Ovarian-sparing surgery for ovarian teratoma in children.

BACKGROUND: Ovarian teratoma (OT) is the most common ovarian neoplasm in children. Oophorectomy has been the standard treatment but may impair fertility. The aim of this study was to investigate the feasibility and outcome of ovarian-sparing surgery (OSS) for OT. PROCEDURE: We retrospectively studied all children treated for OT at a pediatric teaching hospital in Paris, France, between March 1992 and July 2006. OSS was performed when deemed technically feasible in patients who had no lymphadenopathy by preoperative imaging or surgical exploration, normal tumor marker levels, and calcifications on radiographs. RESULTS: We identified 30 patients, including 29 with unilateral OT and 1 with synchronous bilateral OT. Emergent surgery was performed in five patients, among whom four had ovarian torsion requiring oophorectomy and one underwent OSS. Of the 26 OTs in the 25 remaining patients, 10 were managed with OSS and 16 with oophorectomy. Subsequently, ultrasound monitoring detected OT development in the contralateral ovary in 4 (14%) patients, after a median of 3 years (range, 1-14 years); OSS was performed in all four cases. The patient with bilateral synchronous OT, managed by OSS initially, underwent unilateral oophorectomy 3 years later for a recurrence. Overall OSS was performed for 15 (42%) OTs. CONCLUSIONS: Our results suggest recommendations for preserving fertility whenever possible without compromising the oncological prognosis. In particular, OSS should be reserved for patients who meet all criteria for localized mature teratoma. Long-term follow-up is crucial.

Ingestion of neodymium magnet spheres: Three case studies.

We report three pediatric cases of two, five, and 12 neodymium magnet spheres ingested simultaneously. In two cases, endoscopic extraction failed because of the strength of the magnetic attraction and a gastrotomy was performed to remove all magnets. In the third case, the magnets were not accessible endoscopically and were removed by laparotomy. We wish to warn consumer groups and pediatricians about the danger of neodymium magnets.

[Prenatal ultrasound diagnosis of neuroblastoma]. / Neuroblastomes de diagnostic échographique anténatal.

With the progress of foetal ultrasonography, diagnosis of foetal tumors can be made before birth. We report five cases of prenatally detected neuroblastoma of the adrenal glands. Diagnosis was made during the third trimester in all cases. At birth no clinical sign related to the tumor was present, and urinary catecholamines were within normal ranges. On ultrasound scans the tumor was cystic in 1 case, solid in 2 cases and of mixed echogenicity in 2 cases. All children were operated on without pre or postoperative chemotherapy. In each setting the tumor was a stage I neuroblastoma according to the Evans classification. All children are alive and disease-free with a follow-up of 32 months at 14 years.

Intraoperative localization of neuroblastoma in children with 123I- or 125I-radiolabeled metaiodobenzylguanidine.

BACKGROUND: This study describes a novel method of intraoperative localization of neuroblastoma with a gamma-detecting probe, to detect in situ tumor binding of radiolabeled 123I- or 125I-metaiodobenzylguanidine (MIBG) and improve the quality of tumor resection. METHODS: Fifty-eight children underwent 66 surgical procedures with intraoperative detection of radiolabeled MIBG. All patients with positive MIBG scintiscans at diagnosis were included in the study. A tumor/background ratio exceeding 2:1 at the time of operation was considered positive, indicating a significant uptake of MIBG, compatible with the presence of malignant cells. The surgeons were requested to evaluate the contribution of the method to the surgical procedure. Sensitivity and specificity of the method with either 123I-labeled MIBG or 125I-labeled MIBG, on the basis of correlations between probe findings and pathologic analysis of 288 resected specimens, were determined. RESULTS: Intraoperative detection was helpful in 65% of surgical procedures, allowing a better definition of tumor limits and extension to locoregional nodes or detection of small and nonpalpable tumors in sites with difficult surgical access, especially during operation for relapse. The detection was not contributory in 35% of the procedures (well-localized tumors, thoracic neuroblastoma for technical reasons, highly differentiated tumors as ganglioneuroma, and tumors with mainly necrosis or fibrosis). The sensitivity of 123I and 125I was the same (91% and 92%), but the specificity of 125I (85%) was significantly higher than that of 123I (55%) (p < 0.005). CONCLUSIONS: First, this study demonstrates the feasibility of intraoperative detection, with radiolabeled MIBG, of neuroblastoma in children. We advocate the use of 125I rather than 123I. Second, the method is useful to improve the quality of macroscopic resection in widespread neuroblastoma with nodal involvement, in sites with difficult access, and in operations for relapse.

