Journal of Pediatric Surgery (2006) 41, 919 – 922
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Total esophagogastric dissociation: 10 years’ review Antonino Morabitoa,*, Anupam Lalla, R. Lo Piccoloa, Helen McCarthyb, Lisa Kauffmannc, Shiban Ahmeda, Adrian Bianchia a
Department of Paediatric Surgery, Central Manchester and Manchester Children’s University Hospitals, Manchester, M27 4HA, UK b Dietetic Department, Central Manchester and Manchester Children’s University Hospitals, Manchester, M27 4HA, UK c Central Manchester PCT, Central Manchester and Manchester Children’s University Hospitals, Manchester, M27 4HA, UK Index words: Gastroesophageal reflux; Neurological impairment; Total esophagogastric dissociation
Abstract Purpose: Neurologically impaired children run a 12% to 45% risk of recurrent gastroesophageal reflux (GER) after fundoplication. Elimination of the reflux by brescueQ total esophagogastric dissociation (TEGD) encouraged us to use it also as a bprimaryQ form of antireflux surgery in this group of patients. Methods: Twenty-six (14 male, 12 female) patients underwent TEGD between 1994 and 2004, of which 16 were primary and 10 were rescue procedures for failed fundoplication. Results: There was no operative mortality and postoperative complications were limited to one subphrenic collection, one esophagojejunal dehiscence, and one small bowel hernia beneath the jejunal Roux loop. Gastrostomy feeding was usually established by 3 to 5 days and the mean hospital stay was 10.2 days (range, 6-18 days). At follow-up of 7 months to 11 years, there was no recurrence of GER. Four late deaths were unrelated to the surgery. The children’s nutritional status improved with the mean weight standard deviation score showing a statistically significant increase from 2.63 preoperatively to 0.96 postoperatively (Wilcoxon’s signed rank P value V.005). Conclusions: Total esophagogastric dissociation is a safe definitive solution for GER because it eliminates all risk of recurrent reflux. We therefore feel that TEGD can be used as a primary treatment of choice for severely neurologically impaired patients who are experiencing GER and are completely dependant on tube feeds. D 2006 Elsevier Inc. All rights reserved.
Children with neurological impairment are among the group of high-risk patients who commonly develop gastroesophageal reflux (GER) [1,2]. They are commonly referred for antireflux surgery. After all types of antireflux surgery, these children carry a failure rate of 12% to 45%. Redo antireflux procedures have an even higher rate of recurrence (45%) than the initial operation [3]. Total esophagogastric Presented at the 37th Annual Meeting of the Canadian Association of Paediatric Surgeons, Quebec, Canada, September 22-25, 2005. 4 Corresponding author. Tel.: +44 161 276 6542; fax: +44 161 922 2193. E-mail address:
[email protected] (A. Morabito). 0022-3468/$ – see front matter D 2006 Elsevier Inc. All rights reserved. doi:10.1016/j.jpedsurg.2006.01.013
dissociation (TEGD) was described in 1997 as an alternative approach to treat GER in neurologically impaired children with no ability to swallow. It has been shown to be effective in eliminating reflux completely [4,5]. This study assesses our results with TEGD as a bprimaryQ definitive treatment and as a brescueQ operation after failure of conventional antireflux surgery.
