Achalasia is a rare motility disorder of esophagus and is characterized by increased resting pressure of lower esophageal sphincter and reduced motility of the body. This results in functional obstruction and failure of relaxation of the lower esophageal sphincter (LES). The classic presentation is difficulty in swallowing and vomiting of undigested food, and children can often present with chest pain. In some instances, these symptoms can lead to considerable weight loss.
This condition is relatively rare in young children, and although some cases have an early onset, most are diagnosed in late childhood or early adolescence. We are yet to come across any reported case of achalasia in a 6 month old.
The classical treatment of achalasia in children is surgical division of lower esophageal sphincter, which decreases the sphincter pressure (Heller’s cardiomyotomy). This is done either by thoracotomy or by laparotomy, and used to be a formal undertaking. The development of
endoscopy and balloon dilatation of the sphincter, which aims to fracture the sphincter from within, rapidly gained popularity due to its relative non-invasive nature. Although 90% success rate has been claimed during the first year of follow up, the advantage peters away on long-term study, necessitating repeated dilatation. Endoscopy guided injection of Botulinum toxin into the sphincter is another option as Botulinum decreases sphincter pressure. But the results are short lived, and the inflammatory response and fibrosis makes subsequent surgery rather difficult.
The ideal antireflux procedure following laparoscopic Heller myotomy for achalasia is controversial. The authors present a laparoscopic technique of Heller’s cardiomyotomy with partial anterior fundoplication to bolster the myotomy .
A 6 month old baby presented to us with persistent vomiting from 2 months of age and failure to thrive. He weighed 4 kg at 6 month of age. His other systemic examinations were normal. A barium study was done which showed holding up of barium, narrowing of the distal esophagus and delayed passage of barium into the stomach. Findings were consistent with Achalasia cardia (Fig. I). Upper GI endoscopy was not possible because of unavailability of a miniature infant endoscope. In a joint meeting with the pediatric gastroenterologist it was decided worthwhile to consider for surgery as repeated dilations will be more traumatic, stressful and morbid for a 6 month old infant with 4 kg weight. The child was taken up after anesthetist deemed the child fit for a laparoscopic Heller’s cardiomyotomy with Dor’s anterior fundoplication.
A 24 Fr Nasogastric tube was introduced orally (Fig. II), over which the repair was done. A pneumoperitoneum was created using the Hassan’s technique, and 5 ports were inserted (Fig. III). 4 ports were 5 mm and a 3mm epigastric port was used to retract the left lobe of liver. A 5 mm 30 degree scope was introduced through supraumbilical port in the midline. The abdominal esophagus (AO) was identified and the phreno-oesophageal ligament divided. The AO was completely exposed and pulled from the cardio-esophageal junction through the left anterior axillary port. A myotomy was performed from 2cm onto the stomach to the upper limit of the AO, about 5-6cm on the anterior aspect of the lower end of the esophagus to the left side of the anterior vagus nerve. The muscle was split with good exposure (bulge) of the mucosa (Fig IV). An anterior fundoplication was done with 2/0 silk sutures taking 3 separate stitches. A stitch of the fundus to the left myotomy and right myotomy was taken separately to keep the myotomy open. (Fig V). Air was pushed with saline over the myotomy to ensure no leak. In the 3rd bite a part of the fundus was stitched to the angle of the hiatus so that the fundus covers over the bulged esophageal mucosa. The wounds were then closed with absorbable sutures and an adhesive dressing was applied (Fig VI).
Post-operatively, the patient was kept fasting for 48 hours. The baby was commenced on a liquid diet through the nasogastric tube after 2 days. Injectable co-amoxiclav was administered for 5 days with rectal paracetamol suppository for pain relief. Patient’s length of stay was 5 days, till he was started on oral feeds after removing the nasogastric tube. The patient was reviewed in our clinic 6 weeks after discharge from hospital, and was well, he had no difficulty in swallowing with no further episodes of vomiting with satisfactory weight gain.
The predominant pathological process of achalasia involves the loss of ganglion cells from the wall of the esophagus, and this starts at the LES and develops proximally. The loss of nerves along the esophagus causes a lack of peristalsis, which in turn leads to stasis of undigested food and subsequent dilation of the esophagus. There are several forms of treatment for achalasia which include both conservative and surgical management, and the decision of which to choose involves the consideration of multiple clinical and economic factors.
Conservative management includes the use of pharmacological agents which do not eliminate the underlying disease but instead, only temporarily improve the condition. These include calcium channel blockers (e.g. nifedipine) and nitrates (e.g. isosorbide dinitrate), which act by compensating for the decrease in the inhibitory neurotransmitter, nitric Oxide (NO), and this facilitates a decrease in the LES tone. In addition, use of these medications on a long term basis usually results in tolerance, and this significantly diminishes its effects over time. Endoscopic therapy of botulinum toxin has been shown to improve the symptoms of dysphagia and regurgitation, decrease the tone of the LES and improve esophageal emptying. However this may need to be repeated on several occasions to maintain an effect. Pneumatic dilatation of the LES has also been shown to be effective and is thought to achieve symptomatic effect while being able to avoid the risks of more invasive surgery. However, there are risks associated with this procedure itself, like esophageal perforation and worsening symptoms of gastro-esophageal reflux . There are no literature supporting such treatment in a 6 month old infant.
Surgical management is well documented in the literature and with minimally invasive surgery being the current trend in management of many surgical cases, it is no surprise that this is becoming the desired method of treatment. Large reviews have documented a 90% overall improvement of symptoms following surgery and this remains as high as 80% after 5 years . There are quite conflicting results, however about the rate of gastro-esophageal reflux after performing a cardiomyotomy, and so the general trend is to do an anterior fundoplication (also known as a Dor Fundoplication) at the same time . Operative complications, as in any other procedure, can occur, but are rare. These include mucosal tearing, perforation or post operative leakage . Surgical therapy after failed pneumatic dilatation has also been shown to be very effective.
Considering the age of the child and a narrow working space between the liver and the hiatus, it was a very demanding procedure completed successfully. In essence, the morbidity associated with this procedure is minimal, and the laparoscopic approach allows for faster recovery, a decreased period of immobility, shorter time to tolerating oral diet and so, decreases the length of hospital stay. Overall, laparoscopic Heller’s cardiomyotomy provides a safe and effective alternative for children presenting with esophageal achalasia . We report the rare occurrence of Achalasia cardia in a 6 month old infant treated by a demanding laparoscopic Heller’s cardiomyotomy with Dor’s anterior fundoplication.
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About the Authors:
Prakash Agarwal, Professor, Department of Pediatric Surgery, Sri Ramachandra University, Porur, Chennai – 600116.
R.K.Bagdi, Ex Professor, Department of Pediatric Surgery, Sri Ramachandra University, Porur, Chennai – 600116.
S. Balagopal, Professor, Department of Pediatric Surgery, Sri Ramachandra University, Porur, Chennai – 600116.
R Madhu, Associate Professor, Department of Pediatric Surgery, Sri Ramachandra University, Porur, Chennai – 600116.
P. Balamourougane, Associate Professor, Department of Pediatric Surgery, Sri Ramachandra University, Porur, Chennai – 600116.
Moorthy G,Assistant Professor, Department of Pediatric Surgery, Sri Ramachandra University, Porur, Chennai – 600116.
Manoj kumar, Senior resident, Department of Pediatric Surgery, Sri Ramachandra University, Porur, Chennai – 600116.
Dr. Prakash Agarwal
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Manapakkam, Chennai – 600116
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