Prolonged cord separation in a newborn: a rare case of an unusual epithelialised umbilical cord.

A female full-term neonate, accompanied by her parents, was referred to the paediatric surgery department on the day of after birth. She presented with a 9 cm length pathological umbilical cord, of which the first 7 cm was red and wet, with ulceration, necrosis and healing areas. The patient never had a fever. Abdominal palpation showed no umbilical hernia and abdominal Doppler ultrasound was normal. After several days of disinfection, by biseptine antiseptic solution, and a monthly follow-up, most of the umbilical cord fell out. It only remained a 4 cm length navel consisting of 2 cm of excessive skin and 2 cm of mucous tissue. The lesion was surgically excised at 6 months old. The patient was discharged on postoperative day 1. The results of the histology confirmed the diagnosis of an epithelialised umbilical cord. The 1-month follow-up was uneventful.

Seat belt syndrome with caecal perforation and Chance fracture in an adolescent.

Seat belt syndrome describes a combination of injuries a person restrained by a seat belt may have after a high-velocity motor vehicle crash. A seat belt-shaped bruise may be present across the torso. A Chance fracture is a horizontal vertebral fracture, resulting from spinal hyperflexion, and in disruption of the three columns of the vertebral bodies. Its association with intra-abdominal injury is rare in children. A male, in early adolescence, was involved in a high-energy car crash. A total-body CT scan highlighted lacerations of the anterolateral abdominal wall and an acute L4 Chance fracture. Another CT scan performed because of clinical deterioration, showed cecal perforation, caused by a mesenteric tear and non-displaced fracture of the left sacral ala. He underwent a right hemicolectomy with ileocolic anastomosis and protective ileostomy and was immobilised in a body jacket orthosis for 3 months. The ileostomy was closed 3 months later. At 2-year follow-up, he is well.

The Gigli saw, a useful tool for cutting a metal ring causing penile strangulation.

Penile strangulation is a rare pediatric urology emergency. A 13-year-old boy with no psychiatric history was referred to the university hospital 2 h after a metallic ring was self-placed over his penis. Under general anesthesia, a Gigli saw cut the cockring in two places. The patient was discharged home on postoperative day 1. He neither attended a consultation at the one-month follow-up nor saw a psychiatrist. Thirteen similar cases have been reported in children since 1975. We were the first team to use a Gigli saw in a child in our department.

Evaluation of continuous wound infusion with local analgesics in postoperative renal transplantation patients: A retrospective study.

Objectives: The objective is to evaluate the efficacy of the continuous wound infusion (CWI) with Ropivacaine (naropeine 2 mg/ml) on postoperative pain, analgesics consumption, and bowel function in renal transplantation patients. Materials and Methods: A retrospective study trial including 79 patients who underwent renal transplantation. Patients were separated into two groups (catheter or without catheter). We identified 52 (65.8%) patients who received catheter wound infusion during the first 48 h postoperatively. On the other hand, 27 (34.1%) patients received standard without catheter anesthetic technique. Catheter wound infusion was achieved through a 12 cm catheter, inserted subcutaneously after abdominal closure. The catheter was placed above the external oblique aponeurosis. All postoperative data were examined to evaluate the first postoperative 48 h. This study aims to assess three variables: postoperative pain analysis through a visual analog scale, analgesics consumption, and bowel function. Results: The overall score of the three variables was studied. Regarding pain assessment, we have determined that the group of patients with catheter scored better than patients without catheter with borderline significance (66.3 vs. 61.2 consecutively; P = 0.0843). An early bowel function was noted in patients with catheters on the 2nd postoperative day (P = 0.0209). Moreover, patients without catheter consumed more painkillers with nonsignificant difference (P = 0.2499). Conclusion: Patients with catheter showed earlier bowel function than the noncatheter group on the 2nd postoperative day. The catheter group had better pain evaluation.

Long-Term Functional Outcomes of an Anorectal Malformation French National Cohort.

OBJECTIVES: The present study aimed to assess long-term functional outcomes of children with anorectal malformations (ARMs) across a network of expert centers in France. METHODS: Retrospective cross-sectional study of patients ages 6-30 years that had been surgically treated for ARM. Patient and ARM characteristics (eg, level, surgical approach) and functional outcomes were assessed in the different age groups. RESULTS: Among 367 patients, there were 155 females (42.2%) and 212 males (57.8%), 188 (51.2%) cases with, and 179 (48.8%) higher forms without, perineal fistula. Univariate and multivariate statistical analyses with logistic regression showed correlation between the level of the rectal blind pouch and voluntary bowel movements (odds ratio [OR] = 1.84 [1.31-2.57], P < 0.001), or soiling (OR = 1.72 [1.31-2.25], P < 0.001), which was also associated with the inability to discriminate between stool and gas (OR = 2.45 [1.28-4.67], P = 0.007) and the presence of constipation (OR = 2.97 [1.74-5.08], P < 0.001). Risk factors for constipation were sacral abnormalities [OR = 2.26 [1.23-4.25], P = 0.01) and surgical procedures without an abdominal approach (OR = 2.98 [1.29-6.87], P = 0.01). Only the holding of voluntary bowel movements and soiling rates improved with age. CONCLUSION: This cross-sectional study confirms a strong association between anatomical status and functional outcomes in patients surgically treated for ARM. It specifically highlights the need for long-term follow-up of all patients to help them with supportive care.

