- Email: [email protected]
Esophagogastric dissociation in the neurologically impaired: an alternative to fundoplication?
Journal of Pediatric Surgery (2005) 40, 915 – 919
www.elsevier.com/locate/jpedsurg
Esophagogastric dissociation in the neurologically impaired: an alternative to fundoplication? Anju Goyala,*, Basem Khalila, Kelvin Chooa, Khalid Mohammedb, Matthew Jonesa a
Department of Paediatric Surgery, Royal Liverpool Children’s Hospital, Alder Hey, L12 2AP Liverpool, UK Department of Neurology, Royal Liverpool Children’s Hospital, Alder Hey, L12 2AP Liverpool, UK
b
Index words: Gastroesophageal reflux; Neurologically impaired children; Fundoplication; Esophagogastric dissociation; Antireflux surgery
Abstract Background/Purpose: Gastroesophageal reflux is common in children with severe neurological impairment. Fundoplication may produce symptomatic improvement but has a high failure rate. Esophagogastric dissociation (EGD) is an alternative procedure for treatment of gastroesophageal reflux. The aim of this study is to evaluate the results of EGD in our institution and compare them with a neurologically matched group of children who had Nissen fundoplication. Methods: Twenty consecutive patients who had EGD were retrospectively evaluated and the results were compared with a neurologically matched group of 20 consecutive patients who had Nissen fundoplication. Results: Twenty patients had EGD, 17 as a primary procedure. There was no operative mortality but 5 have died of other causes. Resolution of reflux-associated symptoms occurred in all patients. Of the 15 survivors, 5 remain on antireflux medication. Twenty patients had fundoplication. There was no operative mortality, but 8 patients have died of other causes. Failure occurred in 5 patients necessitating further surgery. Of the 10 unreoperated survivors, 6 remain on antireflux medication. Conclusions: Esophagogastric dissociation is an effective antireflux procedure when compared with fundoplication. It has a lower failure rate. We recommend EGD as a primary procedure in selected children with severe neurological impairment. D 2005 Elsevier Inc. All rights reserved.
Gastroesophageal reflux (GER) and gastric dysmotility are common problems in children with severe neurological Presented at the 56th Annual Meeting of the Section on Surgery of the American Academy of Pediatrics, San Francisco, California, October 8-10, 2004. This project is registered in the Clinical Audit Department of the Royal Liverpool Children’s Hospital, Alder Hey, Liverpool, UK, under project number 504. T Corresponding author. Tel.: +44 0 151 2525361; fax: +44 0 151 2525677. E-mail address: [email protected] (A. Goyal). 0022-3468/$ – see front matter D 2005 Elsevier Inc. All rights reserved. doi:10.1016/j.jpedsurg.2005.03.004
impairment (NI). This may compromise their already limited quality of life by causing nutritional, esophageal, respiratory, and pain-related problems. Fundoplication has been the traditional surgical approach to this condition, but it is associated with a high failure rate of 6% to 25% and with the persistence of unpleasant gastrointestinal symptoms [1- 6]. Several alternatives to fundoplication have been described, including bfeeding jejunostomy,Q bfundoplication with pyloroplasty Q [7-9], and esophagogastric dissociation (EGD). The role of EGD in the management of children with severe GER was first described by Bianchi [10] in 1997. Initially
916
A. Goyal et al.
2. Results 2.1. Patients
Fig. 1
Esophagogastric dissociation.
used as a salvage procedure after bfailed fundoplication,Q favorable results have led to an increasing role as a primary antireflux procedure in selected patients [11,12]. The aim of this study is to compare the results of EGD and Nissen fundoplication in children with severe NI in our institution.
1. Methods Twenty consecutive patients who had EGD at The Royal Liverpool Children’s Hospital between January 2001 and June 2004 were included in the study. Esophagogastric dissociation was offered to patients who were nonverbal, nonambulatory, unable to feed orally, and in whom symptoms of retching, vomiting, and discomfort predominated. All had severe NI with a bgross motor functionQ (GMF) score of 5 [13]. The results were compared with the most recent group of 20 consecutive patients with a GMF score of 5 who had Nissen fundoplication (1998-2004). Data were collected from hospital records and by personal interview/telephonic questionnaire.
