Management of nasopharyngeal teratomas associated with cleft palate.

Nasopharyngeal teratomas are rare tumours, responsible for a high birth mortality rate from acute respiratory distress. Palatine localization can lead to an embryopathogenic mechanical obstacle responsible for a cleft palate. The aim of this study was to update current knowledge concerning the management of this rare pathological association. We conducted a multicentre, retrospective study by case analysis. The inclusion criteria were patients of any age under care for a nasopharyngeal teratoma associated with a velopalatine cleft. The diagnosis of the teratoma was confirmed by histological analysis. Seven cases were included in the study: three cases from the University Hospital of Lille, one from the University Hospital of Caen, one from of the University Hospital of Toulouse, and two from of the University Hospital of Amiens. Approximately 30% of patients experienced acute respiratory distress at birth, necessitating oro- or nasotracheal intubation. The surgical excision was performed in the first 5 months of life for all patients and in a single operative time for 70%. There was no recurrence. Therapeutic management of nasopharyngeal teratomas associated with cleft palate at birth is multidisciplinary and is based on surgical excision. In the absence of other associated pathologies, the prognosis is favourable.

[Management of unintentional pediatric female genital trauma]. / Prise en charge des traumatismes du périnée accidentels chez la jeune fille.

INTRODUCTION: Unintentional pediatric female genital trauma is frequent in the daily practice of emergency wards. However, scientific data are rare in the literature, leading to variability in their management. The aim of this study was to evaluate our practice in order to obtain epidemiological data and define clinical guidelines. MATERIAL AND METHODS: We conducted a retrospective study from March 2013 to January 2015 and identified all emergency visits for this pathology. Data were extracted from the patients' charts and a statistical analysis was performed. RESULTS: One hundred and eighteen patients were admitted during the study period, with an average age of 5.9years. Straddle injuries accounted for 73 % of the injury mechanisms. Most wounds involved the majora and minora labia. Sixty-five patients did not require stitches (55.9 %); 29 patients were examined with Meopa® but 43 % required a more precise surgical exploration in the OR, due to the lack of compliance. Forty-six patients were sutured in the OR. Associated lesions (undiagnosed in the emergency department) were diagnosed during surgical exploration in 13 patients (22 %) with two urethral wounds. Significant wound size differences were observed in 69 % of patients between the pre- and intraoperative assessments. CONCLUSION: Surgical exploration under general anesthesia should be proposed for all unintentional female genital trauma unless the patient is older than 8 and allows complete examination at the emergency department consultation.

[From paediatric urological care to adult urology. Assessment of a transition consultation for adolescents]. / De l'urologie pédiatrique à l'urologie adulte, quelle prise en charge de l'adolescent ? État des lieux d'une consultation de transition en urologie.

To provide an adequate lifelong urological care in the complex period of adolescence, a transition consultation conducted by a paediatric surgeon and an urologist was developed in our institution. As a real rite of passage, it allows the follow-up and the adapted care of urological conditions, sometimes complex, and permits the transition between childhood and the world of grown-ups. We reported our experience at the Children Hospital of our institution (paediatric surgery and urology departments). During a 6 months period (January-July 2015), forty-five young adults with a mean age of 17.8±3.6 years were seen in transition consultation. Eight patients had neurogenic voiding disorders (4 spina bifida, 1 multiple sclerosis, 1 mitochondrial encephalopathy, 1 metachromic leucodystrophy, 1 paraplegia), 9 patients had idiopathic voiding disorders, 1 patient had a non obstructive malformative uropathy; and 30 patients had surgery during infancy and childhood: hypospadias in 17 young men and malformative uropathy in 13 patients. This consultation occurred within 4.6±4.5 years after the last consultation with paediatric surgeon. For 6 patients, the transition consultation was the first for the urological problem. After this consultation, 8 patients stayed in paediatric surgery and 37 patients were referred to adult urologist. Among those 8 patients: 2 patients had cognitive and psychiatric disorders; 4 patients refused to be transferred to adult unit; 2 patients wanted to come back at transition consultation. Among the 37 patients transferred in adult urological care: 6 patients had urological surgery, and one patient was referred to a sexology consultation. The remaining 30 patients have initiated long-term monitoring. All reconvened patients came back at the follow-up visit (at least 12 months follow-up). A 16-year-old patient (spina bifida with polymalformative syndrome) developed a depressive syndrome at the end of the consultation, in the motive of an awareness of the definitive nature of his handicap and the need of medical follow-up throughout his life. Transition consultation makes easier the passage from paediatric care to adult urological care. It allows a smooth change of interlocutors, facilitates subsequent care and improves compliance to medical follow-up. It requires a good collaboration between paediatric and adult care units. Transition responds to an increasing request of adolescents, families, and medical teams, since care rupture during adolescence can have functional and psychological consequences. LEVEL OF PROOF: 4.

