CASE REPORT

The patient was planned for exploratory laparotomy. Intra-operatively, to our surprise, the stomach found normal intraabdominal (Figure 5). There was slight dilatation of the esophageal hiatus and of the abdominal part of the esophagus, however, no obvious herniation was identified. On passing a nasogastric tube from the head end, there was copious drainage of dark hemorrhagic contents. The tube was not palpated in the stomach or the abdominal esophagus. A diagnosis of achalasia and megaesophagus was then considered, bearing in mind that the nasogastric tube of small caliber was rolled-up inside the megaesophagus. A wide-bore (36 Fr) gastric calibration tube (GCT) used in bariatric procedures was then passed. Failure of the GCT to pass across the lower esophageal junction (LES) was considered to be confirmatory for the diagnosis of achalasia. A Heller’s cardiomyotomy was performed over the LES (total of 8 cm) and gastric fundus, which enabled the GCT to pass into the stomach. An anterior fundoplication (270°) was used to strengthen the defect and the nasogastric tube was passed till the pylorus.

The patient recovered over 2 weeks postoperatively with enteral and parenteral nutrition. He was discharged on oral feeding on POD 17, and is being considered for elective transthoracic esophagectomy after optimization.

DISCUSSION

Achalasia is an esophageal motility disorder due to neurogenic degeneration which result in hypertension of lower esophageal sphincter (LES), with consequent failure of LES relaxation. Achalasia is categorized based on etiology - primary or idiopathic, and, secondary if caused by parasitic infections like Trypanosoma’s Cruzi seen in chagas disease or, if associated with metabolic disease or tumors [4,5]. Presenting symptoms of achalasia consists of dysphagia, regurgitation and weight loss. The gold-standard test for diagnosis is esophageal manometry which demonstrates esophageal aperistalsis and increase LES pressure.

It is very rare for a patient of achalasia to present with upper gastrointestinal bleeding; the postulated mechanisms are esophageal ulcer or severe esophagitis due to loss of the mucosal protective barrier by the uncontrolled gastric acid reflux and by prolonged stasis [8]. Infections like Candida species, cytomegalovirus, and herpes simplex virus may be contributory in immunocompromised patients. Direct mucosal damage from the ingestion and stasis of caustic agents like tannins, nonsteroidal anti-inflammatory drugs (NSAIDs), bisphosphonates, and certain antibiotics may also complicate a pre-existing achalasia. In extreme cases, achalasia may present as near-fatal esophageal bleeding. Other long-standing complications can be megaesophagus, aspiration pneumonia, esophageal rupture, malnutrition, esophageal squamous cell carcinoma [2,9]. It is evident that patients who develop large episodic hematemesis from underlying ulcers with end-stage complications like megaesophagus, malignancy and cases refractory to medical treatment need to undergo esophagectomy [10].

Our patient presented with upper GI bleed with massive esophageal dilation mimicking hiatus hernia on UGI endoscopy and imaging. The diagnosis of achalasia was confirmed intra-operatively after failure to identify any intra-abdominal pathology. The standard surgical treatment involves pneumatic dilation or Heller’s cardio myotomy which both aim to reduce LES resting pressures. We performed Heller's cardio myotomy with anterior fundoplication as a temporizing measure in our moribund patient; we have further planned for elective transthoracic esophagectomy after optimization.

There are inherent challenges in the diagnosis and management of complicated achalasia, as evident from our case. With a large sigmoid dolichol mega esophagus undiagnosed previously, investigations like CT and endoscopy may also be limited in the emergency situation. The endoscope is often limited by the amount of blood, necrotic luminal contents and the increased tortuous length. Multiple endoscopies may be required to clearly diagnose the condition, since manometry is not a feasible option in these situations. This has also been documented in earlier reports [2]. Probably, intra-operative confirmation as we performed is the only feasible method.

CONCLUSION

This case demonstrates rare case of long-standing achalasia with megaesophagus that mimics hiatus hernia. Multiple endoscopies may be required to clearly diagnose the condition, since manometry is not a feasible option in these situations.