Mesenteric adenitis caused by Yersinia pseudotuberculosis presenting as an abdominal mass.

Infection by Yersinia pseudotuberculosis has become of increasing pathological importance. Patients normally present with symptoms similar to those of appendicitis, due to mesenteric adenitis. We present the case of 3 patients infected by Yersinia pseudotuberculosis who in addition to fever and abdominal pain had a palpable abdominal mass, so great was the enlargement of the mesenteric nodes. In 2 patients a laparotomy was carried out, followed by biopsy of a mesenteric lymph node. The diagnosis of Yersinia infection was confirmed by bacterial culture of the biopsied material and also by serology. In the third patient, serological studies and ultrasonic imaging of the abdomen led to early diagnosis and surgery was avoided. We suggest that a diagnosis of mesenteric adenitis due to Yersinia pseudotuberculosis should now be considered in all patients presenting with an abdominal mass, and in whom there is an appropriate clinical and epidemiological history. The diagnosis should be confirmed by abdominal ultrasound (alternatively Computerised Axial Tomography or Magnetic Resonance Imaging) and serological studies. In this way, unnecessary surgery can be avoided.

Prenatal detection of a congenital pancreatic cyst and Beckwith-Wiedemann syndrome.

We report a case of congenital pancreatic cyst detected prenatally by ultrasound in a fetus with evidence for a diagnosis of Beckwith-Wiedemann syndrome (BWS). Neonatal hypoglycaemia was prevented. The cyst was managed by internal drainage. This is the second reported case of BWS associated with pancreatic cystic dysplasia and the first time that this association has been detected prenatally. Differential diagnosis of cystic abdominal lesions occurring in utero should take pancreatic cysts into consideration. This case suggests that pancreatic cysts should be included in the BWS phenotype.

[Glomerular nephropathy with IgA mesangium deposits and Crohn disease]. / Néphropathie glomérulaire à dépôts mésangiaux d'IgA et maladie de Crohn.

BACKGROUND: Renal disease is an unfrequent extraintestinal manifestation of chronic inflammatory bowel disease. CASE REPORT: A 12-year-old girl suffered from recurrent abdominal pain, diarrhea and growth impairment due to Crohn's disease of ileocaecal region. After six months of nutritional rehabilitation, an ileo-caecal resection was performed because of intestinal stenosis. The surgical procedure was followed by parietal abcess and cutaneous fistula. One year later, a purulent secretion came out of the fistula associated with fever, hematuria and acute renal failure. Renal biopsy confirmed IgA nephropathy. The course was favorable under parenteral nutrition and after surgical closure of the sigmoido-cutaneous fistula. The microscopic hematuria only persisted but the nephropathy did not relapse even during a further digestive exacerbation. CONCLUSION: IgA nephropathy has been reported in association with chronic inflammatory bowel disease. Its mechanism remains unclear: increased mucosal IgA production in inflammatory bowel, increased serum IgA and/or immune complex deposition in the renal mesangium appear the most relevant hypotheses.

[Replacement continent bladder in dogs by opening an antro-pyloric graft onto the abdominal wall (seven cases between 1980 and 1983)]. / Etude d'une vessie continente de remplacement chez le chien par ouverture à la paroi abdominale d'un greffon antro-pylorique (sept cas réalisés de 1980 à 1983).

To determine the usefulness of antral bladder controlled by the pyloric sphincter denervated for urological applications, bladder replacement was performed in 17 dogs using the antral segment with its own sphincter, initially with the pylorus supplied by the left gastro-epiploic artery, and later by the right gastro-epiploic artery. The smooth pyloric muscle was denervated to obtain permanent spasm and pulled through the striated anterior abdominal muscle. Both ureters were anastomosed by a Cohen's procedure. The best results were obtained with a gastric pouch supplied by the right gastro-epiploic artery; dissection and reimplantation of the ureters in the gastric wall are difficult. IVP and cystography indicated good function of the antral pouch, with no reflux. The antral bladder controlled by the denervated pyloric sphincter could be a possible alternative in the surgical management of neurogenic or exstrophic bladder.

