Dysphagia during Pregnancy: Severe Achalasia Diagnosis in the Third Trimester of Pregnancy

Abstract

Purpose: A 30-year-old African-American female who was G8P6 with history of spontaneous abortion of her first pregnancy and WPW Syndrome presented to obstetric clinic with worsening dyspnea on exertion. She also reported 1 year history of dysphagia to both solids and liquids, and 30-pound weight loss. No history of recent travel. On presentation, her vital signs showed episodic tachycardia (HR110 beats per minute) and tachypnea (RR18-24). Physical exam revealed 32 weeks of pregnant abdomen, inspiratory and expiratory stridor best heard in the neck with transmitted sound on bilateral lung fields, and a palpable thyroid with no retrosternal extension. Lab work showed anemia (Hgb 10.6 gm/dL), normal renal function, and LFTs (except albumin 2.4 g/dL). Imaging studies revealed a markedly dilated esophagus with smooth tapering at GE junction and markedly narrowed trachea due to compression from dilated esophagus (Image 1). Upper endoscopy showed severely dilated esophagus with retained fluid and food debris, and tight lower esophageal sphincter suggestive of achalasia. A decision was made and injected a total of 100 IU of Botox (25 IU at each quadrant) in the distal esophagus, 2 cm above the GE junction. Patient was intubated and sedated for the procedure by anesthesia team. An NG was placed for nutrition. Within 4 days after the procedure, patient's symptoms have improved and was discharged with follow-up. Patient delivered the baby at full term as outpatient with no complications. She presented 8 months later with similar symptoms and a repeat EGD showed similar findings suggestive of severe achalasia. A high-resolution manometry study confirmed the diagnosis of achalasia and surgical consultation was obtained for definitive therapy. Achalasia is a rare disease with a prevalence rate of 8 per million population. Its presentation during pregnancy is rare and poses a challenging management scenario. A few published case reports suggested either Botox or pneumatic balloon dilation as options. In our case, we opted to treat with Botox injection which resulted in good relief of symptoms for 2 months during which she was able to complete her pregnancy and delivered the baby with no complications.Figure: CT image showing dilated esophagus filled with food debris and associated tracheal compression.