Intestinal duplication in the tongue: embryological and radiological point of view.

Intestinal duplication in the tongue is a rare entity. Occurrence in the anterior part of the tongue is exceptional. We report an intestinal duplication in the tongue causing eating difficulties and discuss the accuracy of embryologic and histopathology knowledge as radiology.

Hirschsprung's disease: the "Swiss roll" technique revisited.

Purpose: During pull-through for Hirschsprung's disease (HSCR), the assessment of innervation is mainly based on the presence of ganglion cells when conventional Hematoxylin and Eosin (HE) staining is used. In hypoganglionosis, the evaluation is difficult. We adapted a standardized methodology for the examination of resected bowel after HSCR surgery, using the technique described by Moolenbeek on rodent intestine and later by Meier-Ruge in children.

Sudden unexpected death in an infant with L-2-hydroxyglutaric aciduria.

Inherited metabolic disorders are the cause of a small but significant number of sudden unexpected deaths in infancy. We report a girl who suddenly died at 11 months of age, during an intercurrent illness. Autopsy showed spongiform lesions in the subcortical white matter, in the basal ganglia, and in the dentate nuclei.

Role of DNA content analysis and immunohistochemistry in the evaluation of the risk of unfavourable outcome in Wilms' tumours.

Background: Wilms' tumour (WT) is the most common solid tumour affecting young children. Its histological diversity leads to difficulties in predicting the outcome.

Materials And Methods: Image analysis cytometry and immunohistochemistry with a selected panel of antibodies were performed in 23 cases of WT considered of intermediate risk according to the revised International Society of Pediatric Oncology (SIOP) working classification of renal tumours of childhood.

Cervical nerve root synovial sarcoma in a child with chromosomal (X;18) translocation. Case report and review of the literature.

We report on an 11-year-old female with a history of cervicobrachialgia and progressive weakness of the right arm. Cervical spine MRI showed an enhancing heterogeneous intradural mass occupying the right C6-C7 foramen. She underwent a right C6-C7 foraminotomy with a complete macroscopic removal of the lesion.

Posterior quadrantic epilepsy surgery: technical variants, surgical anatomy, and case series.

Objective: Patients with intractable epilepsy due to extensive lesions involving the posterior quadrant (temporal, parietal, and occipital lobes) form a small subset of epilepsy surgery. This study was done with a view to analyze our experience with this group of patients and to define the changes in the surgical technique over the last 15 years. We also describe the microsurgical technique of the different surgical variants used, along with their functional neuroanatomy.

(Poly)acrylonitrile-based hydrogel as a therapeutic bulking agent in urology.

Vesicoureteral reflux and urinary incontinence can be treated by endoscopic injection. Injectables such as collagen and hyaluronic acid-dextranomer are commonly used, however are not offering optimal therapeutic effect yet. In the present study a novel application for poly(acrylonitrile)-based hydrogel (Hypan), already tested in other clinical indications, has been investigated for its aptitude as an injectable bulking agent.

Myxoid fibroblastic tumor of the maxillary sinus in a 21-month-old child: An unusual diagnosis.

Myxoid tumors of the soft tissue are a heterogeneous group of lesions with significant differences in behavior, from being totally harmless to malignant tumors with metastasizing potential. We describe the unusual case of a 21-month-old boy who developed a paranasal low-grade myxoid neoplasm with recurrent potential. The histological findings showed a proliferation of spindle cells within a vascular and myxoid background.

Fetus with two identical reciprocal translocations: description of a rare complication of consanguinity.

We report on a 24-week fetus with multiple organ anomalies secondary to biparental inheritance of an apparently balanced t(17;20) reciprocal translocation. The pregnancy was terminated following the discovery by ultrasound of an abnormal heart and micrognathia. At autopsy, the following anomalies were found: Pierre-Robin sequence, hypoplasia of the right ventricle with muscular hypertrophy, and endocardial fibroelastosis, hypoplastic lungs, dysplastic left kidney, bilateral pelvicalyceal dilatation, central nervous system periventricular heterotopias and right sided club foot.

Meningeal solitary fibrous tumour in a child.

Introduction: Meningeal solitary fibrous tumour is a relatively recent pathological entity that has rarely been described in children. With radiological techniques, it cannot be distinguished from meningiomas, and the diagnosis has to be confirmed histologically.

Case Report: We discuss the possible histogenesis of this tumour and the need for recognizing this lesion as a separate entity.

Association between fetal interleukin-1 receptor antagonist gene polymorphism and unexplained fetal death.

Objective: In spite of extensive clinical examinations or autopsies, as many as 15% to 40% of stillbirths remain unexplained. A systemic fetal inflammatory response is an independent risk factor for severe neonatal morbidity, mediated by proinflammatory cytokines. As a major anti-inflammatory cytokine, interleukin-1 receptor antagonist (IL-1ra) plays a crucial role modulating the proinflammatory response.

Non-functioning pituitary carcinoma.

Null-cell carcinomas of the pituitary are extremely rare. We describe a 41-year-old woman with a large adenohypophyseal neoplasm presenting as a primary nonfunctioning tumor without pituitary insufficiency. Signs of mass effect with progressive unilateral ocular motility disorders and anterior pituitary failure developed rapidly.

Surgical treatment of temporoparietooccipital cortical dysplasia in infants: report of two cases.

Purpose: Extensive multilobar cortical dysplasia in infants commonly is first seen with catastrophic epilepsy and poses a therapeutic challenge with respect to control of epilepsy, brain development, and psychosocial outcome. Experience with surgical treatment of these lesions is limited, often not very encouraging, and holds a higher operative risk when compared with that in older children and adults.

Methods: Two infants were evaluated for surgical control of catastrophic epilepsy present since birth, along with a significant psychomotor developmental delay.

Intraneural synovial sarcoma: two cases.

We report two cases of intraneural synovial sarcoma. The first patient is a 46-year-old female who presented for several months with soft-tissue mass in the right infra-auricular region. The second patient is a 11-year-old girl who fell and then presented with pain in the area innervated by the right C7 spinal root and a nodule identified in the nerve root foramina.

Disconnective hemispherectomy for hemispheric dysplasia.

Twelve patients suffering from intractable epilepsy and presenting with radiological evidence of diffuse hemispheric involvement of a dysplastic process, were treated by disconnective hemispherectomy, either functional hemispherectomy or peri-insular hemispherotomy. The median age at surgery was 4.5 years old and the interval between seizure onset and surgery, 3 years.

Lymphomatoid granulomatosis in a renal transplant patient.

Lymphomatoid granulomatosis is a rare angiocentric and angiodestructive pulmonary angiitis considered as a variant of the lymphoproliferative disorder group. Patients with organ transplantation are at an increased risk for post-transplant lymphoproliferative disorders secondary to their immunosuppression. However, lymphomatoid granulomatosis has rarely been described in patients with renal transplantation.