Altered secretion of Sertoli cells hormones in 2-year-old prepubertal cryptorchid boys: a cross-sectional study.

In cryptorchid boys, failures in germ cell development have been clearly established. Some studies reported some abnormalities in Sertoli cells morphology but the results regarding their endocrine secretion remain controversial. To compare testicular hormone levels in young boys with and without cryptorchidism, we performed a cross-sectional hospital-based study. From surgery appointment records, we identified a case group of boys with unilateral or bilateral cryptorchidism and a control group undergoing dental care, minor osteoarticular or dermal surgery. Blood samples were withdrawn during the surgical procedure to perform testosterone, inhibin B and anti-müllerian hormone (AMH) immunoassays. We included 27 cryptorchid boys and 27 controls aged of 26.6 vs. 24.2 months, respectively (p = 0.172) far from the post-natal mini-puberty and the corresponding hormonal surges. Age-adjusted AMH and inhibin B levels were significantly lower in cryptorchid than in control boys (AMH: 87 ng/mL vs. 135 ng/mL; p = 0.009, inhibin B: 97 pg/mL vs. 133 pg/mL; p = 0.019, respectively). Moreover, AMH and inhibin B levels were significantly lower in the bilateral cryptorchid subgroup, being 50% lower than in the controls (p = 0.011 and 0.019, respectively) and while both hormones levels were independent in controls, they became strongly correlated in bilateral cryptorchid boys (R² = 0.75, p = 0.001). In addition, testosterone levels were still detectable in some boys, with significantly lower levels in cryptorchid group than in controls. Overall, 2-year-old cryptorchid patients presented a simultaneous and significant drop in AMH and inhibin B levels, suggesting a functional defect of Sertoli cells. This deficiency appeared more pronounced in bilateral cryptorchidism and thus, regarding the pivotal role of Sertoli cells in germ cell development, it may explain the compromised fertility found later in men born with such a malformation.

[Conservative management of tracheal injuries in children: Clinical case and literature review]. / Prise en charge conservatrice d'un traumatisme trachéal : cas clinique et revue de la littérature.

Tracheal injuries are a rare but potentially lethal entity, most particularly in pediatrics. While standardized management allows quick therapeutic decisions in adults, based on the results of the initial bronchoscopy, the use of diagnostic exams remains unclear during childhood. We describe the case of a 6-year-old patient with a posterior tracheal wall injury due to a car accident, which was managed without tracheal endoscopy. Based on our experience and on a literature review, we suggest clarifying the management of tracheal injury in children, defining the place of computerized tomodensitometry and endoscopy in the decision process.

[Intussusception in infancy and childhood: Radiological and surgical management]. / Conduite à tenir devant une invagination intestinale aiguë du nourrisson et de l'enfant.

Ileocolic intussusception in infants and children requires emergency treatment and has a very good prognosis. Abdominal ultrasonography imaging has a high sensitivity and specificity in its detection. Management of ileocolic intussusceptions now requires fewer surgical procedures because interventional radiology is usually efficient. Surgery (laparoscopy or laparotomy) is reserved for failed radiological reductions and advanced or immediately complicated intussusceptions (pneumoperitoneum, acute peritonitis, shock). Radiology teams have two reduction techniques: hydrostatic barium enema under fluoroscopic or sonographic guidance and air enema under fluoroscopic guidance. The superiority of one radiological reduction technique over the other cannot be asserted, and the choice depends on experience and available equipment. Current management of intussusceptions should be performed by pediatric radiology, anesthesiology and surgery teams.

[Morgagni-Larrey diaphragmatic hernia in a 3-month-old child affected by Marfan syndrome]. / Hernie de la fente de Larrey chez un nourrisson de trois mois atteint du syndrome de Marfan.

