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Morbidity and mortality in total esophagogastric dissociation: A systematic review
Journal of Pediatric Surgery (2013) 48, 707–712
www.elsevier.com/locate/jpedsurg
Original articles
Morbidity and mortality in total esophagogastric dissociation: A systematic review☆ Robert T. Peters a , Yan Li Goh b , Jessica Maria Veitch b , Basem A. Khalil a , Antonino Morabito a,⁎ a
Department of Paediatric Surgery, Royal Manchester Children's Hospital, Manchester, M13 9WL, United Kingdom School of Medicine, The University of Manchester, Oxford Road, Manchester, M13 9PT, United Kingdom
b
Received 9 March 2012; revised 14 October 2012; accepted 13 November 2012
Key words: Child; Infant; Gastroesophageal reflux; Fundoplication; Esophagogastric dissociation
Abstract Background/Purpose: Total esophagogastric dissociation has been described as both a primary and a rescue procedure for patients with severe gastroesophageal reflux. Although most commonly used in the neurologically impaired, it has also been used in those with no neurological impairment. The main objective of this study was to determine morbidity and mortality for this procedure. Methods: All published cases of esophagogastric dissociation in children were identified. Series were updated where possible by personal communication with the author. Patient characteristics, indications, morbidity, and mortality were analyzed. Results: One hundred eighty-one cases were identified. One hundred seventeen were primary operations and 64 were rescue procedures. There were 29 (16.0%) early complications and 28 (15.5%) late complications with 6 (3.3%) deaths related to the procedure of a total of 35 deaths. Twenty-one patients (11.6%) required re-operation in the study periods. Conclusions: Esophagogastric dissociation has an acceptable morbidity and mortality. It is useful as both a primary and a rescue procedure. © 2013 Elsevier Inc. All rights reserved.
Total esophagogastric dissociation (TOGD) is an antireflux procedure originally described as a “rescue” procedure for use in children with neurological impairment (NI) by Bianchi [1] in 1997. Its use has subsequently been broadened to include neurologically normal children and adults with NI [2]. TOGD is now used as both a primary surgical procedure and as “rescue” therapy following failed fundoplication.
☆ Conflicts of interest: None. ⁎ Corresponding author. Tel.: +44 161 7012194; fax: +44 161 7012767. E-mail address: [email protected] (A. Morabito).
0022-3468/$ – see front matter © 2013 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.jpedsurg.2012.11.049
The procedure involves separation of the esophagus from the stomach just superior to the lower esophageal sphincter with closure of the gastrotomy. An iso-peristaltic Roux-en-Y esophagojejunostomy is performed and, if not already present, a gastrostomy is placed. The patient is then fed enterally via the gastrostomy although they can swallow saliva and some may also be able to take oral nutrition. [1]. The aim of this study was to analyze data from all published cases of TOGD in children with particular attention to patient characteristics, indications, complications and mortality. Where available, unpublished cases were added by personal communication with authors of published
708
R.T. Peters et al.
series. There have been nine reported cases of its use in adults but these have not been included in this review [2–7].
1. Methods Medline, PubMed, National Library of Health and Google Scholarly articles from year 1997 to August 2011 were searched. Search terms used were [total] ([o]esophago-gastric/ gastro-[o]esophageal) (dissociation/disconnection/separation). A primary procedure is defined as the first operation performed (eg for gastroesophageal reflux) and a rescue procedure as a further operation following failed earlier operation(s). Early complications are those occurring in the first 30 days following the procedure or those described as early within analyzed articles. Late complications are those occurring later than 30 days following TOGD or those described as late. Authors of published studies were contacted by e-mail and information on any TOGD performed since their publications was requested. Where available, information on these cases was added to the current study. Raw data were available from Manchester for neurologically impaired children and this was used rather than published series although 26 of these cases had previously been published [8].
2. Results One hundred eighty-one patients who had undergone TOGD were identified from 10 articles, personal communication with authors of these articles and data held at our institution (Table 1, Fig. 1) [2–6,9–13]. A two-center study combining data from Manchester and Rome was analyzed to Table 1 Summary of studies and their contribution by number of patients to this analysis. Study
Neurologically Neurologically impaired children normal children
Danielson and Emmens 26 [3] (+ personal communication) Buratti et al. [13] 7 Islam et al. [4] (+ personal 11 communication) Goyal et al. [11] 20 Ishimaru et al. [12] 2 Lagausie et al. [2] Alberti et al. [6] 6 Lall et al. [5] (Rome) 23 Lall et al. [10] (difficult situations) 1 Boubnova et al. [9] (laparoscopic) Manchester (data from 61 center) Total 157
12
11 1
24
gain the Rome data [5]. Only the most recent publication from a center was analyzed where this previous case series was updated. Seventy-two of the children (39.8%) in the study were treated in Manchester. The majority of identified cases of TOGD were for children with neurological impairment (n = 157, 86.7%). Twenty-four (13.3%) were performed in neurologically normal children. The percentage of each group where TOGD was performed as a primary procedure rather than a rescue procedure was 67% and 50% respectively (Fig. 1). Overall there were 29 (16.0%) early complications and 28 (15.5%) late complications. Of these patients, 21 (11.6%) required re-operation. In the study periods, there were 35 (193%) deaths but only 6 (3.3%) were attributable to the TOGD procedure. The complications, re-operations and mortality are discussed by patient group below and summarized in Tables 2 and 3.