1.1. Operative procedure All EGDs were done by a single surgeon using minor variations of the method described by Bianchi [10] (Fig. 1). The choice of incision (upper midline, left subcostal, or left thoracoabdominal) was dictated by patient habitus and previous operative scars. The stomach and esophagus were mobilized as per fundoplication and assessed. A HeinekeMikulicz pyloroplasty was done to aid gastric emptying. A 30 -cm Roux-en-Y loop was constructed and brought up via a retrocolic/retrogastric route. The stomach was then disconnected from the esophagus and the cardia oversewn. Finally, the Roux loop was anastomosed to the lower end of the esophagus. A gastrostomy was fashioned if not already present.
Twenty children had EGD (7 males and 13 females). Their mean age was 5.5 years (range, 7 months-16 years). All had a GMF score of 5 and were nonverbal, nonfeeding (tube fed), nonambulatory, and unable to support their head against gravity. All had severe GER characterized by pain, retching, vomiting, failure to thrive, and recurrent aspiration. Two patients had 4 previous failed fundoplications and 1 patient had a previous failed feeding jejunostomy. Four patients had a tracheostomy for airway management and 8 patients had videofluoroscopic evidence of aspiration. Twenty patients had Nissen fundoplication (14 males and 6 females). Their mean age was 3.4 years (range, 2 months9 years). All had a GMF score of 5 and were neurologically and symptomatically comparable with the EGD group. Three patients had a tracheostomy for airway management and 11 patients had videofluoroscopic evidence of aspiration.
2.2. Procedures Mean operating time for EGD was 238 minutes (range, 120- 470 minutes). Feeds were started at a mean of 5.0 days (range, 4 -7 days) and full feeds were established by 8.4 days (range, 6 -21 days). Mean intensive care stay was 1.6 days (range, 0-9 days). Mean operating time for Nissen fundoplication was 150 minutes (range, 60-240 minutes). Mean number of days for commencement of feeds was 2.7 days (range, 1- 4 days) and full feeds were established by 9.1 days (range, 4- 49 days). Mean intensive care stay was 4.1 days (range, 1-18 days) (Table 1).
2.3. Morbidity/mortality In the EGD group, there was no operative mortality. Two patients had an early complication (1 wound dehiscence and 1 iatrogenic diaphragmatic herniation) requiring uneventful reoperation. One patient required a subsequent pyloroplasty for delayed gastric emptying because this was not done during the original procedure. Five patients have subsequently died of unrelated causes. In the Nissen fundoplication group, there was no operative mortality. There were no early complications. One patient required a late laparotomy for adhesive obstruction and 5 (25%) patients required further antireflux
Table 1
Postoperative analysis of patients
Variables
EGD
Fundoplication
Average operating time (min) Commencement of feeds (d) Full feeds (d) ICU stay (d) Redo procedures needed
238 5.0 8.4 1.6 0
150 2.7 9.1 4.1 5
Esophagogastric dissociation in the neurologically impaired surgery for recurrent symptoms. Eight patients have subsequently died of unrelated causes.
2.4. Current status In the EGD group, 15 patients have survived without the need for further antireflux surgery at a mean follow-up of 13 months (range, 2-32 months). All patients are tolerating full feeds and are gaining weight. Twelve patients are currently free of reflux symptoms. Five (25%) patients remain on antireflux medication. Three patients have occasional vomiting, which does not preclude full feeding. They were evaluated with contrast follow-through studies via the gastrostomy, which did not reveal any evidence of jejunoesophageal reflux. Presumably, they have intermittent mild jejunoesophageal reflux due to bowel dysmotility, which would account for vomiting although we could not demonstrate it. They continue to be on antireflux medication empirically. Another patient has screaming episodes unrelated to feeds, which have been extensively investigated and for which no cause could be found. She continues to be on omeprazole rather empirically again. The fifth patient has gastrostomy-related local soreness for which he is on omeprazole, which is now being weaned. In the Nissen fundoplication group, 10 patients have survived without the need for further antireflux surgery at a mean follow-up of 42 months (range, 19-72 months). All patients are tolerating full feeds and are gaining weight. Six (60%) surviving patients continue to have reflux symptoms and remain on antireflux medication (Fig. 2). They have symptoms of retching and vomiting and 2 have recurrent chest infections. Four had postoperative evaluation for GER with contrast studies. Only 1 of these had evidence of reflux.
