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December 2008, Volume 58, Issue 12

Original Article

Achalasia in a Gastroenterology Unit of Karachi

Waquar Uddin Ahmed  ( Pakistan Medical Research Council, Research Centre )
Huma Qureshi  ( Pakistan Medical Research Council, Research Centre )
Mumtaz Maher  ( Department of Surgery, Jinnah Postgraduate Medical Centre, Karachi. )
Ambreen Arif  ( Pakistan Medical Research Council, Research Centre )

Abstract

Objective: To study the presentation of Achalasia and compare the response of pneumatic dilatation with surgery.
Methods: Retrospective analysis of patient's records (January 2000-December 2005) from outpatients department of Pakistan Medical Research Council), Jinnah Postgraduate Medical Centre, Karachi was done. All patients with Achalasia were analyzed. As a protocol endoscopy, esophageal manometry, esophageal transit time and barium swallow was done to establish the diagnosis. Surgery and endoscopic guided pneumatic dilatation were offered to these patients as treatment options. Patients undergoing surgery or pneumatic dilatation were later followed to assess the efficacy and those not responding to second dilatation were also operated and follow up of all these cases were noted.
Results: Forty-six patients (24 males, 22 females) with a mean age of 39.8 ± 15.9   years were analyzed. Dysphagia was the primary symptom in thirty eight patients (83%) followed by vomiting and epigastric pain. Pneumatic dilatation was performed in 32 out of forty-six patients. Out of these cases 22 (69%) had single, and 10 (31%) had two dilatations. Two patients (6%) had perforation, one required emergency surgery, another patient was managed conservatively and recovery was unremarkable. Six patients (19 %) later required surgery. Fourteen cases opted for surgery as a primary treatment. Out of 20 patients operated, four (20%) required post operative dilatation and one (5%) developed gastro-esophageal reflux.
Conclusions: Achalasia is prevalent in young age, in both sexes almost equally. Pneumatic dilatation is safe and effective, as it can be managed on outpatient basis with little morbidity and 81 % success rate. Surgery is still an effective procedure with comparable 80 % success rate (JPMA 58:661; 2008).

Introduction

Achalasia is one of the best understood oesophageal motility disorders, derived from Greek terminology meaning "failure to relax". It was described by Thomas Willis in 16741 and treated by dilatation of the lower oesophageal sphincter with whalebone, which relieved dysphagia and other obstructive symptoms.2 It is characterized by the absence of muscular contractions in the distal two-thirds (smooth muscle) of the esophagus which results in failure of the lower oesophageal sphincter to open/relax and obstructs the passage of food into the stomach. People with achalasia experience a progressive difficulty in eating solid food and in drinking liquids which evolves gradually in a period of years. They often experience regurgitation, and sometimes have spasm-type chest pain.
The reasons for failure of the oesophageal muscles to contract normally in patients with motility disorders, including achalasia, are unknown.3 In patients with achalasia there are nerve cells within the muscle layers of the esophagus that appear to degenerate for reasons that are not currently understood. These denervation and reduction of post-ganglionic nerve fibers have been postulated to be related to the hypertensive or non-relaxation of LES in patients with achalasia.4 Recently it has been suggested that the reduction of intramural ganglion cells might be a secondary change, probably due to inflammation triggered by autoimmune mechanisms or a chronic degenerative process of the vagal nerve. The primary lesion could be a severe myopathy of the smooth muscle cells.5
The treatment options of achalasia are medications that relax smooth muscles, botulinum toxin injection pneumatic dilatation6 and surgical myotomy.7,8 The efficacy of pneumatic dilatation has been reported up to 95%.9 Similarly treatment success with botulinum toxin and surgery up to 76% and 89% respectively. However the effect of botulinum toxin injection is short lived and needs retreatment in up to 81% within one year.9,10 This study was conducted to see the response of pneumatic dilatation in achalasia patients and compare its efficacy with the surgical treatment.

Patients and Methods

A retrospective analysis of patient's records from January 2000 to December 2005 was conducted at Pakistan Medical Research Council and Research Centre at Jinnah Postgraduate Medical Centre at Karachi, to see the pattern of Achalasia and its response to pneumatic dilatation versus surgery. Medical history and subjective evaluation of clinical symptoms before endoscopy were recorded. Upper G I Endoscopy, Oesophageal Manometry, Oesophageal Transit Time (OTT) and barium studies were done to establish the diagnosis. Dysphagia due to other causes or achalasia secondary to tumour was excluded.
Patients were given two options of therapy either surgery or pneumatic dilatation. Those who opted for surgery were sent for surgery, whereas those who asked for dilatation, initial dilatation was done using 35 mm polyethylene balloon dilator (Microvasive Rigiflex). The balloon was inflated to a pressure ranging from 10-12 psi for 10 seconds and was then deflated upon withdrawal; presence of blood was noted on the balloon as a confirmation of effective procedure. Endoscope was reintroduced to evident the tear at the site of dilatation (as facility of fluoroscopy was not available). Later on these patients were followed up in out patient's clinic for their response evaluation. Patients who did not respond were subjected to a second dilatation using the same protocol at 4 weeks and then followed in the out patients. Those who did not respond to second dilatation were referred for surgery and then followed up. Similarly those who were electively operated were also followed in the outpatient clinic for at least one year to observe the efficacy of the procedure.

