Clinical Case Conference Hair Apparent: Rapunzel Syndrome Ariel S. Frey, B.A., M.A.T. Initial Psychiatric Consultation The child psychiatry team (A.S.F. and A.M.) met with Milissa McKee, M.D., M.P.H. Emily and her mother the morning before surgery. By her own and her mother’s account, she was happy and Robert A. King, M.D. well adjusted. She had many friends and was enrolled in a mainstream public school second-grade class that she Andrés Martin, M.D., M.P.H. enjoyed. Emily lived in the home of a family friend with her mother (Kim), her older half sister, and her younger brother. Up until 2 years before, Emily had lived with her mother, siblings, and father in their own home. Kim felt Rapunzel had magnificent long hair, fine as spun that family life had been generally good during the mar- gold. riage and that her husband had been a good father; —The Grimm Brothers, Rapunzel (1) Emily was especially close to him. Emily’s father, how- ever, had a substance abuse problem that led to dissolu- Rapunzel’s hair fell to the ground like a rainbow. tion of the marriage 2 years before admission. After this, It was as strong as a dandelion the parents’ relationship became strained, and Emily and as strong as a dog leash. had not seen her father in a year. Emily was briefly in counseling to help her deal with the —Anne Sexton, Rapunzel (2) changes in her life surrounding her parents’ separation, but the counselor felt that she was cop- ing well and did not require further psy- History of the Problem chotherapy. Kim, however, expressed “[It] had a long tail private concern that Emily was not deal- Emily (all names and identifiers have ing with the loss of her father. Mean- been changed to protect confidentiality) that extended past the while, Emily had started at a new school was a 7-year-old girl seen by her pedia- duodenum, a rare and was doing well academically and trician for a routine annual examina- socially. tion. Although Emily had voiced no complication… With this background information, complaints, her mother had noted that further evaluation was deferred until she had appeared paler than usual. referred to as after the surgery, when the identity of Upon physical examination, her pedia- Rapunzel syndrome.” the mass would be elucidated. trician noted a nontender palpable mass in the child’s stomach, leading Surgical Procedure him to order an abdominal ultrasound. The study showed no evidence of any abnormalities, al- Because the mass in Emily’s stomach appeared too though the technician did note that Emily must have just large to be removed laparoscopically, a gastrotomy was eaten because her stomach was full. However, her mother performed instead through a 7-cm median xiphoumbili- reported that Emily had not eaten since breakfast, 4 cal incision. The mass was found to be a trichobezoar, or hours earlier. hairball, 45 cm long and 8 cm in diameter (Figure 2). The On reevaluation the next day, Emily’s pediatrician still hair mass was cast in the shape of the stomach, which it palpated the same mass, leading him to order a com- filled, and extended down through the distal end of the puted tomography (CT) scan of the abdomen. The abdom- duodenum, past the ligament of Treitz. inal CT demonstrated a free-floating mass that filled the entire stomach and was surrounded by a thin rim of con- Trichobezoars trast material (Figure 1). Emily’s pediatrician did not think The smooth surface of hair does not allow for its propa- the mass was consistent with a neoplasm but was con- gation through peristalsis. Thus, when hair is ingested, it cerned that it could be a conglomeration of something she had ingested. Upon further questioning, Emily re- gets trapped in the mucosa of the stomach. As more hair is ported no stomach pain, nausea, vomiting, reflux, diar- added, the resulting mass causes the stomach to cease rhea, flatulence, recent illnesses, or fever. Her mother re- peristalsis completely. This mass is a trichobezoar (3), first ported no change in her bowel habits and stated that described in 1779 by Baudomant (4). Emily never wanted to eat much in one sitting. Trichobezoars are nearly impossible to diagnose by Given the large size and appearance of the mass on plain film alone. Ultrasound has been shown to be effec- tomography, a decision was made to remove it surgi- tive in diagnosing bezoars in up to 88% of cases if a “clean” cally. Just as the elective surgery was scheduled, Emily acoustic shadow, which represents a solid mass, can be vi- was referred for evaluation by the child psychiatry sualized (4, 5). However, a calcified mass, neuroblastoma, consultation-liaison team. aneurysm, abscess, or fecal material can appear similarly 242 http://ajp.psychiatryonline.org Am J Psychiatry 