This document discusses Rapunzel's Syndrome, a rare variant of trichobezoar where a mass of tangled hair extends from the stomach into the small intestine. It presents a case study of a young female patient who underwent surgery to remove a large trichobezoar. Rapunzel's Syndrome is associated with psychiatric disorders like trichotillomania and trichophagia. Open laparotomy is usually required to remove the complex mass. Post-operative psychiatric counseling is important to prevent recurrence by treating the underlying conditions.

1. RAPUNZEL’S SYNDROME-A RARE VARIANT OF
TRICHOBEZOAR-CASE DISCUSSION & LITERATURE
REVIEW
1.DR RAVICHANDRA MATCHA,
ASSISTANT PROFESSOR OF SURGERY,
RANGARAYA MEDICAL COLLEGE,KAKINADA
2.DR V.V.S.N.DORA KUNJAM,
AMMA HOSPITALS,
YELESWARAM

2. INTRODUCTION
 RAPUNZEL’S SYNDROME IS A RARE VARIANT OF TRICHOBEZOAR IN
WHICH THE MASS IN STOMACH EXTENDS INTO SMALLINTESSTINE &
SOMETIMES INTO COLON.
 TRICHOBEZOAR IS A CONDITION IN WHICH A HARD PAINLESS MASS
OF ENTANGLED HAIR MIXED WITH FOOD MATERIAL IS FORMED IN
STOMACH.
 THIS HAPPENS BECAUSE HAIR ESCAPES PERISTALYTIC MOVEMENTS
OF STOMACH BECAUSE OF THE SLIPPERY NATURE OF IT’S SURFACE.
 BECAUSE OF THE CHURNING MOVEMENTS OF STOMACH THE HAIR
ATTAINS THE SHAPE OF STOMACH IN LONG STANDING CASES.
 THE NAME OF RAPUNZEL’S SYNDROME WAS DERIVED FROM A
GRIMM BROTHERS FAIRY TALE OF A 12 YEAR OLD PRINCESS WHO
WAS SHUT IN A TOWER WITH NEITHER STAIRS NOR DOORS BY AN
ENCHANTRESS WHO CLIMBED UP THE TOWER’S WALL BY USING
RAPUNZEL’S LONG HAIR.

3. INTRODUCTION CONTD…………
 THIS CONDITION IS ALWAYS ASSOCIATED WITH PSYCHIATRIC
DISORDERS NAMELY-TRICHOTILLOMANIA & TRICHOPHAGIA IN
WHICH THE PATIENT HAS AN IRRESISTIBLE DESIRE TO PLUCK
THE HEAD HAIR AND INGEST IT.
 THIS HAIR ON GETTING ENTANGLED FORMS A HARD ,
NONTENDER MASS IN STOMACH ALONG WITH FOOD MATERIAL.
 THE GASTRIC PORTION OF MASS ATTAINS BLACK COLOUR DUE
TO DENATURATION OF HAIR PROTIEN KERATIN.(THIS CAN BE
CLEARLY OBSERVED IN THE PICTURE OF THE REMOVED
MASS).
 IN EARLY STAGES IT IS VERY DIFFICULT TO DIAGNOSE BECAUSE
OF IT’S VAGUE SYMPTOMS.
 THIS IS PURELY A DISEASE OF 2ND DECADE & OCCURS IN
CHILDREN & YOUNG WOMEN.
 A STRONG SUSPISION & ELICITATION OF POSITIVE PSYCHITRIC
DISORDER HISTORY IS KEY FOR IT’S DIAGNOSIS.

4. THE PICTURE OF THE MASS REMOVED IN THE
PRESENT CASE
GASTRIC PORTION
INTESTINAL PORTION

5. INTRODUCTION CONTD……
 AS THE MASS IN RAPUNZEL’S SYNDROME EXTENDS INTO SMALL
INTESTINE & SOME TIMES INTO COLON, IN LONG STANDING
CASES IT GIVES RISE TO COMPLICATIONS.
 THE DESCRIBED COMPLICATIONS IN LITERATURE INCLUDE
 PERITONITIS DUE TO HOLLOW VISCOUS PERFORATION
 PANCREATITIS
 OBSTRUCTIVE JAUNDICE
 INTESTINAL OBSTRUCTION --- AS THE MASS IN THE INTESTINES GETS
DETACHED FROM THE MAIN MASS AND CAN CAUSE SMALL INTESTINAL
OBSTRUCTION AND RARELY LARGE INTESTINAL OBSTRUCTION.
 UNTIL NOW APPROXIMATELY 45 CASES OF RAPUNZEL’S
SYNDROME WERE DESCRIBED IN LITERATURE STARTING FROM
1968, THE YEAR IN WHICH 1ST TYPICAL CASE OF RAPUNZEL’S
SYNDROME WAS REPORTED.


6. PATIENT & METHODS
A YOUNG FEMALE PATIENT PRESENTED WITH CHRONIC EPIGASTRIC
PAIN, POST PRONDIAL EMESIS, ANOREXIA, LOSS OF WEIGHT &
EARLY SATIETY.
PHYSICAL EXAMINATION REVEALED MICROCYTIC, HYPOCHROMIC
ANEMIA, ALOPECIA AREATA, GENERALIZED THINNING & A
NONTENDER, HARD LUMP IN EPIGASTRIUM EXTENDING ONTO
THE RIGHT HYPOCHONDRIUM.
HISTORY ELICITED FROM HER MOTHER REVEALED PSYCHIATRIC
DISORDERS NAMELY -- TRICHOTILLOMANIA & TRICHOPHAGIA.
UPPER G.I.ENDOSCOPY REVEALED THE FUNDIC ASPECT OF THE
MASS.
A CECT ABDOMEN REVEALED A MASS IN STOMACH WITH
EXTENSION INTO SMALL INTESTINE.

