Transfer of surgical competences in the treatment of intersex disorders in Togo.

BACKGROUND: To evaluate the impact of scientific seminar on the sexual ambiguity on patients and paediatric surgeons in French-speaking African countries. MATERIALS AND METHODS: This was a report of the proceeding of a teaching seminar on intersex management, which was held from December 4 th to 8 th , 2006, in the Paediatric Surgery Department of Tokoin Teaching Hospital and the Surgery Department of "Saint Jean de Dieu" Hospital of Afagnan, Togo. RESULTS: There were 107 participants [five professors of paediatric surgery, 62 African paediatric surgeons (including 15 from African French-speaking countries), and 40 general surgeons]. The workshop involved a two-day theoretical teaching session (aimed at understanding, recognising, and treating the sexual ambiguities), and practical session; during these sessions different intersexes (one case of mixed gonadal dysgenesis, two of female pseudohermaphroditism, and two of male pseudohermaphroditism), were operated free of charge. Participants expressed satisfaction and confidence with regard to the management of intersex after the seminar. CONCLUSION: This scientific forum allowed possible exchange of competence among the paediatric surgeons with regard to efficient treatment of sexual ambiguities.

Transfer of surgical competences in the treatment of intersex disorders in Togo

Background: To evaluate the impact of scientific seminar on the sexual ambiguity on patients and paediatric surgeons in French-speaking African countries. Materials and Methods: This was a report of the proceeding of a teaching seminar on intersex management; which was held from December 4 th to 8 th ; 2006; in the Paediatric Surgery Department of Tokoin Teaching Hospital and the Surgery Department of ""Saint Jean de Dieu"" Hospital of Afagnan; Togo. Results: There were 107 participants [five professors of paediatric surgery; 62 African paediatric surgeons (including 15 from African French- speaking countries); and 40 general surgeons]. The workshop involved a two-day theoretical teaching session (aimed at understanding; recognising; and treating the sexual ambiguities); and practical session; during these sessions different intersexes (one case of mixed gonadal dysgenesis; two of female pseudohermaphroditism; and two of male pseudohermaphroditism); were operated free of charge. Participants expressed satisfaction and confidence with regard to the management of intersex after the seminar. Conclusion: This scientific forum allowed possible exchange of competence among the paediatric surgeons with regard to efficient treatment of sexual ambiguities"

[Preoperative evaluation of choledochal cyst with MR cholangiopancreatography]. / Bilan préthérapeutique d'une dilatation congénitale de la voie biliaire principale par cholangio-pancréatographie-IRM.

UNLABELLED: The choledochal cyst is a rare congenital disorder usually diagnosed in childhood. It requires a complete surgical resection to prevent complications, particularly the risk of malignant changes. At present, the preoperative examination requires a direct opacification of the biliary tree, but this is an invasive technique with a high risk of infection, especially in pediatric patients. CASE REPORT: A choledochal cyst was diagnosed in a five-year-old girl with recurrent abdominal pain. Diagnosis was made by ultrasound and preoperative evaluation by magnetic resonance-cholangiopancreatography using single-shot fast-spin echo sequences. A complete correlation was observed between surgical, preoperative cholangiography and MRCP data. CONCLUSION: Recent improvement in MRCP techniques provide a complete anatomic analysis of choledochal cysts, enabling one to diagnose an anomalous junction of the pancreaticobiliary duct, even the presence of stones within the biliary tree. This short and noninvasive examination should in the future replace direct opacification of the biliary tree for the preoperative assessment of choledochal cysts.

A curable cause of chronic idiopathic intestinal pseudo-obstruction in children: idiopathic myositis of the small intestine.

We report an intestinal pseudo-obstruction syndrome occurring in a 6-month-old infant girl suffering from acquired major and persistent abdominal distension, which 2 months later required ileostomy. Histologic examination of samples of the small intestine showed considerable inflammatory reaction in the muscular layers of the intestinal wall. Steroid therapy, begun as soon as the histologic results were known, resulted in recovery. In a similar case, reported previously, steroid therapy used after the lesions had already caused fibrosis and atrophy of the intestinal wall, proved ineffective. This particular form of pseudo-obstruction is classified as "idiopathic myositis of the small intestine." It is important to identify the condition because an early course of steroid therapy, before the appearance of fibrotic lesions, could improve the prognosis.

[Urinary tract abnormalities with prenatal diagnosis: neonatal management and outcome of 100 children born 1988-1990 at the Angers CHU (University Hospital)]. / Uropathies malformatives de diagnostic anténatal: prise en charge néonatale et devenir de 100 enfants nés entre 1988 et 1990 au CHU d'Angers.

