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.

Trends of juvenile and adolescent suicides in North Tunisia: a 12-year study.

OBJECTIVES: The aim of our study was to analyze the epidemiological characteristics for suicide methods and factors over a 12-year period among child suicides in Northern Tunisia and to compare juvenile and adolescent suicides. STUDY DESIGN: This is a cross-sectional study. METHODS: We included all child and adolescent suicide cases that took place in the North of Tunisia over a 12 year period (2005-2016). Data were collected from medical records and judicial inquiries and were classified into three sections: sociodemographic data, the circumstances of suicide, and the autopsy findings. Data were then compared between the 'juvenile suicide group' and 'the adolescent suicide group', according to the WHO definition. RESULTS: Casualties were equally males and females, mostly adolescents (74.5%), aged 15 years old on average. Hanging was the most frequent suicidal method. A peak of frequency was observed in 2014. CONCLUSION: Our results suggested to focus, among other preventive measures, on the role of media coverage of child suicides.

[Prenatal diagnosis and management of two cases of bilateral ureteroceles on simplex ureters]. / Diagnostic prénatal et prise en charge de deux cas d'urétérocèles bilatéraux sur uretères simplex.

Ureterocele is a rare urologic disorder characterized by pseudocystic dilatation of the terminal submucosal ureter. Most cases of ureteroceles are associated with complete ureteral duplicity and ureterohydronephrosis, whereas ureteroceles on simplex ureters are rarer. The authors report two cases of bilateral ureteroceles on simplex ureters diagnosed prenatally at 30 and 32weeks gestation. Fetal ultrasound had revealed bilateral ureterohydronephrosis. The delivery was made at term and renal function was normal at birth. Radiological and isotopic studies of the urinary tract confirmed the diagnosis of bilateral ureteroceles on simplex ureters that were obstructive in one case and not obstructive in the other case. Both cases had urinary antiseptic treatment and neither had urinary infection. Endoscopic puncture with electrocoagulation of ureteroceles was performed at 8 and 14months of age, respectively, with a simple postoperative course. Prenatal diagnosis of ureteroceles is essential to plan early multidisciplinary care to avoid long-term renal consequences.

[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.

[Emboligenous hydatid cyst of the right heart]. / Kyste hydatique emboligène du coeur droit.

The cardiac location of the echinococcosis is rare. It is associated with complications potentially severe. Indeed, the break inside the cardiac chambers with pulmonary embolism is the inevitable complication of the echinococcosis of the right heart. Between January 1992 and January 2006, five patients were operated in the department of cardiac surgery of Sousse (Tunisia) for an emboligenous hydatid cyst of the right heart. The average age is of 30 years with extremes from 18 to 65 years. The cardio-pulmonary bypass is the technique of choice. We regretted a single death in immediate postoperative period. All the patients were controlled with an average recession of 36 months. A single late death was noticed. No recurrence was observed.

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.

[Malignant renal tumors before one year of age. Experience of a North African pediatric surgery service]. / Tumeurs rénales malignes avant l'âge d'un an. Expérience d'un service de chirurgie pédiatrique nord Africain.

UNLABELLED: The malignant tumours of the kidney are not very frequent during the first year of life and pose diagnostic and therapeutic problems. The aim of this work is to make an analysis of the epidemiologic, clinical and anatomo-pathological characteristics of these tumours during the first year of life and a development on the therapeutic methods and their results. MATERIAL AND METHODS: [corrected] This is a retrospective study of 8 observations of malignant tumours of the kidney whose first symptomatology appeared during the first year of life. RESULTS: The malignant tumours of the kidney observed before the one year age constituted 18% of the tumours of the kidney in the child. A female prevalence was noted with a sex-ratio of 0.6. The assessment of extension found cutaneous (one case), ganglionic (one case) and pulmonary (two cases) metastases. First chemotherapy was prescribed to five patients. The surgery consisted on a widened nephrectomy in seven cases and a tumorectomy for an infant presenting a nephroblastomatosis. The tumour corresponded to a nephroblastoma in seven cases and a rhabdoide tumour in the last case. Mortality was high (50%) caused by the toxicity of chemotherapy in three cases and an advanced stage of cancer in one case. CONCLUSION: Renal tumoral pathology occurring in infants less than one year of age poses true etiologic and therapeutic problems. The high frequency of the nephroblastoma and the absence of benign tumour in our series encourage us to evocate more often the malignant renal tumours and to practice per cutaneous biopsies in case of diagnostic doubt.

[Urethral duplication in boys: report of 4 cases]. / La duplication urétrale chez le garçon: à propos de 4 cas.

