Koyanagi urethroplasty for proximal hypospadias: A stage procedure?

OBJECTIVE: To report experience and technical refinements with the Koyanagi urethroplasty for proximal hypospadias. METHODS: A retrospective study was conducted between 2004 and 2017. Medical records of patients treated by the Koyanagi technique were investigated. The penile ventral skin was closed either with a Byars flap or using an "Ombredanne's chasuble" (OC). The cohort was divided chronologically into two groups of the same number of patients (early -E- and late experience group-LEG-). Demographics, anatomical findings and surgical outcomes were compared between groups using univariate analysis. A logistic regression was performed to assess factors associated with the occurrence of a postoperative urethrocutaneous fistula or urethroplasty dehiscence (UD). RESULTS: The Koyanagi urethroplasty was performed in 67 patients, 33 in the EEG and 34 in the LEG. The overall surgical complication rate was 64.2% (n=43), including 42 fistula or UD, higher in the EEG (81.8%) than in the LEG (44.1%, P<0.01). However, in the LEG, patients underwent the surgery older and the use of OC more frequent. After multivariate analyses, factors associated with a postoperative fistula or UD were the year of surgery (OR=0.71 [0.53-0.96] P=0.02), the age at surgery (OR=1.11 [1.01-1.22], P=0.03); contrary to the skin coverage method or the stenting duration (P>0.05). An urethral stenosis occurred in 1 patient (1.5%). At last follow-up, 64.2% of patients required a further procedure and 80.6% of urethral meatus were glandular. CONCLUSION: In this study the complication rate, particularly the urethrocutaneous fistula, remained high. Urethral stenosis, were rare but the follow-up was too short to clearly identified them. Considering as a 2 stage procedure koyanagi uretroplasty allows to obtain finally good results. LEVEL OF EVIDENCE: III.

Repeated detrusor injection of botulinum toxin A for neurogenic bladder in children: A long term option?

AIMS: Evaluation of repeated (at least 4) intra-detrusor injections of toxin botulinum A (IDI-TBA) for neurogenic bladder in a pediatric cohort. METHODS: Patients who underwent at least 4 IDI-TBA between 2005 and 2017 for neurogenic bladder related issues were included (detrusor overactivity and low compliance). Clinical and cystometric data were collected before and after the first injection and after the last injection. The primary endpoint was the proportion of patients with non-abnormal cystometry (no detrusor overactivity and normal compliance). Secondary outcomes were the evolution of the observed bladder capacity/expected ratio, surgical complications and acquired kidney impairment. RESULTS: From the 832 patients referred to our institution for neurogenic bladder, 48 underwent IDI-TBA, and 17 at least 4 injections. Among them, a total of 95 procedures were performed (median per patient 5 [4-8]). While the first injection had a significant effect for 82.3% patients, the last injection improved the medical condition for only 53.0% cases. The bladder capacity ratio, initially 36.1%, increased to 80.3% after the first injection but decreased to a level of 57.1% at last. After a median follow-up of 57 [34-102] months, no severe complications were reported but 11.8% of patients presented with repeated pyelonephritis. A bladder augmentation surgery was finally indicated for 35.3% cases. CONCLUSIONS: Despite a low complication rate and impressive cystometric results after the first injection, IDI-TBA efficacy decreased with time and repetition. These findings prone a long-term follow-up and a "à-la-carte" management of this specific population depending on the long-term response to IDI-TBA.

How can we improve perinatal care in isolated multiple intestinal atresia? A retrospective study with a 30-year literature review.

INTRODUCTION: Multiple intestinal atresia (MIA) is a rare cause of neonatal intestinal obstruction. To provide an overview of the current prenatal, surgical, and nutritional management of MIA, we report our experience and a literature review of papers published after 1990. METHODS: All cases of isolated MIA (non-hereditary, not associated with apple-peel syndrome or gastroschisis) treated at our institution between 2005 and 2016 were reviewed and compared with cases found in the literature. RESULTS: Seven patients were prenatally suspected of having intestinal obstruction and were postnatally diagnosed with MIA, with a mean 1.7 (1-2) resections-anastomoses (RA) and 6 (1-10) strictureplasties performed, resulting in a mean resected bowel length of 15.1cm (15-25 cm). Median time to full oral feed was 46 days (14-626 days). All patients were alive and none had orality disorder after a mean follow-up of 3.1 years (0.2-8.1 years). Three surgical strategies were found in the literature review: multiple RA (68%, 34/50) including Santulli's technique in four of 34 (12%) and anastomoses over a transanastomotic tube (32%, 16/50), with a 98% survival rate, and short-bowel syndrome for only two patients. CONCLUSION: Bowel-sparing surgery and appropriate medical management are key to ensuring a favorable nutritional and gastrointestinal outcome and a good prognosis. Prenatal assessment and standardization of the surgical course of treatment remain challenging.

