Lung Transplantation Triggered "Jackhammer Esophagus": A Case Report and Review of Literature
Nineteen-year-old female was diagnosed as idiopathic bilateral bronchiectasis and developed end stage lung disease and respiratory failure. She was referred to King Faisal Specialist Hospital & Research Center in June 2012 for further assessment and possible LTx. During her routine pre-transplant work up, esophageal HRM 24-hour pH impedance monitoring and modified barium swallow were performed to rule out any major esophageal dysmotility or significant GERD, which could predispose her to recurrent aspiration pneumonia and pseudomonas colonization. There was no significant past history of heartburn, regurgitation, chest pain or dysphagia before the LTx. HRM study was performed with a 4.2 mm outer diameter solid state assembly with 36 circumferential sensors spaced at 1-cm intervals (Given Imaging, Los Angeles, CA, USA). Esophageal pressure topography data was analyzed using ManoView™ analysis software. Her pre-transplant, HRM parameters were within normal limits (). Ambulatory impedance pH monitoring study was performed before the LTx, after an overnight fast (off proton pump inhibitors [PPI] for 1 week) on an out-patient basis, using a ZepHr system (Sandhill Scientific, Inc., Highland Ranch, CO, USA), which was equipped with a catheter with 6 impedance electrodes and 2 pH electrodes (first at distal esophagus and second at just below the upper esophageal sphincter). Both the impedance and pH signals were recorded at 50 Hz on a 128 MB compact flash (SanDisk, Milpitas, CA, USA). No significant gastro-esophageal reflux was reported in pre-transplant 24-hour impedance pH evaluation ().\nShe underwent bilateral sequential LTx in October-2012 with no major post-operative complication and discharged after 2 weeks in stable condition on immunosuppressant drugs (mycophenolate mofetil, tacrolimus and prednisolone). She was re-admitted 4 weeks later, when she developed aspiration pneumonia with additional complaints of dysphagia, regurgitation and heartburn. Her second hospital course was again uneventful; she received parenteral antibiotics, PPI and discharged in satisfactory condition. It was suspected that her aspiration pneumonia might have been triggered by GERD secondary to esophageal dysmotility. She was advised to continue her immunosuppressant and standard chemoprophylaxis with valganciclovir, bactrim and itraconazole, in addition to esomeprazole 20 mg twice daily to suppress the symptoms of GERD. Azithromycin 3 times weekly was added as anti-inflammatory agent. The patient's HRM study was repeated 8 weeks after LTx, and her detail HRM datasets were reviewed according to the current Chicago classification. It showed typical spastic hypercontractile contractions (the greatest DCI = 12,437 mmHg · cm · sec) consistent with the diagnosis of Jackhammer esophagus. Surprisingly, her last esophageal HRM returned to normal at 12 weeks after LTx, as shown in the study of esophageal pressure topography with conventional line tracings () without use of any muscle relaxant or sedatives. The data included in the HRM study was LES resting pressure, distal latency, integrated residual pressure, DCI and distal esophageal amplitude at 3, 7 and 11 cm above LES and contractile front velocity before LTx, 4 and 12 weeks after LTx as shown in .\nThe 24-hour pH impedance monitoring study was repeated again on PPI after 8 weeks of LTx (because she was symptomatic on PPI) which was consistent with significant GERD. Gastric emptying study also after the LTx showed severely delayed gastric emptying for solid meal (97% retention at 120 minutes with stagnation of food at 4 hours delayed images) consistent with gastroparesis. Post LTx barium swallow and upper gastrointestinal endoscopy was normal. The impedance pH study (on PPI) was repeated 12 weeks after LTx and surprisingly, no significant abnormality was seen ().