Urethral duplication is a rare anomaly, affecting mainly boys. The clinical presentation varies because of the different anatomical patterns of this abnormality. We report 4 cases of urethral duplication in children. An epispadiac duplication was present in 1 case, hypospadiac duplication in 1 case, Y-duplication in 1 case and a congenital prepubic sinus in the last case. The pre-operative probe was based on urethrocystography and fistulography. The surgical management included excision of the duplicated urethra in 3 cases. Transurethral incision of the intra-urethral septum was performed in the case of hypospadiac duplication. The characteristics of male urethral duplication are reviewed.

[Fetal rhabomyomatous nephroblastoma. Report of 2 cases and review of the literature]. / Le néphroblastome rhabdomyomateux foetal. A propos de deux cas et revue de la littérature.

OBJECTIVE: Fetal rhabdomyomatous nephroblastoma is a particular and very rare histologic variety of nephroblastoma. The aim of this work is to study the principal clinic, therapeutic and evolutive characteristics of the fetal rhabdomyomatous nephroblastoma through two personal cases and a review of the literature. PATIENTS AND METHODS: This is a retrospective study of two observations of fetal rhabdomyomatous nephroblastoma treated in the pediatric surgery departement of Monastir (Tunisia) among 47 cases of nephroblastoma. The diagnosis was confirmed in the two cases by the histologic examination. RESULTS: The two patients were a six and a sixteen months old boy and girl. They were admitted for a voluminous mass occupying the left half-abdomen. The radiologic and biologic explorations load, in the two cases, to the diagnosis of left nephroblastoma. After a first chemotherapy that did not induce a reduction of the tumoral volume, a widened left nephrectomy was performed for the two patients. The histologic examination of the two pieces of nephrectomy concluded to a fetal rhabdomyomatous nephroblastoma with existence in the second case of an extension of the lesions to the renal pelvis and ureter in the form of a pseudo-botryoïde tumor. The tumor was classified stage I in the first case and stage II N0 in the second. The treatment was completed by an adapted post operative chemotherapy according to the SIOP 9 protocol. The two patients are currently in complete remission with an overview of six years and half. CONCLUSION: The fetal rhabdomyomatous nephroblastoma is a special histologic form of nephroblastoma that is characterized by the paucity of pulmonary metastasis, the absence of response to chemotherapy and the possibility of tumoral extension in the renal pelvis and ureter. His prognosis is similar to the classical nephroblastoma.

[Pneumatic dilatation of caustic esophageal stenosis in children. Report of five cases]. / Dilatation pneumatique des sténoses caustiques de l'oesophage chez l'enfant. A propos de cinq cas.

The ingestion of caustic substances is a frequent accident in the child. The treatment of caustic strictures of the esophagus, which constitute the most frightening complication, varies from conservative treatment to esophageal replacement. We report the preliminary results of a prospective work established since July 1998 and aimed at evaluating the role of pneumatic dilatation in the treatment of these injuries. STUDIED POPULATION: The series included five boys with an average age of six years. Two children were neurologically handicapped. RESULTS: The caustic product was soda (three cases), a dishwasher solution (one case) and bleach (one case). Dysphagia was constant and concerned the semi-fluids in four cases. The stricture was extended over an average length of 4 cm. The total number of dilatations was 36, with an average of seven sessions/patient. No complication was noted. The result was good in four cases. The only failure was noted in a child who presented a gastroesophageal reflux secondary to the scarring phenomena. CONCLUSION: Pneumatic dilatation constitute a therapeutic mean whose results are attested. The best treatment remains, however, preventive: it is the setting out of reach of the child the dangerous products.

[Esophageal duplication in children. Report of 7 cases]. / Duplication oesophagienne chez l'enfant. A propos de sept cas.

UNLABELLED: Esophageal duplications represent 10 to 20% of esophageal malformations and 15 to 20% of digestive duplications. METHODS: The authors report a series of seven cases observed between 1985 and 1999. RESULTS: The sex ratio was 2.5. The diagnosis was made before the age of six months in five cases. The clinical presentation included respiratory signs in six cases and digestive signs in five cases. The diagnosis was made on the association of a mediastinal mass behind vertebral malformation in five cases. The duplication was cystic in five cases and tubular in two. A perforation in the pleura occurred in one case of tubular duplication. The intervention consisted of total excision of the duplication in four cases and subtotal resection in two cases. For the seventh patient, who presented a perforation of the duplication in the pleura, the operation comprised a thoracic drainage associated to feeding gastrostomy. The histologic examination of the six operative specimens confirmed the diagnosis of esophageal duplication. Six patients survived and have favorable evolution with an average follow-up of four years. DISCUSSION: Esophageal duplications represent 15 to 20% of digestive duplications. Their clinical presentations are variable. Their diagnosis is often made before the age of two years, when a posterior mediastinal mass is associated with vertebral malformations. Apart from the tubular form, which is rare, diagnosis is confirmed by the histologic study of the operative specimen. CONCLUSION: Esophageal duplications are rare and often pose a problem of positive diagnosis. Their treatment is surgical and should be undertaken before the appearance of complications.