for End-Stage Achalasia

Achalasia subtype

Integrated relaxation pressure (mm Hg)

Peristalsis

Additional considerations

Type 1

>15 mm Hg

100% failed

Premature contractions with DCI <450 mm Hg/s/cm can serve as surrogate for failed peristalsis

Type 2

>15 mm Hg

100% failed

Panesophageal pressurization with ≥20% swallows

Type 3

>15 mm Hg

No normal peristalsis

Premature contractures, DCI >450 mm Hg/s/cm with >20% swallows

../images/473262_1_En_10_Chapter/473262_1_En_10_Fig1_HTML.jpg — Fig. 10.1

End-stage esophagus characterized by dilation, tortuosity, and distal kinking

Achalasia affects 1.6 per 100,000 people, occurring equally in men and women between 30 and 60 years of age [5–7]. Patients with end-stage achalasia typically experience dysphagia, recurrent food impaction, poor esophageal clearance, aspiration, recurrent pneumonia, heartburn, occasionally GI bleeding, and retrosternal chest pain [8]. They are also at increased risk for esophageal squamous cell cancer [9, 10].

Descriptions of the spectrum of achalasia presentation and esophageal morphology fill the literature. One of the earliest studies by F.G. Ellis followed 85 patients diagnosed with achalasia between 1933 and 1948 [11]. Three stages of achalasia were described: onset, silent period, and progressive deterioration. Achalasia was described as a disease with periods of quiescence and sporadic progression. But the exact timing and mechanisms through which a normal-sized esophagus transitioned from normal to dilated size were unclear.

More recent analyses provided description of manometric findings of achalasia. But there is little clarity on the risk of each subtype evolving into megaesophagus [12]. It is presumed that a non-resolved fixed distal esophageal obstruction eventually may lead to the development of a dilated, bag-like esophagus. Probably, it takes between 12 and 15 years for a normal-size esophagus to degenerate into a dilated and often sigmoid one [13, 14].

Achalasia Pathophysiology

Pathologic examination of surgical explants suggests that achalasia is an idiopathic neuronal degenerative disease, caused by T-cell lymphocyte destruction of enteric neurons in the distal two-thirds of the esophageal smooth muscle [15–18]. Infectious and congenital etiologies have also been suggested [19].

There is homology between end-stage achalasia and megaesophagus in Chagas disease. The flagella protozoa Trypanosoma cruzi is transmitted to humans by the Triatominae bug subfamily [20]. Chagas disease occurs in acute and chronic phases [21]. The acute phase can be symptom-free and occurs in patients typically under 1 year of age. If symptoms do occur, they are generalized and nonspecific: fever, inflammation at inoculation site, lymphadenopathy, and palpebral swelling. This phase lasts 4–8 weeks; approximately 30% of patients will develop subsequent systemic sequelae of the disease. In the gastrointestinal tract, this has been described as organomegaly of the esophagus, stomach, duodenum, jejunum, gallbladder, and colon [22]. Visceral explant analysis has shown that Chagas-affected esophagus demonstrates inflammation and fibrosis of the muscularis propria and myenteric plexus; mononuclear cells are surrounded by eosinophils. Mast cells and rare plasma cell also fill the muscularis and myenteric plexus.

Indications for Esophagectomy

Esophagectomy for end-stage achalasia is indicated for symptomatic patients who suffer from megaesophagus, have failed prior treatment (balloon dilation, Heller myotomy or POEM), and show radiographic evidence of disease progression [3, 4]. Careful preoperative assessment must be performed, and patients must receive full counseling on the details and potential complications of esophagectomy.

Achalasia and Esophagectomy History

In the twentieth century, surgical experiences with achalasia and esophagectomy paralleled each other. The fields did not intersect however until the late 1970s.

Surgical History of Esophageal Achalasia

On April 14, 1913, Ernst Heller (1877–1964) performed the first longitudinal esophageal myotomy in a 49-year-old German man who presented with a pharyngeal food impaction [23, 24]. He performed an anterior and posterior myotomy due to his discontent with the intraoperative visual appearance of the esophagus after anterior myotomy alone.

