[Does prenatal diagnosis modify neonatal management and early outcome of children with esophageal atresia type III?]. / Le diagnostic anténatal modifie-t-il la prise en charge néonatale et le devenir à 1 an des enfants suivis pour atrésie de l'œsophage de type III ?

OBJECTIVE: Evaluate neonatal management and outcome of neonates with either a prenatal or a post-natal diagnosis of EA type III. STUDY DESIGN: Population-based study using data from the French National Register for EA from 2008 to 2010. We compared children with prenatal versus post-natal diagnosis in regards to prenatal, maternal and neonatal characteristics. We define a composite variable of morbidity (anastomotic esophageal leaks, recurrent fistula, stenosis) and mortality at 1 year. RESULTS: Four hundred and eight live births with EA type III were recorded with a prenatal diagnosis rate of 18.1%. Transfer after birth was lower in prenatal subset (32.4% versus 81.5%, P<0.001). Delay between birth and first intervention was not significantly different. Defect size (2cm vs 1.4cm, P<0.001), gastrostomy (21.6% versus 8.7%, P<0.001) and length in neonatal unit care were higher in prenatal subset (47.9 days versus 33.6 days, P<0.001). The composite variables were higher in prenatal diagnosis subset (38.7% vs 26.1%, P=0.044). CONCLUSION: Despite the excellent survival rate of EA, cases with antenatal detection have a higher morbidity related to the EA type (longer gap). Even if it does not modify neonatal management and 1-year outcome, prenatal diagnosis allows antenatal parental counseling and avoids post-natal transfer.

Conservative treatment of metanephric adenoma. A case report and review of the literature.

Metanephric adenoma (MA) is a rare kidney tumor, especially in children, with an excellent prognosis and frequent association with polycythemia. We report the case of a 4-year-old girl presenting a MA revealed by polycythemia. Any secondary polycythemia requires checking for a possible kidney tumor even when the erythropoietin level is not elevated. MA is an exclusively epithelial tumor that can be similar to nephroblastoma or papillary renal cell carcinoma; therefore, it requires strict histopathological analysis. There are few radiological characteristics of MA: well circumscribed tumor, slightly enhanced after the injection of an intravenous contrast medium on computed tomography scan or magnetic resonance imaging. Diagnosis can be supported by core-needle kidney biopsy after hemostasis check, following two published cases of associated acquired von Willebrand disease. Chemotherapy can then be avoided and first-line conservative surgical treatment by partial nephrectomy can be considered, as in our patient's case. However, the marginal resection raised the question of frozen sections analysis of the margins. We recommend thorough follow-up visits, combining clinical examination, ultrasonography and, in the case of polycythemia, biological assays.

Laparoscopic transperitoneal partial nephrectomy in children under 2 years old: a single-centre experience.

OBJECTIVE: To report our experience with laparoscopic transperitoneal (TP) heminephrectomy for duplicated pyeloureteral systems in children under 2 years old. PATIENTS AND METHODS: We retrospectively reviewed the intraoperative evolution and patient outcome of 10 laparoscopic partial ureteronephrectomies (7 upper and 3 lower pole nephrectomies) using a TP approach, performed over 4 years. Mean age at surgery was 9.1 months (range 3.5-20.4), with seven children younger than 1 year. Average follow up based on clinical examinations and renal ultrasounds was 13.9 months (range 1.2-38.5). RESULTS: The average operative time was 123 min (range 90-195). There was no need for conversion and no intraoperative complications. Mean postoperative hospital stay was 2.9 days. A perirenal collection with spontaneous regression occurred in one case. The main postoperative complication consisted of a diminution in renal function with pyelic dilation in a 4-month-old child, due to inadequate positioning of the remaining moiety. This child remains asymptomatic 3 years after surgery. CONCLUSIONS: We recommend a TP approach for laparoscopic heminephrectomy due to the improved vascular exposure and control, diminished need for renal mobilization, and greater working space that can be obtained, especially in toddlers with a higher morbidity rate caused by the lack of retroperitoneal space.

Interlaboratory trial on the analysis of alkylphenols, alkylphenol ethoxylates, and bisphenol A in water samples according to ISO/CD 18857-2.