Results

A total of forty-six patients with achalasia (24 males and 22 females), with a mean age of 39.8 + 15.9 years (age range 21-60 years) were retrospectively analyzed. Dysphagia was the primary symptom in thirty eight patients (83%), followed by vomit in 54% and epigastric pain 30%. Endoscopy showed resistance to the passage of scope in 43 (93.4%), a peristalsis/minimal peristalsis in 23 (50%), tortuous/dilated oesophagus in 16 (34.7%) patients, and inability of the scope to bypass cardio-oesophageal junction in 3 (9.3%). The examination was completed after oesophageal dilatation. Dilatation was done with savary gilliard dilator size 12 just to pass the scope and to exclude secondary achalasia due to stricture or growth in the oesophagus and fundus. All the cases with oesophageal stricture or neoplasm were excluded by taking biopsies prior to the inclusion as a case of Achalasia. Oesophageal manometry was done after two weeks, on any patient who required oesophageal dilatation. In this series one patient required a guide wire to pass the manometry nasal catheter, because of a very tight lower oesophageal sphincter.
OTT (oesophageal transit time) showed distal oesophageal hold up of the dye, incomplete relaxation of the lower oesophageal sphincter and delayed oesophageal transit time. Oesophageal manometry showed absence of peristalsis or very low amplitude peristaltic waves, elevated lower oesophageal sphincter pressure with incomplete relaxation of the lower oesophageal sphincter.
Out of forty-six patients, 32 underwent pneumatic balloon dilatation and fourteen patients opted for surgery. In all 32 patients initial dilatation was done using a 35 mm balloon with 10 to 12 psi pressure, repeat dilatation based on symptom assessment was done after 4 weeks in 10 patients and 35 mm balloon was used up to 10-12 psi, pressure for 10 seconds.
Post dilatation complications were observed in 2 patients who had perforation and were referred to the surgical department, where one was conservatively managed with uneventful recovery but other patient was operated upon for repair. Six patients (19%) who had dilatation initially were referred for surgery due to the failure of effective oesophageal dilatation.
Fourteen patients were operated with uneventful recovery. Of the total 20 patients operated, four (20%) required post operative dilatation within one year (two patients after one month, one at five months and one at ten and half months after the surgery) and only one (5%) patient developed  gastro-oesophageal reflux.

Discussion

Achalasia is the best understood oesophageal motility disorder in which definitive treatment can be offered to the patients. Currently there are four options of treatment for idiopathic achalasia: pharmacological therapy, pneumatic dilatation, botulinum toxin injection and surgical (Hellers) myotomy. Pharmacological therapy with: isosorbide nitrate or calcium channel blockers are of limited value due to short term effect and are being used only for patients in poor condition or who decline any other definitive treatment.11,12
There is a long standing controversy between the choice of pneumatic dilatation and Heller myotomy for treating achalasia. Usually gastroenterologists prefer pneumatic dilatation as a first choice and reserve surgery for cases where dilatation fails or ends up with complications like perforation. The rationale behind this thinking is emerging data with good result of pneumatic dilatation ranging from 65 to 90%. It is less expensive,13 requires no hospitalization,14 requires limited recovery period and subsequent rapid return to normal life and there is minimum risk of post dilatation gastro-oesophageal reflux. In this study an open choice was given to the patient to undergo surgery or to have pneumatic dilatation because not everyone would like to have a painful procedure without general anaesthesia. Seventy percent of our patients under went pneumatic dilatation on their own discretion. Out of these cases 31% required a second dilatation. Six patients (19%) had relapse of symptoms within six months and ultimately required surgery. Cumulative success rate of pneumatic dilatation in this series was 81%, which is comparable with previous studies.13 Two patients (6%) in this series had perforation, which is within the range of reported series15 (0-18%, with an average of 5%), one of them managed conservatively and the other required surgical repair. In this study short duration dilatation was done because it has been experienced earlier in a local study that short duration (6-sec) pneumatic dilatation is as effective as longer duration (60-sec) in the treatment of achalasia and is without any perforation.16
Fourteen patients were operated electively and 6 patients who did not respond to pneumatic dilatation were later operated. Out of these 20 patients having surgical Heller's myotomy, four (20%) developed post operative stenosis. Giving a success rate of 80%, showing almost similar efficacy as dilatation, this is contrary to the reported series of a prospective randomized trial of surgery versus dilatation therapy, favouring surgery 93% vs. 62% respectively.17
This study gives a preliminary local data of the outcome of pneumatic dilatation and surgical myotomy with encouraging results. Results of multiple dilatations with gradual increase in the size of dilators with long follow-ups are emerging from local studies.18 The results of laparoscopic myotomy are also being presented,19,20 which will further improve the quality of surgical interventions like shorter hospital stay and recovery time and decreased incidence of postoperative gastro oesophageal reflux.

References

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