7. PATIENTS & METHODS CONTD……..
 SURGICAL PROCEDURE: AN OPEN LAPAROTOMY WAS
PERFORMED USING SUPRAUMBILICAL MID LINE INCISION.
 AN ANTERIOR GASTROTOMY INCISION WAS GIVEN & THE
GASTRIC PORTION WAS REVEALED.
 AS THE MASS WAS EXTENDING ONTO SMALLINTESTINE ,
CAREFULLY PLANNED AND PLACED ENTEROTOMY INCISIONS
FECILITATED THE EASY REMOVAL WITHOUT CAUSING INJURY
TO THE BOWEL.
 THE GASTROTOMY & ENTEROTOMY INCISIONS WERE DEALT
WITH A 2 LAYERED CLOSURE.

8. PATIENTS&METHODS CONTD……..
 RESULTS: THE POST OPERATIVE PERIOD WAS UNEVENTFUL.
THE PATIENT RECOVERED WELL BUT FOR A MINOR SURGICAL
SITE INFECTION.
 THE PATIENT WAS SENT FOR PSYCHIATRIC CONSULTATION AT
THE TIME OF DISCHARGE TO TREAT THE UNDERLYING
PSYCHIATRIC CONDITIONS --- TRICHOTILLOMANIA &
TRICHOPHAGIA & TO RULE OUT RECURRENCES.
 FOLLOW UP: THE PATIENT WAS FOLLOWED UP FOR A PERIOD
OF 2 YEARS. DURING THIS PERIOD THE PATIENT REGAINED HER
LOST WEIGHT & ALL HER NUTRITIONAL PARAMETERS WERE
IMPROVED. THERE WERE NO RECURRENCES DURING THIS
FOLLOW UP PERIOD.

9. DISCUSSION
 BEZOAR MEANS A HARD MASS OF COLLECTED FIBERS OF
VEGETABLE OR HUMAN ORIGIN.
 PHYTOBEZOARS AND LACTOBEZOARS WERE REPORTED IN
THE LITERATURE.
 THE NAME BEZOAR WAS DERIVED FROM AN ARABIC WORD
BEDZEHR OR PERSIAN WORD PADZHAR MEANING
PROTECTING AGAINST POISON.
 TRICHOBEZOAR IS MAINLY A DISEASE OF 2ND DECADE.
 IT COMMONLY OCCURS IN FEMALES BECAUSE OF THEIR
LONG HAIR.
 A BEZOAR OF COTTON WAS REPORTED IN AN 18 YEARS OLD
MALE PATIENT.
 A TYPICAL RAPUNZEL’S SYNDROME WAS REPORTED IN A
MALE PATIENT AND THE SOURCE OF HAIR WAS RECOGNISED
AS HIS SISTER.

10. DISCUSSION CONTD……..
 AN AUTHOUR REPORTED A SERIES OF 4 CASES. IN ALL THE
CASES THE MASS WAS REMOVED BY OPEN LAPAROTOMY &
IT STILL REMAINS THE GOLD STANDARD TREATMENT FOR
TYPICAL RAPUNZEL’S SYNDROME BECAUSE OF THE
COMPLEXITY OF THE MASS.
 A RARE CASE WAS REPORTED IN POST PARTUM PERIOD.
 EARLY SMALL BEZOARS CAN BE REMOVED BY UPPER
G.I.ENDOSCOPY OR LAPAROSCOPICALLY.
 INSTRUMENTS LIKE TRICHOTOMES & BEZOTOMES WERE
USED IN SOME CASES FOR REMOVAL.
 EXTRA CARPOREAL BEZOTRIPTORS WERE USED IN SOME
CASES AND IT HAS TO BE STILL EVALUATED FOR IT’S
EFFICIENCY.

11. CONCLUSION:
 RAPUNZEL’S SYNDROME IS TRICHOBEZOAR EXTENDING INTO
SMALLINTESTINE/COLON.
 IT IS ALMOST ALWAYS A DISEASE OF SECOND DECADE
OCCURING MOST COMMONLY IN YOUNG FEMALES.
 OPEN LAPAROTOMY REMOVAL STILL REMAINS THE GOLD
STANDARD THERAPY FOR FULL BLOWN RAPUNZEL’S
SYNDROME.

12. CONCLUSION CONTD…….
 PSYCHIATRIC COUNSELLING IS A MUST IN ALL CASES AS THE
RAPUNZEL’S SYNDROME IS ALWAYS INVARIABLY ASSOCIATED
WITH UNDERLYING PSYCHIATRIC DISORDERS---
 TRICHOTILLOMANIA
 TRICHOPHAGIA
 OTHERWISE THERE IS ALWAYS A SCOPE FOR RECURRENCE AS
THIS CONDITION IS ALWAYS ASSOCIATED WITH THESE
PSYCHIATRIC DISTURBANCES.
A DIAGNOSIS OF THIS CONDITION
IN EARLY STAGES WARRANTS A
STRONG SUSPISION OF DIAGNOSIS IN
THOSE PRESENTING WITH VAGUE
SYMPTOMS & ELICITING A POSITIVE
MEDICAL HISTORY REGARDING THE
ASSOCIATED PSYCHIATRIC
CONDITIONS.
TREATING THE PSYCHIATRIC
CONDITIONS SUCCESSFULLY,
PREVENTS THE RECURRENCE OF THIS
DISEASE.