The authors report on a case of diaphragmatic hernia occurring in a 3-month-old child affected by Marfan syndrome. Diagnosis was made on a chest X-ray and cardiac ultrasounds, performed because of the association of poor general condition, failure to thrive, and signs of respiratory distress. As a reminder, we emphasize the association between Marfan disease and diaphragmatic hernias as well as the diagnostic approach to reach an appropriate diagnosis.

[Prenatal diagnosis of a right thoracic congenital ectopic kidney with a diaphragmatic hernia: a combination with a good prognosis]. / Diagnostic anténatal d'un rein droit thoracique et d'une hernie de coupole diaphragmatique droite: une association de bon pronostic.

Ectopic intrathoracic kidney is a rare congenital anomaly, usually asymptomatic. This anomaly is sometimes associated with a diaphragmatic hernia. Few cases of this combination have been described, often in the absence of a prenatal diagnosis. We report on the case of a female newborn infant who was diagnosed with an ectopic intrathoracic right kidney and a diaphragmatic hernia upon 33 weeks of gestation. The patient underwent surgery on the first day of life and the respiratory and renal outcomes were simple. We review the literature and discuss the seemingly good prognosis of this combination.

[Epidemiology of malformations associated with cleft lip and palate: a retrospective study of 324 cases]. / Épidémiologie des malformations associées aux fentes labiales et palatines à propos d'une étude rétrospective de 324 cas.

PURPOSE: Children with cleft lip and/or palate may have associated malformations, whether or not they are included in a syndromic form. This study's purpose was to provide a better understanding of the epidemiology and distribution of malformations and syndromes associated with these clefts. METHODS: Retrospective study of 324 patients with cleft lip or palate born between 1994 and 2011. The associated malformations were diagnosed during the 1st year of life. RESULTS: Cleft lip or labioalveolar clefts were less frequently associated with other malformations than cleft palate. These nonsyndromic malformations preferentially affected the urogenital and renal system in case of cleft palate (48.5%) and the cardiovascular system for clefts with a lip defect (30.5%). The syndromic forms were rare in the cleft lip and labioalveolar clefts (3.47%). In contrast, cleft palate appeared much more frequently included in a syndromic form, with 52 children out of 151 (34.4%). The Pierre-Robin sequence was the most frequent syndrome with more than 25% of the cleft palate population. The defect of the palate was associated with a higher rate of other malformations or syndromes (36.51% vs 29.9% for the entire population with a cleft) (n=324) (P<0.001). CONCLUSIONS: The distribution of malformations appears to be linked to the type of cleft. These findings underline the importance of conducting a systematic neonatal malformation workup in children born with clefts in order to diagnose abnormalities and organize effective and consistent management.

[Thymoma in children: a rare and difficult diagnosis]. / Thymome de l'enfant : un diagnostic parfois difficile.

Thymoma is extremely rare within the pediatric age range, which could lead to delayed diagnosis. Based on the clinical case of a mediastinal tumor in an 8-year-old patient, we detail the key points in the management of this disease highlighted by our recent experience.

[Biliary peritonitis after traumatic rupture of a choledochal cyst]. / Péritonite biliaire par rupture traumatique d'un kyste du cholédoque.

Choledochal cysts are rare congenital malformations of the biliary tract. Traumatic rupture of a choledochal cyst can be misleading. An 11-year-old boy was admitted for peritonitis and intestinal occlusion after blunt abdominal trauma, evolving over 48 h. Laparotomy revealed bile ascites and a suspected duodenal perforation. After referral to our center, a CT scan showed a perforated choledochal cyst. Six months later, a complete excision of the cyst was successfully performed. This treatment is mandatory because of the risk of further complications such as lithiasis, pancreatitis, cholangitis, biliary cirrhosis, and malignant transformation (cholangiocarcinoma).

[Long-term outcomes after hypospadias surgery: Sexual reported outcomes and quality of life in adulthood]. / Vie sexuelle des jeunes adultes opérés dans l'enfance d'un hypospadias postérieur.

