Metanephric adenoma diagnosed on biopsy in an infant: a case report.

BACKGROUND: Metanephric adenoma is a rare benign renal tumor of the kidney, uncommonly observed in children. It is often misdiagnosed preoperatively as a malignant neoplasm, leading to an unnecessary nephrectomy. The challenge is to make the right diagnosis preoperatively and therefore manage it with conservative surgery. We report a case of a child with metanephric adenoma who underwent nephron-sparing surgery. CASE PRESENTATION: A renal tumor was discovered fortuitously in an 18-month-old Caucasian girl with several congenital malformations. Investigations showed a 28 × 27 × 27 mm left renal mass centrally located, well defined, nonvascularized, with no calcifications and which compressed the adjacent renal tissue. Furthermore, there were no signs of metastasis. The decision of a multidisciplinary meeting was to perform a computed tomography (CT)-scan-guided biopsy. Histologic examination concluded it was a metanephric adenoma. We performed a left open partial nephrectomy via a flank retroperitoneal incision. The final histopathological examination confirmed the diagnosis. The postoperative course was uneventful. CONCLUSION: Preoperative diagnosis of metanephric adenoma is challenging. Because of the high probability of unnecessary radical nephrectomy, preoperative biopsy can be safe and determining to guide a more conservative approach so nephron-sparing surgery can be performed.

Duodenal Atresia Associated with Apple Peel Atresia and Situs Inversus Abdominus: A Case Report.

Duodenal atresia is rarely associated with situs inversus abdominus. We report a case of duodenal atresia associated with small bowel atresia of apple peel type and situs inversus abdominus.

[Gastric volvulus in children: five case reports]. / À propos de 5 cas de volvulus gastrique chez l'enfant.

UNLABELLED: Gastric volvulus is an abnormal rotation of all or a part of the stomach around one of its axes. It is a rare cause of intestinal obstruction in children. This anomaly can be primary, due to abnormalities of the gastric ligaments, or secondary to other congenital malformations. We report on the cases of five children treated between January 1994 and December 2011, four boys and one girl, with a medium age of 7 months. Diagnosis was based on clinical features, particularly in the upper gastrointestinal Rx contrast study, which confirmed the diagnosis. Four out of the five children underwent laparoscopic surgery with fixation of the stomach. A diaphragmatic hernia was associated in one case. Antireflux surgery was performed in three cases, and a diaphragmatic defect was closed in one case. The follow-up was uneventful after a medium period of 7 years. CONCLUSION: good knowledge of this anomaly is the guarantee of early diagnosis and optimum treatment to ameliorate the prognosis.

[Cervical teratoma in a child]. / Tératome cervical chez l'enfant.

Teratomas are unusual tumors derived from all 3 germs cells layers: endoderm, mesoderm, and ectoderm, with varying proportions. The cervical area is exceptionally affected. We report 4 cases of cervical teratoma. The clinically and radiologically suggested diagnosis was confirmed by histology. We describe herein the main clinical, radiological, and histological aspects and outcomes of this disease. Despite its most often benign histologic nature, cervical teratoma may threaten newborn infants' life due to airway compression. A multidisciplinary approach to the disease starting at delivery is required to improve the prognosis.

[Isolated congenital tracheoesophageal fistula]. / Fistules oesotrachéales congénitales isolées.

INTRODUCTION: Isolated tracheoesophageal fistula without esophageal atresia is a rare congenital malformation. Its etiology is obscure. Diagnosis is difficult but must be made early. PURPOSE: To study the clinical, radiological, and evolutionary sights of this malformation. PATIENTS AND METHODS: We report 4 cases of tracheoesophageal fistula, collected in the department of pediatric surgery of Monastir Hospital and in the neonatology unit of Sousse Hospital during the period between January 2001 and December 2010. RESULTS: The clinical picture consisted in a coughing bout and cyanosis after each feeding. Thoracic and abdominal imaging showed aspiration pneumonia, atelectasis, and gas within the colon. Gastrointestinal opacification demonstrated the fistula in 2 cases. Tracheoscopy visualized the tracheoesophageal fistula in the other 2 cases. Treatment was surgical and consisted in the section-ligation of the tracheoesophageal fistula with pleural interposition in all cases. The course was simple in two cases with a 3-year and 3.5-year follow up, respectively, but the infants died in the other 2 cases. CONCLUSION: Although a rare malformation, tracheoesophageal fistula should be suggested as a diagnosis when respiratory symptoms occur during feeding starting during the neonatal period.

[Esophageal strictures in children with epidermolysis bullosa]. / Sténoses œsophagiennes de l'épidermolyse bulleuse.

UNLABELLED: Esophageal stricture is a rare but often severe complication of recessive dystrophic epidermolysis bullosa in children. The purpose of the study was to review this digestive complication with emphasis on diagnostic modalities and therapeutic management. PATIENTS AND METHODS: This was a retrospective study of two pediatric cases of esophageal stenosis that occurred during generalized recessive dystrophic epidermolysis bullosa of the Hallopeau-Siemens type. RESULTS: The 2 patients were aged 8 years 8 months and 11 years 5 months, respectively. Dysphagia was of early onset, before the age of 10 years in both cases. Esophageal opacification led to the diagnosis of esophageal stenosis located in the upper 1/3 of the esophagus in 1 case and at the junction between the middle and the lower 1/3 of the esophagus in the other case. None of the 2 patients received medical treatment, and pneumatic dilation was the treatment method that was advocated. Esophageal endoscopy showed the stenosis and helped guide the positioning of the balloon catheter. These patients underwent 2 and 3 sessions of dilation, respectively, at intervals of 2 months and 1 year. Balloon dilation has allowed the patients to have a more comfortable life with decreased dysphagia and a substantial improvement in nutritional status. However, this improvement was transient (1 patient had symptomatic recurrence of stenosis after 3 years), which shows that monitoring of the patients and the resumption of dilatation sessions may be necessary. CONCLUSION: Esophageal strictures in dystrophic epidermolysis bullosa of the Hallopeau-Simens type are severe and difficult to support. Pneumatic dilatation is the treatment of choice for the fragile esophagus. It gives satisfactory results and can be repeated without significant risk.