3. Discussion Patients with severe neurological disability suffer from a combination of oropharyngeal incoordination, gastric dys-
917 motility, and GER, which seriously compromises their already limited quality of life. They are prone to malnutrition, recurrent aspiration, and esophageal ulceration and frequently experience debilitating discomfort that is often refractory to conventional medical therapy. Fundoplication has been the traditional surgical approach to this problem but is known to have indifferent results in this group of patients, with a high incidence of recurrent symptoms and revision rates ranging from 6% to 25% [1- 6]. This high failure rate has been attributed to a number of factors such as gut dysmotility, seizure disorders, aerophagia [14,15], and central (ie, derived from the central nervous system) pain. Ravelli and Milla [16] propose that many of these symptoms may result from gastric dysmotility and that these children suffer from inappropriate reflex vomiting in addition to passive reflux. They performed electrogastrography in 50 children with central nervous system disorders who suffered from retching/vomiting and found gastric dysrhythmias in 62% of these. Dysrhythmias result from abnormal activation of the efferent limb of the emetic reflex or from lack of inhibition of excitatory fibers as a consequence of either disturbed input to the hindbrain from higher centers or anatomic and functional disturbances of the vomiting center. Although fundoplication may prevent the physical act of vomiting, it would do little to alleviate the underlying neurological problem. Esophagogastric dissociation offers the advantage that by disconnecting the esophagus from the stomach, it eliminates all possibility of GER and also, by virtue of complete vagotomy at the time of operation, eliminates abnormal extrinsic innervation of the foregut. The combined pyloroplasty aids gastric emptying. Esophagogastric dissociation was initially advocated as a salvage procedure after failed fundoplication, but subsequent good results encouraged Bianchi [10] to propose it as primary procedure in this select group of patients. Early reports suggest that EGD produces very good symptomatic results, but concerns remain over the magnitude of the procedure and the potential for serious operative complications. These may include bleeding, anastomotic leakage, necrosis of the Roux loop, perforation, wound dehiscence, diaphragmatic hernia,
Successful Procedures EGD (20) Alive (15)
Nissen (15) Dead (5)
Dead (5)
Symptomatic (3)
Symptomatic(6)
No symptoms 12
No Symptoms 4
Fig. 2
Outcome of patients who did not need reoperation.
Alive (10)
918 adhesive obstruction, and death [17]. Several authors have published their experience with EGD, with significant complications requiring surgical intervention occurring in 20% to 30% of cases [4,12,18]. This high incidence of complications has led some authors to recommend that EGD be reserved as a procedure of last resort [17]. In our series, 2 (10%) patients had early complications requiring operative intervention. In both cases, the children made an uneventful recovery. Despite the apparent magnitude of EGD, postoperative debilitation and bICU stay Q were no worse than for fundoplication. We believe that with appropriate experience, careful patient selection, and good anesthetic support, EGD should not pose a greater risk than other antireflux procedures. We have been very pleased with the symptomatic improvement of patients who have had EGD and have been particularly struck by the general satisfaction of the children’s parents. Spontaneous parental comments have included phrases such as babsolutely fantastic,Q bbest thing that’s ever happened to him,Q and bshe has slept for the first time in seven years.Q Other authors have reported similar satisfaction. However, we have found it very difficult to establish objective outcome measures because these children suffer from numerous comorbid problems that preclude the use of such standard outcome parameters as hospital attendance and hospital stay. Twelve of the 15 surviving patients are largely asymptomatic and are thriving; however, 3 patients continue to have some minor gastrointestinal difficulties. They have occasional minor vomiting but this does not compromise their tolerance of feeds. The vomiting may result from an associated degree of panintestinal dysmotility resulting in jejunoesophageal reflux. Also in retrospect, we feel that in 1 of these 3 patients the Roux loop may have been a bit short. Another child experiences intermittent bouts of unexplained discomfort, which have been extensively investigated, without any obvious explanation becoming apparent. The discomfort is not debilitating, and she remains otherwise well. All in all, we feel that although these patients have residual gastrointestinal symptoms, it is difficult to attribute these to any intrinsic deficiency of the EGD procedure.