OBJECTIVE: To evaluate outcomes and long-term sexual quality of life after hypospadias surgery. Seventeen-years-old patients operated for a posterior hypospadias in childhood were included in a transversal study. PATIENTS AND METHODS: Fifteen patients, among the forty children treated since 1997, accepted to participate. These young men (mean age at the first surgery was 27.9±20months) were clinically reviewed and responded to questionnaires (EUROQOL 5, IIEF15 and non-validated questionnaire). This study arises about 8.4±5years after the last visit in paediatric department. RESULTS: Mean study age was 21.2±4.7years. One third of patients thought that global quality of life was distorted. Although 33% of the patients had erectile dysfunction, 80% were satisfied with their sexual quality of life. The most important complains were relative to the penile appearance. Number of procedures was not predictive of patient's satisfaction about penile function and appearance. Thirty-three percents of the patients would have been satisfied to have psychological and medical support. They would be interested in having contact with patients who suffered from the same congenital abnormality. CONCLUSION: These patients had functional and esthetical disturbances. This visit leads to a specific visit in 20% cases. In this study, medical follow-up does not seem to be counselling and had to be adapted. Adequate follow-up transition between paediatric and adult departments especially during adolescence seems to be necessary.

[Management of urachal remnants in children: Is surgical excision mandatory?]. / Prise en charge des résidus de l'ouraque au cours de l'enfance : l'exérèse chirurgicale est-elle obligatoire ?

INTRODUCTION: The classical management of urachal remants consists in surgical resection, in order to prevent infections and long term malignancies. However, some reports have recently spread a wait and see management. The aim of our study was to report the results of the surgical management in our center. MATERIAL AND METHODS: We conducted a retrospective, monocentric review of all patients managed for urachal remnants from January 2005 to December 2014. RESULTS: Thirty-five patients have been operated during the study period (18 girls and 17 boys). Mean age at surgery was 4,9±4,4 years old. Twenty-seven patients were referred due to symptoms whereas 8 were discovered incidentally (4 by ultrasound scan and 4 during laparoscopy). Among them, 10 were urachal cysts, 15 were urachus sinusa and 10 were patent urachus. Thirty were operated using an open approach and 5 using a laparoscopic approach. Mean length of stay was 3,8±1,7days (1-10) with a mean duration of bladder drainage of 2,5±1 days. No major complications occurred. No abnormal tissue was discovered at the histological analysis. CONCLUSION: Presentation of urachal remnants is variable but surgical outcomes remain excellent in our experience. When symptoms occur, the surgical decision is easy, but when the diagnosis is incidental, the decision is much more complicated. Official guidelines could ease the decision process and the management of urachal anomalies.

[Tibial nerve transcutaneous stimulation for refractory idiopathic overactive bladder in children and adolescents]. / Stimulation transcutanée du nerf tibial dans le traitement des hyperactivités vésicales réfractaires de l'enfant et de l'adolescent.

INTRODUCTION: The purpose of this study was to evaluate safety and tolerability of transcutaneous tibial nerve stimulation (TENS) in patients under 15years of age with refractory overactive bladder. MATERIALS AND METHODS: A retrospective analysis was conducted on outcomes of TENS (1daily 20-minute session, 10Hz) in patients with refractory overactive bladder, excluding patients with neurogenic bladder. Treatment efficacy was evaluated on symptomatic improvement and voiding schedule. Healing was defined as following: no recurrence of urinary tract infection, normal urodynamic voiding parameters, no nighttime continence disorder, normal uroflowmetry. RESULTS: Nineteen consecutive patients with refractory overactive bladder were treated from November 2010 to March 2012 (11girls, 8boys, age 12.1±2.7 years). Three patients reported only daytime voiding disorders, the others reported daytime and nighttime voiding disorders. Ten patients reported febrile urinary tract infection (1 boy, 9 girls). The average length of treatment was 6 months. Two patients were lost to follow-up. Thirteen patients had only tibial TENS; 3 patients had tibial TENS and trospium chloride or desmopressin. At 1-month assessment, 16 patients out of 17 (94%) reported symptomatic improvement. At the end of treatment, 12 patients out of 17 (70%) met healing criteria (5 boys, 7girls), without relapse within 9 months. Three boys (18%) had partial improvement (no daytime wetting, but increased daytime frequency). No patient reported side effects. CONCLUSION: Tibial TENS is a safe, non invasive and effective treatment in refractory overactive bladder in children. The success rate is 70%, with no side effect and no relapse at the end of the treatment in our study.