ISO/CD 18857-2 (International Organization for Standardization, Geneva) describes a new international standard method for the determination of octylphenol, nonylphenol, their mono- and diethyoxylates, and bisphenol A in nonfiltered samples of drinking, ground, surface, and wastewater. The method is based on the extraction of the analytes from an acidified water sample by solid phase extraction, solvent elution, derivatization, and determination by gas chromatography with mass spectrometric detection. For validation of this method, 14 laboratories from 4 different countries in Europe and Canada participated in an interlaboratory trial to determine the performance characteristics of the method, which are intended for publication in the corresponding standard. The interlaboratory trial was evaluated according to ISO 5725-2 and included two duplicate nonfiltered water samples: surface water containing the target compounds in an analyte concentration range from 0.05 to 0.4 microg/L and wastewater containing the target compounds in a concentration ranged from 0.1 to 5 microg/L. The repeatability variation coefficients (within-laboratory precision) varied for all samples and compounds between 1.9 and 7.8%, showing a sufficiently high repeatability of the method. The reproducibility variation coefficients (between-laboratory precision) were found to vary within a satisfactory range of 10.0-29.5% for surface water and 10.8-22.5% for wastewater. The recoveries as a measure of accuracy varied from 98.0 to 144.1% for surface water and from 95.4 to 108.6% for wastewater. The determined concentrations of the samples compared well to the "true" values, thus showing very satisfactory accuracy of the method. In the chromatogram of the surface water sample, a high unresolved background made up of coextractable matrix compounds was apparent. It is conceivable that compounds from this background may be responsible for enhanced recoveries of 144.1% for 4-nonylphenol (mixture of isomers) and of 123.4% for 4-nonylphenol monoethoxylate (mixture of isomers) in the surface water samples. The isotope-marked standard compounds developed in this context proved to be reliable internal standards that allow a precise and accurate quantitation of all compounds specified in ISO/CD 18857-2. The results of the interlaboratory trial confirmed that the analytical method is robust and reliable and can be used as a standard method to analyze the target compounds in water samples.

Comparison of detection limits in environmental analysis--is it possible? An approach on quality assurance in the lower working range by verification.

Detection limit, reporting limit and limit of quantitation are analytical parameters which describe the power of analytical methods. These parameters are used for internal quality assurance and externally for competing, especially in the case of trace analysis in environmental compartments. The wide variety of possibilities for computing or obtaining these measures in literature and in legislative rules makes any comparison difficult. Additionally, a host of terms have been used within the analytical community to describe detection and quantitation capabilities. Without trying to create an order for the variety of terms, this paper is aimed at providing a practical proposal for answering the main questions for the analysts concerning quality measures above. These main questions and related parameters were explained and graphically demonstrated. Estimation and verification of these parameters are the two steps to get real measures. A rule for a practical verification is given in a table, where the analyst can read out what to measure, what to estimate and which criteria have to be fulfilled. In this manner verified parameters detection limit, reporting limit and limit of quantitation now are comparable and the analyst himself is responsible to the unambiguity and reliability of these measures.

[Post-traumatic intrasplenic arteriovenous fistula in a child. Spontaneous regression]. / Fistule artério-veineuse intrasplénique post-traumatique chez un enfant. Régression spontanée.

We report on an 11-year-old boy who developed splenic arteriovenous fistula resulting from blunt abdominal trauma. This fistula disappeared spontaneously after 8 months of follow-up without any complication.

Retroperitoneal laparoscopic nephrectomy in children: preliminary report of six cases.

Laparoscopic nephrectomy is a new procedure that must be evaluated in adults and children. This technique allows a reduction in complications and sequelae. The majority of indications, such as renal dysplasia and destroyed kidneys due to obstructive or refluxing uropathy, are suitable for laparoscopic nephrectomy. Contraindications are Wilms' tumor and trauma, which represent only 20 percent of nephrectomies in our experience. As in open surgery, to perform nephroureterectomy for benign disease, a retroperitoneal approach seems more logical than transperitoneal approach, which is the usual approach for laparoscopic surgeons. We have attempted six retroperitoneal laparoscopic nephrectomies in children from 3 months to 14 years old. The patient is positioned in a lateral position after creation of a retropneumoperitoneum under visual control; three or four ports are needed and renal vessels are dissected then clipped, or coagulated if small. Destroyed kidneys are generally of small size, so they can be extracted via a 10- or 12-mm cannula site without morcellation. Operative time ranges from 35 to 210 mm (median 120 mm). We have had no complications or conversions. Retroperitoneal laparoscopic nephrectomy in children is a feasible and safe procedure in well-trained hands.

Retroperitoneal laparoscopic nephrectomy in children. Preliminary report of 18 cases.