[Total colonic form of Hirschsprung disease. Treatment and long-term follow-up in 16 cases]. / Forme colique totale de la maladie de Hirschsprung. Traitement et suivi á long terme á propos de seize cas.

From 1971 to 1994, 16 cases of total colon Hirschsprung's disease were treated at the University Hospital in Rennes. Diagnosis have been at 2 days to 3 months. Two children had a family history of Hirschsprung disease among which one associated megacolon and multiple endocrine neoplasia. This family had a mutation of the RET proto oncogene. Six children died before complete surgical cure, among whom 4 before total parenteral nutrition. Six were treated according to Lester Martin, 3 according to Duhamel, and 1 to Swenson. Diarrhea and occlusions happened during the first postoperative years. None had any enterocolitis. Eight of 9 followed children are continent. Technique had no influence on long term outcome. Early neonatal occlusion management seems to decrease enterocolitis's incidence. We abandoned Lester's technique and kept Duhamel's technique. The problems encountered during ileostomy period do not encourage us to forward the age of definitive surgery procedure.

Urinary fungal bezoars in children--report of two cases.

We report two cases of urinary obstruction by fungal bezoars in full-term neonates who presented a uropathy detected antenatally. Early percutaneous urinary diversion was performed to relieve renal impairment secondary to a primary megaureter in the first case and to bilateral pelvi-ureteral obstruction in the second. Acute fungal obstruction occurred first on the side of the primary megaureter and then on the healthy side in the first patient. Symptoms of infection and impaired renal function led to a diagnosis of fungal bezoar. In the second patient the development of the bezoar was more insidious and occurred after surgical correction of the obstructive pelvi-ureteral junction on the left side. Candiduria was the first sign in both cases. Ultrasonography is the best method to visualize fungal masses within the collecting system. In most cases, percutaneous nephrostomy allows relief of the obstruction, sampling of urine for culture and irrigation with amphotericin B. However, additional surgical intervention may be necessary. Systemic antifungal treatment using mainly 5-flucytosine is also given.

[Digestive stenoses following ulcero-necrotizing enterocolitis]. / Les sténoses digestives post-entérocolite ulcéro-nécrosante.

Over a 10-year period, the authors followed the course of 112 neonates treated for primary acute necrotizing enterocolitis. Ten cases of gastrointestinal stricture were diagnosed, regardless of initial treatment, after an interval of 20 days to 6 months. The site of the stricture was colonic in 6 patients, ileal in three other patients and ileal and colonic in the remaining patient. The stricture was treated surgically in 9 patients (resection-anastomosis in 6 cases, resection with gastrointestinal diversion in 2 cases and isolated gastrointestinal diversion in 1 case). A follow-up barium enema in 1 patient showed spontaneous resolution of the stricture. With a mean follow-up of 5 years, the over all result was considered to be good in 7 cases and poor in one case. Two deaths were observed, one on the 10th day and the other at the 3rd postoperative day. These complications appeared to be related to the delayed diagnosis and treatment of the stricture.

Remarks about the prognosis in case of antenatal diagnosis of gastroschisis.

We report our experience of 15 cases of gastroschisis which occurred between 1981 and 1993. All but one were diagnosed antenatally by ultrasound between 16 and 32 weeks of pregnancy. We made a termination of the pregnancy in 3 cases, for multiple malformations in 2 cases and one case of very early premature rupture of the membranes (PROM). When checked (11 cases), the karyotype was normal. We made a cesarean section in 11 cases: the indication was a complication for 6 (fetal distress, PROM, polyhydramnios, large dilatation of the gut). We noted growth retardation in 7 newborns and prematurity in 5/12 (mean gestational age of 36.8 weeks). The preoperative study of the gut noted 5 cases with intestinal damage and one case of complete necrosis of the gut. The global prognosis is not as good as usual, with a perinatal mortality of 41.6% (5/12). We discuss this latter point and examine the literature.

