Annals of Pediatric Surgery Vol 5, No 4, October 2009, PP 228-232 Original Article Cricopharyngomyotomy as a Complimentary Procedure to Nissen's Fundoplication in Mentally Retarded Patients Talal A Al-Malki College of Medicine and Medical Sciences, Taif University, Department of Pediatric Surgery, Alhada Armed Forces Hospital. Taif, Kingdom of Saudi Arabia Background/Purpose: Gastroesophageal reflux (GER) in mentally retarded patients is often associated with cricopharyngeal spasm which manifests itself by repeated aspiration pneumonia and chest infections. This work aims to evaluate Cricopharyngomyotomy as a complimentary procedure to Nissen's fundoplication in mentally retarded children diagnosed with gastroesophageal reflux associated with cricopharyngeal spasm. Materials & Methods: The study includes nine patients. Preoperative workup included esophagogram, esophagoscopy, and cine-radiography to diagnose concomitant cricopharyngeal achalasia. All cases had Nissen's fundoplication and Cricopharyngomyotomy, together with a feeding gastrostomy. Results: Early post operative complications occurred in three cases and included chest infection, wound infection and intestinal obstruction. Late complications included recurrence GER, tight fundoplication and aspiration pneumonia. There were three mortalities. Operative management strategy effectively controlled the disease and its concomitant associates . Conclusion: A high index of suspicion is required to diagnose concomitant existence of cricopharyngeal spasm with GER. Cricopharyngomyotomy is an excellent complimentary procedure to Nissen fundoplication when cricopharyngeal spasm is present. Index Word: Cricopharyngeal achalasia, Gastroesophageal reflux, Mental retardation, Nissen's fundoplication. INTRODUCTION brahams and Burkitt 1 were the first 7. Another problem sometimes encountered in A investigators to establish the association of management of GER in mentally retarded patients is mental retardation (MR) with gastro-esophageal cricopharyngeal achalasia. The spasm of the reflux (GER). Significant vomiting occurs in 10-15% of cricopharyngeal muscle may be secondary as a severely mentally retarded children 2, of whom 75% compensatory mechanism with gastroesophageal have GER 3. The tendency to ascribe the vomiting to reflux 8, or secondary to the associated diseases such psychological causes has led to delayed diagnosis and as Chiari malformation 9, 10 or syringomyelia 11. It can has often resulted in complications from reflux also be secondary to poliomyelitis, cerebrovascular esophagitis4. Medical management of GER in this accident, neuritis, thyrotoxicosis, trauma, muscular group has a high failure rate 5, whereas surgical dystrophy, pseudo bulbar palsy, amyotrophic lateral management results in excellent long-term outcome 5- sclerosis, oculopharyngeal syndrome, and post ------------------------------------------------------------------------------------------------------------------------------------------------------------------------ Correspondence to: Dr. Talal A. Al-Malki, College of Medicine and Medical Sciences,Taif University, P. O. Box 888, Taif, Kingdom of Saudi Arabia, Telephone: 00 966 2 7242295,Fax: 00 966 2 7250528 E-mail: [email protected] Al-Malki T. vagotomy states 8. A primary form of cricopharyngeal were six boys and three girls, with average age of 3.2 achalasia was also described, but very rarely years (range 5 months to 9 years).The etiology of the encountered and only fewer than 50 pediatric cases mental retardation was microcephaly in three cases, have been reported 12. When GER is associated with birth asphyxia (two cases) and Down's syndrome cricopharyngeal achalasia, the condition manifests (four cases). Epilepsy was an association in four cases. itself by recurrent chest infection and aspiration Only two children (both with Down syndrome) were pneumonia. A third problem that should be of average weight. The rest were all less than fifth addressed in the management of this group of percentile. All patients were fed through a nasogastric patients is that they tend to be more malnourished tube. The presenting symptoms were vomiting, than patients with normal central systems and GER 13. dysphagia, recurrent chest infection, hematemesis, Feeding of the severely MR patient by mouth is, at apnea and salivation (Table 1). Investigations best, difficult, as evidenced by severe malnutrition. included barium swallow/ upper GI series and Nasogastric feedings incur the risks of improper tube esophagoscopy to demonstrate reflux, the presence of placement, reflux aspiration pneumonia and esophageal stricture, and the presence of a esophageal stricture. Nasoduodenal and nasojejunal concomitant pathology such as hiatus hernia. All nine feeding