Gastric Bezoar in a Girl With Sickle Cell Disease
A 12-year-old African American girl with hemoglobin SS genotype sickle cell disease (SCD) presented to the emergency department (ED) and was admitted to our hospital after having experienced 5 weeks of unrelenting abdominal pain. She described the pain as intermittent, nonradiating, midepigastric, aggravated by eating or lying flat, and improved when lying down on her left side. She had used prescribed hydrocodone for pain management at home, but the medication had been ineffective.
The girl had noted a decreased tolerance to solid foods and that she had an easier time with liquids. She also reported having fullness after meals. Her mother had noticed a 4.5-kg weight loss over 5 weeks.
She had been seen for similar complaints in the emergency department (ED) 3 times in the past 5 weeks prior to her admission. The results of abdominal ultrasonography performed during her ED visit a week prior were reported as normal; she was treated for presumed muscle spasm and discharged home. Of note, she frequently had complained of abdominal pain in the past, often in association with SCD-related vaso-occlusive crisis. Over the past few weeks, however, her pain had been more constant, and she had not had pain at other sites.
The girl denied headaches, chest pain, constipation, dyspnea, vomiting, fever, cough, congestion, abdominal point tenderness, tingling, numbness, or changes in mental status. She had received a diagnosis of asthma 2 years ago for which she used a bronchodilator and inhaled corticosteroid. She also took antihistamines to control allergic rhinitis symptoms, and she took hydroxyurea for her SCD. Her immunizations were up to date. Psychosocial evaluation findings were significant for anxiety and for her father having been incarcerated.
Significant findings on physical examination were the palpation of a hard, transverse, elongated mass, measuring approximately 2 × 6 cm, located in the epigastrium and extending to the left upper quadrant of the abdomen. The edge of the liver was palpable, but the spleen’s tip could not be palpated.
Results of abdominal ultrasonography were unchanged from the study done a week prior. Results of an abdominal computed tomography (CT) scan with contrast showed the stomach lumen to be completely filled with a large inhomogeneous mass measuring approximately 4.9 × 6 × 18 cm (A and B). The CT findings were consistent with gastric bezoar.
Further history was obtained from the patient and parent. The girl denied pica or eating any nonfood substances. We consulted our surgery team; esophagogastroduodenoscopy revealed a large ball of hair in the stomach that was unable to be removed endoscopically during the procedure. The surgery team proceeded with an exploratory laparotomy and gastrostomy and removed a large hair mass from the stomach measuring 20 × 10 × 7 cm (C). The patient tolerated the procedure well.
Five days later, upper gastrointestinal tract imaging results ruled out leakage from the stomach. Her diet then was gradually advanced. She was discharged in good condition 7 days after surgery. Psychiatric follow-up was scheduled to address her pica.
Discussion
SCD is an inherited disorder that affects hemoglobin, resulting in the creation of abnormal sickle-shaped red blood cells that have a tendency to obstruct blood flow. Among the most common complications of SCD are frequent pain crises, respiratory problems, chronic pain episodes, cerebral infarcts, an increased risk of infection, and damage to vital organs.1
Although vaso-occlusive pain crisis, particularly abdominal pain, is a common presentation in patients with SCD, it can present a diagnostic challenge and requires the consideration of a broad differential diagnosis. The most urgent presentation of abdominal pain in SCD is acute splenic sequestration, leading to the second most common cause of death in these patients. Children with SCD can have appendicitis, intestinal obstruction, or other condition presenting as acute surgical abdomen.
Persons with SCD also are at increased risk for behavioral and emotional problems,2-4 including pica.5 According to the fifth edition of the Diagnostic and Statistical Manual of Mental Disorders, a diagnosis of pica is made if eating of non-nutritive substances persists for at least 1 month and cannot be attributed to a cultural practice or another mental disorder.6 Ingestion of nonedible substances can lead to intestinal obstruction, gastric bezoar, electrolyte abnormalities, and other complications.7-9 While the association of pica with SCD is not well understood, it is postulated to be multifactorial, with metabolic, nutritional, behavioral, and psychological factors having roles.
Although rare, bezoars can lead to abdominal pain resulting from gastric obstruction, and they can be misdiagnosed and mistreated as pain crisis secondary to SCD. The rarity of the association of gastric bezoars, pica, and SCD has resulted in limited published research. Still, children and adults with SCD have a comparatively high prevalence of pica, with reported rates of more than 60%.5,10 Trichotillophagia, the ingestion of hair, is a rare type of pica that can result in the formation of a trichobezoar, as in our patient’s case.
The rare case presented here demonstrates the importance of considering gastric bezoars in the differential diagnosis of patients with SCD who present with abdominal pain. Gastric outlet obstruction is a potentially life-threatening complication of bezoar that requires timely emergent surgical intervention. The case also highlights the need to screen children with SCD for pica during routine health examinations or during inpatient hospitalizations for abdominal pain.
References
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