Visualization of the focus in congenital hyperinsulinism by intraoperative sonography.

In surgery for focal congenital hyperinsulinism (CHI), the identification and complete resection of the focus without collateral damage is of utmost importance. In a pilot study we applied intra-abdominal high-frequency sonography during surgery for focal CHI in 2 infants. The focus could be identified, its relation to the pancreatic and common bile duct could be shown, and the typical octopus-like tentacles could be demonstrated.

[Comorbidity and psychosocial need in children and adolescents with anorectal malformations].

Anorectal malformations (ARM) are not externally visible and have an uncertain medical course. Only about half of the patients with ARM have satisfactory bowel functions. Studies of ARM have reported reduced quality of life and psychosocial problems in up to 73% of the patients.

Evaluation of [18F]fluoro-L-DOPA positron emission tomography-computed tomography for surgery in focal congenital hyperinsulinism.

Context: In congenital hyperinsulinism (CHI), the identification and precise localization of a focal lesion is essential for successful surgery.

Objective: Our objective was to evaluate the predictive value and accuracy of integrated [18F]fluoro-L-DOPA ([18F]FDOPA) positron emission tomography (PET)-computed tomography (CT) for the surgical therapy of CHI.

Design: This was an observational study.

Duplication and exstrophy of the bladder with clitoris and vagina duplex as well as uterus bicornis.

The variants of bladder exstrophy with duplication of the bladder are extremely rare and associated with further malformations of the genitourinary system. Initially, the clinical appearance is that of a classic exstrophy. In this case a second, regularly configured bladder with a normal supravesical urinary tract was found covered by an omphalocele like a cutaneous bag.

Isolated left-sided congenital diaphragmatic hernia: cardiac axis and displacement before fetal viability has no role in predicting postnatal outcome.

Objectives: The purpose of this retrospective study was to determine whether objective assessment of cardiac shifting on two-dimensional ultrasonography can predict postnatal outcome in fetuses with isolated left-sided congenital diaphragmatic hernia (CDH).

Materials And Methods: Still images at the level of the four-chamber view were obtained in 23 fetuses with left-sided CDH. A group of 12 fetuses (3 non-survivors and 9 survivors) were examined at two periods, between 20 and 30 weeks and between 31 and 40 weeks.

Rhabdomyomatous dysplasia of the newborn lung associated with multiple congenital malformations of the heart and great vessels.

Interstitial proliferation of striated muscle in the lung is extremely rare. Most cases are associated with other congenital malformations, such as lung sequestration, diaphragmatic hernia, or cardiac malformations. We describe a newborn with rhabdomyomatous dysplasia of the lung associated with multiple congenital malformations of the heart and great vessels.

Myoid differentiation in mesoblastic nephroma: clinicopathologic and cytogenetic findings of a rare case.

The authors report the case of a benign renal mesenchymal tumor in a baby boy detected by ultrasound scanning during prenatal diagnosis. Histologically, the tumor was diagnosed as a congenital mesoblastic nephroma (CMN) with myoid differentiation. The tumor normally is characterized by a fascicular proliferation of bland, spindle-shaped cells.