Infants affected by Hirschsprung disease (HSCR), a neurodevelopmental congenital disorder, lack ganglia of the intrinsic enteric nervous system (aganglionosis) in a variable length of the colon, and are prone to developing severe Hirschsprung-associated enterocolitis (HAEC). HSCR patients typically show abnormal dense innervation of extrinsic cholinergic nerve fibers throughout the aganglionic rectosigmoid. Cholinergic signaling has been reported to reduce inflammatory response.
View Article and Find Full Text PDFBackground & Aims: Hirschsprung's disease (HSCR) is a congenital intestinal motility disorder defined by the absence of enteric neuronal cells (ganglia) in the distal gut. The development of HSCR-associated enterocolitis remains a life-threatening complication. Absence of enteric ganglia implicates innervation of acetylcholine-secreting (cholinergic) nerve fibers.
View Article and Find Full Text PDFBased on a case report of an intrascrotal lipoblastoma in childhood, we present the preoperative diagnostic algorithm and the main differential diagnoses in testicular and paratesticular tumors as well as their surgical management. An 8-week-old infant was admitted with a scrotal tumor known since birth. MRI showed a pinnate vessel supply, originating in the left internal iliac artery.
View Article and Find Full Text PDFWe report on an acute calcinosis in infants as a differential diagnosis to a trauma or an infect of the wrist.
View Article and Find Full Text PDFBackground: We hypothesize that the current practice of sphincter assessment in anorectal malformations (ARMs) by direct muscle stimulation can be improved by sacral nerve stimulation (SNS). Focusing on the specific adjustments for infants, we highlight the anatomical and neurophysiologic basis of SNS and its further diagnostic and therapeutic implications.
Patients And Methods: We examined 20 patients: 12 patients with ARM, 3 with Hirschsprung disease, 3 with sacrococcygeal teratoma, and 2 with cloacal exstrophies.