1. Materials and methods Between 1994 and 2004, 26 neurologically impaired children (14 male and 12 female) underwent TEGD under
920 Table 1
A. Morabito et al. The neurological problems in the patients
Neurological problems
Incidence
Cerebral Palsy Chromosomal abnormalities Prematurity Hydrocephalous Muscle dystrophy VACCTERL and cerebral atrophy
53.8% 10.4% 10.4% 7.6% 3.8% 3.8%
(14/26) (4/26) (4/26) (2/26) (1/26) (1/26)
two consultant pediatric surgeons in Manchester, UK. Sixteen children had a definitive primary TEGD and 10 had a rescue TEGD of which 9 were for recurrent GER and 1 for esophageal obstruction after fundoplication. The indications for primary TEGD were severe neurological impairment as graded by the American Psychiatric Association [6], swallowing difficulties, severe GER refractory to medical treatment, recurrent respiratory infections, failure to thrive, and poor quality of life both for the children and for the carers. Parents are asked to consider several options including gastrostomy alone, feeding jejunostomy, TEGD, and fundoplication with gastrostomy. During the consultation, it is made explicitly clear that the choice of procedure should carry the least risk dependant on the condition of their child. Furthermore, where possible, it should be definitive in eliminating the possibility of GER. Children who can swallow and are not dependant on nasogastric tube or gastrostomy feeds are not considered for primary TEGD. At the time of surgical referral, the children’s neurological status had already been determined (Table 1). Six patients had postinfection cerebral palsy, 3 had birth trauma, and 5 had perinatal hypoxia. Chromosomal abnormalities included deletion of chromosomes 5, 3p, 9p, and 6. All the premature patients (32, 30, 24, and 26 weeks) who developed severe mental disability required major resuscitation at birth. Twelve patients also had a severe scoliosis. The symptoms varied from failure to thrive, vomiting, and recurrent chest infections (Table 2). All of these patients were studied preoperatively with video fluoroscopy for assessment of swallowing, a contrast study for assessing reflux and gastric emptying, a 24-hour esophageal pH study, and an esophagoscopy to assess degree of esophagitis. The operative procedure is carried out under general anesthesia with muscle relaxants as described by Bianchi [4].
Table 2 patients
The incidence of preoperative symptoms in the
Symptoms
Incidence
Failure to thrive Recurrent chest infection Vomiting Dysphagia Retching
24/26 25/26 20/26 19/26 25/26
(92.3%) (96.1%) (76.9%) (73.0%) (96.1%)
Fig. 1 The esophagus is transected above the gastroesophageal junction and the stomach oversewn (A). An isoperistaltic antirefluxing Roux-en-Y loop of jejunum is brought without tension, through the transverse mesocolon, passing behind the stomach to anastomose with the lower esophagus. Bowel continuity is established by end-to-side jejunojejunostomy at 40 cm from the esophagojejunal anastomosis (B).
A transverse or oblique incision was made in the left hypochondrium and the left lobe of the liver transposed medially. Unraveling any residual fundal wrap at the gastroesophageal junction restored lower esophageal anatomy. The esophagus was transected just above the gastroesophageal junction and the stomach oversewn (Fig. 1A). An isoperistaltic antirefluxing Roux-en-Y loop of jejunum on a reliable mesenteric vascular pedicle was brought without tension, through the transverse mesocolon, passing behind the stomach to anastomose with the lower esophagus (Fig. 1B). Bowel continuity was established by end-to-side jejunojejunostomy at 40 cm from the esophagojejunal anastomosis. At our center, pyloroplasty was not regarded as essential for primary TEGD unless there was evidence of delayed gastric emptying. Concern or suspicion of vagal nerve injury in rescue patients was also an indication for pyloroplasty. When possible, a preexisting gastrostomy was preserved; otherwise, a new gastrostomy was fashioned. Twelve patients had an intermittently catheterizable nonrefluxing gastrostomy fashioned from the greater curve of the stomach [7]. Free gastrostomy drainage was maintained postoperatively and the esophagojejunal anastomosis was protected by nasojejunal tube drainage until return of bowel activity. The weight was recorded for all patients preoperatively and was regularly obtained postoperatively from the child’s local dietetic service. Nutritional status was defined using weight for age standard deviation scores (z scores). A z score of 2 is indicative of malnutrition as defined by the World Health Organization [8]. In the follow-up visits, the patient’s weight, number of chest infections needing hospitalization, and any other problems were recorded. The parents and caregivers were specifically asked questions regarding the
Total esophagogastric dissociation: 10 years’ review ease of childcare, quality of time spent with their child, and overall enjoyment of the child.