Pleuroperitoneal shunt in the management of chylothorax caused by thoracic lymphatic dysplasia.

Three cases of intractable chylothorax secondary to thoracic lymphatic dysplasia were treated by pleuroperitoneal shunt insertion. These cases included one with Gorham's syndrome, and one case with a bilateral chylothorax and chylous ascites. Pleuroperitoneal shunts allowed an adequate internal drainage in all cases, alleviating protein and lymphocyte losses caused by recurrent pleural taps. In the third case with chylous ascites, valved shunts were used to avoid reflux between the peritoneal cavity and the pleural space. Such palliative therapy did not change the bad prognosis of these patients with lymphatic disorders but improved the children's quality of life.

Postpneumonectomy syndrome in children: advantages and long-term follow-up of expandable prosthesis.

BACKGROUND/PURPOSE: Pneumonectomy in children can be complicated by a severe mediastinal shift, which leads to bronchial stretching resulting in severe respiratory failure. This postpneumonectomy syndrome can be corrected by inserting a prosthesis in the empty side of the chest. METHODS: Forty-two children, from 6 months to 15 years old, underwent a pneumonectomy. Seven of these patients were treated surgically for severe manifestations of postpneumonectomy syndrome. First insertion of an expandable prosthesis was followed up in 5 cases by its replacement with a breast prosthesis in adolescence. The expandable prosthesis was injected periodically with saline solution to maintain the mediastinum in a midline position as the children grew. RESULTS: The mean delay between pneumonectomy and first prosthesis implantation was 5 years (range, 11 months to 8 years). Pulmonary function tests showed a substantial improvement in the obstructive syndrome in all patients except one, in whom the functional improvement was moderate. The mean follow-up after the expandable prosthesis implantation was 6 years (range, 6 months to 10 years) and all patients are doing well. CONCLUSIONS: The insertion of an intrathoracic prosthesis can dramatically improve the clinical symptoms and reduce the functional obstructive syndrome. The expandable prosthesis allowed for progressive, well-tolerated recentering of the mediastinum and adjustment for growth.

Laparoscopic diagnosis of contralateral patent processus vaginalis in children under 1 year of age with unilateral inguinal hernia: comparison with herniography.

PURPOSE: The effectiveness of laparoscopic diagnosis of contralateral patent processus vaginalis (CPPV) in children with unilateral inguinal hernia was evaluated. METHODS: Ninety-three consecutive children under the age of 1 year were operated on for a unilateral inguinal hernia. A contralateral CPPV was diagnosed by laparoscopy via the inguinal hernia sac before ligation. The laparoscopy results of this technique were correlated with those of herniography or inguinal exploration. RESULTS: Laparoscopy was performed on 88 patients; sensitivity was 71% and specificity 89%. The only complication arising from the procedure was wound infection in two patients. CONCLUSION: This method is a simple, safe, and accurate procedure for selecting children for contralateral surgical exploration.

Venovenous single lumen cannula extracorporeal lung support in neonates. A five year experience.

The authors have developed a venovenous extracorporeal lung support technique with an original single lumen cannula to avoid the carotid ligation of venoarterial extracorporeal membrane oxygenation (ECMO). During a 5 year period, the authors have used the technique in 107 neonates (weight: 3.045 +/- 0.6 1 kg; gestational age: 38.1 +/- 2.2 weeks). All of the neonates had severe respiratory failure despite maximal conventional treatment and the same indications as those for ECMO. The venovenous technique associates extracorporeal CO2 removal and apneic oxygenation. The system includes a single lumen cannula, an alternating clamp that generates a tidal flow, and an original non-occlusive roller pump that avoids the use of a venous bladder. The PaCO2 was normal (34.6 +/- 3.9 mmHg) with a blood flow of 40-50% of the total cardiac output. Under apneic oxygenation, PaO2 improved rapidly, allowing a decrease in FiO2 and mean airway pressure, minimizing barotrauma. The mean duration of bypass was 117.8 +/- 83.9 hr, and 91 of the 107 (85%) neonates were weaned from AREC. The technical complications were less important than those associated with venoarterial ECMO. The authors conclude that AREC is as effective as venoarterial ECMO and is easier to use.

[Pickrell intervention in children for anal incontinence secondary to anorectal malformation]. / Intervention de Pickrell chez l'enfant pour incontinence anale secondaire à une malformation anorectale.