[Laparoscopic transposition of lower polar vessels for pyelo-ureteral junction obstruction: preliminary experience]. / Transposition des vaisseaux polaires pour syndrome de la jonction pyélo-urétérale : expérience préliminaire.

AIM OF THE STUDY: Lower pole vessels are a classical cause of PUJO even in children. The "gold standard" in the management of PUJ obstructions remains a dismembered pyeloplasty as described by Anderson and Hynes. However, some authors have developed an alternative procedure to this approach with encouraging results. The aim of our study was to evaluate our preliminary results concerning laparoscopic vascular hitch for crossing vessels. MATERIAL AND METHODS: We conducted a retrospective, monocentric study of all patients managed by this technique from January 2010 to December 2012. RESULTS: Eleven patients (7 boys, 4 girls) were managed by laparoscopy at a mean age of 10.7 years (5.4-17). They were referred to our center for clinical symptoms (intermittent pain 7, high blood pressure 1, UTI 1), antenatal diagnosis or accidental discovery. Obstruction was confirmed by MAG3 nephrogram and the presence of obstructive vessels by tomodensitometry or MRI. Mean operative time was 90.2minutes (48-184). Seven patients over 11 were strictly managed by laparoscopic transposition of lower pole vessels. Four required a classical video-assisted dismembered pyeloplasty due to a potential intraluminal stenosis. The latter were suspected by a distension test with furosemid in all four cases. Mean follow-up was 12.9±3 months. Nine patients over 11 were totally non symptomatic, whereas 2 still present mild intermittent pain. In all cases, ultrasound scans show an improvement of the pelvic dilatation. CONCLUSION: Laparoscopic transposition of lower pole vessels is a suitable and feasible alternative for the management of obstructive PUJ. Our preliminary experience emphasizes the need for a precise preoperative selection of patients along with a per operative evaluation of the obstruction. Further experience seems required to improve our criteria in this indication. LEVEL OF EVIDENCE: 5.

Free tubularised vesical mucosa graft for congenital stenosis of the urethra in children.

BACKGROUND: Reconstruction of urethral strictures in children remains a challenge to the pediatric surgeon as these are often related to different rare congenital anomalies with various clinical presentations that endanger renal function and should be repaired in young children. Multiple techniques have been described for their repair. We aimed to determine whether the use of a free tubularised bladder mucosal graft associated to a prior tubeless vesicostomy was feasible and sure, as this technique of reconstruction using tubularised grafts has not been described yet in young children. RESULTS: Two newborn male patients were referred to our department. Both presented a congenital stenosis of the urethra as a part of a complex urethral malformation. Surgery involved prior tubeless vesicostomy, free bladder mucosal graft for urethral reconstruction, and vesicostomy closure for both children. Postoperative evolution was satisfying in both children and cystourethroscopy showed permeable urethra. Satisfying cosmetic and functional results have been obtained in the two cases. CONCLUSIONS: The prior vesicostomy prevents kidney damage in the context of complex genital and urinary malformations. Bladder mucosa's immunohistological properties are the most similar to those of the urethral tissue, and are appropriate for this type of correction, making our technique feasible and sure. LEVEL OF EVIDENCE: 5.

[Magnet ingestions in children: a French multicenter study]. / Ingestion de corps étrangers aimantés chez l'enfant: enquête multicentrique française.

Digestive complications related to the ingestion of magnetic foreign bodies in children are increasing, especially in Asia and North America. In France, several case reports have been reported since 2008. We conducted a retrospective multicentric study to evaluate the frequency of ingestion of magnet foreign bodies and to describe the complicated cases that have occurred in France over the last 5 years. We report 40 cases of which 60% were multiple magnet ingestions. Eighty-eight percent of the children of the group who had swallowed multiple magnets needed interventional management by endoscopy (33%) or surgery (58%). Only two children (12.5%) of the group who swallowed one magnet required removal. This problem is not uncommon in France (2% of the 1132 foreign bodies investigated in the Toulouse center over 5 years), which justifies clear information for healthcare professionals and caregivers in order to avoid potential intestinal complications. We suggest interventional management or very close monitoring in the cases of multiple magnet ingestion. Meanwhile, in the majority of confirmed cases of simple magnet ingestions, we propose home monitoring.