Reform of the 3rd cycle in surgery training.

The main objectives of the reform of the 3rd cycle of medical studies in France that was instituted in 2017 after eight years of preparation, are to train future specialists in a consistent and equitable fashion and to replace the previous time-based qualification by training based on the progressive acquisition of skills. This reform was an opportunity for the 13 different French surgical specialty Colleges involved to share reflections on what a surgeon actually was and to define training in surgical sub-specialties. The current reform is well adapted to these specifications and has fostered training models that are consistent with each other. This article discusses the historical construction of this reform, what will change in the training of future surgeons, as well as some points that warrant caution. The third cycle reform has also triggered a reform of the second cycle, which is expected to come into force for the 2020 academic year. Its objective will be to eliminate the guillotine effect created by the National Classifying Examinations and to allow students to better understand and test their desire and skills for a given specialty. It will be up to these same surgical Colleges to determine how to do this for the sub-specialties of the "surgery" discipline.

Indications and results of diagnostic biopsy in pediatric renal tumors: A retrospective analysis of 317 patients with critical review of SIOP guidelines.

OBJECTIVES: According to the Renal Tumor Study Group (RTSG) of the International Society of Paediatric Oncology (SIOP), diagnostic biopsy of renal tumors prior to neoadjuvant chemotherapy is not mandatory unless the presentation is atypical for a Wilms tumor (WT). This study addresses the relevance of this strategy as well as the accuracy and safety of image-guided needle biopsy. METHODS: Clinical, radiological, and pathological data from 317 children (141 males/176 females, mean age: 4 years, range, 0-17.6) consecutively treated in one SIOP-affiliated institution were retrospectively analyzed. RESULTS: Presumptive chemotherapy for WT was decided for 182 patients (57% of the cohort), 24 (8%) were operated upfront, and 111 (35%) were biopsied at diagnosis. A non-WT was confirmed after surgery in 5/182 (3%), 11/24 (46%), and 28/111 (25%), respectively. Age at diagnosis was the most commonly (46%) used criterion to go for biopsy but a nine-year threshold should be retrospectively considered more relevant. Tumor volumes of clear cell sarcoma of the kidney and WT were significantly higher than those of other tumors (P = 0.002). The agreement between core-needle biopsy (CNB) and final histology was 99%. No significant morbidity was associated with CNB. CONCLUSION: The use of SIOP criteria to identify patients eligible for presumptive WT neoadjuvant chemotherapy or upfront surgery avoided biopsy in 65% of children and led to a 97% rate of appropriate preoperative chemotherapy. Image-guided CNB is a safe and accurate diagnostic procedure. The relevance of SIOP biopsy criteria might be improved by using an older age threshold (9 years instead of 6 years) and by adding initial tumor volume.

One-stage combined delayed bladder closure with Kelly radical soft-tissue mobilization in bladder exstrophy: preliminary results.