A. Goyal et al.
[3]
[4]
[5]
[6]
[7] [8]
[9]
[10] [11]
[12]
[13]
[14]
[15]
[16]
[17]
[18]
influence of central nervous system impairment. J Pediatr Surg 1988;23(7):638 - 40. Martinez DA, Gin-Pease ME, Canniano DA. Sequelae of antireflux surgery in profoundly disabled children. J Pediatr Surg 1992;27(2): 267 - 73. Gatti C, di Abriola GF, Villa M, et al. Esophagogastric dissociation versus fundoplication: which is best for severely neurologically impaired children? J Pediatr Surg 2001;36(5):677 - 80. Pimpalwar A, Najmaldin A. Results of laparoscopic antireflux procedures in neurologically impaired children. Semin Laparosc Surg 2002;9(3):190 - 6. Rice H, Seashore JH, Touloukian RJ. Evaluation of Nissen fundoplication in neurologically impaired children. J Pediatr Surg 1991;25(6):697 - 701. Smith D, Soucy P. Complications of long-term jejunostomy in children. J Pediatr Surg 1996;31(6):787 - 90. Gilchrist BF, Luks FI, DeLuca FG, et al. A modified feeding Rouxen-Y jejunostomy in the neurologically damaged child. J Pediatr Surg 1997;32(4):588 - 9. Okuyama H, Urao M, Starr GA, et al. A comparison of the efficacy of pyloromyotomy and pyloroplasty in patients with gastroesophageal reflux and delayed gastric emptying. J Pediatr Surg 1997;32(2): 316 - 9. Bianchi A. Total esophagogastric dissociation: an alternative approach. J Pediatr Surg 1997;32(9):1291 - 4. Dall’Oglio L, Gatti C, Villa M, et al. A new and successful chance in surgical treatment of gastroesophageal reflux in severely neurologically impaired children: Bianchi’s procedure. Eur J Pediatr Surg 2000;10(5):291 - 4. Danielson PD, Emmens RW. Esophagogastric disconnection for gastroesophageal reflux in children with severe neurological impairment. J Pediatr Surg 1999;34(1):84 - 6. Palisano R, Rosenbaum P, Walter S, et al. Gross motor function classification system for cerebral palsy. Dev Med Child Neurol 1997; 39:214 - 23. Wheatley MJ, Coran AG, Wesley JR, et al. Redo fundoplication in infants and children with recurrent gastroesophageal reflux. J Pediatr Surg 1991;26(7):758 - 61. Turnage RH, Oldham KT, Coran AG, et al. Late results of fundoplication for gastroesophageal reflux in infants and children. Surgery 1989;105(4):457 - 64. Ravelli AM, Milla PJ. Vomiting and gastroesophageal motor activity in children with disorders of the central nervous system. J Pediatr Gastroenterol Nutr 1998;26:56 - 63. 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 [Electronic publication, May 12, 2004]. Islam S, Teitlebaum DH, Buntain WL, et al. Esophagogastric separation for failed fundoplication in neurologically impaired children. J Pediatr Surg 2004;39(3):287 - 91.
4. Conclusion We believe that EGD is a safe and effective alternative to fundoplication and recommend that it be considered for the treatment of GER in children with severe NI.
References [1] 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(11):1169 - 73. [2] Tuggle DW, Tunnel WP, Hoelzer DJ, et al. The efficacy of Thal fundoplication in the treatment of gastroesophageal reflux: the
Discussion Phil Frickman, MD (Los Angeles, CA ): I enjoyed your paper very much. One of the questions I had about your slides was that your follow-up for the EGD group was only 13 months on average, whereas your follow-up for the fundoplication group was substantially longer, which may account for your higher death rate in your other patients. I’m not sure these results support your conclusions necessarily. Could you comment on that?