Renal cystic tumours in children--a diagnostic challenge.

A series of 8 cystic renal tumours is reported in seven-months to four-years-old children. The final diagnosis was cystic nephroma (multilocular cyst) in 4, cystic, partially differentiated nephroblastoma in 3 and partially cystic nephroblastoma in one. Pre-operative distinction between those three types is difficult and inadequate therapeutic approach may result from a wrong diagnosis. Progress in imaging techniques allows a better analysis of cysts and septa. If no solid part can be detected in the tumour, total nephrectomy is sufficient to obtain a favourable outcome.

[Cloacal exstrophy: what therapeutic approach for what result?]. / L'exstrophie du cloaque: quelle attitude thérapeutique pour quel résultat?

The authors report 11 cases of cloacal exstrophy and analyse the anatomical features and the results of surgery. The outcome of three patients aged 20, 18 and 9 years is related. Advances in prenatal assessment of malformations is likely an explanation for the lack of new cases in the recent years. However the surgical procedure now available provides a better quality of life to patients with such a disabling malformation.

[Renal agenesis, bicornuate uterus and cyst of the Gartner's duct]. / Agénésie rénale, utérus bicorne et kyste du canal de Gartner.

A cyst of Gartner's duct with a bicornuate, unicervical uterus and ipsilateral renal agenesis were seen in a 13-month-old girl. Left renal agenesis had been suggested by antenatal ultrasound findings. After birth, there were no symptoms but ultrasonography demonstrated a multicystic mass in the pelvis. Diagnosis was confirmed by histological study of the mass which had been completely removed during surgery. This infrequent genitourinary malformation is due to a developmental abnormality of the wolffian duct early in fetal life. This case is the eighth report in the literature. Course can be complicated by abscess formation and malignant transformation but most patients remain symptom-free. Treatment is discussed.

[Anterior rectal duplication. Value of ultrasonic diagnosis]. / Duplication rectale antérieure. Intérêt de l'échographie.

A case of neonatal anterior rectal duplication is reported. Abdominal ultrasonography revealed a prerectal cystic mass. The different types of rectal duplications and the main differential diagnosis are described. Mechanical obstruction and neoplastic risk make early surgery necessary.

[Duplication of the urethra in a young girl. Apropos of a case]. / Duplication urétrale chez la fille. A propos d'un cas.

Duplication of the female urethra is quite uncommon. A case of complete urethral duplication is reported in a 2.5 year old girl seen for pyuria. Physical examination showed and epispadiac meatus with dribbling urine just above a bifid clitoris associated with an orthotopic meatus. The two urethras were patent and situated on the same sagittal plane as seen on the cystography. The treatment consisted of excising the whole epispadic urethra using a combined perineal and suprapubic approach enlarged by symphyseal section for a better repair of the bladder outlet. Postoperatively, the patient was content and free of symptom. The main features of urethral duplication in girl are reviewed.

[Torticollis in children: do not forget the Sandifer syndrome]. / Torticolis de l'enfant: ne pas oublier le syndrome de Sandifer.

We report a case of Sandifer syndrome with chronic torticollis and gastroesophageal reflux (GER). The infant exhibited regurgitations and vomiting from birth. Torticollis with a permanent tilt of the head towards the right developed at age six months. At 16 months, persistence of the vomiting and abnormal attitude of the head and neck led to a CT scan that outruled a brain tumor. Esophageal pH recordings disclosed severe gastroesophageal reflux (pH less than 4 for 46% of the time over 24 hours) and endoscopy showed ulcerated peptic esophagitis. Surgical treatment of the GER ensured both resolution of the reflux and disappearance of the torticollis, establishing the causal relationship between the former and latter manifestations.

[Diffuse esophageal leiomyomatosis. Apropos of 3 cases]. / La léiomyomatose oesophagienne diffuse. A propos de 3 observations.