BACKGROUND: The radical soft-tissue mobilization (RSTM, or Kelly repair) is an anatomical reconstruction of bladder exstrophy generally performed as a second part of a two-step strategy, following successful neonatal bladder closure. OBJECTIVE: The objective of this study is to determine the feasibility of a combined procedure of delayed bladder closure and RSTM in one stage without pelvic osteotomy, in both primary and failed initial closure. DESIGN, SETTING, AND PARTICIPANTS: From 11/2015 to 01/2018, 27 bladder exstrophy patients underwent combined bladder closure with RSTM by the same surgical team at four cooperating tertiary referral centers for bladder exstrophy, including 20 primary repairs (delayed bladder closure, median age 3.0m [0.5-37m]) and seven secondary repairs after failed attempt at neonatal closure, median age 10m [8-33m]. INTERVENTION: RSTM included full mobilization of the bladder plate, urogenital diaphragm, and corpora cavernosa from the medial pelvic walls, followed by anatomical reconstruction with antireflux procedure, bladder closure, urethrocervicoplasty, muscle sphincter approximation, and penile/clitoral reconstruction. OUTCOME MEASUREMENTS: The main criteria were bladder dehiscence or prolapse. Secondary outcomes included bladder neck fistula or urethral fistula, urethral stenosis, and parietal hernia. Continence and voiding have not been addressed at this stage. RESULTS AND LIMITATIONS: All bladder exstrophy cases were successfully closed without osteotomy, with no case of bladder dehiscence after 12 m [3-30] follow-up. COMPLICATIONS: Urethral fistula or stenosis occurred in eight patients: 4/5 fistulae closed spontaneously in less than 3 months; four urethral stenoses were successfully treated with 1-3 sessions of endoscopic high-pressure balloon dilatation or meatoplasty; one patient with persistent bladder neck fistula is currently awaiting repair. Although the follow-up is short, it does allow examination of the main outcome criterion, namely bladder dehiscence, which is usually expected to happen very early after surgery. CONCLUSION: The Kelly RSTM can be safely combined with delayed bladder closure without osteotomy in both primary and redo cases in classic bladder exstrophy.

Total midgut volvulus in adults with intestinal malrotation. Report of eleven patients.

OBJECTIVE: Total small-intestinal volvulus with malrotation (TSIVM) classically presents in the neonatal period; it occurs much less frequently in the adult and is often misdiagnosed. Prognosis is directly related to the degree and duration of intestinal ischemia. Our goal is to describe our experience with TSIVM in the adult, to identify any specific findings and to discuss its management. METHOD: Eleven patients who had undergone surgery for TSIVM at three centers between 1992 and 2012 were included. Surgery was performed as an emergency for five patients and surgery was elective for six. RESULTS: Mean follow-up was 63 months (range: 12-270). Six patients had had previous abdominal surgery. In nine cases, the diagnosis of TSIVM was made preoperatively, mainly by CT scan in eight cases. Seven patients had associated congenital failure of retroperitoneal fixation of the right colon and all of these underwent a Ladd procedure. The mortality rate was zero. Of the five patients who underwent emergency surgery, three required intestinal resections, one of whom developed a short bowel syndrome. The six patients who underwent surgery electively had no surgical complications. CONCLUSION: TSIVM is a very unusual finding in adult patients. The diagnosis can be made by CT scan with IV and oral contrast, but it often comes to light only at the time of surgery, even though the patients have often had recurrent episodes of abdominal symptomatology that dated back to childhood. The Ladd procedure, consisting of division of Ladd's bands, widening of the mesentery, and incidental appendectomy, remains the standard surgical repair. Digestive surgeons who care for adults should be familiar with this procedure, and it should be performed, as often as possible, with the assistance of a pediatric surgeon.

[Latex-free hospitals: benefits and disadvantages]. / Hôpitaux pédiatriques sans latex: avantages et contraintes.

The objective of this article is to inform health-care personnel, especially those of pediatric hospitals (pediatricians, surgeons, anesthetists, etc.), about the incidence of latex allergy in children and the adverse effects that may result if avoidance measures are not taken. The prevalence of this allergy is increasing because of repeated exposure to this ubiquitous material (in medical equipment or household products such as bottle teats or balloons). The risks are allergic reactions, ranging from benign local dermatitis to anaphylactic shock. This problem is well known and progress has been made: there is increased production of latex-free products and screening for latex-related allergy or sensitization during the pre-anesthetic evaluation to take preventive actions when planning surgery. The younger the patient exposed to latex, the higher the risk of sensitization. Therefore, the main issue raised in this article is the potential benefit of moving to latex-free health-care facilities so as to minimize the risk of allergic incidents. Many studies showed a significant decrease in the risk of allergy (sensitization and allergic reaction) when avoiding latex, but we must also consider the feasibility, the cost, and the effectiveness of such a policy.

[Recurrent small-bowel intussusceptions revealing a disseminated BCG infection related to severe combined immunodeficiency]. / Invagination intestinale récidivante révélatrice d'une bécégite disséminée liée à un déficit immunitaire combiné sévère.

