RAPUNZEL SYNDROME – An ultrasound diagnosis

“A large & long intestinal Trichobezoar causing intussusception like bowel mass & obstruction”

Case presented by-

Dr.Subhash Tailor [ MD Radiology ]

Dr.Gaurav Bahety [M Ch pediatric surgery ]

BHILWARA [ Rajasthan]

The Rapunzel syndrome is a trichobezoar with a long tail extending from the stomach to small bowel .  To our knowledge , this is a very rare & uncommon case in a male child [ as most of the reported cases of Rapunzel syndromes in pediatric age were females ] in which the trichobezoar has passed from stomach into jejunam , & caused bowel mass & obstruction involving the entire bowel upto sigmoid colon .  Ultrasound revealed a large , elongated , curved multilayedred bowel mass[appearing like that of intussusception] encasing thick central echogenicity [ the trichobezor & its tail ] .Hence this should be suspected in the appropriate clinical & imaging circumstances.

Key Points  

-Trichobezoars are usually asymptomatic until they reach a large size .

-The Rapunzel syndrome [ a trichobezoar with a long tail extending from the stomach to the small bowel ] is a rare cause of intussusceptions & obstruction.

Case Details-

A   6 yrs old boy having h/o chronic pain abdomen  , presented with acute pain abdomen ,vomiting  &  distension , was asked for  abdominal sonography . His laboratory tests were noncontributory ,  and  abdominal radiograph revealed few air fluid levels.

USG  abdomen  showed  a large elongated  multilayered bowel mass [ like intussusception] occupying most of the left side abdomen and extending in pelvis  with  few dilated small bowel loops & mild ascites . The bowel mass was typically simulating intussusception & showing long curved course , with a long intraluminal/central echodense area[suspected trichobezoar ] within it. The proximal part of the central  echodense area was a bit large [the trichobezoar ] , &  rest appeared thin & long extending in entirety of bowel mass[tail of bezoar ]  . Color Doppler scan revealed mural vascularity within mass , indicating adequate bowel perfusion . In light of USG findings probable diagnosis of intussusception like bowel mass ,may be due to intraluminal  elongated bezoar , with mild obstruction & ascites was made.[ see US images ]  Further evaluation by CT scan was suggested.

Operative details  – Pt. was taken for urgent surgery to avoid impending gut gangrene & perforation. A large & elongated trichobezoar , found within lumen from jejunum to proximal sigmoid colon colon , making clustered redundant looped obstructed bowel mass , was removed successfully with appropriate surgical steps . The proximal  large part of trichobezoar was ,of stomach shape & crossed pyloric canal ,seen lodged in proximal jejunum . The rest thin elongated  bazoar tail was extending in rest of the redundant clustered  bowel loops to reach upto sigmoid colon [ see per op.photos ].

Figure 1- Large intraluminal echodensity in left upper abdomen ,representing proximal part of bezoar in jejunum

Figure 2 –Large elongated  multilayered bowel mass [like intussusception] in left side abdomen  extending into pelvis, containing long central echodensity - consistent with trichobezoar tail

Figure 3 – Multilayered bowel mass with mural vasculature present on doppler

Figure 4 – Per op. photograph showing  large proximal part of  bazoar in jejunum

Figure 5 –Per operative trichobezoar removal in progress