Rapunzel syndrome:A rare case - Dr Kelkar

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Rapunzel Syndrome: The Rapunzel syndrome is an extremely rare intestinal condition in humans resulting from eating hair (trichophagia). The syndrome is named after the long-haired girl Rapunzel in the fairy tale by the Brothers Grimm. Trichophagia is sometimes associated with the hair pulling disorder trichotillomania.

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Rapunzel syndrome:A rare case - Dr Kelkar

  1. 1. By Dr. Sanjeevanee Kelkar She is a general surgeon & for last 16 years has been working as a full time General Surgeon in a Charitable Trust Hospital in Nagpur. She is also a Senior Consultant & has been the Medical Superintendent also for last 5 years. 
  2. 2. <ul><li>Trichobezoar extending from Stomach till terminal Ileum. </li></ul><ul><li>“ Trichotillomania” </li></ul><ul><li>Trichobezoar is a rare clinical condition. </li></ul><ul><li>Stomach is the common site of occurance. </li></ul><ul><li>Intestinal obstruction due to trichobezoar is extremely rare. </li></ul><ul><li>Tricho=hair ,Bezoar=mass or ball. </li></ul><ul><li>Adolescent girls - 90% </li></ul><ul><li>H/O swallowing hair,,eating hair is found in 1:2000 children. </li></ul><ul><li>10% patients show psychiatric abnormalities or mental retardation. </li></ul><ul><li>First case of human trichobezoar was reported by Baudamant in1779. </li></ul>
  3. 3. <ul><li>Problem-Stomach not able to exteriorize hair & other substances out of lumen because the friction surface is not sufficient for propulsion by peristalsis. </li></ul><ul><li>Indigestible matter trapped by mucus in stomach, forms shape of stomach gradually . </li></ul>
  4. 4. <ul><li>Age occurance----1year to 56 years </li></ul><ul><li>Common age---15 to 20 years & 90% girls </li></ul><ul><li>Symptoms—Epigastric pain,dyspepsia,postprandial fullness. </li></ul><ul><li>Gastric bleeding—6%. </li></ul><ul><li>Intestinal obstruction or perforation—10% </li></ul><ul><li>Iron def.anemia. </li></ul>
  5. 5. Investigations <ul><li>Proper history=suspicion.only </li></ul><ul><li>Xray,only barium meal if done. </li></ul><ul><li>Usg-high echogenicity of hair & presence of multiple acoustic interfaces created by trapped hair & food limits usg diagnosis. </li></ul><ul><li>Endoscopy. </li></ul><ul><li>CT & MRI. </li></ul>
  6. 6. Ms. Poojabai Patel.(from Damoh M.P.) <ul><li>14 year old girl was admitted in CIIMS on 20 th July evening with acute colicky abdominal pain of 25 days duration. </li></ul><ul><li>She was persistently vomiting and was restless for 7 days. </li></ul><ul><li>Her vitals were B.P.110/70mmhg,pulse 110/mt,pale dehydrated . </li></ul><ul><li>Her xray standing abdomen revealed subacute intestinal obstruction. </li></ul><ul><li>She was managed conservatively,R.T,I/V fluids Enemas, Duphalac ,responded by passing motion, reduction in distention ,hence conservative management was continued. On 3 rd day she was again the same ,hence decided to be taken for exploratory laparotomy. </li></ul>
  7. 7. <ul><li>Laparotomy –grossly distended small bowel loops with multiple perforations ,adhesions ,intussusception—causing intestinal obstruction. </li></ul><ul><li>grossly dilated terminal ileum,in which some funny cordlike thing was felt. </li></ul><ul><li>perforations revealed trichobezoar.Evacuated. </li></ul>
  8. 8. <ul><li>Laparotomy –grossly distended small bowel loops with multiple perforations ,adhesions ,intussusception—causing intestinal obstruction. </li></ul><ul><li>grossly dilated terminal ileum,in which some funny cordlike thing was felt. </li></ul><ul><li>perforations revealed trichobezoar. Evacuated. </li></ul>
  9. 9. <ul><li>Multiple mesenteric Lymph nodes enlarged. </li></ul><ul><li>Resection & anastomosis was done. </li></ul><ul><li>Stomach was inspected & found huge bezoar. </li></ul><ul><li>Gastrotomy done and huge stomach –shaped trchobezoar evacuated. </li></ul><ul><li>Lavage, hemostasis,drainage & closure. </li></ul>
  10. 10. <ul><li>Postoperatively first 48 hours was o.k./, then bp started fluctuating ,shifted to ICU,INR deranged, </li></ul><ul><li>In spite of doing everything died after 72 hours, the pathetic ending . </li></ul>

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