Esophagogastric dissociation in the neurologically impaired Anju Goyal, MRCS (Liverpool, UK ): Yes, as you can see, the follow-up in the fundoplication group was much longer. This is because we tried to take the patients who were neurologically matched, and since we have started doing the EGD procedure, we have done very few fundoplications so we had to actually go back and take patients who had fundoplications probably a few years prior to the EGD. So naturally the follow-up was longer, but the deaths in all the cases were unrelated so I suppose that should probably not have any influence on the conclusions. Jed Nuchtern, MD, FAAP (Houston, TX): Do you feel there are any absolute contraindications to this procedure? Anju Goyal, MRCS (Liverpool, UK ): We tend to use EGD only in patients who are severely neurologically handicapped and who are not feeding orally. We do
919 not tend to use EGD in those patients who are feeding by mouth. Dave Rodeberg, MD (Rochester, MN): What was the first fundoplication procedure that was performed in the majority of the patients for which they required further operations? Anju Goyal, MRCS (Liverpool, UK ): What were the further operations? Five patients required further operations. Four of them had redo fundoplication, and one had a feeding jejunostomy. Two of the fundoplications were successful reoperative cases. Two failed and went on to have further EGD, and one patient who had a feeding jejunostomy following a failed fundoplication that failed again and went on to have an EGD. So out of these five failures, basically three went on to have an EGD ultimately.
www.elsevier.com/locate/jpedsurg
Esophagogastric dissociation in the neurologically impaired: an alternative to fundoplication? Anju Goyala,*, Basem Khalila, Kelvin Chooa, Khalid Mohammedb, Matthew Jonesa a
Department of Paediatric Surgery, Royal Liverpool Children’s Hospital, Alder Hey, L12 2AP Liverpool, UK Department of Neurology, Royal Liverpool Children’s Hospital, Alder Hey, L12 2AP Liverpool, UK
b
Index words: Gastroesophageal reflux; Neurologically impaired children; Fundoplication; Esophagogastric dissociation; Antireflux surgery
Abstract Background/Purpose: Gastroesophageal reflux is common in children with severe neurological impairment. Fundoplication may produce symptomatic improvement but has a high failure rate. Esophagogastric dissociation (EGD) is an alternative procedure for treatment of gastroesophageal reflux. The aim of this study is to evaluate the results of EGD in our institution and compare them with a neurologically matched group of children who had Nissen fundoplication. Methods: Twenty consecutive patients who had EGD were retrospectively evaluated and the results were compared with a neurologically matched group of 20 consecutive patients who had Nissen fundoplication. Results: Twenty patients had EGD, 17 as a primary procedure. There was no operative mortality but 5 have died of other causes. Resolution of reflux-associated symptoms occurred in all patients. Of the 15 survivors, 5 remain on antireflux medication. Twenty patients had fundoplication. There was no operative mortality, but 8 patients have died of other causes. Failure occurred in 5 patients necessitating further surgery. Of the 10 unreoperated survivors, 6 remain on antireflux medication. Conclusions: Esophagogastric dissociation is an effective antireflux procedure when compared with fundoplication. It has a lower failure rate. We recommend EGD as a primary procedure in selected children with severe neurological impairment. D 2005 Elsevier Inc. All rights reserved.
Gastroesophageal reflux (GER) and gastric dysmotility are common problems in children with severe neurological Presented at the 56th Annual Meeting of the Section on Surgery of the American Academy of Pediatrics, San Francisco, California, October 8-10, 2004. This project is registered in the Clinical Audit Department of the Royal Liverpool Children’s Hospital, Alder Hey, Liverpool, UK, under project number 504. T Corresponding author. Tel.: +44 0 151 2525361; fax: +44 0 151 2525677. E-mail address: [email protected] (A. Goyal). 0022-3468/$ – see front matter D 2005 Elsevier Inc. All rights reserved. doi:10.1016/j.jpedsurg.2005.03.004
impairment (NI). This may compromise their already limited quality of life by causing nutritional, esophageal, respiratory, and pain-related problems. Fundoplication has been the traditional surgical approach to this condition, but it is associated with a high failure rate of 6% to 25% and with the persistence of unpleasant gastrointestinal symptoms [1- 6]. Several alternatives to fundoplication have been described, including bfeeding jejunostomy,Q bfundoplication with pyloroplasty Q [7-9], and esophagogastric dissociation (EGD). The role of EGD in the management of children with severe GER was first described by Bianchi [10] in 1997. Initially