Congenital infantile fibrosarcoma of the forearm.

Congenital infantile fibrosarcoma is a rare soft tissue neoplasm in the infant of which only a few cases are reported as congenital. This tumor has a rapid growth and extensive local invasion, but metastasis rarely occurs. Distal extremities involvement is more common and metastasis are rare. We report a case of a congenital infantile fibrosarcoma of the forearm, which was initially confused with a hemangioma, treated successfully by surgical resection.

Spontaneous perforation of the extrahepatic bile duct in infancy: report of two cases and literature review.

BACKGROUND: Spontaneous perforation of the bile duct (SPBD) is a rare disease in infancy. The pathogenesis, diagnostic modalities and treatment options for this condition are reviewed and discussed. METHODS: The authors report 2 new observations of SPBD in 2 male newborns aged respectively 27 and 21 days. RESULTS: The 2 newborns presented with cholestatic jaundice. Abdominal sonography showed an extrahepatic mass. Exploratory laparotomy revealed that the perforation was located in the cystic duct in the first case and in the common bile duct (CBD) in the second. The site of perforation was repaired and both patients underwent simple external biliary drainage. The postoperative course was uneventful for both patients with follow-up ranging from 2 to 4 years. CONCLUSIONS: SPBD is a rare, but important cause of surgical jaundice in infants. The pathogenesis of SPBD is unknown and multifactorial and diagnosis is often problematic. Surgical management is always required and a conservative approach is usually recommended. The prognosis is good with early surgical management.

[Intussusception owing to pathologic lead points in children: report of 27 cases]. / Les causes inhabituelles d'invagination intestinale aiguë: à propos de 27 cas.

OBJECTIVE: Intussusception owing to pathologic lead points is a challenging condition for pediatric surgeons. The aim of this study was to review the particularities of clinical presentation, the place of morphologic investigation in depicting the presence of an organic lesion and the management of secondary intussusception. PATIENTS AND METHODS: The authors report a series of 27 patients treated from 1986 to 2004, for secondary intussusception. RESULTS: Nineteen boys and 8 girls, aged from 45 days to 11 years (mean age: 40 months) presented with secondary intussusception: Meckel's diverticulum (13 cases); lymphoma (8 cases); intestinal duplication (3 cases); heterotopic pancreas (2 cases); intestinal polyp (1 case). All patients were operated upon after failure of hydrostatic reduction. An intestinal resection with an end to end anastomosis was done for 26 patients. The biopsy of a large abdominal mass after an easy reduction of the intussusception was performed in 1 case. Chemotherapy was started at the sixth postoperative day for the 8 children having lymphoma. Two of them died during therapy. For the 25 others, the postoperative course was uneventful with a mean follow-up of 4 years. COMMENTARY: The improvement of the management and the prognosis of secondary intussusception requires an early diagnosis. Morphologic examination must not be limited to the diagnostic of intussusception but must aim at searching a lead point. The reduction of this particular form is based exclusively on surgery.

[Morgagni hernia in infancy: report of 7 cases]. / Les hernies rétrocostoxiphoïdiennes chez l'enfant: à propos de 7 cas.

BACKGROUND: Morgagni or retrosternal hernia is a rare entity. It's representing almost 4% of all types of congenital diaphragmatic hernias in children. POPULATION AND METHODS: The data concerning 7 infants with Morgagni hernias (MH) have been retrospectively reviewed by the authors. RESULTS: Their ages at presentation ranged from 7 months to 11 years. There were 4 males and 3 females. The majority of patients had repeated chest infections. The diagnosis was made on chest radiograph and barium enema. All patients were operated through the abdomen (5 upper midline, 2 laparoscopic approach). The hernia sacs were excised and the defects repaired in all patients. DISCUSSION: Clinical awareness, early diagnosis and surgical treatment especially with laparoscopic correction, are important factors. CONCLUSION: Surgical correction is needed for asymptomatic MH in children to obviate the risk of complications and because the treatment is easy.

[Congenital esophageal stenosis due to tracheobronchial remnants: report of 2 cases and literature review]. / Sténose congénitale de l'oesophage par hétérotopie trachéobronchique: à propos de 2 cas et revue de littérature.

Congenital esophageal stenosis due to tracheobronchial remnants is a rare malformation whose diagnosis may be difficult. It is characterised by the abnormal presence of congenital tissue of tracheal origin in the esophageal wall, which is responsible for the narrowing of the esophagus. We report 2 cases whose treatment was surgical after failure of esophageal dilations. The presence of tracheal-bronchial tissue was confirmed by histological examination of the operative piece. Outcome was favourable and the final result was excellent. Recently, endoscopic ultrasonography has been proved useful in the diagnosis of congenital esophageal stenosis due to tracheobronchial remnants by showing the presence of cartilage, which explains the failure of dilation. The high rate of perforation in these cases is due to brutal fragmentation of the cartilaginous rings. Surgical resection of esophageal stenosis with the tracheobronchial tissue appears the only treatment susceptible to completely suppress the stenosis and its consequences.