Sunday, August 24, 2014

ULTRASOUND IN NEONATAL HIRSCHSPRUNG DISEASE


CASE HISTORY 
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 )


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