2. Results There was no operative mortality in either the primary TEGD or the rescue groups. Bowel activity and gastrostomy feeding were established by 3 to 5 days. The mean hospital stay was 10.2 days (range, 6-18 days). The mean operative time for primary TEGD was 125 minutes (range, 90-200 minutes) with minimal blood loss and 240 minutes (range, 190-420 minutes) for rescue procedures with a greater blood loss and blood transfusion in one patient. Morbidity after primary TEGD was limited to one left subphrenic collection requiring drainage and one small bowel hernia beneath the jejunal Roux loop, which required operation. In the rescue group, one esophagojejunal dehiscence was seen, which was managed conservatively. There were 4 late deaths in the rescue group, two died of deterioration of their neurological condition, one died of chronic lung disease and congenital heart disorder, and one died because of hypovolemic shock secondary to severe diarrhea and vomiting (Table 3). The deaths occurred 5 to 79 months after the TEGD and were not related to the operative intervention. The follow-up ranged from 7 months to 11 years with no instance of recurrence of reflux. A suspicion of GER was refuted by a contrast barium study in one child and in two others by radioisotope milk scans. Twenty-four–hour pH studies in the same children, with the probe placed at the esophagojejunal anastomosis, did not show any acid or alkaline reflux. Therefore, we now avoid routine postoperative investigations because it obviates unnecessary stress for the children. The patients who have some ability to swallow still enjoyed oral feeding. The gastrostomy feeds were better tolerated as bigger bolus feed could be administered without any risk of reflux and it became easier for both the children and the carers. Retching was present initially in 96% of our patients; it was observed in follow-up visits that retching slowly improved and became manageable over a period of 1 year in 75% of these cases. The mean weight standard deviation score (z score) at the time of surgery was 2.63 (range, 6.1 to 0). Of the Table 3
Causes of late death in the rescue group
Cause of death Chronic lung disease and congenital heart disease Neurological deterioration Diarrhea and vomiting hypotension and shock and arrest Severe kyphoscoliosis and neurological deterioration
Time after TEGD 5 mo 79 mo 20 mo 59 mo
921 26 patients, 24 showed an improvement in their z score. The mean postoperative weight z score was 0.96 (range, 6.32 to 1.25). Nutritional failure in two patients related to failure on the part of the parents to comply with an adequate feeding regimen. Data were analyzed using Wilcoxon’s signed rank test (nonparametric paired data), with statistical significance demonstrated at P V .005. Analysis of preoperative and postoperative weight z score was considered statistically significant ( P = .005). Two patients (one from each group) required hospitalization for non-aspiration chest infections. Analysis of the questionnaire regarding satisfaction of patients and carers showed improvement in the quality of life of the patient and caregivers.
3. Discussion It is generally accepted that children with profound neurological disability have a markedly decreased life expectancy [9,10]. Martinez et al [10] state that these children will survive for longer if they remain free of GER. Excellent results are obtainable with conventional antireflux management as long as it effectively eliminates reflux. However, Pearl et al [11] sum up the literature when they report that 71% of neurologically impaired children returned within 1 year of conventional antireflux surgery with clinical complaints attributable to GER. The need for a more reliable treatment of GER in neurologically impaired children had long been apparent. In 1997, Bianchi [4] described TEGD as an alternative approach for the management of GER in mentally impaired children, many of whom also experience pharyngeal neuromuscular incoordination and swallowing difficulties. Other series have since been reported, all of which confirm the complete elimination of reflux by TEGD [3,12-15]. Most series report a reduction in hospitalization as a result of the reduction in food aspiration and chest infections. They comment on the improvement in the child’s general health and in the quality of life for both the child and the carer. We believe that the management of GER should be patient specific. Fundoplication (open or laparoscopic) with or without gastrostomy is a good option for children with the ability to swallow, even accepting the risk of failure of the fundoplication. It has been suggested that a feeding jejunostomy (open or laparoscopic) is a less complex procedure [16] and we indeed advocate it for the very high-risk ill children where major surgery carries a far greater risk. However, a feeding jejunostomy, although useful for nutrition, does not stop acid reflux with possible catastrophic consequences. We firmly believe that this is therefore a less acceptable long-term solution. When the ability to swallow is clearly compromised, primary definitive TEGD and gastrostomy offer a superior solution in eliminating reflux and feed aspiration with no possible risk of GER recurrence. It is evident that neither fundoplication
922 nor TEGD or indeed feeding jejunostomy will eliminate the risk of pharyngeal aspiration of saliva or other orally ingested materials. It is difficult to continue to accept that TEGD should only be reserved for rescue after a failed fundoplication because the risk of recurrence is appreciable [11] and secondary surgery is far more difficult. The review of the available literature and our own series suggest that the reported complications after TEGD are minimal and not procedure related. It is our view that TEGD satisfies all the requirements in children with severe neurological impairment with pharyngeal neuromuscular incoordination and severe GER. We feel that as a primary procedure, it offers the benefits of eliminating GER and avoiding recurrence with consequent multiple antireflux surgeries. It remains, however, an excellent rescue procedure after failed fundoplication.
