Sindrom Rapunzel – rijedak uzrok akutnog pankreatitisa: prikaz slučaja

Cilj: Osvijestiti o mogućnosti postojanja sindroma Rapunzel u adolescentica s akutnim pankreatitisom. Prikaz slučaja: U hitnu pedijatrijsku ambulantu javila se petnaestogodišnja djevojka zbog povremenih bolova u gornjem dijelu abdomena koji su se intenzivirali par dana pred dolazak. Prilikom fizikalnog pregleda nađena je u epigastriju i lijevom hipohondriju bolna rezistencija veličine otprilike 10 cm u promjeru. Opsežnom laboratorijskom analizom našle su se povišene koncentracije enzima gušterače te se postavila dijagnoza akutnog pankreatitisa. Daljnjom obradom indicirala se gastroskopija kojom se prikazala masa kose na ulazu u želudac. Kompjutoriziranom tomografijom uočilo se da ona seže sve do bulbusa duodenuma. Nakon provedenog konzervativnog liječenja pankreatitisa abdominalni bolovi su regredirali, došlo je do normalizacije enzima gušterače te je bilo indicirano operativno liječenje. Učinila se eksplorativna laparotomija i gastrotomija. Ekstirpirao se trihobezoar težine 1 kg i dužine 25 cm oblika odljeva lumena želuca i početnog dijela duodenuma. Postavila se dijagnoza sindroma Rapunzel. Dječji psihijatar potvrdio je prisutnu trihotilomaniju i trihofagiju te se djevojku uključilo u psihoterapijski tretman. Zaključak: Sindrom Rapunzel vrlo je rijetko stanje, ali treba biti uključeno u diferencijalnu dijagnozu akutnog pankreatitisa u adolescenata. Specifični simptomi trihotilomanije i trihofagije u kombinaciji s nespecifičnim simptomima, poput dugotrajnog bola u abdomenu, trebali bi pobuditi sumnju na razvoj trihobezoara.
Aim: To raise awareness about the existence of Rapunzel syndrome in female adolescents with acute pancreatitis. Case report: A 15-year-old girl came to the emergency pediatric clinic due to the occasional pain in the upper abdomen, which had intensified a few days before her arrival. During physical examination, a painful resistance of approximately 10 cm in diameter was palpated in the epigastrium and the right hypochondrium. Extensive laboratory analysis revealed raised concentrations of pancreatic enzymes so a diagnosis of acute pancreatitis was made. Further examination indicated gastroscopy, which showed a mass of hair at the entrance to the stomach. Computed tomography revealed that the mass reached all the way to the duodenal bulbus. After the conservative treatment of pancreatitis, the abdominal pain regresses and the pancreatic enzymes returned to normal so the operative treatment was indicated. Exploratory laparotomy and gastrotomy were performed. A kilogram heavy and 25 cm long trichobezoar in the shape of the gastric lumen and the initial part of the duodenum were exploited. Rapunzel syndrome was diagnosed. The child psychiatrist confirmed the presence of trichotillomania and trichophagia, so the girl was referred to the psychotherapy treatment. Conclusion: Rapunzel syndrome is a very rare condition but should be included in the differential diagnosis of acute pancreatitis in adolescents. Specific symptoms of trichotillomania and trichophagia in combination with non-specific symptoms such as prolonged abdominal pain should raise the suspicion of the development of trichobezoars.