Primary hypospadias repair outcomes: results from a retrospective cohort of 292 children.

PURPOSE: Hypospadias surgery is challenging. Numerous techniques have been described with variable complication rates. The main objectives of our study were to evaluate the urethroplasty complication rate of primary hypospadias repair and to identify risk factors for complications. METHODS: We retrospectively evaluated the outcomes of all patients who underwent primary hypospadias repair in our department between January 2012 and December 2020. Demographic, anatomical, operative, and postoperative data were reported. Univariate and multivariate logistic regression analyses were performed to identify variables associated with complications. Statistical significance was set at p < 0.05. RESULTS: Of 292 patients, 203 (69.5%) had distal hypospadias, 63 (21.6%) had mid-penile hypospadias, and 26 (8.9%) had proximal hypospadias. The mean age at the time of surgery was 22.8 months. Seventy-eight patients (26.7%) had urethroplasty complications. The rate of complications was higher for proximal hypospadias (57.7%), onlay island flap (44.4%), and Koyanagi (75%) procedures. The mean follow-up duration was 29 months. Two predictors of complications were identified by multivariate analysis: catheterization duration and urethroplasty technique. The reoperation rate was 30.5%. CONCLUSION: Urethroplasty complications frequently occur after primary hypospadias repair. The complication rate was higher in proximal hypospadias. Catheterization duration and surgical technique were significant predictors of complications. A longer and more standardized follow-up is needed for a better assessment of hypospadias repair outcomes.

[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.

[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.

[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.

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.

[Enterocolitis as a complication of late-diagnosed Hirschsprung disease]. / Entérocolite compliquant une maladie de Hirschsprung diagnostiquée tardivement.

Enterocolitis is the most serious complication of Hirschsprung disease. Early management of these patients can decrease the severity of this complication. Hirschsprung disease is more common in patients with trisomy 21 than in the general population. Furthermore, the risk of developing enterocolitis is higher in this population. We report on an infant with trisomy 21 who developed enterocolitis as a complication of Hirschsprung disease that was diagnosed late when the infant presented with septic shock leading to death of the patient.

[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.

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.

Management of Wilms tumors in Drash and Frasier syndromes.

BACKGROUND: Children with WT1 gene-related disorders such as Denys-Drash syndrome (DDS) and Frasier syndrome (FS) are at increased risk of Wilms tumor and end-stage renal disease. We investigated whether Wilms tumors in these patients displayed a specific phenotype or behavior and whether nephron-sparing surgery was beneficial. PROCEDURE: We retrospectively studied all patients with DDS, FS, or other WT1 mutations treated at our institutions between 1980 and 2007. RESULTS: We identified 20 patients, of whom 18 had benign or malignant tumors. Wilms tumors occurred in 15 patients, being unilateral in 10 and bilateral in 5 (20 tumors). Median age at Wilms tumor diagnosis was 9 months. No patients had metastases. According to the International Society of Pediatric Oncology Working Classification, there were 19 intermediate-risk tumors and one high-risk tumor; no tumor was anaplastic. In patients with nephropathy who underwent unilateral nephrectomy for Wilms tumor or nephron-sparing surgery for bilateral Wilms tumor, mean time to dialysis was 11 or 9 months, respectively. Other tumors included three gonadoblastomas (in two patients), one retroperitoneal soft-tissue tumor, and one transitional cell papilloma of the bladder. Two patients, both with stage I Wilms tumor, died from end-stage renal disease-related complications. The median follow-up time for the 18 survivors was 136 months (range, 17-224 months). CONCLUSION: Most Wilms tumors in children with WT1-related disorders were early-stage and intermediate-risk tumors, with a young age at diagnosis. In patients without end-stage renal disease, nephron-sparing surgery should be considered for delaying the onset of renal failure.

[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.

Esophageal replacement in children by an isoperistaltic gastric tube: a 12-year experience.

The colonic segment is the most frequently used material for replacing the esophagus in children; however, the use of a gastric tube has become a reliable alternative operation. Since 1987, we have used an isoperistaltic gastric tube to replace the esophagus in children, and we present a series of 21 patients. Indications for operation included caustic injury (nine), esophageal atresia (eight), peptic stricture (two), congenital stricture (one), and esophageal duplication (one). There was no death or necrosis of the graft during the early postoperative period. The esophagogastric anastomosis leaked in two cases, but both of them closed spontaneously. A temporary dumping syndrome was encountered in two children. Two patients had strictures of their upper anastomosis responding to dilatations. The two patients who had a pharyngogastric anastomosis developed either intractable stricture or nonfunctioning anastomosis. One of them died 9 months later from aspiration pneumonitis. At follow-up, 16 of 21 patients could accept a normal diet (13 were entirely asymptomatic, and three suffered occasional mild dysphagia). Two patients suffered significant dysphagia (one had a durable dilation of his gastric tube), and three needed a feeding jejunostomy. Acid secretion of the gastric tube was proved in nine cases. Two patients were shown to have cervical Barrett's esophagus above the anastomosis. These findings indicate the need for lifelong endoscopic follow-up for these patients.

[Postnatal management of urinary tract anomalies after antenatal diagnosis]. / Prise en charge post-natale des uropathies de découverte anténatale.

