[Feminizing genitoplasty for prepubertal children and teenagers female]. / Génitoplastie féminisante dans les anomalies du développement sexuel de l'enfant pré-pubère et de l'adolescente au Cameroun. À propos de 9 cas.

OBJECTIVE: The aim of this study was to report our experience after 10 years of practice of feminizing genitoplasty in prepubertal and adolescent patients with disorders of sex development (DSD) assigned females as females in a developing country. METHODOLOGY: This was a cross-sectional, descriptive and retrospective study over a period of 9 years. All pre-pubertal (8-12 years) and adolescent patients female sex assigned with DSD who had willfully consented to the surgery with their guardians and underwent feminizing genital surgery were enrolled in the study. Data collection included: age at presentation, precise diagnosis, surgical procedures, complications, cosmetic result and duration of follow-up. Each patient had a precise diagnosis and the surgery was planned after discussion with the multidisciplinary team. Cosmetic results were assessed based on: appearance of the clitoris and separation of the vaginal and urethral openings. RESULTS: Nine patients raised as females with a median age of 8 years (IR: 10.75) were recorded. Surgery was performed at a median age of 11 years (IR: 9.5). In this series, 6 had a 46, XY karyotype with varying diagnoses: partial androgen insensitivity syndrome (n=2); 5-alphareductase insufficiency (n=2); 17-ketoreductase insufficiency (n=2); gonadal dysgenesis with a mutation in the NR5A1 gene (n=2), 2 had ovostesticular DSD, (karyotypes 46, XX), and 1 had mixed gonadal dysgenesis (karyotype 45, X/46, XY). Partial or total gonad(s) removal in accordance with assigned gender was the most common associated procedure. It was bilateral in 7 cases and unilateral in 2 cases. Follow-up ranged from 3 months to 4.5 years (median: 26 months, IR:18.25). One patient had acute urinary retention in the early follow-up. No other complication such as incision bleeding was recorded. The cosmetic appearance of the external genitalia was satisfactory in all patients. CONCLUSION: Feminizing genital surgery in Cameroon remains a major challenge and should seldom be realized without a precise diagnosis. Late age at presentation is peculiar to our setting; however, it gives room for the patients' participation and input to decisions that will have a life-long personal impact on their lives in terms of psychosocial development and fertility. LEVEL OF EVIDENCE: 3.

Liver function tests in children with blunt abdominal traumas.

BACKGROUND: Previous studies have suggested that there are cut-off values for liver function tests (LFTs) beneath which significant liver injury can be excluded after blunt abdominal trauma in children. Our objective is to test this hypothesis in our patient population. METHODS: The LFTs of all consecutive patients admitted in Geneva from January 1, 2001 to December 31, 2004 following blunt abdominal trauma were analysed and compared to radiological (ultrasound and/or computed tomography scan) findings and final outcome. RESULTS: Of 115 patients identified, sixteen had radiological evidence of liver injury. These patients had significantly (p < 0.01) increased aspartate aminotransferase (AST) and alanine aminotransferase (ALT) values (474 +/- 369 IU/l and 442 +/- 383 IU/l, respectively) compared to patients without liver injury (AST 88 +/- 161 IU/l and ALT 68 +/- 137 IU/l). Among the sixteen patients with liver injury, ten (63 %) had AST < 450 IU/l and seven (44 %) had ALT < 250 IU/l. Two patients had radiological evidence of OIS grade 3 liver injury with AST as low as 95 and 92, and ALT of 80 and 86, below all cut-off values recommended in the literature. CONCLUSION: In our experience, low LFT values at admission could not rule out significant liver injury. The diagnosis of such lesions still relies on clinical and radiological findings, as do other intra-abdominal organ injuries.

A case report of Cowper's syringocele in an 18-months old infant at the Yaoundé Gynaeco-Obstetric and Pediatric Hospital.

Syringocele or dilatation of the duct of the bulbo-urethral (Cowper's) gland is usually of congenital origin but can be acquired. It is a very rare deformity, <10 cases have been reported in literature. The main objective is to describe an additional case of syringocele of Cowper's glands and review the literature. An 18-month-old infant presented with a history of acute urinary retention 3 days after birth and a cystostomy was done. Voiding cystourethrogram was normal and cystourethroscopy showed a syringocele. Endoscopic incision was performed in our patient with satisfactory results. No complications were noted. Syringocele or cystic dilatation of Cowper's gland duct usually has a congenital aetiology. Diagnosis is confirmed by endoscopy. Treatment is by marsupialisation in the urethra by endoscopy. Syringocele is a rare pathology usually congenital. It should be suspected in all case of lower urinary tract obstruction in children.