Infant small-bowel intussusceptions, most of the time idiopathic, may exceptionally reveal a severe digestive disease. We report the case of a 4-month-old infant who presented multiple and simultaneous ileal intussusceptions associated with severe acute gastroenteritis. Initially, the infant showed a protein-losing enteropathy with a clear alteration of the general state of health and undocumented fever, resistant to broad-spectrum antibiotic therapy. Skin and splenic nodules associated with hepatosplenomegaly and pancytopenia set in progressively. The etiologic evaluation led to the diagnosis of a Mycobacterium bovis BCG infection related to severe combined immune deficiency. The treatment consisted in anti-tuberculosis quadruple therapy in association with immunoglobin supplementation. Secondarily, the patient underwent gene therapy in a clinical trial. An early BCG vaccine in the first weeks of life, before the outbreak of infection revealing the immune deficiency, is a risk factor in triggering a disseminated BCG infection in immunodepressed infants. This clinical case is the first reported of severe combined immune deficiency revealed by small-bowel intussusceptions related to a disseminated BCG infection.

[Hypnosis as an effective management of a child with posttraumatic stress disorder after perineal trauma]. / Panser par l'hypnose après un traumatisme périnéal : prise en charge d'une jeune fille en état de stress post-traumatique par l'hypnothérapie.

Children and teenagers may face trauma that threatens their life, but also their psychological integrity. These injuries can lead to posttraumatic stress disorder (PTSD), which is the most common psychopathological consequence after a trauma. Age is not a protective factor and this disorder can be severe and may last over a long-term period. Effective therapies on PTSD are scarce and research on this topic is rare in children. We report a case of a 12-year-old girl affected by PTSD after a carousel accident at the age of 4 years. A therapy based on hypnosis and psychological support was rapidly effective. This psychotherapeutic option was chosen on the basis of common features shared by hypnosis and the posttraumatic symptoms. Clinical manifestations of PTSD disappeared after 4 weeks of therapy and the patient remained symptom-free during a 1-year follow-up. Our report suggests that hypnosis could be an effective therapy for children with PTSD. Prospective studies on a larger number of patients are needed to validate this hypothesis.

[Endoscopic management of urinary incontinence in neurogenic bladder due to spinal cord lesions in children]. / Prise en charge endoscopique de l'incontinence urinaire des vessies neurologiques d'origine médullaire de l'enfant.

INTRODUCTION: The urological management of urinary incontinence in neurogenic bladder due to spinal cord lesions in children is intended to achieve social continence while preserving the upper urinary tract, combining clean intermittent catheterization with anticholinergic agents. The objective of this study was to report the results on continence of endoscopic management of bladder and/or sphincter of children with failure or intolerance to first intention therapy. PATIENTS AND METHODS: Of the 364 children followed for neurologic bladder in our institution, 22 required endoscopic management between 2000 and 2012. Urinary incontinence was related to detrusor overactivity in 16 children and sphincter deficiency in 13 children, requiring one or several intradetrusor injections of botulinium toxin-A (BTA) and/or dextranomer/hyaluronic acid (Dx/Ha) injection in the bladder neck. Continence was reassessed between six and eight weeks after the last injection using the Schulte-Baukloh score. RESULTS: At the end of the mean follow-up of four years, 16 children received 54 injections of BTA and 13 children had 24 injections of Dx/Ha. Social continence, defined as a score between 0 and 1, was acquired quickly after injection of BTA and required to repeat the injections every 8.7 months (6-12) with a very low morbidity. After the first injection of Dx/Ha, 69% of the children improved significantly their incontinence score (from 1 to 0 or from 2 or 3 to 1) with better results for girls. CONCLUSION: An appropriate endoscopic management has enabled an improvement of the continence of two-thirds of children who fail first intention treatment for their neurogenic bladder. This is an alternative to delay or avoid major surgery.

Beckwith-Wiedemann syndrome: growth pattern and tumor risk according to molecular mechanism, and guidelines for tumor surveillance.

