34. Prepubertal Vaginal Bleeding: A Rare Presentation of Prepubertal Metastatic Vulvar Crohn's Disease

Abstract

<h3>Background</h3> Metastatic Crohn's disease (MCD) is a rare manifestation of inflammatory bowel disease. Vulvar lesions are seen in only 2% of female patients with Crohn's disease (CD) and frequently predate gastrointestinal manifestations in the pediatric population. Subsequently, MCD is a particularly challenging diagnosis in pediatric and adolescent patients with long-term comorbidities and overall decreased quality of life (QOL). <h3>Case</h3> An 8-year-old female presented to her pediatrician with prepubertal vaginal bleeding, described as initially light pink spotting with wiping that progressed to episodes of bright red bleeding that worsened with activity. Her only other complaints were constipation and generalized groin discomfort. Prior work up from her pediatrician included labs which were significant for iron deficiency anemia out of proportion to her described blood loss. Imaging had also been obtained including a normal pelvic ultrasound and a CT scan which showed diffuse mesenteric fat stranding, characterized as nonspecific but possibly secondary to underlying inflammatory process, and prominent lymph nodes noted within the colon. On physical exam, she was Tanner stage I with significant bilateral vulvar edema and erythema without obvious lesions. The urethral meatus was normal, but the vaginal introitus was difficult to visualize in the office due to the vulvar edema. She was also noted to be at the first percentile for weight and zero percentile for height. She was taken to the operating room for further evaluation, and underwent exam under anesthesia (EUA) with vaginoscopy and vulvar biopsies. EUA again showed significant bilateral vulvar edema with no visible lesions; vaginoscopy showed diffuse inflammation of the vagina and cervix but otherwise no lesions or other abnormalities. Vulvar biopsies revealed lymphangiectasia circumscriptum, which has been associated with CD, neoplasia, and hidradenitis suppurativa. Completion of work up with pediatric gastroenterology is pending at time of this submission, but we anticipate confirmation of diagnosis of CD in the next couple of weeks. <h3>Comments</h3> Chronic, untreated MCD can lead to significant scarring, disfigurement, increased morbidity, and overall decreased QOL. Unfortunately MCD manifestations do not consistently correspond with gastrointestinal symptoms or flares, and thus require frequent follow-up and surveillance. Given the rarity of the condition, there is limited data regarding vulvar MCD, and first line treatment remains corticosteroids with compression garments for symptomatic control of vulvar edema. A multidisciplinary approach is essential for these patients, and a high degree of suspicion is necessary for accurate diagnosis and treatment.