Value of ultrasonographic detection of normal ovarian tissue in the differential diagnosis of adnexal masses in pediatric patients.

Objective: The aim of the study was to evaluate whether the presence of normal ovarian tissue, the 'ovarian crescent sign' (OCS), could assist in the differential diagnosis between benign and malignant adnexal masses in young patients.

Methods: This was a prospective observational study, from March 2004 to January 2006, which included 98 patients younger than 20 years of age (including two with bilateral tumors) with an ultrasonographic diagnosis of an adnexal mass. Morphological assessment using the Ueland index was performed in each case.

A complex rearrangement involving cryptic deletion of ETV6 and CDKN1B genes in a case of childhood acute lymphoblastic leukemia.

We report on a case of childhood B-cell lineage acute lymphoblastic leukemia (ALL). Conventional cytogenetic analysis at diagnosis showed the karyotype: 47,XY,add(3)(q?),-12,+2mar[4]/46,XY[18]. Fluorescence in situ hybridization (FISH) revealed a complex rearrangement: 47,XY,der(3)(3pter->3q29::12q13->12q24.

True duplicate bladder exstrophy: a rare lesion.

A two-day-old newborn male child with a rare variant of exstrophy bladder was managed in our institute. The child has true duplicate bladder exstrophy which is extremely rare and only 8 cases reported in the world literature so far. We describe another one and briefly review the literature.

Ectopia cordis associated with Cantrell's pentalogy.

Cantrell's pentalogy with ectopia cordis is an extremely rare and lethal congenital anomaly, with a reported incidence of 1:100,000 births in developed countries. We report a neonate who presented with ectopia cordis along with cleft lower sternum, upper abdominal wall defect, ectopic umbilicus, diaphragmatic defect, and interventricular septal defect. The neonate had respiratory distress with peripheral cyanosis and died because of acidosis and electrolyte imbalance before surgical intervention could be undertaken.

Congenital bronchopulmonary malformations: a single-center experience and a review of literature.

Purpose: To present a single-center experience with 25 cases of bronchopulmonary malformations and the review the literature.

Materials And Methods: We conducted a retrospective analysis of the medical records of patients with congenital bronchopulmonary malformations who were operated between July 1997 and July 2007 in our institute; we examined the modes of presentations, management, and outcome. Outcome of all patients was assessed over a short follow-up period (average 1.

Unusual variant of Cantrell's pentalogy?

A 12-hour-old male infant presented with prolapsed abdominal content through a defect on left side of chest wall with respiratory distress. A thorough clinical examination suggested absence of ectopia cordis, abdominal wall defect, and any bony anomaly. The child expired after 6 hours of admission because of respiratory distress and electrolyte imbalance.

Recombinase-activating gene 1 immunodeficiency: different immunological phenotypes in three siblings.

Unlabelled: We report different immunological phenotypes in three siblings from consanguineous family with recombinase-activating gene 1 (RAG1) gene mutations. Null mutations of RAG genes result in severe combined immunodeficiency (SCID) with absent T and B cells. Hypomorphic mutations with retained activity of RAG genes may lead to a 'leaky' SCID with some features of Omenn syndrome (OS) or typical OS.

Torsion of malignant ovarian tumors in childhood and adolescence.

Aim: To investigate the frequency of torsion of malignant ovarian tumors in children and adolescents.

Methods: We evaluated all patients treated for adnexal torsion in a tertiary care referral pediatric institution during the last 20 years. Presentation, tumor markers and pathology reports were evaluated.

Successful treatment of hodgkin lymphoma in nijmegen breakage syndrome.

Nijmegen breakage syndrome (NBS) is a rare DNA repair disorder characterized by microcephaly, immunodeficiency, and predisposition to malignancy. We report on a 5-year-old patient with NBS who presented with nodular sclerosing type of Hodgkin disease stage IVB. Chemotherapy consisting of COPP/ABV regimen with reduction at 75% of full doses was employed.

Single-stage repair of vestibular and perineal fistulae without colostomy.

Purpose: This retrospective study was undertaken to evaluate the feasibility of primary anorectoplasty without a covering colostomy using the anterior sagittal anorectoplasty (ASARP) or posterior sagittal anorectoplasty (PSARP) technique in patients having vestibular and perineal fistulae, its complications, results, and remote outcome in our institute.

Methods: From January 2000 to June 2007, patients with vestibular and perineal fistulae subjected to single-stage surgical correction at our institute were reviewed retrospectively from the data available in hospital records and follow-up complaints of patients and their parents in the outpatient department. Patients who had undergone a staged repair were excluded from the study.

Neonatal oral tumors: congenital epulis and epignathus.

Oroparyngeal tumors in neonates protruding from the mouth are extremely rare. They have the potential to cause respiratory distress, or even death of the child during the perinatal period owing to airway obstruction and may need a multidisciplinary team approach at the time of birth. Congenital epulis and epignathus are the 2 rare lesions usually presenting like this and clinical manifestations depend on their position and size.

Right lung agenesis.

Congenital pulmonary agenesis or aplasia is extremely rare. Although more than fifty percent of patients die before first five years of age, some individuals may remain asymptomatic throughout their life. A three-month-old female child with right pulmonary agenesis presented to us with severe respiratory distress.

Supernumerary testis: a case report and review of literature.

Supernumerary testis or polyorchidism is a rare anatomical and congenital anomaly, and approximately 100 cases have been reported in the literature so far. It is often associated with processus vaginalis anomalies and with increased risk of malignancy and infertility. We report a case and its management, having left-sided duplex testis and found incidentally during inguinal hernia repair, and review the literature.