STUDY AIM: The aim of this study was to report the results of unilateral Pickrell procedure in children with fecal incontinence secondary to anal atresia. PATIENTS AND METHOD: Twenty three children were operated between 1975 and 1997. The mean age at the time of the operation was 8.5 years. Functional results were systematically evaluated by Kelly's clinical score and by anal sphincter electromyography and anal manometry for some patients. RESULTS: There was no postoperative mortality. One colostomy was performed for recurrent local sepsis. The functional result with a mean follow-up of 6 years was estimated to be good in 25%, intermediate in 45% and poor in 30%. Clinical improvement of continence was obtained in 70% of cases. CONCLUSION: The improvement of continence after Pickrell procedure essentially depends on the patient selection criteria, pre- and postoperative rehabilitation and regular enemas. In the case of poor results, the authors propose bilateral Pickrell procedure which improved the results in eight out of nine children in this group.

[Spermatic cord torsion in the newborn: role of surgical exploration]. / Torsion du cordon spermatique chez le nouveau-né: que faut-il attendre de l'exploration chirurgicale?

UNLABELLED: Torsion of the spermatic cord is frequent in neonates suffering from acute scrotal distress. A retrospective study was carried out to determine the viability and the outcome of testis treated by surgical exploration. PATIENTS AND RESULTS: Surgical exploration of 30 neonates presenting acute scrotal distress found: 18 torsions of the spermatic cord, six intrascrotal bleedings or hematoma due to delivery trauma, five tunica vaginalis inflammations and one ischemia without torsion. The torsion occurred in utero in 16 cases and after birth in two cases. The contralateral uninvolved testis was fixed to the scrotum in all cases. The torsion was extravaginal in 17 cases and intravaginal in one case. In all cases of prenatal torsion, there was total necrosis of the testis. One case of postnatal torsion operated on four hours after the beginning of the torsion was saved. CONCLUSION: Although the viability of the torsioned testis is compromised in cases of prenatal torsion, surgical exploration is necessary to confirm diagnosis and to avoid any contralateral torsion because bilateral torsion exists. Only emergency surgical exploration can save a testis that has undergone post-natal torsion as reported in others series.

[Gorham disease with prominent pleuropulmonary manifestation]. / Maladie de Gorham à manifestation pleuropulmonaire prédominante.

Gorham's disease usually manifests as diffuse osteolysis but may be complicated with pleural effusion. We describe the case of a 12 year-old boy who had repeated pleural effusions. Radiographs show a mediastinal widening and an interstitial syndrome related to hemolymphangiomatous involvement.

[Cholelithiasis in children with sickle cell disease: experience of a French pediatric hospital]. / Lithiase biliaire chez l'enfant drépanocytaire: expérience d'un hôpital pédiatrique parisien.

BACKGROUND: Gallstones are frequently encountered in sickle cell disease. Their complications are difficult to distinguish from vaso-occlusive abdominal pain and they can sometimes threaten the patient's life. The aim of this study was to describe our local experience with cholelithiasis in children with sickle cell disease. PATIENTS AND METHODS: We analyzed the follow-up records and abdominal sonography results of 185 children with sickle cell anemia, aged zero to 18 years, followed up in Trousseau Children's Hospital (Paris) from 1982 to 1998. RESULTS: Cholelithiasis was detected in 26 patients. The youngest patient was five years old. Cholelithiasis was discovered because of clinical manifestations in 12 patients. Asymptomatic cholelithiasis patients developed clinical manifestations in 28% cases in a maximum delay of two and a half years after its diagnosis. Laparoscopic cholecystectomy was performed in nine cases and open cholecystectomy in 17 cases. The mean postoperative length of stay was significantly shorter in the group of patients with laparoscopy in comparison with the group with open cholecystectomy. Histologic analysis of the gallbladders noted 85% of acute or chronic cholecystis. CONCLUSION: We suggest that cholelithiasis should be carefully sought in the presence of abdominal manifestations in sickle cell patients. We recommend that annual abdominal sonography be performed in sickle cell patients as early as seven years of age and elective cholecystectomy be performed on patients with cholelithiasis.

[The role of surgery in tuberculosis in children]. / Place de la chirurgie dans la tuberculose de l'enfant.