UNLABELLED: Malformative uropathies diagnosed in utero are increasing in number. This work describes the decision strategy adopted in Angers concerning the neonatal handling of those abnormalities. PATIENTS AND METHODS: One hundred children born between 1988 and 1990, with prenatally suspected fetal uropathy, were included in the study and followed for a period of 3 years. In every case, an ultrasound scan was performed at birth. In cases with persistent abnormality, a voiding cystourethrography was done in the first week of life. An intravenous urograph and/or a nuclear renography were performed during the second month of life. RESULTS: Twenty-nine children were normal. Seventy-one were affected by 126 isolated or related uropathies; the most frequent ones were the ureteropelvic junction obstruction syndrome (48), ureterovesical junction obstruction (18) and multicystic kidneys (13). A vesicoureteral reflux was associated in 22% of cases. The diagnosis was perfectly correlated with the prenatal diagnosis in 50% of cases. Fourteen of the normal children had a later ultrasound scan control, between 2 and 9 months; later on, three of them showed a moderate ureteropelvic junction obstruction syndrome. Amongst the 48 ureteropelvic junction obstruction syndromes, 22% have been operated on. The others obstructive uropathies remained stable or spontaneously improved. DISCUSSION: These results require us to discuss as matter of priority the large number of spontaneously regressive prenatal hydronephrosis and the necessity to establish a consensus for the pre and postnatal pathological thresholds of the anteroposterior pelvic diameter, the interest in carrying out a voiding cystography after the birth when dilatation is confirmed, the interest of nuclear renography for the diagnosis and follow-up of obstructive uropathies and the absence of urgency for surgery. CONCLUSION: We propose a decision tree specifying the action to take when facing the diagnosis of a prenatal hydronephrosis. It could be a part of the reflection for the medical teams handling these uropathies is the aim of a consensual attitude which is now essential.

[Congenital dilatation of the prostatic utricle. Embryologic review, diagnosis and treatment. Apropos of 2 pseudo-tumoral cases, without hypospadias]. / La dilatation congénitale de l'utricule prostatique. Rappel embryologique, diagnostic et traitement. A propos de deux observations pseudo-tumorales, sans hypospadias.

The authors report two pediatric cases of congenitally dilated prostatic utricle. First a newborn with prenatal ultrasound diagnosis of abdomino-pelvic cystic mass and secondly a 4-year-old boy with urinary incontinence and lower abdominal mass. Unilateral renal hypoplasia was noted in both cases, but no hypospadias. The study of embryogenesis and pathogenesis shows that the anomaly happens before the 8th week of pregnancy. When hypospadias is associated, the anomaly develops later and has a hormonal cause. Prostatic utricle is not only an embryologic Müllerian remnant, but comes from the mesodermic and endodermic tissues. The first clinical signs are often urinary signs or an abdominopelvic mass. The prostatic implantation and the median localization of this cystic mass are both revealed by urethrography and ultrasonography. Computed tomography and especially Magnetic Resonance Imaging may be performed. MRI provides a detailed demonstration of the anatomy and a large field of view in all three planes. It helps to plan surgical therapy. The treatment of prostatic utricle may be percutaneous, endoscopic or surgical. The indications are based on the symptoms, but the risk of malignancy must be kept in mind. In our cases, the marked dilatation of the prostatic utricle led to surgical treatment and retrovesical excision was successful.

[Nephrogenic nephroma]. / Le néphrome néphronogène.

Nephrogenic nephroma was individualized by A. Pagès in 1980 as a rare renal neoplasia that mainly occurs in adolescents and adults and presents a benign outcome. The tumor is composed of very small tubular, papillary, and glomeruloid structures. Immunohistochemistry and electron microscopy show that the tumor is a proliferation of primitive epithelial cells that mimic the embryonal nephron. This neoplastic entity has often been confused with a nephroblastoma, although it is a benign tumor that is cured by simple resection. Further studies are needed to define its exact incidence and its histogenesis.

[2,8-dihydroxyadenine lithiasis. 2 new pediatric cases of an unknown metabolic deficit. The use of extracorporal lithotripsy]. / La lithiase 2,8-dihydroxyadéninique. 2 nouvelles observations pédiatriques d'un déficit métabolique méconnu. Apport de la lithotrypsie extra-corporelle.