Atypical phenotype of a patient with Bardet-Biedl syndrome type 4.

BACKGROUND: Bardet-Biedl syndrome (BBS) is a multisystemic disorder characterized by rod-cone dystrophy, truncal obesity, postaxial polydactyly, cognitive impairment, male hypogonadotropic hypogonadism, complex female genitourinary malformations, and renal abnormalities. There is a large clinical and also genetic heterogeneity in BBS. Here, we report a patient with polydactyly, hyperechogenic kidneys increased in size with normal corticomedullary differentiation, anal imperforation, and malformation of genitals with presence of a genital tubercle with ventral urethral meatus associated with two unfused lateral genital swelling and absent urethral folds, in the context of 46, XY karyotype. METHODS: Karyotype and solo exome sequencing were performed to look for a genetic etiology for the features described in our patient. RESULTS: We identified a homozygous in-frame deletion of exons 4 to 6 in the BBS4 gene (NM-033028 (BBS4-i001): c.[(157-?)_(405 +?)del] p.(Ala53-Trp135del), which is classified as pathogenic variant. This analysis allowed the molecular diagnosis of BBS type 4 in this patient. CONCLUSION: Complex genital malformations are only reported in female BBS6 patients yet, and genital abnormalities and anal imperforation are not reported in male BBS4 patients to date. We discuss the possible hypotheses for this phenotype, including the phenotypic overlap between ciliopathies.

Esophageal Atresia and Respiratory Morbidity.

BACKGROUND AND OBJECTIVES: Respiratory diseases are common in children with esophageal atresia (EA), leading to increased morbidity and mortality in the first year. The primary study objective was to identify the factors associated with readmissions for respiratory causes in the first year in EA children. METHODS: A population-based study. We included all children born between 2008 and 2016 with available data and analyzed factors at birth and 1 year follow-up. Factors with a P value <.10 in univariate analyses were retained in logistic regression models. RESULTS: Among 1460 patients born with EA, 97 (7%) were deceased before the age of 1 year, and follow-up data were available for 1287 patients, who constituted our study population. EAs were Ladd classification type III or IV in 89%, preterm birth was observed in 38%, and associated malformations were observed in 52%. Collectively, 61% were readmitted after initial discharge in the first year, 31% for a respiratory cause. Among these, respiratory infections occurred in 64%, and 35% received a respiratory treatment. In logistic regression models, factors associated with readmission for a respiratory cause were recurrence of tracheoesophageal fistula, aortopexy, antireflux surgery, and tube feeding; factors associated with respiratory treatment were male sex and laryngeal cleft. CONCLUSIONS: Respiratory morbidity in the first year after EA repair is frequent, accounting for >50% of readmissions. Identifying high risk groups of EA patients (ie, those with chronic aspiration, anomalies of the respiratory tract, and need for tube feeding) may guide follow-up strategies.

Testicular rupture after blunt scrotal trauma in children: A case report and literature review.

Blunt testicular trauma with rupture of albuginea is one of the rarest emergencies in children. Medical history and Testicular Doppler Ultrasound lead to diagnosis. Appropriate management is necessary to preserve the testis. Follow-up to adulthood is recommended to assess the impact on fertility.

Risk Factors of Early Mortality and Morbidity in Esophageal Atresia with Distal Tracheoesophageal Fistula: A Population-Based Cohort Study.