Congenital urethral hypoplasia with urethral fistula without imperforate anus: report of two cases.

Two male children with ano/recto-urethral fistula without anorectal malformation were managed in our institute. These patients had variable clinical manifestations and associated anomalies including congenital urethral hypoplasia, renal agenesis and patent urachus. Both were treated successfully according to their status of disease-complexes.

Isolated tubercular liver abscess in children treated with percutaneous isoniazid infusion.

Isolated Tubercular liver abscess is mainly reported in adult patients. We report two cases of isolated tubercular liver abscess in paediatric patients. Diagnosis was made by clinical and ultrasound guided aspiration of pus showing acid fast bacilli in one case.

Bilateral eventration of the diaphragm with malrotation and unilateral renal agenesis.

Bilateral eventration of the diaphragm associated with malrotation of gut and unilateral renal agenesis is a very rare entity. The authors treated a seven mth old male child who presented to them with respiratory distress and gastrointestinal problem. The child was anemic and was in type II respiratory failure.

Minimal invasive treatment of cervical rhabdomyosarcoma in an adolescent girl.

Rhabdomyosarcomas are the most common soft tissue sarcomas in childhood and adolescence. In cases of minimal cervical invasion, less invasive local excision in combination with adjuvant chemotherapy has resulted in excellent survival rates with complete preservation of the bladder, rectum, uterus and vagina. Herein we present the case of an adolescent girl with a large rhabdomyosarcoma of the uterine cervix treated with minimal invasive therapy.

Upper lung lobe systemic artery-pulmonary vein fistula with signs and symptoms of congestive heart failure: successful treatment with coil embolization.

Systemic artery-pulmonary vein fistula with a pulmonary left-left shunt is extremely rare. In this report, we present the case of a 3-month-old infant with enlarged right upper lobe systemic artery-pulmonary vein fistula and signs and symptoms of vascular tracheal compression and congestive heart failure. Two major aberrant arteries with separate origins came from the descending thoracic aorta and entered the right upper pulmonary lobe where they branched into many vascular channels and promptly drained into the enlarged right upper pulmonary vein.

Epithelial ovarian tumors and CA125 in premenarchal girls.

Purpose: This is a review of our 18-year experience with premenarchal girls with epithelial ovarian tumors. Special attention was focused on the predictive value of CA125 serum levels.

Methods: Analysis of premenarchal patients with resected or biopsied ovarian masses from 1988 to 2005 was performed.

Age-related role of ambulatory electrocardiographic monitoring in risk stratification of patients with complete congenital atrioventricular block.

Aims: The aim of the paper was to assess the importance of 24 h electrocardiographic Holter monitoring in determining predictive factors for Adams-Stokes (AS) attacks and heart failure (HF) in children and adolescents with complete congenital atrioventricular block (CCAVB).

Methods And Results: Forty-five patients were divided into two groups according to the presence of AS attacks and HF and six age-related subgroups. The following parameters of 24 h electrocardiographic Holter monitoring were analysed: (i) minimum heart rate (HR), (ii) maximum HR, (iii) average HR, (iv) daytime HR (v) rhythm and conduction disturbance.

Pre-operative differentiation of pediatric ovarian tumors: morphological scoring system and tumor markers.

Objective: To investigate the importance of morphological scoring systems in differentiation of ovarian tumors in childhood.

Methods: Morphological assessment using DePriest's index was performed for all patients with histopathological confirmation of ovarian tumor, with evaluation of tumor markers, from January 1997.

Results: Fifty-three girls (age range 13 months to 19 years) were surgically treated for 59 ovarian tumors, including six bilateral.

Echocardiographic analysis of the subtypes of right ventricular restrictive physiology in surgically treated patients with tetralogy of Fallot.

Background: Our study focuses on echocardiographic assessment of the right ventricular diastolic function and adaptive right ventricular response to volume overload resulting from pulmonary and tricuspid regurgitation in surgically treated patients with tetralogy of Fallot.

Method And Results: We included 60 patients subsequent to surgical correction of tetralogy of Fallot, dividing them into two groups - a group of 18 patients with restrictive physiology, having antegrade flow to the pulmonary arteries greater than 30 centimetres per second in late diastole in five consecutive beats, and a group of 42 patients with non-restrictive physiology. Based on the cardiothoracic ratio, being more or equal to, or less than 0.

Acute mastoiditis in infants.

We present a retrospective study of 37 infants who were operated for acute mastoiditis during the period 2000-2004 in Mother and Child Health Care Institute, Belgrade, Serbia and Montenegro. About 23 patients (62.2%) were male and 14 (37.

Fever of unknown origin in 185 paediatric patients: a single-centre experience.

Aim: We conducted a prospective study to evaluate the causes and outcome in children with fever of unknown origin (FUO).

Methods: From 1990 to 1999, 185 children with FUO were evaluated. Initial evaluation included routine haematological analysis, Epstein-Barr virus (EBV) serology, urine, stool or blood cultures, chest X-ray and tuberculin probe.

Burkitt lymphoma-induced ileocolic intussusception in Wiskott-Aldrich syndrome.

A 12-year-old patient with Wiskott-Aldrich syndrome (WAS) was referred because of recurrent abdominal pain and bloody stools. Ileocolic invagination was diagnosed and resection of the terminal ileum was performed. Pathologic examination identified submucosal tumor as the leading point of intussusception.