Inherited adenine phosphoribosyltransferase (APRT) has a recessive transmission. When it is very important, adenine can't be restored into nucleic acids pool and will changed into 2,8-dihydroxyadenine (2,8-DHA) by xanthine oxidase. To date in all countries but Japan, 2,8-DHA urolithiasis is observed only into homozygotic subjects with complete APRT deficiency Commonly, its onset is observed in childhood often dramatically. The authors report two new pediatric cases into new french families. First a 8 years old boy with spontaneous elimination of two lithiasis after right lumbar pain. Secondly an infant (nineteen months) who has presented an acute renal failure with anuria. Bilateral lithiasis included into pyelourectal junctions have been pulled out by bilateral surgical pyelotomy. In each case, lithiasis were radiolucent and diagnosis made by ultrasonography. The uric acid metabolism was normal and it is the infra red spectrophotometric study of stones that had recognised the 2,8-DHA component. In the second case, bilateral residual lithiasis have been broken by piezoelectric extra-corporeal lithotripsy with good tolerance and favorable result. The two children received preventive treatment. After 36 and 19 months they have no recurrence. In the literature, the frequency of 2,8-DHA lithiasis is very more low than the theoretical of homozygotics in population (1/100,000). The common confusion with uric lithiasis is one possible explanation. So spectrophotometric study of radiolucent stones was meant to be realised when uric metabolism is not disturbed.(ABSTRACT TRUNCATED AT 250 WORDS)

[Munchausen's syndrome by proxy and chronic intestinal pseudo-obstruction]. / Syndrome de Munchausen par procuration et pseudo-obstruction intestinale chronique.

A case of intestinal pseudo-obstruction in a 4 1/2 year-old boy is reported. All etiologic investigations remained negative. Management successively required continuous enteral feeding, ileostomy then total parental nutrition. The proof of a chronic barbiturate intoxication, induced by the mother, was made only after 2 1/2 years of follow-up, when the patient was in a critical condition. Separation of the child from his family led to complete disappearance of symptoms within a few days.

[Isolated congenital stenoses of the extrahepatic bile ducts. Review of the literature apropos of a new case]. / Les sténoses congénitales isolées des voies biliaires extrahépatiques. Revue de la littérature à propos d'une nouvelle observation.

The authors describe a case of congenital stenosis of the common hepatic duct, in ten-year-old girl, with jaundice for two months. Ultrasonography showed an intra and extrahepatic duct dilatation. The operative cholangiogram demonstrated severe stenosis at the end of the common hepatic duct. A Roux-en-Y anastomosis of the jejunum to this duct relieved the obstruction. Histopathological examination of the removed stricture showed non inflammatory fibrous structures. The congenital biliary strictures are exceptional. The review of the literature allowed to collect nine other cases, which symptoms began before fifteen years old. Only four were treated in childhood. The arguments for a malformative origin of the stricture are the patient's age, the seat of the stenosis and its histological structure. Several physiopathological theories allowed to explain the usually delayed beginning of first symptoms: distortion of the stenosis by gradual distension, biliary sludge, lithiasis and infection. The preoperative diagnosis of the stricture may now be made by transhepatic or endoscopic cholangiography, but before to assert its congenital feature, it must eliminate many intrinsic or extrinsic causes: post operative, traumatic, inflammatory and tumoral. The Roux-en-Y hepaticojejunostomy is the usual treatment.

[Multiple congenital esophago-tracheal fistulas (excluding atresia of the esophagus). Review of the literature, apropos of a new case]. / Les fistules oeso-trachéales congénitales multiples (atrésie de l'oesophage exclue). Revue de la littérature, à propos d'une nouvelle observation.

Congenital tracheoesophageal fistulas (CTOF) without esophageal atresia are uncommon and exceptional when multiple. Authors report a new personal case treated in two times. Others eight cases in literature are studied. Only one pre-operative diagnosis was made. Two old no-operated cases were discovered at autopsy. For five, no clinical modification was noted after the first CTOF surgical care. In them two no-reoperated died and the second fistula was seen after. For the others, the same radiological or endoscopic exams that have not marked the second fistula were positive after surgery. No explanation was done. For a better result, systematic complete investigation of trachea and esophagus are necessary even if first fistula is diagnosed. Luckily per-operative discovery of second fistula (one case) do not allowed initial extensive surgery. To support a second congenital tracheoesophageal fistula after a surgical time, many criterias are necessary: 1. a undoubted fistula recognised during the first intervention; 2. recurrent symptoms after surgery with no free interval; 3. identification by radiology or endoscopy of a new tracheoesophageal shunt in other place that the first; 4. its surgical discovery in no previous dissected area. In all cases where fistulas are described, the aspect is not the same: the upper fistula is a "H" type and the lower is shorter and larger ("X" type). To note a case of three simultaneaous fistulas in the same patient.

[2,8-dihydroxyadenine lithiasis. 2 new pediatric cases of this misdiagnosed metabolic abnormality. The value of extracorporeal lithotripsy]. / La lithiase 2,8-dihydroxyadéninique 2 nouvelles observation pédiatriques d'un déficit métabolique méconnu. Apport de la lithotrypsie extra-corporelle.