916
A. Goyal et al.
2. Results 2.1. Patients
Fig. 1
Esophagogastric dissociation.
used as a salvage procedure after bfailed fundoplication,Q favorable results have led to an increasing role as a primary antireflux procedure in selected patients [11,12]. The aim of this study is to compare the results of EGD and Nissen fundoplication in children with severe NI in our institution.
1. Methods Twenty consecutive patients who had EGD at The Royal Liverpool Children’s Hospital between January 2001 and June 2004 were included in the study. Esophagogastric dissociation was offered to patients who were nonverbal, nonambulatory, unable to feed orally, and in whom symptoms of retching, vomiting, and discomfort predominated. All had severe NI with a bgross motor functionQ (GMF) score of 5 [13]. The results were compared with the most recent group of 20 consecutive patients with a GMF score of 5 who had Nissen fundoplication (1998-2004). Data were collected from hospital records and by personal interview/telephonic questionnaire.
1.1. Operative procedure All EGDs were done by a single surgeon using minor variations of the method described by Bianchi [10] (Fig. 1). The choice of incision (upper midline, left subcostal, or left thoracoabdominal) was dictated by patient habitus and previous operative scars. The stomach and esophagus were mobilized as per fundoplication and assessed. A HeinekeMikulicz pyloroplasty was done to aid gastric emptying. A 30 -cm Roux-en-Y loop was constructed and brought up via a retrocolic/retrogastric route. The stomach was then disconnected from the esophagus and the cardia oversewn. Finally, the Roux loop was anastomosed to the lower end of the esophagus. A gastrostomy was fashioned if not already present.
Twenty children had EGD (7 males and 13 females). Their mean age was 5.5 years (range, 7 months-16 years). All had a GMF score of 5 and were nonverbal, nonfeeding (tube fed), nonambulatory, and unable to support their head against gravity. All had severe GER characterized by pain, retching, vomiting, failure to thrive, and recurrent aspiration. Two patients had 4 previous failed fundoplications and 1 patient had a previous failed feeding jejunostomy. Four patients had a tracheostomy for airway management and 8 patients had videofluoroscopic evidence of aspiration. Twenty patients had Nissen fundoplication (14 males and 6 females). Their mean age was 3.4 years (range, 2 months9 years). All had a GMF score of 5 and were neurologically and symptomatically comparable with the EGD group. Three patients had a tracheostomy for airway management and 11 patients had videofluoroscopic evidence of aspiration.
2.2. Procedures Mean operating time for EGD was 238 minutes (range, 120- 470 minutes). Feeds were started at a mean of 5.0 days (range, 4 -7 days) and full feeds were established by 8.4 days (range, 6 -21 days). Mean intensive care stay was 1.6 days (range, 0-9 days). Mean operating time for Nissen fundoplication was 150 minutes (range, 60-240 minutes). Mean number of days for commencement of feeds was 2.7 days (range, 1- 4 days) and full feeds were established by 9.1 days (range, 4- 49 days). Mean intensive care stay was 4.1 days (range, 1-18 days) (Table 1).
2.3. Morbidity/mortality In the EGD group, there was no operative mortality. Two patients had an early complication (1 wound dehiscence and 1 iatrogenic diaphragmatic herniation) requiring uneventful reoperation. One patient required a subsequent pyloroplasty for delayed gastric emptying because this was not done during the original procedure. Five patients have subsequently died of unrelated causes. In the Nissen fundoplication group, there was no operative mortality. There were no early complications. One patient required a late laparotomy for adhesive obstruction and 5 (25%) patients required further antireflux
Table 1
Postoperative analysis of patients
Variables
EGD
Fundoplication
Average operating time (min) Commencement of feeds (d) Full feeds (d) ICU stay (d) Redo procedures needed
238 5.0 8.4 1.6 0
150 2.7 9.1 4.1 5
Esophagogastric dissociation in the neurologically impaired surgery for recurrent symptoms. Eight patients have subsequently died of unrelated causes.