References [1] Kubiak R, Spitz L, Kiely EM. Effectiveness of fundoplication in early infancy. J Pediatr Surg 1999;34:295 - 9. [2] Orestein SR, Di Lorenzo C. Postfundoplication complications in children. Curr Treat Options Gastroenterol 2001;4:441 - 9. [3] Islam S, Teitelbaum DH, Burtain WL, et al. Esophagogastric separation for fundoplication in neurologically impaired children. J Pediatr Surg 2004;39(3):287 - 91. [4] Bianchi A. Total esophagogastric dissociation: an alternative approach. J Pediatr Surg 1997;32:1291 - 4.
A. Morabito et al. [5] Esposito C, Montupet P, Rothenberg S. Gastroesophageal reflux in infants and children. Chapter 31. Berlin7 Springer-Verlag; 2004. p. 225 - 8. [6] Diagnostic and Statistical manual of mental disorder. 3rd ed. rev. Washington (DC)7 American Psychiatric association; 1987. [7] Bianchi A, Pearse B. The non-refluxing gastrostomy: an evaluation. Pediatr Surg Int 1997;12(7):494 - 6. [8] World Health Organisation. Physical status: the use and interpretation of anthropometry. W.H.O. Technical Report Series No. 854, 1995. [9] Eyman RK, Grossman HT, Chaney RH, et al. The life expectancy of profoundly handicapped people with mental retardation. N Engl J Med 1990;323:584 - 9. [10] Martinez DA, Pease MEG, Caniano D. Sequelae of antireflux surgery in profoundly disabled children. J Pediatr Surg 1992;27:267 - 73. [11] Pearl RH, Robie DK, Ein SH, et al. Complications of gastroesophageal antireflux surgery in neurologically impaired versus neurologically normal children. J Pediatr Surg 1990;25:1169 - 73. [12] Danielson PD, Emmens RW. Esophagogastric disconnection for gastroesophageal reflux in children with severe neurological impairment. J Pediatr Surg 1999;34:84 - 7. [13] Gatti C, Abriola F, Villa M, et al. Esophagogastric dissociation versus fundoplication: which is the best for severely neurologically impaired children? J Pediatr Surg 2001;36:677 - 80. [14] Buratti S, Kamenwa R, Dohil R, et al. Esophagogastric disconnection following failed fundoplication for the treatment of gastroesophageal reflux disease (GERD) in children with severe neurological impairment. Pediatr Surg Int 2004;20:786 - 90. [15] Dall’Oglio L, Gatti C, Villa M, et al. A new and successful change in surgical treatment of gastroesophageal reflux in severely neurologically impaired children: Bianchi’s procedure. Eur J Pediatr Surg 2000;10:291 - 4. [16] Han-Geurts IJ, Lim A, Stijenen T, et al. Laparoscopic feeding jejunostomy: a systematic review. Surg Endosc 2005;12(5).