Four years later, Heller’s original longitudinal esophagomyotomy evolved into an anterior myotomy (“modified Heller”) [25, 26]. This procedure was quickly popularized and, with the addition of a partial fundoplication to reduce postoperative gastroesophageal reflux, is currently the accepted surgical technique for treating achalasia [4, 27]. The first laparoscopic Heller myotomy was performed in 1991 by Cuschieri et al. [28]. Current outcomes with Heller myotomy demonstrate up to 90% symptom improvement. Achalasia guidelines recommend Heller myotomy as the first-line treatment for achalasia. Balloon dilation and POEM also offer less invasive and promising short-term results [29, 30]; POEM and balloon dilation do not provide anti-reflux treatment.

Esophagectomy History

In 1913, Dr. Franz Torek at the German Hospital in New York performed the first successful esophagectomy [31]. Through a left thoracotomy, Torek removed the esophagus of a 67-year-old woman suffering from squamous cell esophageal carcinoma. Gastrointestinal continuity was reestablished through an external prosthetic tube connecting a cervical esophagostomy to gastrostomy. The prosthesis was manually removed after meals. The patient lived 13 years postoperatively.

Subsequent esophagectomy outcomes were poor [32]. Bleeding, uncontrolled pneumothorax, esophageal leak, mediastinitis, esophageal necrosis, pneumonia, and death were commonly observed complications. Suboptimal patient selection, poor understanding of esophageal carcinoma, limited anesthetic capability, lack of standardized surgical technique, and critical care and antimicrobial deficiencies contributed to these results.

Interest in esophagectomy was revived in the late 1930s. In 1938, Adams and Phimester performed esophagectomy with restoration of GI continuity through a left thoracotomy [33]. Sweet replicated this technique and published favorable results of 141 consecutive patients [34, 35].

Sweet’s experience revived esophagectomy and additional surgical techniques were developed. In 1946, Ivor Lewis performed an esophagectomy through a right thoracotomy and midline laparotomy [36]. In 1969, K.C. McKeown performed esophagectomy through right thoracotomy, laparotomy, and right cervical incision [37, 38]. In 1976, Dr. Marc Orringer popularized the transhiatal esophagectomy (THE) [39].

Minimally invasive esophagectomy was developed in the 1990s. Dallemagne et al. performed the first minimally invasive McKeown esophagectomy [40]. Azagra et al. published a series of eight patients who underwent McKeown esophagectomy with thoracoscopic esophageal mobilization, cervical mobilization, and laparotomy [41]. Laparoscopic pioneers from Japan and the United States published outcomes showing reduced morbidity compared to open esophagectomy [42–45]. As the experience with laparoscopy and thoracoscopy grew, minimally invasive procedures have become more popular. Meta-analysis and prospective comparison of thoracic and non-thoracic esophagectomy for cancer patients have shown that transthoracic procedures have higher risk of pulmonary complications, lymphatic leak, and wound complications. Transhiatal surgery has a higher risk of anastomotic leak and recurrent laryngeal nerve injury.

Esophagectomy for Achalasia

In 1977, H.W. Pinotti published the technique of esophagectomy through a trans-mediastinal tunnel in a Brazilian patient suffering from end-stage esophageal dilation due to Chagas disease [46]. The high prevalence of Chagas disease in South America led the largest early series of esophagectomy for megaesophagus to come from Brazil.

In 1988, Pinotti’s group published a series of 108 patients who underwent THE for achalasia, reporting a 3.4% mortality [46]. In 1989, Devaney et al. published a series of 26 patients who underwent THE for end-stage achalasia at the University of Michigan, which observed a single reported death [47]. Several other series have been published, reporting favorable results with esophagectomy for end-stage achalasia. Tables 10.2, 10.3, 10.4, and 10.5 detail various aspects of these series.