2.4. Current status In the EGD group, 15 patients have survived without the need for further antireflux surgery at a mean follow-up of 13 months (range, 2-32 months). All patients are tolerating full feeds and are gaining weight. Twelve patients are currently free of reflux symptoms. Five (25%) patients remain on antireflux medication. Three patients have occasional vomiting, which does not preclude full feeding. They were evaluated with contrast follow-through studies via the gastrostomy, which did not reveal any evidence of jejunoesophageal reflux. Presumably, they have intermittent mild jejunoesophageal reflux due to bowel dysmotility, which would account for vomiting although we could not demonstrate it. They continue to be on antireflux medication empirically. Another patient has screaming episodes unrelated to feeds, which have been extensively investigated and for which no cause could be found. She continues to be on omeprazole rather empirically again. The fifth patient has gastrostomy-related local soreness for which he is on omeprazole, which is now being weaned. In the Nissen fundoplication group, 10 patients have survived without the need for further antireflux surgery at a mean follow-up of 42 months (range, 19-72 months). All patients are tolerating full feeds and are gaining weight. Six (60%) surviving patients continue to have reflux symptoms and remain on antireflux medication (Fig. 2). They have symptoms of retching and vomiting and 2 have recurrent chest infections. Four had postoperative evaluation for GER with contrast studies. Only 1 of these had evidence of reflux.
3. Discussion Patients with severe neurological disability suffer from a combination of oropharyngeal incoordination, gastric dys-
917 motility, and GER, which seriously compromises their already limited quality of life. They are prone to malnutrition, recurrent aspiration, and esophageal ulceration and frequently experience debilitating discomfort that is often refractory to conventional medical therapy. Fundoplication has been the traditional surgical approach to this problem but is known to have indifferent results in this group of patients, with a high incidence of recurrent symptoms and revision rates ranging from 6% to 25% [1- 6]. This high failure rate has been attributed to a number of factors such as gut dysmotility, seizure disorders, aerophagia [14,15], and central (ie, derived from the central nervous system) pain. Ravelli and Milla [16] propose that many of these symptoms may result from gastric dysmotility and that these children suffer from inappropriate reflex vomiting in addition to passive reflux. They performed electrogastrography in 50 children with central nervous system disorders who suffered from retching/vomiting and found gastric dysrhythmias in 62% of these. Dysrhythmias result from abnormal activation of the efferent limb of the emetic reflex or from lack of inhibition of excitatory fibers as a consequence of either disturbed input to the hindbrain from higher centers or anatomic and functional disturbances of the vomiting center. Although fundoplication may prevent the physical act of vomiting, it would do little to alleviate the underlying neurological problem. Esophagogastric dissociation offers the advantage that by disconnecting the esophagus from the stomach, it eliminates all possibility of GER and also, by virtue of complete vagotomy at the time of operation, eliminates abnormal extrinsic innervation of the foregut. The combined pyloroplasty aids gastric emptying. Esophagogastric dissociation was initially advocated as a salvage procedure after failed fundoplication, but subsequent good results encouraged Bianchi [10] to propose it as primary procedure in this select group of patients. Early reports suggest that EGD produces very good symptomatic results, but concerns remain over the magnitude of the procedure and the potential for serious operative complications. These may include bleeding, anastomotic leakage, necrosis of the Roux loop, perforation, wound dehiscence, diaphragmatic hernia,
Successful Procedures EGD (20) Alive (15)
Nissen (15) Dead (5)
Dead (5)
Symptomatic (3)
Symptomatic(6)
No symptoms 12
No Symptoms 4
Fig. 2
Outcome of patients who did not need reoperation.
