Rapunzel Syndrome Complicated by Escherichia coli Sepsis, Bowel Perforation, and Pancreatitis in an 11-year-old Malnourished Female

{"Background": "Rapunzel syndrome, first described in 1968 by Vaughn et al, is a rare form of trichobezoar with extension of the bezoar into the small bowel. The most common presenting symptoms include abdominal pain (37%), nausea and vomiting (33.3%), obstruction (25.9%), and peritonitis (18.3%). Less common presentations include weight loss (7.4%) and intussusception (7.4%). Exceedingly rare complications include gastric ulceration, obstructive jaundice, acute pancreatitis, and malabsorptive complications such as protein-losing enteropathy, iron deficiency, and megaloblastic anemia. A 2016 structured PubMed search reported sepsis occurring in only 2% of patients with Rapunzel syndrome. Since 2016, only one additional case of sepsis complicating Rapunzel syndrome has been reported. This paper presents a case of Rapunzel syndrome in an 11-year-old female complicated by Escherichia coli sepsis, malnutrition, pancreatitis, cholangitis, and bowel perforation.", "Methods": "This is a single case report. The patient was an 11-year-old female with a 5-year history of poor weight gain presenting with a 3-week history of constant nonradiating epigastric abdominal pain, nausea, vomiting, and decreased appetite. Initial evaluation by her primary care provider with a proton pump inhibitor and dicyclomine provided no relief. Upon emergency department arrival, physical exam revealed malnutrition (weight z-score for age \u20132.15, BMI < first percentile per CDC 2\u201320 years girls), dehydration (heart rate 109 bpm), and mild epigastric abdominal pain. Initial labs showed albumin 2.9 g/dL, ALT 151 \u00b5/L, AST 100 \u00b5/L, total bilirubin 0.4 mg/dL, direct bilirubin 0.1 mg/dL, lipase 1030 \u00b5/L, triglycerides 65 mg/dL, WBC 11.7 K/mm\u00b3 (81.9% neutrophils, 10.5% lymphocytes), Hg 14.4 g/dL, and MCV 83.3 fL. Right upper quadrant ultrasound showed a normal gallbladder with no gallstones and a common bile duct measuring 11 mm; the pancreas was not visualized. On hospital day 2, the patient became febrile to 102.1\u00b0F orally. Blood and urine cultures were obtained before initiating piperacillin-tazobactam. Blood cultures returned positive for E. coli within 6 hours. An abdominal x-ray for NGT placement revealed the tube taking an unusual course along the periphery of the stomach. Repeat labs showed ALT 419 \u00b5/L, AST 720 \u00b5/L, total bilirubin 1 mg/dL, and direct bilirubin 0.6 mg/dL. MRCP revealed no intraductal stones, a nonlayering low signal gastric substance, and fluid-filled loops of small bowel. Liver function tests continued to rise (total bilirubin 2.2 mg/dL, direct bilirubin 1.6 mg/dL). Upper endoscopy on hospital day 3 revealed a large trichobezoar occupying most of the stomach and extending past the pylorus into the third portion of the duodenum, with the ampulla noted to be prominent and erythematous.", "Key Results": "Emergent laparotomy revealed a large gastric bezoar containing hair, lice, nail clippings, and plant-like material extending from the stomach to the terminal ileum with several rope-like turns causing kinking of the small bowel. Several areas of full thickness necrosis were noted along the mesenteric border of the small bowel. The bezoar was extracted through a gastrostomy and 2 small bowel enterotomies. A temporary abdominal closure was placed with a second look performed 48 hours later. The patient developed disseminated intravascular coagulation and hypotension requiring vasopressor support. Second look revealed multiple patches of partial thickness necrosis along the mesenteric border and a full thickness area of necrosis that had perforated. The bowel was repaired in single-layer fashion. Abdominal closure was performed 72 hours later, and the patient was successfully extubated. She completed a 14-day course of piperacillin/tazobactam and a 7-day course of fluconazole. Further workup revealed iron level <10 \u00b5g/dL and total iron binding capacity of 144 \u00b5g/dL. Psychiatry evaluation revealed trichotillomania and trichophagia. The patient had no visible hair thinning or alopecia. She was a picky eater eating the same meals daily (pasta and chips) and would gag when protein was encouraged. Cognitive behavioral therapy was recommended, and she was discharged to an inpatient nutritional and physical rehabilitation program.", "Clinical Implications": "This case highlights that Rapunzel syndrome can present with life-threatening complications including E. coli sepsis, bowel perforation, and acute pancreatitis, even in the absence of obvious signs of trichotillomania on physical exam. The authors note that bowel perforation is reported more frequently than sepsis in Rapunzel syndrome, and subacute symptoms of abdominal pain with delayed presentation may contribute to the development of sepsis. Acute pancreatitis is thought to occur due to irritation and obstruction of the ampulla of Vater by mass effect of the bezoar tail. Trichobezoars may go unrecognized until they are large enough to cause gastrointestinal symptoms, as the behavior often occurs in private. Cognitive behavioral therapy is the cornerstone of treatment, and a thorough psychiatric evaluation is warranted given the high incidence of comorbid conditions such as mood, anxiety, eating, and substance use disorders."}