Laparoscopic Heller Myotomy in a Patient With Achalasia and Isolated Situs Inversus of the Liver

Abstract

Achalasia is a rare esophageal motility disorder characterized by incomplete lower esophageal sphincter (LES) relaxation, increased LES tone, and absent peristalsis in the esophagus. Management of achalasia includes pneumatic dilation (PD), Botulinum toxin A (BTA) injections to LES, per oral endoscopic myotomy (POEM), and a laparoscopic Heller myotomy (LHM). Situs inversus is a rare congenital condition in which the abdominal and thoracic organs are located in a mirror image of the normal position in the sagittal plane. We herein present a case of a patient with Type II achalasia who underwent an LHM and toupet fundoplication in the setting of an isolated laterality malposition of the liver on the left side of the abdomen. Single organ congenital lateralization defects are extremely rare with literature describing few case reports and case series. A much rarer condition is isolated organ situs inversus. In the foregut, most reports of isolated situs inversus are limited to isolated gastric situs inversus, dextrogastria. Most isolated liver malposition has described situs ambiguous, at the midline, usually associated with polysplenia. Our patient had the normal position of the foregut structures, including the stomach, spleen, pancreas, and duodenum, except for the isolated situs inversus of the liver. Because of the unusual anatomy, performing an LHM was quite challenging. Our workup approach and intraoperative considerations are described. By displacing the larger left lobe of the liver, we were able to safely complete a standard heller myotomy with adequate length and distally across the gastroesophageal junction. Our patient had an uncomplicated post-operative course, and at follow-up has continued to show improvements in her dysphagia and her quality of life.

Keywords: abdominal situs inversus; dextrohepatica; esophageal achalasia; esophagus; foregut; heller myotomy; isolated situs; minimally invasive; minimally invasive laparoscopy; visceral situs malposition.

Figure 1. Dilated esophagus (above with blue arrow). Tight lower esophageal sphincter (below with black arrow)

Figure 2. Hiatal hernia on retroflex (blue arrow)

Figure 3. Esophagram showing dilated esophagus (blue arrow) and minimal to no flow through the gastroesophageal junction with bird's beak appearance (black arrow)

Figure 4. HRM consistent with type II achalasia; DCI of 360 (blue arrow). Pan esophageal pressurization (black bracket) and IRP of 21 (black arrow)

HRM, high-resolution manometry; DCI, distal contraction integral; IRP, integrated relaxation pressure

Figure 5. CT scan showing a dilated esophagus (blue arrow)

Figure 6. Isolated situs inversus of the liver (dextrohepatica) showing the left lobe of the liver on the right side of the patient (blue arrow)

Figure 7. Trocar placement for a LHM showing the location and the size of the trocars

LHM, laparoscopic Heller myotomy

Figure 8. The left lobe of the liver to the patient's right side above the labeled IVC. Liver retractor under the right lobe on the patient's left side

IVC, inferior vena cava

Figure 9. The right lobe of the liver on the patient's left side displaced by the liver retractor

Figure 10. View of the hiatus after complete dissection

Figure 11. Preparation for Toupet fundoplication following a Heller myotomy, blue lines depict the cut edge of the esophageal muscles

Figure 12. POD #1 esophagram showing decreased esophageal dilatation (black arrow) and flow through the gastroesophageal junction (blue arrow)