Medical College of Georgia Philadelphia, PA, United States
Michael Coles, MD1, Muaaz Masood, MD1, John Erikson Yap, MD2 1Medical College of Georgia, Augusta, GA; 2Medical College of Georgia at Augusta University, Augusta, GA
Introduction: We present a case of idiopathic angioedema of the tongue in a patient being evaluated for endoscopic placement of a nasogastric tube and parenteral nutrition supplementation.
Case Description/Methods: A 65-year-old African-American female with a history of T2N0M0, ER positive, HER-2 negative breast cancer treated with chemotherapy and radiation and obstructive sleep apnea presented with acutely worsening macroglossia. Her macroglossia (Fig. 1A) progressed over a 5-year time span resulting in difficulty with phonation and severe obstructive sleep apnea. Her swelling remained fixed only with interval worsening. High dose steroids, antihistamines and epinephrine provided no significant benefit. Hereditary angioedema types I-II ruled out with normal C4 and C1INH function and quantity. She denied pruritic/urticarial symptoms consistent with Histaminergic idiopathic angioedema. Hormonal workup demonstrated normal cortisol, LH, FSH, ACTH, estradiol, IGF-1, and growth hormone levels. MRI (Fig.1B) and tissue biopsy was negative for amyloidosis or granulomatosis. Her medication regimen was scrutinized for potentially causative agents such as ACEi/ARBs or calcium channel blockers. Patient admitted with worsening of macroglossia and airway obstruction necessitating urgent tracheostomy. Treatment with IV dexamethasone provided equivocal effect. Gastroenterology was consulted for consideration of NGT placement to assist in PO intake. We recommended straw use for liquid PO intake to circumvent the macroglossia after clearance by a speech-language pathologist. Endoscopic placement of the NGT and ultimately PEG tube for nutritional supplementation was deemed unnecessary after caloric intake met per the nutritionist. Given the fixed nature of her swelling, lymphatic drainage obstruction remains atop the differential diagnosis.
Discussion: 30-50% of angioedema cases are characterized as idiopathic with nebulous pathophysiology. This case underscores the importance of multidisciplinary, pre-procedural planning to avoid unnecessary and potentially harmful interventions directed to the enteric tract. As gastroenterologists, seemingly simple endoscopic interventions may expedite treatment. However, continuous consideration of the holistic clinical picture should be maintained as simple non-invasive, treatment modalities may prove equally effective with less potential for risk. In this particular case, angioedema of the tongue may present unrecognized airway management risks during GI tract instrumentation procedures.
Figure: Figure 1A: Gross photo of macroglossic tongue. Figure 1B: MRI with contrast of demonstrating macroglossia in the sagittal view.
Michael Coles indicated no relevant financial relationships.
Muaaz Masood indicated no relevant financial relationships.
John Erikson Yap indicated no relevant financial relationships.
Michael Coles, MD1, Muaaz Masood, MD1, John Erikson Yap, MD2. P0434 - The Plastic Straw: A Simple Tool for Nutritional Optimization of Acute Idiopathic Angioedema of the Tongue, ACG 2021 Annual Scientific Meeting Abstracts. Las Vegas, Nevada: American College of Gastroenterology.