injection endoscopique de polytétrafluoroéthylène (teflon). A propos d'une série de 100 reflux chez 72 enfants.

The endoscopic subureteral injection of teflon is a new alternative to correct vesicorenal reflux in children. We report the use of this procedure in 72 children presenting with 100 refluxing ureters. In 98 refluxing units the disappearance of reflux was noted on a cystogram performed immediately (success rate: 98%). Followup after 6 months, the reflux recurred in 6 ureters of 52 controlled units (success rate 88.5%). Obstruction was not observed in any cases by repeated ultrasonography. The procedure is simple and reliable. In a failure that required an open operation, surgical reimplantation was not difficult. An endoscopic technique to cure reflux, of various grades would be a valuable alternative. The procedure certainly has advantages for difficult surgical reimplantations (neurogenic bladder, failed reimplant ureters). However, many years follow up are needed to demonstrate lasting success and absence of complications.

[Vesico-ureteral reflux. Ultrasonographic aspect of endoscopic treatment (excluding follow-up)]. / Reflux vésico-urétéral. Aspect échographique du traitement endoscopique (en dehors de la surveillance).

Thirty four children, mean age 6 years, with reflux of 56 ureters were treated by an endoscopic approach. The technique, a recent innovation, attempts to reinforce the intravesical trajectory of the ureter by the injection of a Teflon disk below the vesical mucosa at the ureteral meatus level. The Teflon disk used is radiotransparent but could always be detected by ultrasound imaging, whatever its size, as a very reflecting image with a posterior cone shadow.

[Foreign bodies in the body following vascular surgery (11 cases)]. / Les corps étrangers de l'organisme après chirurgie vasculaire (11 observations).

Eleven cases of foreign body (F.B.) (3 autochthonous and 8 of other sources) demonstrate the rarity but also the persistent reality of these accidents. Incidence between 1970 and 1985 was close to one F.B. per 3,000 vascular procedures. In 4 cases the F.B. followed surgical non-reconstructive surgery and in the other 7 cases revascularization procedure, material involved being most often textile, sponges in 7 cases and towel in one case. Circumstances leading to let F.B., without disculpating the surgeon, included long and difficult operations conducted as emergencies or requiring one or more recovery operations. Clinical findings of the F.B. were of post-operative sepsis (10 cases) of varied clinical expression. General (4 cases of septicemia) and local (3 thrombosis, 1 anastomotic hemorrhage) consequences were serious. Prognosis in patients with F.B. closely follows that of vascular sepsis, with maximum seriousness after bifurcated prosthetic shunt. An essential prognostic factor is early diagnosis of F.B.: it is dependent on a series of clinical and paraclinical etiologic arguments, particularly results of radiography of the operated zone, fistulography and echography. Surgical intervention is a function of initial operation and is that proposed for all cases of vascular sepsis, with the common denominator of the total revision of the operated zone and removal of foreign bodies. Prophylaxis of F.B. in vascular surgery follows the rules for general surgery; these have been enounced for the last 50 years but have failed to prevent the postoperative F.B. This rare but always possible accident justifies its knowledge by even the most serious, experienced vascular surgeon.

[Aneurysms of the infrarenal aorta and visceral cancer. Therapeutic problems]. / Anévrysmes de l'aorte sous-rénale et cancers viscéraux. Problèmes thérapeutiques.

The association of an abdominal aortic aneurysm (AAA) and a long-standing or progressing cancer is a frequent finding: 14 cases among the 112 infrarenal aortic aneurysms treated by one of us (J.C.) are discussed in this report. The marked predominance in bronchial and ORL epithelioma (50%) is explained by the common pathogenic factors of these neoplasms and atheroma. Surgical treatment is difficult because of the potentially lethal character of the two lesions: it must allow for size and possible progressive nature of aneurysm and prognosis of the neoplasm as defined by the TNM classification. Detection of an AAA in a patient with a history of neoplasm means that the opportunity for aortic surgery is dependent of therapeutic control (or otherwise) of the neoplastic disease and therefore frequently the length of follow up period after therapy. When detection of the AAA and neoplasm is simultaneous, the aneurysm progressing or ruptured, surgical complications leave little choice with regard to operative strategy. In 3 cases, simultaneous treatment of an AAA and a neoplasm was possible, particularly in the case of Grawitz tumors of cortical development without pyelocaliceal invasion. In most patients, separate operative stages are necessary in order to ensure asepsis of AAA surgery. Aneurysmal occlusion with an extrafocal shunt can allow one-stage surgery when aneurysm and neoplasm are equally menacing.

[The urorectal syndrome caused by abdomen-levator incoordination in children]. / Le syndrome urorectal par incoordination abdomino-lévatorienne chez l'enfant.

We have studied thirty four children (mean age 6.6 years) with severe functional constipation, associated or not with encopresis. In twenty three cases the defecography showed a persistent anorectal angle due to failure of puborectalis sling relaxation during defecation straining. Voiding disorders, unknown to parents, but demonstrated by obstructive uroflowmetry, are also seen in nineteen of these children. Therefore is definite a true urorectal outlet obstruction syndrome by spastic pelvic floor mechanism. The exact etiologic factor of these functional abnormalities remains undefined. All children were managed by biofeedback training. A satisfactory result (with six months follow up), has obtained in fourteen cases.