tubes are difficult to place and maintain 14. cases had cine-esophagogram to document the Investigation modalities for GER include barium presence of cricopharyngeal achalasia. esophagogram, to demonstrate the reflux and if complicated with a distal esophageal stricture, Pre-operative preparation esophagoscopy to document reflux esophagitis and pH monitoring probe 13. If cricopharyngeal achalasia Patients were admitted few days before surgery. All is expected, investigations are advised to include cine- patients were put on Bethanechol and/or esophagogram with fluoroscopic observation of the Metclopramide, antacids, nasogastric tube feeding in swallowing which shows regular posterior narrowing an upright position. Respiratory therapists were on the posterior wall of the pharyngoesophageal involved in pre- and postoperative care to help in junction (posterior bar) at the level of C 4, 5 with alleviating and preventing postoperative pulmonary enlargement of the hypopharynx 15, 16. Manometric complications. A broad spectrum antibiotic was given study of the upper esophageal sphincter (UES) shows with the anesthetic medications. hypertensive UES with failure of relaxation at the highest pressure of the pharynx suggesting Surgery incoordination in deglutition. This study is difficult to Nissen's fundoplication and gastrostomy: A plastic perform in infants because the distance from the loop was passed behind the abdominal part of the pharynx to the cricopharyngeal muscle is short and esophagus to pull it out. Mobilization of the the manometric probe is prone to slip out at abdominal esophagus was carried out and a portion 17 swallowing . Treatment strategy of this group of of the gastric fundus was passed from the left side patients should not only deal with GER but also behind and around to the right side of the esophagus. address the problem of the concomitant Sutures were taken from fundus to esophagus to cricopharyngeal spasm if present. The aim of the fundus thereby securing a circumferential wrap of present study was to evaluate the cricopharyngeal stomach around the esophagus. The operation was myotomy as a complimentary procedure to Nissen ended by performing a gastrostomy in the standard fundoplication in mentally retarded patients with way. GER and documented cricopharyngeal achalasia.. Cricopharyngomyotomy: The cricopharyngeus muscle was approached via a left transverse cervical PATIENTS AND METHODS incision at the level of the cricoids’ cartilages. The sternomastoid muscle was separated, and the The study was conducted during the period April omohyoid was divided or retracted. The posterior 2000 to June2007. Out of 50 severely retarded children part of the pharyngoesophagus was exposed by with significant gastroesophageal reflux, nine patients rotating and retracting the larynx anteriorly and the had documented cricopharyngeal dysfunction. They carotid sheath posteriorly. The transverse fibers of the 229 Vol 5, No 4, October 2009 Al-Malki T. cricopharyngeus muscle were identified and incised infection and intestinal obstruction. (Table 3). Late longitudinally with extension of the incision above morbidity (after hospital discharge) occurred in and below the muscle over approximately four another three patients including recurrence of GER (in centimeters. This incision cut through all layers and one patient, necessitating redo fundoplication), tight stopped short of mucosa. All muscle fibers were fundoplication (in one patient, necessitating a short divided for the myotomy to be effective. A stretch of term dilatation schedule) and aspiration pneumonia the muscle fibers helped to achieve complete division. in one case. Mortality occurred in three cases. Two This stretch could be done by lower pharyngeal and cases died during the hospitalization period due to esophageal distension with an esophageal dilator or a sepsis, which did not respond to medical treatment. balloon dilator, taking care not to injure delicate Another late mortality (40 days post operative) structures and leading to recurrent laryngeal nerve occurred due to aspiration pneumonia. All nine damage or pharyngocutaneous fistula. patients had immediate dramatic improvement after Crico-pharyngomyotomy in terms of deglutition and relief from repeated chest infection. Following the Postoperative care case for an average of 24 months (range 12-36 A broad spectrum antibiotic was used to cover the months), the average percent increase in body weight next 24 hours. Respiratory therapists were involved to (calculated by dividing the current /pre operative give pulmonary toilet. Patient was kept fasting. weight *100) was 25% (range 10-50%) When intestinal movement was resumed (usually on 3rd post operative day), feeding from gastrostomy tube was started.