Alive (10)
918 adhesive obstruction, and death [17]. Several authors have published their experience with EGD, with significant complications requiring surgical intervention occurring in 20% to 30% of cases [4,12,18]. This high incidence of complications has led some authors to recommend that EGD be reserved as a procedure of last resort [17]. In our series, 2 (10%) patients had early complications requiring operative intervention. In both cases, the children made an uneventful recovery. Despite the apparent magnitude of EGD, postoperative debilitation and bICU stay Q were no worse than for fundoplication. We believe that with appropriate experience, careful patient selection, and good anesthetic support, EGD should not pose a greater risk than other antireflux procedures. We have been very pleased with the symptomatic improvement of patients who have had EGD and have been particularly struck by the general satisfaction of the children’s parents. Spontaneous parental comments have included phrases such as babsolutely fantastic,Q bbest thing that’s ever happened to him,Q and bshe has slept for the first time in seven years.Q Other authors have reported similar satisfaction. However, we have found it very difficult to establish objective outcome measures because these children suffer from numerous comorbid problems that preclude the use of such standard outcome parameters as hospital attendance and hospital stay. Twelve of the 15 surviving patients are largely asymptomatic and are thriving; however, 3 patients continue to have some minor gastrointestinal difficulties. They have occasional minor vomiting but this does not compromise their tolerance of feeds. The vomiting may result from an associated degree of panintestinal dysmotility resulting in jejunoesophageal reflux. Also in retrospect, we feel that in 1 of these 3 patients the Roux loop may have been a bit short. Another child experiences intermittent bouts of unexplained discomfort, which have been extensively investigated, without any obvious explanation becoming apparent. The discomfort is not debilitating, and she remains otherwise well. All in all, we feel that although these patients have residual gastrointestinal symptoms, it is difficult to attribute these to any intrinsic deficiency of the EGD procedure.
A. Goyal et al.
[3]
[4]
[5]
[6]
[7] [8]
[9]
[10] [11]
[12]
[13]
[14]
[15]
[16]
[17]
[18]
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4. Conclusion We believe that EGD is a safe and effective alternative to fundoplication and recommend that it be considered for the treatment of GER in children with severe NI.
References [1] 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(11):1169 - 73. [2] Tuggle DW, Tunnel WP, Hoelzer DJ, et al. The efficacy of Thal fundoplication in the treatment of gastroesophageal reflux: the
Discussion Phil Frickman, MD (Los Angeles, CA ): I enjoyed your paper very much. One of the questions I had about your slides was that your follow-up for the EGD group was only 13 months on average, whereas your follow-up for the fundoplication group was substantially longer, which may account for your higher death rate in your other patients. I’m not sure these results support your conclusions necessarily. Could you comment on that?
Esophagogastric dissociation in the neurologically impaired Anju Goyal, MRCS (Liverpool, UK ): Yes, as you can see, the follow-up in the fundoplication group was much longer. This is because we tried to take the patients who were neurologically matched, and since we have started doing the EGD procedure, we have done very few fundoplications so we had to actually go back and take patients who had fundoplications probably a few years prior to the EGD. So naturally the follow-up was longer, but the deaths in all the cases were unrelated so I suppose that should probably not have any influence on the conclusions. Jed Nuchtern, MD, FAAP (Houston, TX): Do you feel there are any absolute contraindications to this procedure? Anju Goyal, MRCS (Liverpool, UK ): We tend to use EGD only in patients who are severely neurologically handicapped and who are not feeding orally. We do
919 not tend to use EGD in those patients who are feeding by mouth. Dave Rodeberg, MD (Rochester, MN): What was the first fundoplication procedure that was performed in the majority of the patients for which they required further operations? Anju Goyal, MRCS (Liverpool, UK ): What were the further operations? Five patients required further operations. Four of them had redo fundoplication, and one had a feeding jejunostomy. Two of the fundoplications were successful reoperative cases. Two failed and went on to have further EGD, and one patient who had a feeding jejunostomy following a failed fundoplication that failed again and went on to have an EGD. So out of these five failures, basically three went on to have an EGD ultimately.