Our experience of proximal hypospadias repair using the Cloutier-Bracka technique at the Gynaeco-Obstetric and Paediatric Hospital, Yaounde-Cameroon.

BACKGROUND: In parts of Africa, routine circumcision is practised and sometimes even on children with hypospadias. The lack of preputial foreskin renders urethroplasty more difficult and often requires to use of a mucosal graft as described by Bracka. OBJECTIVE: The authors describe their experience of hypospadias repair using Bracka's technique. MATERIALS AND METHODS: Over a period of 5 years, 100 cases of proximal hypospadias were operated in our institution. All patients aged 0-18 years who had already been circumcised were included in this study. RESULTS: The outcome of the 12 cases operated according to Bracka's technique was analysed. The mean age was 11.5 years. The ectopic meatus was penoscrotal in three cases, scrotal in one case and perineal in eight cases. After reconstruction, the new meatus was sutured at the top of the glans in one case, at the prepuce in seven cases and at the penile midshaft in one case. The main complications noted were surgical site infection, wound dehiscence, residual chordee and urethrocutaneous fistula. No neourethral stenosis nor uretrocele was recorded. DISCUSSION: The buccal mucosal graft urethroplasty as described by Bracka is associated with a lower risk of meatal strictures compared to other free mucosal grafts. The buccal mucosa is easier to harvest and causes less scarring than bladder mucosa. CONCLUSION: Repair of severe hypospadias remains a challenge for paediatric surgeons. The functional and cosmetic outcomes depend on the choice of the donor site for the graft and objective assessment of successful reconstruction criteria during follow-up.

Comparison of totally implanted and external catheters in paediatric oncology patients.

From June 1982 until December 1989, 93 permanent central venous catheters [59 external catheters (ECs) and 34 implanted catheters (ICs)] were placed in 69 patients. The median age of these patients at placement was 5.6 years for ECs and 8.8 years for ICs (P less than 0.05). Follow-up evaluation was possible on 86 catheters (58 ECs and 28 ICs). The median time of insertion was 236 days and 316 days for ECs and ICs, respectively (P less than 0.05). The median number of open days was 58 for ECs and 66 for ICs (not significant). 17 catheters (6 ECs and 11 ICs) were transiently obstructed (P less than 0.005). 30 episodes of bacteraemia were documented in 20 patients. The incidence of catheter sepsis and bacteraemia of unknown source was one in 278 and 283 open days for ECs and ICs, respectively (not significant). In this retrospective study, ECs appeared to be as safe as ICs when infection was correlated with use of the catheter, but this finding should be confirmed in a randomised design.

Monosegmental liver transplantation from an adult to an infant.

A shortage of small pediatric organ donors has led to the development of reduced size liver transplantation in children. However, the discrepancy between donor and recipient weight can limit the use of this procedure despite transplantation of the left lobe only. Monosegmental liver transplantation using segment III only was recently described. We report here the case of an 11 month old, 6.9 kg boy who received another monosegmental graft (segment II) from a 78 kg donor on an urgent basis. Because of the lack of parenchymal landmarks between segments II and III, sterile methylene blue solution was injected into the portal vein of segment III: parenchyma of this segment colored immediately and was resected accordingly. Three and a half years later, the growth, development, and nutrition of this child were normal. This procedure seems to be helpful when the left lobe of the graft is obviously too large.

Use of Gianturco Z stents in the treatment of vascular compression of the tracheobronchial tree in childhood. A feasible solution when surgery fails.

Severe tracheomalacia secondary to extrinsic vascular compression following a switch operation for transposition is reported. Two attempts at surgical correction failed but successful treatment has been achieved by implantation of two endobronchial Gianturco Z stents. Nonabsorbable stenting in children should be used only in severe obstruction as a last resource, but this technique proved to be feasible in a child weighing 6.2 kg.

A silicone-coated nylon dressing reduces healing time in burned paediatric patients in comparison with standard sulfadiazine treatment: a prospective randomized trial.