PURPOSE: Antenatal diagnosis of urinary tract anomalies raises the question of appropriate therapeutic management. Two risks should be avoided: excessive treatment, belated treatment. Postnatal management of these infants should be determined before birth. The aim of this study is to define indications more precisely. MATERIALS AND METHODS: A comprehensive review of the literature on postnatal treatment of antenatally diagnosed urinary tract anomalies was performed in order to propose a model of immediate management. RESULTS: Neonates with hydronephrosis should be placed on antibiotic prophylaxis. Postnatal sonographic exploration should be done immediately after birth in case of bilateral pathology, and if renal function is compromised: posterior urethral valves, bilateral dilatation with parenchymal thinning; exploration is done at day 10 in other cases, when diuresis is normalized. Voiding cystourethrogram is performed soon after birth if posterior urethral valves are suspected, later in other cases. In cases of intermittent renal pelvic dilatation, often an indirect sign of reflux, the need for a voiding cystogram is controversial. Diuretic renogram with mercaptoacetyl triglycine (MAG 3) is used to access renal function and efficiency of kidney drainage, both are needed for later monitoring. Surgical treatment is indicated in the event of deteriorated renal function with permanent obstruction. The most common uropathies are ureteropelvic junction obstruction, vesicoureteral reflux, posterior urethral valves, and primary obstructive megaureter. CONCLUSION: Antenatal diagnosis of urinary tract anomalies allowed immediate prophylactic treatment of urinary infections, and decrease the risk of severe complications previously observed. Long-term beneficial effects on preserving the renal parenchyma are yet to be determined.

Vascular ring abnormalities: a retrospective study of 62 cases.

BACKGROUND: The authors analyzed clinical signs of vascular ring anomalies together with appropriate complementary examinations and factors predictive of outcome after surgical treatment. METHODS: The authors reviewed the files of 62 patients with vascular ring abnormalities treated at Necker-Enfants Malades Hospital between January 1990 and January 2000, to analyze age at symptom onset, results of paraclinical examinations, the type of vascular ring abnormality, the surgical indications and type of surgery, and postoperative outcome. Outcomes were divided into 3 categories: cure, partial improvement, and no improvement. The chi2 test corrected with Fischer's Exact test was used for statistical analysis. RESULTS: Vascular ring abnormalities were diagnosed at birth in 28% of cases and during the first year of life in 68%. Sixteen percent of patients had associated abnormalities. Recurrent pulmonary and bronchial infections occurred after one year of age. An esophagogram was done in 76% of cases and showed impression images. Endoscopy was done in 63% of cases and showed malacia in 41% of patients and stenosis in 51%. Fifty-eight children were treated surgically. The average hospital stay was 7.4 days. The average follow-up was 37.4 months (12 to 159 months). Complete improvement was obtained in 68% of cases, partial improvement in 17%, and no improvement in 15%. Preoperative malacia was more frequent in patients with partial improvement or no improvement. CONCLUSIONS: Surgical treatment of congenital vascular ring abnormalities is effective and safe. Complications are rare. Prognostic factors include the quality of preoperative preparation of respiratory function, the type of abnormality, and the degree of malacia.

Surgical management and genotype/phenotype correlations in WT1 gene-related diseases (Drash, Frasier syndromes).

BACKGROUND/PURPOSE: The WT1 gene plays a role in urogenital and gonadal development. Germline mutations of this gene have been observed in patients with Drash or Frasier syndrome (Sd). The purpose of this report is to compare phenotype and genotype of these patients. METHODS: Retrospective study of 12 patients treated since 1980 for WT1 gene-related disorders was conducted. RESULTS: End-stage renal disease (ESRD) occurred in 9 patients, mostly because of diffuse mesangial sclerosis (DMS) or focal and segmental glomerular sclerosis (FSGS). Seven patients underwent kidney transplantation, and 2 died. Eleven tumors occurred: 8 Wilms' tumors, one soft tissue tumor, one bladder papilloma, and one gonadoblastoma. Wilms' tumors occurred at a younger age than expected. Eight patients had a 46,XY karyotype. One of these XY patients had female phenotype (Frasier syndrome); she was raised as a girl with bilateral gonadectomy. Seven XY patients had ambiguous phenotype; 4 have been raised as boys and 3 as girls. Four patients had a 46,XX karyotype; they had female genitalia and were raised as girls. WT1 gene analysis was performed in 10 patients and showed heterozygous germline mutations in exon 9 (n = 6), intron 9 (n = 1), exon 3 (n = 1), exon 4 (n = 1), or exon 7 (n = 1). CONCLUSIONS: ESRD was secondary to DMS when exon 9 was mutated, and secondary to FSGS when intron 9 was mutated. When exon 3, 4, and 7 were mutated, no nephropathy has been observed. Wilms' tumors occurred with any kind of WT1 mutation except intron 9. Abnormal sexual differentiation has been observed in all XY patients with WT1 mutation, and the most profound inversion of phenotype was observed with mutation in intron 9. Correlation between phenotype and genotype provides better understanding of the role of WT1, and can help the surgeon in the management of these patients.

Successful intrauterine shunting of a sacrococcygeal teratoma (SCT) causing fetal bladder obstruction.

We report a case of a sacrococcygeal teratoma (SCT) diagnosed at 22 weeks with a substantial intrapelvic cystic extension leading to bladder outlet obstruction and hydronephrosis at 27 weeks. Prenatal percutaneous shunting of the cystic teratoma was performed at 28 weeks to avoid prolonged fetal pelvic compression by the tumour that could have adverse effects by stretching the pelvic plexus and sacral nerves. Urinary dilatation resolved completely after shunting and a 3880 g baby girl was delivered at 39 weeks. The potential benefits of in utero shunting to avoid urological complications of SCTs with intrapelvic extension are discussed.