Three cases of diffuse esophageal leiomyomatosis are discussed. Two of these are familial one, the mother being affected. These familial cases can occur in association with Alport's syndrome. The occurrence of a case of esophageal leiomyomatosis imply a familial survey and the search of a renal or ocular disease. The surgical treatment of this affection in sub total oesophagectomy with esophageal substitution (with the colon especially). The long term prognosis is unknown, with the risk of renal failure if Alport's syndrome is associated or of other leiomyomatous localisation.

[Millard's flap for sequelae of high anorectal malformations. Apropos of 6 cases]. / Plastie de Millard pour séquelles de malformation ano-rectale haute. A propos de six cas.

Mucosal prolapse is a frequent sequela after surgical treatment of the high type of anorectal malformation whatever was the technic used for the pull-through operation. The authors report six cases treated with the spiral flap described by Millard. The initial procedures had been a Romualdi-Rehbein pull through in three cases with secondary revision of anoplasty and a Stephan's operation in the three other cases. Before Millard's plasty, all the patients were continent and two of them had an associated stricture. The anatomical result was good in all the cases with reconstruction of a cutaneous anal funnel and no stricture. Improvement of continence was noted in two cases, uncertain in two. Incontinence remained unchanged in two other cases. The advantages of the Millard's plasty are analysed. The prevention of mucosal prolapse is possible in the initial pull through operation using the same plasty or whatever plasty which provides a cutaneous anal canal.

[Anterior hypospadias and chordee: a one-stage cure using a tubulated and reversed flap]. / Hypospadias antérieur et coudure: cure en un temps par tubulisation inversée.

A one stage urethroplasty using a tubulated and reversed flap was carried out by the authors in 74 cases of hypospadias with chordee between 1978 and 1988. The procedure is derived from the technic of Mustarde and the flip flap of Devine and Horton. The experience led to limit its indication to the patients who have a sufficient length of ventral skin of good quality between the hypospadic meatus and the peno scrotal junction to build the urethra up to the apex of the glans after correction of the chordee. This technic is simple and reliable: complications have been encountered in only two of the last forty cases (one fistula and one urethral stenosis) since the design of the flap was simplified.

[A duplication of the alimentary tract with unusual localization in the submaxillary region]. / Une duplication digestive de localisation inhabituelle dans la loge sous-maxillaire.

Duplication cysts developed from the intra-oral digestive tract are exceedingly rare lesions of which only 15 cases have previously been reported. We report an additional case in a neonate with a large cystic lesion in the right submandibular area. Clinico-pathologic findings are described and the literature is reviewed. Criteria for the diagnosis of digestive tract duplication are recalled. Differential diagnosis and etiopathogenic theories are discussed.

[Accidental ingestion of caustics in children. Apropos of 100 cases]. / Ingestion accidentelle de caustiques chez l'enfant. A propos de 100 observations.

One hundred children (67 boys, 33 girls) underwent early fiberoptic endoscopy (without general anesthesia in 96 cases) for caustic ingestion between January 1985 and June 1988. The intended use of the product was household (83) industrial (4) farm (6) or medicinal (7). Caustic burns were classified as grade 1 (mucosal hyperaemia), grade 2 (ulceration) and grade 3 (necrosis). Endoscopy was negative in 52, grade 1 in 41 who had evidence of esophagitis and/or gastritis, grade 2 and/or grade 3 in 7 cases, 4 of whom had ingested farm products. Eighty-seven children were discharged after examination, 6 were hospitalized for 24 hours. Outcome was favorable for the 5 cases with grade 2 lesions after total parenteral nutrition for an average period of 79 days. The 2 cases with grade 3 injuries went on to develop an esophageal stricture requiring endoscopic dilation and an antral stricture which was treated by antrectomy. Authors emphasize the severity of lesions secondary to the ingestion of dairy pipeline cleaners, the advantages of fiberoptic endoscopy and the role of parenteral nutrition.