A 20 days old neonate with distension of abdomen , at ultrasound showed - slight narrowing of rectum ( dotted arrow ) & gross distension of sigmoid & descending colon ( arrow ) , & mild distension of entire colon. Rest of the abdomen was unremarkable . On the basis of USG findings low (rectal) obstruction suspected with colonic distension , & possibility of Hirschsprung disease proposed . On barium enema study classical findings of narrowing with coning ( zone of transition ) & distension of sigmoid noted , which supported further the diagnosis . The case underwent primary colostomy with rectal biopsy , which confirmed aganglionic nature Hirschsprung disease .
Fig-1 suprapubic US scan shows rectal narrowing with coning ( dotted arrow ) , & distended sigmoid ( small arrow ) . UB seen empty . The image classically similar to barium study .
Fig 2 - Quad US scan shows distended sigmoid , descending & other parts of colon with rectal narrowing .
Fig 3 - Lateral barium enema radiograph
Fig 4- AP barium enema radiograph
Both fig 3 & 4 barium enema radiographs
shows narrowing of rectum with coning / funnelling of zone of transition & distended sigmoid colon , classical for Hirschsprung disease .
Case 2-
Fig 5- Sono-Recto-sigmoidogram,showing rectal narrowing / stricture with sigmoid fullness-classically resembling with barium study consistent with possible diagnosis of "Hirschsprung disease " (story of a 30 days neonate with constipation & abdominal distension)
TAKE HOME- To diagnose Hirschsprung disease in neonatal period is quite feasible and rewarding. The liquid meconium in colon & recto-sigmoid region acts as a contrast ( echogenic liquid in bowel) during sonography & can delineate the rectal narrowing ( stricture as beak sign) with proximal sigmoid distension. In the late age the constipated & fecal matter loaded sigmoid obscures the stricture details due to fecal matter & gas shadowing .
PS - Barium enema radiographic images & operative f/up courtesy : Dr Gaurav Bahety , M. Ch. pediatric surgeon , Bhilwara ( raj , India )