OBJECTIVE: To identify the risk factors for early mortality and morbidity in a population with distal esophageal atresia (EA)-tracheoesophageal fistula. STUDY DESIGN: Cohort study from a national register. Main outcomes and measures included early mortality, hospital length of stay (LoS), need for nutritional support at 1 year of age as a proxy measure of morbidity, and complications during the first year of life. RESULTS: In total, 1008 patients with a lower esophageal fistula were included from January 1, 2008, to December 31, 2014. The survival rate at 3 months was 94.9%. The cumulative hospital LoS was 31.0 (17.0-64.0) days. Multivariate analysis showed that intrahospital mortality at 3 months was associated with low birth weight (OR 0.52, 95% CI [0.38-0.72], P < .001), associated cardiac abnormalities (OR 6.09 [1.96-18.89], P = .002), and prenatal diagnosis (OR 2.96 [1.08-8.08], P = .034). LoS was associated with low birth weight (-0.225 ± 0.035, P < .001), associated malformations (0.082 ± 0.118, P < .001), surgical difficulties (0.270 ± 0.107, P < .001), and complications (0.535 ± 0.099, P < .001) during the first year of life. Predictive factors for dependency on nutrition support at 1 year of age were complications before 1 year (OR 3.28 [1.23-8.76], P < .02) and initial hospital LoS (OR 1.96 [1.15-3.33], P < .01). CONCLUSIONS: EA has a low rate of early mortality, but morbidity is high during the first year of life. Identifying factors associated with morbidity may help to improve neonatal care of this population.

Robot-assisted continent urinary diversion according to the Mitrofanoff principle: results of a bicentric study.

PURPOSE: To assess the outcomes of continent urinary diversion according to the Mitrofanoff principle by robot-assisted laparoscopic surgery in terms of continence, catheterization and repeat surgery. METHODS: All adult patients who underwent procedures for continent urinary diversion via a robot-assisted laparoscopic route between October 2014 and November 2018 were enrolled retrospectively. Abdominal continence and clean intermittent catheterization ability were noted, as well as patient characteristics, details of the technique, intra- and perioperative parameters, and complications. RESULTS: Ten patients were included. Bladder-sphincter disorders were due to neurological causes in nine patients, and one patient had idiopathic bladder sphincter dyssynergia. A continent catheterizable channel was created using the appendix in six cases and the ileum in four cases. The median operative duration was 245 min (IQR 228-370). Two patients had a Clavien 3 complication requiring laparoscopy to separately drain a haematoma and a pelvic abscess. The median follow-up was 21 months (IQR 17-27). Abdominal continence without further intervention was obtained in 6/10 patients (60%). Two patients needed an open revision of the continent urinary diversion, one required injection of Deflux® and one an intradetrusorian injection of botulinum toxin type A (Botox®). Further surgery was needed for two patients to improve urethral continence. At the end of follow-up, all patients were continent and clean intermittent catheterization was possible. CONCLUSION: Continent urinary diversion performed according to the Mitrofanoff principle is possible via a robot-assisted laparoscopic route and yields good outcomes on abdominal continence in the short term in patients with clean intermittent catheterization ability.

[Parental representations of children's acute appendicitis and impact on its surgical management in three departments]. / Représentations parentales de l'appendicite de l'enfant et leurs conséquences sur le traitement chirurgical.

INTRODUCTION: Appendicitis is well known by the general and medical population. Yet, the number of appendectomies varies by a factor of one to four depending on French departments.Purpose of research: This study aimed to compare, according to French schooling departments, the representations and attitudes of parents facing abdominal pain of their children, and their use of health care system. We selected three departments which have different proportions of use of appendectomy according to the DRESS's rapport of 2014. Meurthe-et-Moselle's rank is between 15.65 and 23.48/10 000 people, Seine-et-Marne's rank is between 23.48 and 31.30/10 000, and Jura's rank is superior to 31/10 000. RESULTS: 797 questionnaires were analyzed (45.2%). Significant differences exist in the answers of the three departments. Residents of the Jura region and, in a lesser measure, Seine-et-Marnaises answered differently than Meurthe-et-Mosellanses. The first two were the populations who experienced the most appendectomies and the Meurthe-et-Mosellanses the least. Parents who lived in departments of high rank of appendectomy thought more of acute appendicitis when confronted with their children's abdominal pain, and feared less of appendectomy. They were also more in favor of surgical treatment than parents from Meurthe-et-Moselle. CONCLUSION: Knowledge, attitude and use of health care system seem to influence management of acute appendicitis in children's population and can explain, at least partially, the variations of the appendectomy rate in France. Targeted information for physicians may improve the understanding of patients and reassure them if needed in the purpose of reducing this factor of influence of practices.

Co-existing infantile hepatic hemangioma and mesenchymal hamartoma in a neonate.

Infantile hepatic hemangioma (IHH) and mesenchymal hamartoma (MH) form the first and second most common benign hepatic tumors in children. In this case report, we present a newborn child in whom a growing hepatic mass was discovered at the age of 7 days. She suffered also from anemia, respiratory and renal failure. No signs of heart disease or cutaneous lesions were detected. Alpha-fetoprotein was elevated for her age (3562.2 ng/ml). Imaging studies showed multifocal large cystic lesions associated with heterogeneous small solid lesions with arterial enhancement of the liver. Non-anatomical liver resection was performed initially and synchronous MH and IHH GLUT-1 positive were discovered in the pathological analysis. Segmental IV hepatic resection and later on selective cysts excision were done for persistent hepatic lesion despite medical treatment. After 14 months of beta-blockers treatment, clinical follow-up shows a healthy child with no residual cystic lesions.