BACKGROUND: Beckwith-Wiedemann syndrome (BWS) is an overgrowth syndrome associated with an increased risk of pediatric tumors. The underlying molecular abnormalities may be genetic (CDKN1C mutations or 11p15 paternal uniparental isodisomy, pUPD) or epigenetic (imprinting center region 1, ICR1, gain of methylation, ICR1 GOM, or ICR2 loss of methylation, ICR2 LOM). AIM: We aimed to describe a cohort of 407 BWS patients with molecular defects of the 11p15 domain followed prospectively after molecular diagnosis. RESULTS: Birth weight and length were significantly higher in patients with ICR1 GOM than in the other groups. ICR2 LOM and CDKN1C mutations were associated with a higher prevalence of exomphalos. Mean adult height (regardless of molecular subtype, n = 35) was 1.8 ± 1.2 SDS, with 18 patients having a final height above +2 SDS. The prevalence of tumors was 8.6% in the whole population; 28.6 and 17.3% of the patients with ICR1 GOM (all Wilms tumors) and 11p15 pUPD, respectively, developed a tumor during infancy. Conversely, the prevalence of tumors in patients with ICR2 LOM and CDKN1C mutations were 3.1 and 8.8%, respectively, with no Wilms tumors. CONCLUSION: Based on these results for a large cohort, we formulated guidelines for the follow-up of these patients according to the molecular subtype of BWS.

[Video-assisted surgery in children: current progress and future perspectives]. / Vidéochirurgie chez l'enfant : progrès actuels et perspectives.

This review presents the evidence of video-assisted surgery in the pediatric population and discusses future progress in this field. Videosurgery minimizes the cosmetic impact and the pain induced by open procedures and has been in constant development in adults and children. Earlier training of surgeons and residents combined with advances in anesthetics and technology have expanded the use of videosurgery for more complex interventions. Although most feasible surgical procedures have been performed by laparoscopy, the literature has not yet defined it as the gold standard for most interventions, especially because of the lack of evidence for many of them. However, laparoscopy for cholecystectomy is now the preferred approach with excellent postoperative outcomes and few complications. Although no evidence has been demonstrated in children, laparoscopy has been shown to be superior in adults for gastroesophageal reflux disease and splenectomy. Laparoscopic appendectomy remains controversial. Nevertheless, meta-analyses have concluded in moderate but significant advantages in terms of pain, cosmetic considerations, and recovery for the laparoscopic approach. Laparoscopy is now adopted for undescended testes and allows both localization and surgical treatment if necessary. For benign conditions, videosurgery can be an excellent tool for nephrectomy and adrenalectomy. However, laparoscopy remains controversial in pediatric surgical oncology.

[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.

[Bladder and bowel function in children with congenital spinal lipomatus malformations. A retrospective study of 114 cases]. / Devenir vésicosphinctérien et anorectal des enfants ayant un lipome du cône. Analyse rétrospective sur 114 cas.

OBJECTIVE: To clarify bladder and bowel function of children with lipomas of the conus, without, before and after neurosurgery. PATIENTS AND METHODS: Retrospective analysis of 114 children with a lipomas of the conus, followed in our pediatric neuro-urology department from 1993 to 2010. Several data were collected: bladder and bowel symptoms, bladder and anorectal continence, neurosurgical indication and age, clinical modification after neurosurgery, investigations carried out in pre- and post-surgery treatment, associated bladder and bowel treatment. RESULTS: Forty-nine of the 77 children (63.6%) operated on had never been seen before surgery in our neuro-urology department. Seventy-seven children (67.5%) underwent a neuro-surgery, 60% indicated due to a neurogenic bladder. Before neurosurgery, 66 children (85.7%) had spontaneous miction. Five children (6.5%) had bladder intermittent catheterization. Forty of these patients (56.3%) were continent. After neurosurgery and a specialized consultation in neuro-urology, 54 children (70.1%) were continent. Thirty-seven children (48%) had spontaneous miction. Thirty-seven children (48%) had bladder intermittent catheterization and drug of overactive detrusor. Fifty-two children (67.5%) were constipated after surgery. Seventy-seven percent of the treatments for bowel symptoms were effective in terms of continence. CONCLUSION: The existence of a neurogenic bladder was one of the main indications for neurosurgery. These results suggest that the complexity of care requires neurosurgical, urological surgeon and neuro-urology physician to achieve the explorations and urinary and digestive treatment in order to preserve renal function and both continences.

[Prenatal intestinal volvulus: A life-threatening event with good long-term outcome]. / Volvulus prénatal du grêle : risque vital immédiat mais bon pronostic à long terme.