Table 10.2

Surgical technique , anastomotic location , and follow-up period

Author (year)	N	Surgical technique	Anastomosis location	Follow-up range (mean)
Pinotti et al. (1988) [48]	108	THE – 108 (100%)	Neck – 108 (100%)	NA
Orringer et al. (1989) [49]	26	THE – 24 (92%) McKeown – 2 (8%)	Neck – 26 (100%)	3–91 months (30 months)
Miller et al. (1995) [14]	37	THE – 9 (24%) IL – 12 (32%) McKeown – 11 (29.5%) Distal esophagectomy RY – 5 (13%)	Neck – 20 (54%) Chest – 17 (46%)	1.4–16 years (6.3 years)
Peters et al. (1995) [50]	15	McKeown – 15 (100%)	Neck – 15 (100%)	1–14 years (median 6 years)
Banbury et al. (1999) [51]	32	THE – 21 (66%) Transthoracic – 11 (34%)	Neck – 30 (94%) Chest – 2 (6%)	3–115 months (43 months)
Hsu et al. (2003) [52]	9	Left thoracoabdominal – 9 (100%)	Chest – 9 (100%)	1–12 years (6 years)
Devaney et al. (2001) [53]	93	THE – 87 (93%) McKeown – 6 (7%)	Neck – 93 (100%)	1–190 months (38 months)
Gockel et al. (2004) [54]	8	THE – 6 (75%) McKeown – 2 (25%)	Neck – 8 (100%)	3–92 months (median 43.5 months)
Crema et al. (2005) [55]	30	Laparoscopic THE – 30 (100%)	Neck – 30 (100%)	Not provided
Glatz et al. (2007) [13]	8	IL – 8 (100%)	Chest – 8 (100%)	(median 6 years)
Schuchert (2009) [56]	6	McKeown – 6 (100%)	Neck – 6 (100%)	NA
Crema (2009) [57]	60	Laparoscopic THE – 60 (100%)	Neck – 60 (100%)	6–118 months (NA)
Crema (2017) [58]	231	Laparoscopic THE – 231 (100%)	Neck – 231 (100%)	7 months– 20 years (NA)

THE transhiatal esophagectomy, McKeown three field esophagectomy, IL Ivor Lewis, NA not recorded

Table 10.3

Intraoperative complications

Author (year)	Bleeding	Airway injury	Unplanned conversion to thoracotomy
Pinotti et al. (1988) [48]	2(1.8%)	1(0.9%)	NA
Orringer et al. (1989) [49]	2(7.7%)	0(0%)	2 (7.7%)
Peters et al. (1995) [50]	1(6.7%)	0(0%)	NA
Miller et al. (1995) [14]	2(5.4%)	0(0%)	2 (5.4%)
Banbury et al. (1999) [51]	0(0%)	0(0%)	5 (15.6%)
Devaney et al. (2001) [53]	2(2%)	1(1%)	2 (2%)
Hsu (2003) [52]	0(0%)	0(0%)	NA
Gockel et al. (2004) [54]	0(0%)	0 (0%)	0 (0%)
Crema et al. (2005) [55]	0(0%)	0(0%)	0 (0%)
Glatz et al. (2007) [13]	0(0%)	0(0%)	NA
Schuchert (2009) [56]	1(16.7%)	0(0%)	NA
Crema (2009) [57]	0(0%)	0(0%)	0 (0%)
Crema (2017) [58]	0(0%)	0 (0%)	0 (0%)

NA not recorded

Table 10.4

Postoperative complications

Author (year)	Anastomotic leak	Conduit necrosis	Dysphonia	Pneumonia	Pleural effusion	Pulmonary embolism
Pinotti et al. (1988) [48]	9(8.3%)	0(0%)	NA	9 (8.3%)	23 (21%)	NA
Orringer et al. (1989) [49]	1(3.8%)	0(0%)	2 (7.7%)	NA	NA	NA
Peters et al. (1995) [50]	0(0%)	0(0%)	0 (0%)	1 (6.7%)	1 (6.7% – chylothorax)	NA
Miller et al. (1995) [14]	2(6.2%)	0(0%)	2 (6.2%)	2 (6.2%)	NA	2 (6.2%)
Banbury et al. (1999) [51]	4(13%)	0(0%)	2 (6%)	7 (22%)	1 (3% – chylothorax)	NA
Devaney et al. (2001) [53]	9(10%)	1(1%)	5 (5%)	2 (2%)	2 (2% – chylothorax)	1 (1%)
Hsu et al. (2003) [52]	0(0%)	1(11%)	0 (0%)	0 (0%)	0 (0%)	0 (0%)
Gockel et al. (2004) [54]	1(12.5%)	0(0%)	NA	NA	1 (12.5% – chylothorax)	0 (0%)
Crema (2005) [55]	2(6.7%)	0(0%)	7 (23%)	0 (0%)	0 (0%)	0 (0%)
Glatz et al. (2007) [13]	0(0%)	0(0%)	0 (0%)	0 (0%)	0 (0%)	0 (0%)
Crema et al. (2009) [57]	4(6.7%)	0(0%)	9 (15%)	0 (0%)	8 (13.3%)	0 (0%)
Schuchert et al. (2009) [56]	1(16.7%)	0(0%)	0 (0%)	0 (0%)	1 (16.7%)	0 (0%)
Crema (2017) [58]	11 (4.76%)	0(0%)	18 (7.8%)	NA	22 (9.52%)	NA