Despite increasingly rigorous preventive measures which have limited the number of cases in France, the number of children undergoing surgery for tuberculosis has remained unchanged. Over the last ten years we operated on 40 children in our pediatric unit. In 10 cases, surgery was indicated for diagnosis, in 19 cases surgery was required in an emergency situation during treatment and in 11 the operation was performed at the stage of sequellae. The type of surgery depended on the clinical situation. Thoracoscopy was used for surgical diagnosis and to empty caseous nodes in the lateral tracheal chain but was generally impossible at the sequellae stage.

[Testicular ectopy. Current concepts]. / L'ectopie testiculaire. Notions actuelles.

Testicular descent is a complex mechanism influenced by hormonal and anatomical factors. Whatever the age of the patient or the descent technique used for either one or both testes, there is a risk of degeneration and sterility counts. Anatomical studies and the results of sperm are important, hence the importance of establishing a precise anatomical and therapeutic classification.

[Repair of anterior hypospadias with transurethral vesical catheter: comparison of ambulatory surgery with the "between 2 diapers" catheter and traditional hospitalization]. / La cure d'hypospadias antérieur avec une sonde vésicale trans-urétrale: comparaison des hospitalisations ambulatoire avec la sonde "entre 2 couches" et traditionnelle.

OBJECTIVE: To evaluate the morbidity of ambulatory repair of anterior hypospadias with a transurethral vesical catheter (TUVC) draining the urine between 2 diapers. MATERIAL AND METHODS: 34 children divided into two comparable groups were operated for anterior hypospadias. Group A comprised 17 children operated by ambulatory surgery, with catheter drainage of urine between 2 diapers. Group B consisted of 17 children operated by a conventional inpatient procedure, with the catheter connected to a urine collector. Postoperative complications and the results of a satisfaction questionnaire sent to the parents of children in group A are reported. RESULTS: In group A, one case of TUVC dysfunction was reported and 13 parents declared to be satisfied. One urethral fistula occurred in group B. CONCLUSION: Ambulatory repair of anterior hypospadias with TUVC draining urine between 2 diapers does not induce a higher complication rate than when repair is performed by conventional hospitalisation with the TUVC connected to a urine collector. Urine drainage by TUVC is not a contraindication to ambulatory surgical repair of anterior hypospadias in children.

[Spermatic cord torsion in children]. / Les torsions du cordon spermatique de l'enfant.

OBJECTIVES: Definition of prognostic factors for torsion of the testis in children. MATERIAL AND METHODS: From January 1986 to December 1996, 91 children were operated for torsion of the testis. Torsions were observed at all ages of childhood, but with two frequency peaks: during the neonatal period and in adolescence. Typical clinical features were observed in only three-quarters of cases. RESULTS: Thirty-nine testes were considered to be necrotic versus 52 with a chance of recovery. Factors of poor prognosis were: neonatal forms, delayed management or tight torsion with several turns. 28 of the necrotic testes were conserved: two were complicated by purulent necrosis, but 14% retained normal trophicity on long-term examination. CONCLUSION: The possibility of torsion at all ages of childhood and the frequently incomplete clinical features means that emergency surgical exploration must always be performed in the case of testicular pain. A conservative attitude is motivated by the possibility of recovery of testes considered to be necrotic at operation.

[Abnormalities of the penis in boys]. / Anomalies du pénis chez l'enfant.

Abnormalities of the male genitalia have increased in the last 2 decades in numerous developed countries and remain a frequent reason of consultation in pediatric surgery. The diagnostic spectrum is wide, and surgeons should pay particular attention to these abnormalities because of their potential psychological effect. Anatomically, these abnormalities can affect one of three parts of the penis. First, the foreskin may not be fully retracted. This is normal at birth and can be caused by prepuce adherents that can continue until adolescence. Today, true phimosis is treated with topical corticoids from the age of 3 years. If medical treatment fails, a surgical procedure is required. Second, the urethra can be affected by hypospadia, which is the most frequent abnormality of the urethra. It is associated with ectopic urethral meatus, hypoplastic foreskin, and penis curvature. Its pathogenic background is not clearly understood. Surgery options differ according to the type of hypospadia and according to the surgeon's experience. It is sometimes hard to deal with, especially in a perineal form, where genetic and hormonal studies are recommended. These interventions can lead to complications ranging from stenosis to fistula. Therefore, parents have to be informed of the benefits and risks of the surgical procedures. Epispadias is rare but more serious because of the increasing risk of urinary incontinence. Finally, abnormalities of the corpora cavernosa - often associated with hypospadias - can include penis curvature and micropenis, for which an endocrinological analysis is essential.