Priapism in a sickle cell prepuberal child.

Between 1985 and 1995, six sickle cell children listed SS were hospitalized in our department for priapism persisting after 24 hours of medical treatment. Two cases regressed after 6 hours of treatment follow-up. Four children were treated by a corpus cavernosum puncture, through the gland, leading to priapism detumescence in 3 cases. In one of our cases, the persistence of priapism induced us to perform a spongiocavernous anastomosis at the 72nd hour. One of the children developed a cerebrovascular accident 10 days after the beginning of the priapism. The other children have been re-examined. They have not presented any reccurence and have normal erection after puberty. Starting from a literature review, we explain the principles we adopted. The physiopathology of priapism in sickle cell patients remains unclear, for both high and low flows do exist. In addition to the risks related to blood products, the treatment by transfusional exchange involves neurological risks and must not delay any surgery. Analgesic treatment is often required. Corpus cavernosum puncture is efficient, with no fibrosis risk. In case of failure, a cavernospongious shunt, with a biopsy needle, can be performed at the same time. Surgical anastomoses are then proposed in case of priapism persistence. General evolution is satisfactory. But the sickle cell patient's priapism needs a long-term follow-up in order to recognise any minor recurrences. The latter could be the principal cause of fibrosis and impotency.

Congenital fibrous hamartoma of the hand: a case report.

A patient with a rare localization of a congenital fibrous hamartoma of the hand is described, and the differential diagnosis is discussed. A review of the literature shows the rare frequency of localization of this lesion on the limb extremities.

[Pubic osteitis caused by Salmonella indiana. Apropos of a case]. / Ostéomyélite pubienne à Salmonella Indiana. A propos d'un cas.

INTRODUCTION: The authors report the case of a pubic osteomyelitis caused by a salmonella indiana bacterium in a 12 year old child. CLINICAL CASE: A post traumatic left side pubic pain in a 12 year old child was associated to feverish and inflammatory syndrome, without any predisposing factor. Salmonella Indiana bacterium was found in fecal samples. Radiographs showed an asymetry of the iliopubic branches and an irregularity of the upper left border of the pubic branch. Bone scan showed a public hyperfixation and CT revealed a left-sided pubic osteolysis. The bacteriological examination of the osseous biopsy confirmed the presence of Salmonella Indiana bacterium. Recovery was obtained within 5 months with intravenous antibiotherapy. DISCUSSION: Since 1987, an increase of minor salmonella infections and particularly of Salmonella Indiana bacterium can be observed in metropolitan France (250 isolated in 1991). This increase is not experienced in Martinique. The contamination mode is yet unknown and the osseous localization is secondary to bacteriema. The patient had no predisposition excepted a chronic digestive carrier. This localization is unfrequent in children. The diagnostic has been achieved by bacteriological samples and osseous biopsy. The treatment based was on third generation cephalosporins and on quinolones.

[Accessory thymus in cervical ectopy: apropos of a case]. / Thymus accessoire en ectopie cervicale: à propos d'un cas.

Cervical tumors of thymic origin are considered to be uncommon lesions in the differential diagnosis of neck masses. They can be either cystic or solid. The authors present one case of solid cervical thymic tumor in a 2 month-old baby. The embryogenesis of the thymus explains the cervical location of these tumors. Theories of physiopathology are presented. The clinical presentation is variable and their nature is often recognized only upon surgery and preoperative pathologic examination. Some cases of thymoma and respiratory complications resulting from ectopic thymus have been described in the literature, so that total excision of the mass must be performed. This lesion may be more common than suggested in the literature.

[Sacrococcygeal teratomas in antenatal diagnosis]. / Tératomes sacro-coccygiens de diagnostic anténatal.

The authors report eight cases of antenatal diagnosis of sacro-coccygeal teratoma (SCT) in five girls and three boys in whom the diagnosis was made between the 19th and 34th week of amenorrhea (mean = 27 weeks). The ultrasound pictures taken antenatally of the SCT assist in the discovery of a mass that is usually heterogenous, attached to the distal end of the sacrum, and the discovery is usually made fortuitously or because the height of the uterus is too great. A different series of antenatal diagnoses for SCT have made it possible to work out certain criteria of seriousness to be able to predict intra-uterine death: the presence of anasarca or of hydramnios, the discovery of the lesion before the 30th week of amenorrhoea, the relative weight of the teratoma as against the weight of the fetus being above 50%. We think from our experience that it is important to add the scale of the antenatal growth of the teratoma. A rapid growth of the SCT will lead to a tumour mass which is great as compared to the size of the fetus. Similarly in certain cases the vascular bed will increase in size, and intratumour haemorrhages can occur and give rise to fetal heart failure and also to fetal anaemia, hypoproteinaemia and the appearance of anasarca or of hydramnios. The child dies in utero or immediately after birth because of prematurity from the haemorrhagic state or from cardiac insufficiency. Furthermore accelerated growth of the tumour is nearly always in the immature tumour cells and that means that the child, if it is born alive, should be followed up for a long time because there is a risk of it becoming locally malignant. In practice the monitoring of SCT and the antenatal discovery of the condition should be carried out very seriously in order, in some cases, if it is viable to produce a living child in conditions where the rapid growth of tumour would make it likely that the child would die in utero.

[Idiopathic perforation of the small intestine in newborn infants. Apropos of 2 cases]. / Perforation idiopathique de l'intestin grêle chez le nouveau-né. A propos de deux cas.

Two cases of spontaneous ileal perforation in neonates have been reported. The diagnosis is often difficult due to the lack of clinical or radiological signs. Other possible etiologies are excluded at laparotomy. Hypotheses concerning the etiology of spontaneous intestinal perforations have been discussed.

[Extrahepatic biliary tract atresia and a common biliopancreatic canal. Review of the literature apropos of a case]. / Atrésie des voies biliaires extra-hépatiques et canal biliopancréatique commun. Revue de la littérature à propos d'une observation.

The authors report the case of an 3 months old caucasian boy which had an extrahepatic biliary atresia associate with a congenital biliary cyst and an anomalous pancreaticobiliary junction. The patency between gallbladder and duodenum permits the operative opacification of this long common pancreaticobiliary channel. The congenital biliary cyst was located on the upper part of the common bile duct and was obstructed by biliary concretions. Unfortunately, no patency between intrahepatic bile ducts and this cyst was found. Because this long common pancreaticobiliary channel may cause biliary dilatation or biliary cancer, we performed an hepatic portoenterostomy rather an hepatic portocholecystostomy. A few weeks later, the bile flow was restored and the jaundice decreased. With a follow up of 18 months, this boy is currently anicteric, despite the hepatic fibrosis found on the operative biopsy. The association extrahepatic biliary atresia-long common pancreaticobiliary channel is uncommon and only 20 cases were reported. In our case, the pancreatic juice reflux into the biliary tract might have caused the dilatation of the common bile duct and the biliary atresia by chronic obstructive cholangiopathy.