Anal canal duplication in children: a monocentric experience of 12 cases.

PURPOSE: Anal canal duplication is a rare malformation characterized by a second perineal opening positioned behind the anus, which is generally observed at 6 o'clock in the lithotomy position. The purpose was to describe six new cases of anal canal duplication (in addition to our previously reported series of six patients) with the aim of providing further clinical information about this anomaly. METHODS: We described 6 new cases of anal canal duplication in terms of symptoms, anatomical disposition, imaging results, and histopathology. Clinical details of these cases and those already reported (n = 12) were summarized and compared to existing literature. RESULTS: A total of 12 cases were reported over 34 years. 17% of the patients were male, constituting the only subgroup to present a communication with the digestive tract. A single patient, diagnosed at 12 years, was symptomatic. Half of the patients had at least one associated malformation. All patients underwent surgery, either with a posterior sagittal or perineal approach. CONCLUSION: Diagnosis of anal canal duplication should be suspected when a perineal opening positioned behind the anus is present, and necessitates further exploration by a comprehensive clinical examination and imaging. Surgery is always required, typically performed via a posterior sagittal approach. The postoperative course is usually uncomplicated.

Predictors of the Performance of Early Antireflux Surgery in Esophageal Atresia.

OBJECTIVE: To identify predictors of and factors associated with the performance of antireflux surgery during the first year of life in children born with esophageal atresia. STUDY DESIGN: All patients were included in a French registry for esophageal atresia. All 38 multidisciplinary French centers completed questionnaires about perinatal characteristics and one-year outcome for children born with esophageal atresia. RESULTS: Of 835 infants with esophageal atresia born in France from 2010 to 2014, 682 patients, excluding those with long-gap esophageal atresia, were included. Three patients had type I, 669 had type III, and 10 had type IV esophageal atresia. Fifty-three children (7.8%) received fundoplication during the first year of life. The median age at the time of the end-to-end esophageal anastomosis was 1.1 day (range 0-15). Multivariate analysis identified three perioperative factors that predicted the need for early antireflux surgery: anastomotic tension (P = .004), associated malformations (P = .019), and low birth weight (P = .018). Six other factors, measured during the first year of life, were associated with the need for antireflux surgery: gastroesophageal reflux (P < .001), anastomotic stricture (P < .001), gastrostomy (P < .001), acute life-threatening event (P = .002), respiratory complications (P = .045), and poor nutritional status (P < .001). CONCLUSIONS: Gastroesophageal reflux disease, low birth weight, poor nutrition, and surgical anastomosis difficulties predicted the performance of antireflux surgery in the first year of life in infants with esophageal atresia.

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.

Mixed epithelial and stromal renal tumour in a 12-year-old boy.

Mixed epithelial and stromal tumour of the kidney (MESTK) is a rare kidney neoplasm that occurs almost exclusively in perimenopausal women. Long-term oestrogen replacement appears to play a major role in its pathogenesis. Around 70 cases have been described in the international literature, none of which involve male children. Herein, we describe an atypical case of MESTK diagnosed in a 12-year-old prepubertal boy who presented with hematuria. Pathology and immunohistochemistry revealed a typical MESTK. The child was free of disease at 2-year follow up after a partial nephrectomy and tumour excision.

[Polyorchidism in child (a case report and review of literature)]. / Polyorchidie chez l'enfant (à propos d'un cas avec revue de la littérature).

Polyorchidism is an urogenital curiosity defined by the presence of more than two testes confirmed by histology. This anomaly is extremely rare and only a hundred world cases were described in the literature. Although it can remain asymptomatic, polyorchidism is often associated to processus vaginalis anomalies in childhood (hernia, hydrocele) and undescended testis. The review of the literature finds cases of polyorchidism revealed by testis torsion and an increased risk of malignancy and infertility. We report the case of polyorchidism in a 14 year old child, at whom a painful testis mass indicate surgical exploration and documented the histological diagnosis of polyorchidism. Through this observation and review of the literature, authors describe pathological and managment findings of polyorchidism.

Perforated tubular duplication of the transverse colon: a rare cause of meconium peritonitis with prenatal diagnosis.

The transverse colon is an exceptional location of intestinal duplication. Perforated duplications are rarely described in neonates. Meconium peritonitis (MP) can originate from prenatal perforated intestinal duplication. The authors report a case of a baby girl with prenatal diagnosis of MP. Rapid worsening of clinical aspects at birth and the presence of a pneumoperitoneum on systematic abdominal plain radiographs led to urgent surgery on the 1st day of life. Laparotomy showed a perforated necrotizing tubular duplication of the transverse colon. Removal of the duplication followed by limited segmental colonic resection and double colostomy were carried out. Follow-up was uneventful.

[Testicular Leydig cell hyperplasia in children]. / Hyperplasie à cellules de Leydig du testicule chez l'enfant.

INTRODUCTION: Leydig cell hyperplasia (LCH) of the testis is rarely described in children. The authors report a case of incidental discovery of LCH on ultrasound examination. CASE REPORT: The authors report the case of a 9-year-old boy presenting with isolated and painless increased volume of the left testis, with no clinically palpable mass. Scrotal ultrasound revealed an echogenic mass, 12 mm in diameter, with a solid appearance and several hypoechoic areas, without calcification, situated in the lower pole of the testis. Tumour markers and gonadotropin axis hormonal assessment were normal. On surgical exploration, the testis had a macroscopically normal appearance; opening of the tunica albuginea revealed the lesion and enucleation was performed. Histological examination confirmed the presence of LCH, 5 mm in diameter. The postoperative course was uneventful. Physical examination and scrotal ultrasound have remained normal with a follow-up of two years. DISCUSSION: The authors recall the characteristics of LCH, which usually presents, in children, in the form of signs of precocious puberty or more rarely by gynaecomastia. The lesion is rarely palpable. Scrotal ultrasound reveals a homogeneous mass with several hypoechoic nodules. An endocrine assessment must always be performed (frequent elevation of LH). Surgery should be as conservative as possible (enucleation-resection). Histological diagnosis may be difficult.