Mepitel is a new grid like silicone coated nylon dressing containing no additional biological compounds. We describe a prospective randomized pilot study comparing Mepitel to the standard silver sulfadiazine cream (Flamazine) dressing for the topical treatment of paediatric burns. Seventy-six children presenting within 24 h of injury with a non previously treated burn were randomly assigned to Mepitel treatment (group M) or Flamazine treatment (group F). Age, sex, surface area of burn and causal agent were noted at admission. The depth of the burn, cumulative number of dressings, presence or absence of a complete epithelial cover, infection, bleeding and allergy were noted at each dressing change. There were 41 children in group M and 35 children in group F. Five children were subsequently withdrawn from each group because they required skin grafting. Analysis of the above mentioned criteria showed no statistical difference between the two groups except for the healing time (group M: 7.58+/-3.12, group F: 11.26+/-6.02, p < 0.01) and the number of dressings (group M: 3.64+/-1.5, group F: 5.13+/-2.9, p < 0.05). Mepitel has proved to be an easy-to-remove dressing, adhering only to intact skin. The faster healing time found in the Mepitel group may be related to a direct effect of silicone on epithelial growth or to a decrease in surface-cell damage compared to the silver sulfadiazine group. This attractive product will be further assessed on a larger scale trial to confirm our observations.

An unusual association of small bowel atresia and biliary atresia: a case report.

The embryological development of the hepatobiliary bud presupposes that biliary and duodenal malformations are often associated. On the other hand, the association of biliary atresia with more distal small bowel malformations, possibly due to a vascular accident, seems to be fortuitous. We herewith report such a case and, on reviewing the literature, have found only two previous similar observations.

Postoperative chylothorax in children: differences between vascular and traumatic origin.

Twenty-four children with postoperative chylothorax were encountered among 1,264 consecutive thoracic operations over a 7-year period and form the basis of this study. Chylothorax was caused by direct lesion to the thoracic duct or lymphatic vessels in 17 patients and was associated with superior vena cava (SVC) obstruction in seven. Of the latter, five had bilateral chylothorax. Chylothoraces secondary to venous hypertension and thrombosis have a longer interval between operation and diagnosis compared with direct trauma as well as a longer duration and larger volume of chylous drainage. Treatment was entirely nonoperative in 16 patients and operative in 8. Nonoperative treatment consisted of pleural needle aspiration or suction drainage in association with a medium chain triglyceride (MCT) diet (n = 11) or total parenteral nutrition (TPN) after failure of MCT (n = 5). Direct operation on the thoracic duct was performed in 5 patients, four had pleurodesis, and 2 had pleuroperitoneal shunts inserted. All patients were cured of their chylothorax and there were no deaths. Patients with major vein thrombosis were the most difficult to treat. On the basis of this experience, we suggest a step-by-step approach: (1) insertion of chest tube after 3 to 4 pleural punctures; (2) 1-week trial of MCT diet, with intravenous support to correct protein losses; (3) TPN if chylothorax increases or persists with large volumes; (4) Doppler echocardiography or phlebography to rule out obstruction of major thoracic veins; and (5) insertion of TPN line in inferior vena cava in case of such obstruction; and (6) direct surgical approach to the thoracic duct after 4 weeks of unsuccessful nonoperative treatment.(ABSTRACT TRUNCATED AT 250 WORDS)

Intestinal obstruction caused by an ectopic fallopian tube in a child: case report and literature review.

The authors present the case of a prepubertal 14-year-old girl who was admitted for an acute abdominal pain, fever, and vomiting. She was in a poor general state, having recently suffered a weight loss of 5 kg. A plain abdominal x-ray disclosed signs of mechanical ileus. An abdominal ultrasound scan showed a normal uterus, a normal right-sided ovary, but no left ovary. An emergency laparoscopy found a normal uterus with complete absence of the left ovary and salpinx, the upper left dome of the uterus being smooth with no visible horn. The right ovary and salpinx were normal. Intestinal obstruction was caused by a strangulating cordlike structure of unclear origin. After converting to a laparotomy, we found an abnormal fallopian tube inserted in the left parieto-colic groove. The tube extended next on the lateral sigmoïd mesentery and wrapped itself around the ileum, provoking a local strangulation and an ischemic covered bowel perforation. The bowel perforation was treated by a segmental bowel resection. Careful dissection of the cordlike structure disclosed a true rudimentary fallopian tube with hypotrophic fimbriae and a small distal round structure containing ovarian tissue. These structures were removed entirely. A review of the literature on this rare situation is presented and discussed.

Improvement of the effect of hepatocyte isograft in the Gunn rat by cotransplantation of islets of Langerhans.