Results from the French National Esophageal Atresia register: one-year outcome.

BACKGROUND: The aim of the present national prospective population-based study was to assess the early morbidity of esophageal atresia (EA). METHODS: All 38 multidisciplinary French centers that care for patients with EA returned a specific questionnaire about the 1-year outcome for each patient. This information was centralized, checked, and entered into a database. RESULTS: From the total population of 307 EA patients born in 2008 and 2009, data about the 1-year outcome were obtained from 301 (98%) patients, of whom 4% were lost to follow-up and 5% died. Medical complications occurred in 34% of the patients: anastomotic leaks (8%), recurrent tracheoesophageal fistula (4%), and anastomotic stenosis (22%); all of the latter group needed dilation (median, 2 dilations/patient). A new hospitalization was required for 59% of patients (2.5 hospitalizations/patient) for digestive (52%) or respiratory (48%) reasons. Twelve percent of patients required antireflux surgery at a median age of 164 days (range, 33-398 days), and 1% underwent an aortopexy for severe tracheomalacia. The weight/age Z-score was -0.8 (range, -5.5 to 3.7 months) at 12 months. Fifteen percent of patients were undernourished at 12 months of age, whereas 37% presented with respiratory symptoms and 15% had dysphagia at the last follow-up. Significant independent factors associated with medical complications were anastomotic esophageal tension (p = .0009) and presence of a gastrostomy (p = .0002); exclusive oral feeding at discharge was associated with a decreased risk of complications (p = .007). CONCLUSIONS: Digestive and respiratory morbidities remain frequent during the first year of life and are associated with difficult anastomosis and lack of full oral feeding.

Esophageal atresia: data from a national cohort.

PURPOSE: A prospective national register was established in 2008 to record all new cases of live-birth newborns with esophageal atresia (EA). This epidemiological survey was recommended as part of a national rare diseases plan. METHODS: All 38 national centers treating EA participated by completing for each patient at first discharge a questionnaire validated by a national committee of experts. Data were centralized by the national reference center for esophageal anomalies. Quantitative and qualitative analyses were performed, with P-values of less than 0.05 considered statistically significant. Results of the 2008-2009 data collection are presented in this report. RESULTS: Three hundred seven new living cases of EA were recorded between January 1, 2008, and December 31, 2009. The male/female sex ratio was 1.3, and the live-birth prevalence of EA was 1.8 per 10,000 births. Major characteristics were comparable to those reported in the literature. Survival was 95%, and no correlation with caseload was noted. CONCLUSIONS: Epidemiologic surveys of congenital anomalies such as EA, which is a rare disease, provide valuable data for public health authorities and fulfill one important mission of reference centers. When compared with previous epidemiological data, this national population-based registry suggests that the incidence of EA remains stable.

[Nephrogenic adenoma of the bladder in children younger than 10 years: report of 5 cases]. / Adénome néphrogénique de la vessie chez l'enfant de moins de 10 ans: a propos de 5 cas.

INTRODUCTION: Nephrogenic adenoma (NA) of the bladder is a rare transitional cell tumour of the bladder in children under the age of 10 years. The objective of this study was to report our experience and review the data of the literature in order to define a coherent therapeutic approach. PATIENTS AND METHODS: We report 5 cases of NA diagnosed between 1995 and 2003 in 3 boys and 2 girls with a mean age of 6.38 years and a mean follow-up of 7.1 years. RESULTS: All patients presented a risk factor for bladder irritation. Initial symptoms were nonspecific and radiology contributed to the diagnosis in two cases. Treatment was performed by transurethral resection (3 cases) or cystotomy (2 cases). One recurrence was observed 8 years after the initial diagnosis, but no cases of malignant transformation were observed during follow-up. CONCLUSION: NA of the bladder is a benign tumour which does not predispose to malignancy. The initial treatment consisted of endoscopic resection combined with elimination of risk factors. Despite the frequency of recurrence, endoscopic surveillance does not appear to be justified. Urine cytology could contribute to the diagnosis of recurrence.

Colonic triplication associated with anorectal malformation: case presentation of a rare embryological disorder.

Tubular colonic triplication is an extremely rare hindgut malformation, with only 2 reports in the literature to date. The present authors describe the new and unusual case of a boy born with an imperforate anus, rectovesical fistula, and 3 distinct left colons. The bladder was divided by an incomplete septum. Prenatal ultrasound suggested colonic duplication. Surgical management involved resection of the triplicated segment and posterosagittal anorectal pull through.