Laparoscopic nephrectomy is a new procedure which remains to be evaluated in adults and children. This technique enables the reduction of parietal complications and sequelae. The majority of indications, e.g. renal dysplasia, destroyed kidneys due to obstructive or refluxing uropathy, are suitable for laparoscopic nephrectomy. Contraindications are Wilm's tumor and trauma which represent only 20% of nephrectomies in our experience. As in open surgery, a retroperitoneal approach seems more logical and better adapted than a transperitoneal approach to perform nephroureterectomy for benign disease. From August 1993 to December 1995, we attempted 18 retroperitoneal laparoscopic nephrectomies in children aged from 3 months to 14 years. The patient is placed in a lateral position, and after creation of a retropneumoperitoneum under direct vision control without balloon dissection, three or four ports are needed, renal vessels are dissected, then clipped or coagulated if small. Destroyed kidneys are generally of small size, so they can be extracted via a 10- or 12-mm cannula site without morcellation. Operative time is 35-210 min (median 106 min). There were no major complications and only one conversion. In conclusion, retroperitoneal laparoscopic nephrectomy in children is a feasible and safe procedure in well-trained hands.

Temporal and structural features of infant calls in relation to caregiving behaviour in common marmosets, Callithrix j. jacchus.

The role of infants in the communal rearing system of marmosets and tamarins has received little attention although their demands may not be complied by inadequate helpers. As acoustic signalling plays an important role in their social communication, it is expected to be an effective way for infants to signal their demand for care. As a first approach, this study focussed on the use and quality of 'tsik' calls given by infants of common marmosets (Callithrix j. jacchus) in the context of caregiving. The acoustic and non-acoustic behaviour of a group of common marmosets were recorded during the first two weeks of the life of two infants (twins). Vocalizations were classified, and the structure of the infants' 'tsik' calls was quantified by measuring specific signal parameters. Infants gave 'tsik' calls in close temporal relation to caregiving behaviour. Specific parameters covaried with the subsequent behaviour of potential caretakers. Values of call duration, start frequency, peak frequency, and range were lower when infants' calls were followed by no reaction or an approach of group members, whereas these parameters showed higher values when followed by a transfer of infants. Our results suggest that the actual demand of infants is encoded in the variations of 'tsik' calls.

[Spontaneous colonic perforations revealing Ehlers-Danlos syndrome type IV]. / Perforations coliques spontanées révélatrices d'un syndrome d'Ehlers-Danlos de type IV.

BACKGROUND: The malignant form of Ehlers-Danlos syndrome type IV owes its bad reputation to a proneness to spontaneous rupture of bowel or large vessels, which may reveal the disease. CASE REPORT: A girl suffered acute rupture of the sigmoid at the age of 5 years and rupture of the left colon, twice, at the age of 11 and 13 years, respectively. These ruptures required colostomy and finally colectomy. A proneness to bruisability, history of dislocation of hips, hypermobile joints, ovarian cysts and some minor abnormalities of her face resembled that of the Ehlers-Danlos syndrome which was confirmed by optic and electronic microscopy of the skin biopsy. CONCLUSION: This is the youngest case of rupture of bowel reported in Ehlers-Danlos syndrome. Long-term prognosis is influenced by repetition of intestinal ruptures and occurrence of vascular complications.

[Clinical experience with totally implantable venous access systems in pediatric hematology and oncology]. / Expérience clinique des systèmes veineux totalement implantables en hématologie et oncologie pédiatriques.

Forty-three children with malignant diseases who received 48 totally implanted venous accesses (TIVA) were retrospectively analyzed. More than half the patients had acute leukemia. Mean age was 6 years 10 months. Mean duration of use of the TIVA was 473 +/- 50 days (range 28 to 1,285 da; median 424 days). Removal of the TIVA was required because of an adverse event in 33% of cases. Main reasons for removal included infection (22.9%), thrombosis (6.25%), and catheter dysfunction (4.16%). Catheter-related infections were most often due to staphylococci (90%), especially S. epidermidis (63%). Infection rate was 0.48 per 1,000 patient-days. Flushing with a vancomycin-heparin solution can be expected to decrease this rate. Selection of the implantation site is discussed. In children under 6 years of age, the cephalic vein and external jugular vein are often frail or absent and are therefore less appropriate than the internal jugular vein or subclavian vein.

Transverse mucosal preputial flap for repair of severe hypospadias and isolated chordee without hypospadias: a 350-case experience.

Three hundred patients with hypospadias and 50 patients with isolated chordee without hypospadias were operated on and followed by the same surgeon. A very low postoperative urethrocutaneous fistula rate (4.8%) was related to complete cutaneous coverage of the neourethra. The incidence of stenosis, noted in 8% of the cases, has progressively decreased with improvement of technical details and with growing experience. A total of 75% of the patients were corrected with a single operative procedure. Good disponibility of penile skin rendered a second intervention always easy.

