Laparoscopic adrenalectomy in children.

BACKGROUND: The aim of this study was to describe the indications and results of laparoscopic adrenalectomy in children. METHODS: This retrospective multicenter study included six children (four boys, two girls) aged 2-16 years (mean, 9.5). Three children had hypertension. In three cases, the adrenal mass was discovered incidentally. The tumors were bilateral in two children, right-sided in two cases, and left-sided in two cases. The mean tumor size was 4 cm (range, 1-7). Each child underwent MIBG scintigraphy and MRI before the operation. RESULTS: Four right and two left adrenal glands were resected by laparoscopy (transperitoneal), and two left glands were resected by retroperitoneoscopy. Two conversions were necessary for two left adrenal glands (one retroperitoneoscopy, one laparoscopy). Two partial resections and six adrenalectomies were performed. Histological examination of the tumors revealed two ganglioneuromas, one neuroblastoma, and five pheochromocytomas (two bilateral). One child had an involved node (pheochromocytoma). Both bilateral pheochromocytomas had von Hippel-Lindau disease. There was no morbidity. Mean hospitalstay was 6 days. Postoperative evaluation at 1 month was normal in all children. CONCLUSION: The indications for laparoscopic adrenalectomy in children are benign tumors and pheochromocytomas. In these cases, laparoscopic adrenalectomy is feasible and safe, even in cases of pheochromocytoma.

[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.

Modification of cefixime bioavailability by nifedipine in humans: involvement of the dipeptide carrier system.

We studied the action of nifedipine on the bioavailability of cefixime, a molecule absorbed via the gut wall dipeptide carrier system in the rat, and on the bioavailability of D-xylose, which is absorbed via a pH (and Na(+)-)-dependent transporter. Each compound was administered alone or in combination with 20 mg of nifedipine to eight healthy male volunteers. Nifedipine significantly increased the absorption rate of cefixime (20.7 +/- 4.3 versus 16 +/- 3.5 mg/h in the absence of nifedipine). The absolute bioavailability of cefixime alone was 31% +/- 6% compared with 53% +/- 1% (P < 0.01) in the presence of nifedipine. The observed peak concentrations in serum were significantly different (2.5 +/- 0.3 mg/liter without nifedipine and 3.7 +/- 1.1 mg/liter with nifedipine; P < 0.02). In contrast, nifedipine induced no significant differences in the pharmacokinetic profile of xylose following oral administration. We conclude that (i) cefixime is absorbed in humans by an apparently active process which can be enhanced by a calcium channel blocker, in this case, nifedipine; and (ii) nifedipine does not modify the activity of the pentose transporter.

[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.

[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)

[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.

[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.

[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.

[Neonatal ovarian cysts. Apropos of 4 cases]. / Les kystes ovarients néonatals. A propos de quatre cas.

When the authors had seen four cases of ovarian cysts in the newborn during the course of a few months they decided to describe this pathology. All four cases were discovered in utero during the course of ultrasound examination at gestationaL ages between 28 and 36 weeks. Pictures were obtained of round abdominal liquid areas measuring between 45 and 75 mm in diameter, which were separate from the bladder and the kidneys in female fetuses. One of the cysts showed two different ultrasound zones, of which one seemed to be a deposit of blood which was taken to be evidence of torsion of the ovary in utero. The diagnosis was confirmed after birth by clinical examination, straight X-ray of the abdomen, intravenous urography and ultrasound examination of the newborn. The pathological cause of these cysts is still unknown, but suggests that there was probably an abnormal stimulation by the mother's H.C.G. or abnormal enzyme activity of the theca interna. In each case delivery was vaginal. Although in each of these cases the cysts were benign they were treated surgically because all authors agree that there are risks of complications due to torsion or rupture and because one cannot be certain that they are benign.

[One complication can mask another: chance discovery of a rare lesion during screening for arthritis of the hip]. / Une complication peut en cacher une autre: découverte fortuite d'une lésion rare au décours d'une surveillance pour arthrite de hanche.

The authors report the case of a new born baby in whom at the fourth day of life, a severe hypocalcemia was discovered. An umbilical arterial catheterization was performed. The evolution was characterized by a staphylococcic septicemia with hip arthritis and partial immune deficit. The mid term survey of the joint status, allowed the unexpected discovery, during an examination held under general anesthesia, of a probable mycotic aneurysm, by abdominal palpation. The lesion was localized on the right common iliac artery and was successfully treated by resection; arterial continuity was possible without venous patch nor prosthesis.