Inherited adenine phosphoribosyltransferase (APRT) has a recessive transmission. When it is very important, adenine can't be restored into nucleic acids pool and will changed into 2,8-dihydroxyadenine (2,8-DHA) by xanthine oxydase. To date in all countries but Japan, 2,8-DHA urolithiasis is observed only into homozygotic subjects with complete APRT deficiency. Commonly, its onset is observed in childhood often dramatically. The authors report two new pediatric cases in two new french families. First a 8 year old boy with spontaneous elimination of two lithiasis after right lumbar pain. Secondly an infant (nineteen months) who has presented an acute renal failure with anuria. Bilateral lithiasis incluted into pyeloureteral junctions have been pulled out by bilateral surgical pyelotomy. In each case, lithiasis were radioluscent and diagnosis made by ultrasonography. The uric acid metabolism was normal and it is the infra red spectrophotometric study of stones that had recogniseed the 2,8-DHA component. In the second case, bilateral residual lithiasis have been broken by piezoelectric extracorporeal lithotrypsy with good tolerance and favorable result. The two children received permanent preventive treatment. After 36 and 19 months they have no recurrence. In the literature, the frequency of 2,8-DHA lithiasis is very more low than the theorical incidence of homozygotics in population (1/100,000). The common confusion with uric lithiasis is one possible explanation. So spectorophotometric study of radioluscent stones was meant to be realised when uric metabolism is not disturbed. Prevention associates alimentary diet without purins and permanent treatment by allopurinol (10 mg/kg/day in a child). Not used to date, piezo-electric extracorporeal lithotrypsy seems to take a place for treatment of initial, residual or recurrent 2,8-DHA lithiasis like for our young patient.(ABSTRACT TRUNCATED AT 250 WORDS)

[A severe form of juvenile polyposis coli]. / Forme grave de polypose juvénile colique.

The authors report a case of a life-threatening form of juvenile polyposis coli in a 7-year-old girl. The cure of this affection required a total coloproctectomy with ileoanal anastomosis.

[The accessory scrotum. Apropos of 2 cases]. / Le scrotum surnuméraire. A propos de deux observations.

The authors report two new cases of accessory scrotum. It is an exceptional pathology. The review of the literature allowed to collect thirteen other cases. The aspect of accessory scrotum was a soft mass with scrotal skin and no content. All were located on perineal area; on the midline for ten, lateral to the raphe for four (two on the left and two on the right). Both testes were palpable in the normal scrotum. There was no polyorchidism. For all but five, the accessory scrotum was a single anomaly. Five children presented another genital problem with for four of them a second infrequent scrotal malformation (bifid scrotum or peno-scrotal transposition). Surgical treatment is easy. Histologic examination shows a true scrotal skin with muscular fibers in subcutaneous tissue, like tunica dartos. The "tumor" contains an homogeneous fat tissue. The origin of accessory scrotum is not clear. It does not seem to be a teratoma. Probably it is the result of abnormal migration with a division of labio-scrotal swellings. It is not the same as ectopic scrotum, in which localization is variable, testis is present in the abnormal scrotum or its area and in which there is only one opposite half-scrotum in its normal place.

[Mediastinal pseudocyst in hereditary pancreatitis]. / Faux kyste médiastinal au cours d'une pancréatitie héréditaire.

A 6 year-old child was hospitalized for thoracoabdominal pain. There was a clinical and radiological right pleural effusion. Ultrasonography showed a pseudocyst of the head of the pancreas. CAT scan and operative opacification showed a mediastinal extension of the pseudocyst, with no communication with the pleural effusion. This pseudocyst illustrates an unusual variation of a known complication of this disease. Recovery was obtained via fistulization into the jejunum.

[The calcium bile syndrome in children]. / Le syndrome de la bile calcique chez l'enfant.

The "limy bile" is uncommon in adults and very exceptional in childhood. The authors report a new case of a 7 years old girl. The review of the literature since Churchman's description (1911) allowed to collect eleven other cases of children, less than fifteen old. The usual sexual distribution in biliary diseases with female predominance is not true for them: eight boys for four girls. The youngest was three years and six months old. All but one have presented mainly right upper abdominal pain and vomiting. The diagnosis was made by an abdominal plain film showing a spontaneous visualization of the gallbladder which contained a material made of carbonate of calcium. One was an operative detection. All presented a stone of neck of gallbladder or cystic duct. The origin of "limy bile" is unknown. Nine children have been operated (cholecystectomy) with good result. Spontaneous disappearance of "limy bile" was noted in three cases. It was marked by acute abdominal pain, inconstant jaundice or pancreatic reaction. No surgical care was brought with nor further problems or recurrence.