[Tracheal compression by an aberrant brachiocephalic trunk: diagnosis by magnetic resonance]. / Compression trachéale par un tronc artériel brachio-céphalique aberrant: diagnostic par résonance magnétique.

In a full-term infant who underwent surgery for type III esophageal atresia, attempts to remove the tracheal tube postoperatively failed. Bronchoscopy disclosed external compression of the trachea in the chest. Selection of the investigations most likely to provide the etiologic diagnosis was discussed. Because of the patient's poor general condition, MRI of the mediastinal area was performed first and showed an abnormal origin of the brachiocephalic trunk which was the cause of tracheal compression. No attempt at corrective surgery was made and the patient was successfully extubated at four months of age.

Psychosocial factors related to perceived compliance with cystic fibrosis treatment.

The present research was designed to investigate the adjustment of patients with cystic fibrosis (CF) and their families as well as the relationship between adjustment and physician perceptions of compliance with CF treatment. Patient and family adjustment was assessed by means of the Personality Inventory for Children as well as measures of marital adjustment, depression, and social isolation completed by mothers of CF patients. Results indicated no characteristic pattern of psychopathology or adjustment problems. However, higher levels of perceived compliance with CF treatment were found to be associated with less satisfactory marital relationships and with less frequent maternal social contacts. These findings are discussed in terms of achieving a balance between compliance and psychological adjustment.

[En bloc transplantation of liver, stomach, pancreas and small intestine in an infant. Apropos of a case]. / Transplantation en bloc du foie, de l'estomac, du pancréas et de l'intestin grêle chez un tout-petit. A propos d'un cas.

The now common practice of joint kidney and pancreas or heart or lung transplantation is being completed by other combinations. This is shown by our case of en bloc liver-pancreas-stomach-duodenum-small bowel transplantation in an 18-month-old infant with small bowen atresia complicated by biliary cirrhosis secondary to total parenteral feeding, after the failure of an intraperitoneal visceral transplant at 1 year of age. The graft was taken from an 8-year-old donor and was not pretreated. Being made of the whole intraperitoneal visceral mass, it had to be adapted to the recipient's size by ex vivo exeresis of the right liver, of the spleen, of the terminal ileon and of the colon. Following intraperitoneal visceral exenteration in the recipient, the graft was inserted in an orthoptic position with a digestive reconstruction by esogastric anastomosis and terminal ileostomy. Immunosuppression combined steroids, azathioprine, ciclosporine, and the biological and immunological follow-up regarded the hepatic and pancreatic functions. The intestinal graft was controlled by repeated biopsies through the stomy. Rectal biopsies and lymphocyte typing in the peripheral blood allowed watching for the occurrence of a possible graft-versus-host disease. The outcome was marked by the persistence of massive lymphorrhea during three months and severe central neurological disorders caused by the difficulties to adapt the level of ciclosporine. The hepatic and pancreatic functions became normal within a few days, and the intestinal function allowed progressively suppressing parenteral feeding.(ABSTRACT TRUNCATED AT 250 WORDS)

Analysis and perspectives of endoscopic treatment of vesicoureteral reflux in children with a 20-month follow-up.

We report our experience with the endoscopic treatment of vesicoureteral reflux by a submucosal injection of Teflon in children. During a period of 20 months, 291 refluxing ureters were injected by 4 members of our surgical team. A cystography was performed 3 months later. Because 53 children have a follow-up of less than 3 months, we only consider the remaining 201 ureters. The success rate is 92% which is similar to that of surgical treatment. We insist on some precautions and contraindications. Endoscopic treatment is an undeniable progress, but surgical treatment is still required in some cases.

[Angioma of the umbilical cord. Apropos of a case]. / L'angiome du cordon ombilical. A propos d'une observation.

We report a case of umbilical cord angioma in a female newborn. Ultrasonographic antenatal diagnosis had suspected either an omphalocele or a tumor of the umbilical cord. A caesarean had been decided at term. Surgery was necessary to ligate the umbilical vessels. The cord was 37 cm long and 7 cm large in several places. It weighed 495 g. The interest of this case resides in its rarity. First description of this kind of tumor was made by Gerdes in 1864 and since, only 20 cases have been reported. Antenatal diagnosis difficulties in umbilical cord tumors are reviewed. Vitelin cyst, angioma, and teratoma represent the most common diagnosis which are histologically and clinically discussed. Associated malformations and complications of umbilical cord angioma are reviewed in the literature.