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.

[Prenatal diagnosis of abdominal wall defects]. / Diagnostic anténatal des anomalies de la paroi abdominale antérieure.

Anterior abdominal wall defects (AAWD) correspond to a wide spectrum of congenital defects affecting 6.3/10,000 pregnancies. They have in common a closure defect of the anterior abdominal wall and can be fatal or expose the fetus and the neonate (NN) to many complications. This study was based on a retrospective study of 22 cases of AAWD collected between May 2009 and December 2014. Its purpose was to specify the importance of prenatal ultrasonography in the diagnosis and prognosis assessment of these defects. These 22 AAWDs consisted in 13 cases of omphalocele (including four cases of Beckwith-Wiedemann syndrome), four of gastroschisis, one of pentalogy of Cantrell, three of vesical exstrophy and one of cloacal exstrophy. Prenatal ultrasonography provided the diagnosis of 14 of these defects with a changing sensitivity with the gestational age varying from 17% in the first trimester to 71.4% and 77.8% in the second and third trimesters, respectively. The relevance of this examination was improved when performed by an imaging specialist. The prenatal diagnosis of these defects indicated an amniocentesis in eight cases, allowing the diagnosis of two cases of trisomy 18. It also motivated a therapeutic termination of the pregnancy (TTP) in ten cases. Prenatal ultrasonography allowed better prenatal follow-up and planning of the delivery of the continued pregnancies. It indicated an emergency C-section in only one case by showing intestinal complications of gastroschisis. Four NNs died (two cases of omphalocele and two of gastroschisis), three of which postoperatively and the prenatal diagnosis did not improve survival. Prenatal ultrasonographic diagnosis provided a precise morphological study determining the type of the AAWD, a complete malformation assessment, and the prognosis factors. This resulted in adequate multidisciplinary pre and postnatal care, including a rigorous ultrasound follow-up, a TTP in case of associated defects, and emergency delivery once the complications of poor diagnosis are detected.

La méatotomie moyenne fonctionnelle : facteurs influençant les résultats thérapeutiques

Objectifs :Analyser les résultats fonctionnels et anatomiques de la méatotomie moyenne fonctionnelle et dégager les facteurs qui les influencent.Population et méthodes : étude rétrospective ayant inclus tous les patients ayant eu une méatotomie moyenne fonctionnelle monocentrique de Janvier 2002 à Décembre 2014.Résultats :Cent six patients ont été inclus dans cette étude, répartis en 58 hommes et 48 femmes. L'âge moyen était de 34 ans. L'indication a été posée pour 83 cas de sinusites rhinogènes et 23 cas de sinusites odontogènes. Le taux global de bon résultat fonctionnel de 73,6%. Il était de 74,5% pour le résultat anatomique.L'analyse univariée a permis de retenir comme facteurs de mauvais résultat aussi bien anatomique que fonctionnel après MM, le caractère bilatéral de la sinusite (p=0,000), l'existence decomorbidités à savoir l'allergie nasosinusienne (p=0,045), l'hyperréactivité nasale (p=0,000) et l'hyposmie(p=0,011), un score de Lund Mackay (SLM) global élevé (p=0,00), un SLM de l'ethmoïde antérieur élevé (p=0,009) et un SLM COM global élevé (p=0,001). En analyse multivariée, on a identifié comme facteur prédictif de de bon résultat anatomique un SLM global < 5 (p=0,001)alors que l'étiologie rhinogène unilatérale (p=0,027), l'absence d'hyperréactivité nasale (p=0,009) et un délai d'évolution inférieur à 24 mois (p=0,019) sont des facteurs qui influencent positivement le résultat fonctionnel.Conclusion : L'indication de la MM fonctionnelle doit être posée après une évaluation exacte du terrain, un examen endoscopique et une analyse scanographique précise afin de garantir les meilleurs résultats thérapeutiques

[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.

Endoscopic endonasal management of congenital intranasal meningocele in a 2-month-old infant.

INTRODUCTION: Nasal meningocele, which is usually congenital, is a rare anomaly resulting from meningeal herniation into the nasal cavities through a bone defect in the skull base. CASE REPORT: An 8-day-old boy was referred with respiratory disturbance and nasal obstruction. Examination showed a cyst-like grayish swelling filling the right nasal cavity. CT scan showed opacity, of fluid-like density, filling the right nasal fossa, in contact with a small bony defect in the right cribriform plate. MRI ruled out herniated brain parenchyma and enabled diagnosis of meningocele. The patient was operated on at the age of 2 months through a transnasal endoscopic approach. Immediate postoperative course was favorable. MRI control at 8 months was normal. DISCUSSION: Modern imaging (CT scan and MRI) is of paramount importance in the preoperative evaluation of nasal meningocele. Endoscopic endonasal (EE) surgery is currently the treatment of choice. CONCLUSION: Steady progress in instrumentation, technique and skills will increase the feasibility of skull-base surgery using an endonasal approach in the pediatric population.

[Iliopsoas abscess: a rare complication of pyogenic sacroiliitis in a child]. / Abcès du muscle iliopsoas : complication rare de la sacro-iliite à pyogène chez l'enfant.

Pyogenic sacroiliitis is a rare entity in children. Diagnosis is often delayed because of its variable clinical presentation, low suspicion by the examining physician, and rare findings on radiographs. Delayed diagnosis, however, results in complications such as iliopsoas abscess. We report the case of a 12-year-old girl hospitalized with a 21-day history of fever, pain in the left iliac fossa, and flexion contracture of the hip. On examination, she had fever (38.9 degrees C), psoitis, localized tenderness at the left sacroiliac joint, and pain elicited by lateral compression of the pelvis. The abdominal examination was normal. The erythrocyte sedimentation rate was 130 mm in the first hour, C-reactive protein was 186 mg/l, and the white blood cell count was 18,400/mm(3), with 79% neutrophils. Urinalysis was normal. Blood cultures were negative. Radiographs of the pelvis showed irregular left sacroiliac borders. The CT scan provided the diagnosis of sacroiliitis complicated by an ilioapsoas abcsess. Treatment was based on antibiotic therapy associated with surgical drainage. Bacteriologic investigation revealed Staphylococcus aureus. The patient's temperature returned to normal on the second day. Antibiotics were continued for 3 months, leading to full recovery.

[Isolated hypophyseal tuberculoma: often mistaken]. / Tuberculome hypophysaire isolé: un diagnostic souvent oublié.

Hypophyseal tuberculoma is extremely rare and difficult to diagnose without a clearly suggestive context. Its radiologic features are not specific and are better recognized on MRI with a mass of a variable signal related to the percentage of caseous necrosis. A frequently associated thickening of the pituitary stalk suggests tuberculosis, requiring the search for another tuberculosis location. This paper reports an original case of hypophyseal tuberculoma since it occurred with no other tubercular infection, which delayed the diagnosis despite thickening of the pituitary stalk on MRI.

[Intralingual dermoid cyst: imaging features of a giant cyst]. / Kyste dermoïde de la langue: aspects en imagerie d'un kyste géant.

Dermoid cysts of the oral cavity are rare and most commonly involve the floor of mouth. Intralingual dermoid cysts are extremely rare and usually large at the time of diagnosis in adult patients. We report the case of 6 year old girl with macroglossia and swallowing and respiratory difficulties due to a giant intralingual dermoid cyst. Ultrasonography showed a large cystic intralingual lesion. MRI allowed accurate evaluation of the size, the extension and the relationship of the cystic mass with adjacent structures, the fat component of the cystic mass confirmed the dermoid origin. This case illustrates the diagnostic contribution of US and MRI in this disease.