PURPOSE: Hepatocyte transplantation (HcTX) has been investigated for many years as an alternative therapy to orthotopic liver transplantation to treat hepatic congenital enzymatic deficiency disease. The animal model most used is the Gunn rat, which presents a hyperbilirubinemia caused by the lack of uridine-diphosphate-glucuronyl-transferase. Some investigators have clearly described a hepatotrophic effect mediated by islets of Langerhans (IL) when transplanted with hepatocytes (Hc). In this study, the functional effect of cotransplanted IL on hepatocytes (co-HcTX) in Gunn rats in an isograft model is assessed. METHODS: Two groups are compared: group 1 (n = 6), HcTX to group 2 (n = 6), co-HcTX. Cells isolated by enzymatic digestion are transplanted directly into the splenic parenchyma. Blood samples are taken regularly until day 100 to measure the unconjugated bilirubin (UB). Histological examination of the spleen is performed at the end of the experiment. RESULTS: Both groups show a significant decrease of the UB: group 1, 47%; group 2, 65%. The decrease is statistically more pronounced with co-HcTX. The histological analysis shows a trophic effect of the IL on the grafted hepatocytes in the co-HcTX group. CONCLUSIONS: The HcTX and the co-HcTX correct partially the hyperbilirubinemia of the Gunn rat. A functional assessment has been performed to evaluate the effect of cotransplanted IL on HcTX.

Blunt injuries to liver, spleen, kidney and pancreas in pediatric patients.

96 children with blunt injuries to liver, spleen, pancreas and kidney were analysed. The aim of this review was to determine the accuracy of radiologic imaging and to define clinical factors present on admission which could be predictive of major injuries resolving only by operation. Two thirds of the lesions could be treated non-operatively. Diagnostic accuracy of ultrasound examination (US) varies between 60 and 90%, depending on the type of the injured organ. Peritoneal lavage was progressively abandoned in favour of US. CT scan has a 100% accuracy in the determination of the type and the extension of the injury. Our review revealed 6 clinical factors present on admission with a statistically significant predictive value for surgical intervention.

Liver transplantation in children: past, present and future.

The tremendous progress accomplished during the last twenty years in liver transplantation has permitted the treatment of infants and children who can now benefit from a new organ before an and stage liver disease. The main indications in paediatrics are congenital biliary obstructions, metabolic disorders leading to cirrhosis and fulminant hepatitis. Nevertheless, in the future, other treatments for metabolic and viral diseases will be possible. The shortage of paediatric donors has been partially alleviated by the method of reduced liver, however the general shortage of organs has led to the use of split livers and living-related donors. Overall survival in children can be expected above 80%. In Geneva, 15 paediatric patients were transplanted, 3 twice, over a 6-year period with a survival rate of 86%. The indications were the same as in other centers. Acute rejection was often noted, but easily treated, mainly by steroid bolus. 13 patients have been followed up for more than 1 year and have had satisfactory growth and normal liver function tests. Whenever a liver transplantation is performed, paediatricians have hopes and fears; hope of an improvement of growth and neuro-psychological developments and fears of side effects of immunosuppressive drugs, such as renal function impairment or lymphoproliferative syndrome. The future in the field of liver transplantation will require new methods, with the aim of decreasing the necessity of whole organ transplantation. This includes alternative treatments for metabolic disorders, transplantation of isolated hepatocytes, possibly after gene therapy, and the use of an artificial liver. Some of this future is already present.

Mesenchymal liver hamartoma in a newborn; case report.

A newborn boy had a severe, unexplained apnoea. On clinical examination a palpable abdominal mass was found extending over the right hemiabdomen. Further investigations by Ultrasound, CT scan and MRI revealed a liver tumor (7 x 5 x 9 cm). Histology from an open liver biopsy confirmed the diagnosis of a benign mesenchymal hamartoma. Operative resection of the tumor was performed when the patient was 2 1/2 months old. Postoperative management and clinical follow-up for twelve months were without complications. Primary liver tumors are rarely found in children. About half of them are malignant and associated with high mortality rates (e.g.hepatoblastoma). The most common benign liver tumors are mesenchymal hemangiomas and hamartomas. In this group of tumors prognosis has improved mainly due to progress in imaging techniques and progress in liver surgery during recent years.

Splenic cysts in pediatric patients--a report on 8 cases and review of the literature.

8 cases of splenic cysts are reported: treatment was surgical in 4 large cysts (greater than 5 cm, 3 epidermoids and 1 pseudocyst), and non-operative in 4 small cysts (less than 5 cm, 3 post-traumatic pseudocysts and 1 congenital cyst, probably epidermoid). Follow-up was over a period of 3 months to 7 years. 3 among the 4 post-traumatic pseudocysts were followed up by ultrasound until resolution (3 months to 3 years), and 1 large pseudocyst was operated on due to rupture following benign trauma. 3 large cysts were epidermoid in origin, 1 was electively operated, and 2 were discovered and operated on because of complications (rupture, infection). Only one total splenectomy was performed; surgery preserved the spleen in all other cases; but 1 epidermoid cyst presents now with a recurrence. Based on this experience, we suggest frozen section of the cyst wall during surgery, to decide the type of partial splenectomy to be performed: total cystectomy or marsupialization.