162:2, February 2005 CLINICAL CASE CONFERENCE FIGURE 1. Abdominal Computed Tomography Showing a Mass in the Stomach of a 7-Year-Old Childa Right Left a Orally ingested contrast material has been displaced against the lining of the gastric wall. on an ultrasound (4). An abdominal CT with contrast has removal (10). In the case of Emily, the trichobezoar had a been shown to diagnose 97% of bezoars (5), which appear long tail that extended past the duodenum, a rare compli- as free-floating filling defects in the stomach (3). cation of trichotillomania referred to as Rapunzel syn- Although trichobezoars can be removed in several dif- drome in the surgical literature. ferent ways, Emily’s was too large to avoid open surgery. Trichotillomania has many different clinical presenta- Smaller trichobezoars can be removed by endoscopy after tions. Patients with trichotillomania usually pull hair from fragmentation by water pick, laser, or extracorporeal the scalp, but eyelashes, eyebrows, legs, armpits, and pu- shock-wave lithotripsy (6, 7) or by enzymatically dissolv- bic regions are also targets for pulling. Although by defini- ing the mass. Finally, small bezoars can be removed lap- tion, trichotillomania entails hair pulling to the point of aroscopically through a small incision (6). However, none alopecia, there is a much larger number of individuals of these techniques are successful for very large bezoars who pull their hair but not to the point of noticeable thin- (over 20 cm), which usually require removal through open ning (11, 12). Not all hair pullers experience the sense of surgery (3). tension before pulling followed by relief afterward that is described in the DSM-IV definition; one study showed Trichotillomania, Trichophagia, that among college students, although 1.5% of men and and Rapunzel Syndrome 3.4% of women had chronic hair pulling, only 0.6% met the full DSM criteria for trichotillomania (9, 13). Clinical Presentation Clinical studies of trichotillomania delineate two types Trichotillomania was first described in the literature in of the disorder, which may coexist in one individual (14). 1889 (3) and first recognized by APA as a distinct disorder The “focused” type is trichotillomania in which time and in 1987 (8). The prevalence of trichotillomania is 0.6%– attention are set aside specifically for the purpose of hair 1.6% if DSM-IV criteria are used (9). Among those who suf- pulling. This type of hair pulling is associated with ten- fer from trichotillomania, only 30% will engage in tricho- sion before and relief after pulling, and with negative af- phagia, or eating their hair, and of these, only some 1% fect. More common is the “automatic” or “sedentary” will go on to eat their hair to the extent requiring surgical type of hair pulling, in which subjects pull their hair while Am J Psychiatry 162:2, February 2005 http://ajp.psychiatryonline.org 243 CLINICAL CASE CONFERENCE FIGURE 2. Trichobezoar From a 7-Year-Old Childa a Specimen preparation and photograph courtesy of Dr. Jonathan Bennett, Department of Pathology, Yale-New Haven Hospital and Yale University School of Medicine. engaged in other activities, such as lying in bed, driving, dren who begin hair pulling before the age of 8, the disor- or watching television (15, 16). Patients with the latter der is usually benign and self-limited, although not all type are unaware that they are pulling their hair at all or cases disappear. Most of these cases are of the sedentary become aware only when a clump of hair “appears” in type, and the children, when verbal, often deny pulling their hands (13). Many patients with trichotillomania (15). In one case, a 28-month-old girl was found to be pull- have other associated habits; about 48%–70% have some ing her own hair, and upon review of her history, it was form of oral behavior after pulling hair, such as running found that she had pulled and eaten her dolls’ hair as early the hair across their lips, biting the root, or eating the hair as 6 months of age. Thus, in early-onset trichotillomania, (13, 16). pulling and eating dolls’ hair can be a first symptom of tri- chotillomania (17). Although habits such as thumb suck- Developmental Features ing have been noted in early-onset trichotillomania, other Trichotillomania is far more common in girls than boys. comorbid features have not been found (15). The age at onset of trichotillomania is bimodal; it begins Trichotillomania occurring in the later school years and either in early childhood or in adolescence (15). adolescence is more likely to be chronic and accompanied Of the early-onset cases, one-third develop it before the by comorbid psychiatric disorders, such as depression, age of 10, and 14% develop it before the age of 7.