BACKGROUND AND AIMS: To describe the outcome of neonates with prenatal intestinal volvulus. PATIENTS AND METHODS: All neonates with prenatal intestinal volvulus managed in our institution between May 2004 and December 2010 were retrospectively studied. All neonates with prenatal or neonatal diagnosis of prenatal intestinal volvulus were included. We analyzed age at diagnosis, fetal ultrasound (US) scan and magnetic resonance imaging (MRI) findings, clinical signs at birth, surgical findings, management, and postoperative outcome. RESULTS: Ten neonates with prenatal intestinal volvulus were identified. Prenatal US scans or MRI demonstrated evidence of meconium peritonitis in one fetus and bowel dilatation in 2 others. The mean gestational age at birth was 36 weeks (range, 31-38 weeks) and the mean birth weight was 2811g (range, 2050-3700g). One premature neonate developed respiratory distress and required ventilatory support at birth. In 7 neonates, clinical examination showed distended abdomen and emesis, whereas plain abdominal radiographs showed intestinal obstruction. All neonates underwent surgery and all had normal intestinal rotation, except one with total intestinal volvulus secondary to malrotation. Other causes of volvulus were suspected in 4 neonates: mesenteric defect (n=1), intestinal atresia (n=2) and narrow mesentery (n=1). Detorsion of total volvulus, ileostomy, or intestinal resection with primary anastomosis was performed in 2, 5, and 3 neonates, respectively. One patient with total intestinal volvulus secondary to malrotation died, whereas all other neonates survived. In one patient, the postoperative course was complicated by intestinal dysmotility of the distal small bowel requiring a secondary jejunoileostomy. Stoma closure was subsequently performed at 1 year of age with good outcome. One patient developed angiocholitis treated successfully with antibiotics. Median time to initiate enteral feeds was 7 days (range, 4-16 days) and all patients were subsequently weaned from parenteral nutrition. Median duration of parenteral nutrition was 29 days (range, 6-667 days). None of the patients had cystic fibrosis. Unlike postnatal volvulus, most prenatal volvulus occurs without malrotation. Although prenatal volvulus is a life-threatening condition, our results suggest that good long-term outcome can be achieved in most cases.

[Tongue cyst in children: foregut duplication, a possible diagnosis]. / Kyste lingual de l'enfant : savoir évoquer la duplication digestive.

INTRODUCTION: Foregut duplication is a heterotopy of the digestive mucosa. The tongue localization is relatively uncommon, it presents as a cystic lesion. We report a series of five patients presenting with cysts of the tongue. PATIENTS AND METHODS: This retrospective study was made on cases of foregut duplication of the lingual area, diagnosed between 1977 and 2008. We documented the patient's age, gender, symptoms, lesion localization, clinical radiological and pathological features, treatment, and outcome. RESULTS: Four boys and one girl were included. Two cysts were diagnosed during antenatal screening, two during early infancy, and one at the age of 12 (after infectious complication). In every case, the diagnosis could be confirmed only after surgical removal. DISCUSSION: Foregut duplication of the tongue is rare and its incidence is probably underestimated. This diagnosis should be suggested in case of congenital intraoral cysts. Treatment is surgical excision, and pathological examination confirms the diagnosis.

[PELVIS or LUMBAR syndrome: the same entity. Two case reports]. / Du PELVIS au LUMBAR syndrome : à propos de 2 cas.

INTRODUCTION: The association of a pelvic hemangioma and malformations in the pelvic region are described as SACRAL or PELVIS syndrome. More recently, the acronym of LUMBAR syndrome has been used to describe the association of lower-body hemangioma and other cutaneous defects, urogenital anomalies, ulceration, myelopathy, bony deformities, anorectal malformations, arterial anomalies, and renal anomalies. We report herein 2 representative cases. PATIENTS: Two girls presented with infantile hemangioma associated with genital malformation. One case was associated with spinal anomalies and the other one with the anus in the genital position. CONCLUSION: Segmented hemangiomas are commonly associated with extracutaneous abnormalities. By analogy with PHACE syndrome, PELVIS/SACRAL/LUMBAR syndromes describe the association of segmented hemangioma of the lower extremities associated with other trunk or lower-body malformations. The syndrome is often incomplete.

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.