Effects of glucose on chemically induced acute hepatic failure in rats.

TOPIC: Acute hepatic failure (AHF) is a physiopathological entity difficult to reproduce experimentally but necessary to evaluate new therapies such as bioartificial liver (BAL) or hepatocyte transplantation (HcTX). Two main animal models are available: surgical or chemical induction of an AHF. Many authors have assessed BAL or HcTX with such models and observed increased survival rate in the treated groups. However, during AHF, severe hypoglycemia takes place. Some authors have shown that adjunction of glucose in beverage after surgically induced AHF may induce by itself an improvement of survival rate. METHOD: The purpose of the present study is to test if the adjunction of glucose leads to the same effect in an AHF chemically induced by D-Galactosamine (D-Gal). A group of animals (rats) receiving glucose 20% as beverage immediately after intoxication (Group 1), is compared with a control group receiving tap water (Group 2). RESULTS: The required dose to achieve AHF is 2.75 g/kg body weight (Gr. 0). Survival rates are as follows: Gr. 1: Day 3:20%; day 5: 10%; day 8: 0%. Gr. 2: Day 3: 0%. The difference in survival rate is not statistically significant between the two groups. Liver enzymes peak at 24 hours, then return to normal values; blood sugar levels show no difference between groups. Histological examination of Gr. 0 animals surviving and sacrificed on day 28 shows restitutio ad integrum of hepatic structure. CONCLUSION: Glucose adjunction does not alter survival rate. We can conclude that this particular model of AHF is reliable.

Does hepatocyte transplantation in a chemically induced acute hepatic failure make sense?

PURPOSE: Acute hepatic insufficiency (AHF) is one of the major challenges of intensive care medicine. Liver transplantation is the current solution to unsuccessful medical management. Owing to the lack of organ donors, other methods such as hepatocyte transplantation (HcTX) and bioartificial livers need to be explored. The aim of our experimental study is to evaluate the effect of hepatocyte transplantation on the survival in AHF animals intoxicated with D-Galactosamine (D-Gal). METHODS: The first step consists of the determination of the dose of D-Gal needed to induce at least 80% mortality between 48 and 72 hours. Two groups of a single strain of male Wistar rats are then compared, one being intoxicated with D-Gal (control group), the other receiving and HcTX in the splenic parenchyma 48 hours after intoxication. RESULTS: The required dose to achieve AHF is 3 gr/kg body weight (Gr. 0). Survival rates are as follow: Gr. 1: D0: 93%; from D1 to D28: 13%. Gr. 2: D0: 80%; D1 and D2: 33%; D3: 20%; D4: 13%, from D5 to D28: 6%. (D0 = Day of transplantation). Liver enzymes show a peak of deterioration at 24 hours, then return to normal values in both groups. Histological examination of those animals still alive and sacrificed on day 28 demonstrates a restitutio ad integrum of hepatic structure. In Group 2, it is possible to observe remaining living hepatocytes in the splenic parenchyma. CONCLUSIONS: When HcTX is performed 48 hours after D-Gal intoxication, i.e., when the animals begin to develop AHF symptoms, animal survival only significantly improves between days 0 and 3. Unlike other trials, we cannot demonstrate an improvement in long-term survival. Thus, according to this particular experimental model, HcTX is not an alternative for the treatment of AHF.

Escalator injuries in Genevan children: a report of 6 cases.

We present a retrospective survey of the mechanism and the lesions caused by escalators in Genevan children. Though these accidents are rare, they are often very serious. They occur primarily among children around the age of three and affect especially the hand. It is not economically feasible to replace or modify current escalators. It is therefore important that the current warning signs on escalators be improved. As there is a specific risk to children of catching fingers in the escalator and most parents are unaware of this type of danger, a warning sign with an explicit illustration could be of value. Also an insertion of a plastic bar just above the moving stairs on each lateral wall would block any potential extremities from being caught in the gap between the steps and the side of the staircase.

Selective hepatic artery ligation for hepatic haemangioendothelioma: case report and review of the literature.

Although benign, hepatic haemangioendotheliomas (HHE) are rare vascular tumours of the infant which have a high mortality rate secondary to high output congestive heart failure. The management of these tumours is still controversial and none of the different medical or surgical options has been unanimously accepted. We report the case of a neonate with congestive heart failure (CHF) due to a localized HHE, treated successfully by selective ligation of the left hepatic artery branch irrigating the tumour, under perioperative ultrasound Doppler control. A review of the literature showed 35 cases of HHE treated by hepatic artery ligation (HAL) with a survival rate of 80 %.