2.1. Children with severe neurological impairment The largest group in the identified cases was children with neurological impairment, compromising 157 children (86.7%). One hundred five (66.9%) of these patients underwent TOGD as a primary procedure. When stated, the indications were almost always for gastroesophageal reflux (GOR) with vomiting, failure to thrive and recurrent aspiration pneumonia being the most commonly stated clinical features. The group that underwent TOGD as a rescue procedure (n = 52) had mostly undergone one or more fundoplications but two had a surgical jejunostomy formed. Mean ages at time of operation are given in Fig. 1. The children undergoing TOGD as a rescue procedure were on average 2 years older than those having a primary procedure. Complications with those requiring re-operation are highlighted and mortality are shown in Tables 2 and 3. There were nine early complications requiring re-operation. Three of these were for leak from the esophagojejunal anastomosis (one also had leak from the pyloroplasty), although others with such leaks were able to be managed conservatively. Slow gastric emptying was noted in four patients and one required re-operation and pyloroplasty. The other early reoperations were for iatrogenic diaphragmatic hernia, cecal volvulus, necrosis of the Roux-en-Y loop, enterocutaneous fistula and drainage of a subphrenic collection. There were 10 late complications requiring re-operation. There were four paraesophageal hernias with one leading to extensive gastric necrosis. Two children had small bowel herniation under the Roux-en-Y loop, one of these occurring several years following TOGD. There were further late small bowel obstructions, one of which required operative intervention. Two esophagojejunal strictures required balloon dilatation and in one child a displaced gastrostomy was reinserted intra-abdominally leading to their death. Highlighting the underlying conditions of this group of children, 23 (14.6%) died of causes unrelated to the TOGD within the study periods. There were 5 (3.2%) deaths that
Morbidity and mortality in TOGD
709 TOGD (181)
Children with NI (157)
Fig. 1
Children with no NI (24)
Primary (105)
Rescue (52)
Primary (12)
Rescue (12)
[4.6y (0.1-15)]
[6.6y (1-16)]
[2.6y (0.1-4.6)]
[3.5y (1.8-6)]
Breakdown of patients by group and type of procedure (number of patients) [mean age years (range)].
the rescue group a child presented with paraesophageal hernia 6 months following TOGD. This was repaired and he subsequently did well. Prior to TOGD he had undergone two failed fundoplications and had recurrent aspiration pneumonias. One patient died 2 years following TOGD as a result of the procedure as described previously. A further six died of unrelated causes. Four of these died of progressive respiratory deterioration and respiratory arrest and two died of pneumococcal infection.
could be directly attributed to the procedure. Three children died of small bowel obstruction. One of these had undergone re-operation for a paraesophageal hernia but died during the second episode of obstruction before reaching the hospital. One child who experienced necrosis of the Roux-en-Y loop died following repeated episodes of abdominal sepsis. As described previously there was a death following incorrect re-insertion of gastrostomy.
2.2. Children with no neurological impairment There were 24 (13.3%) neurologically normal children who underwent TOGD. Underlying conditions and indications for procedure are shown in Table 4. The most common indication was severe GOR in children born with esophageal atresia. In those who had previously undergone colonic interposition, the procedure performed was cologastric dissociation rather than TOGD but results are included in this study. Five of the esophageal patients had previously undergone between one and four fundoplications each. There were no reported early complications. In the primary group, two children experienced chronic diarrhea which resolved with dietary modification. Two years following the original operation a child who had TOGD for congenital short esophagus secondary to congenital diaphragmatic hernia presented with herniation of small bowel into the chest. He died of the resulting peritonitis. In
Table 2
We have identified 181 cases of TOGD in the literature and from our own experience. There are nine cases reported of TOGD use in adults but they have not been included here as the focus is on children. The original description of the use of TOGD was in five children as a rescue procedure [1]. Encouraged by these results, Bianchi proposed its evaluation as a primary procedure in children in whom enteral feeding was not expected to be established. Several of the studies summarized in this review did just that [3,5,6,8,11]. All of these papers stated that they considered TOGD as a primary procedure in severely NI children with severe GOR. Most will have no ability to swallow and will be completely
Complications, re-operation rates and mortality by group.
Group
Children Children Children Children
3. Discussion
Patients
with NI (primary) with NI (rescue) no NI (primary) no NI (rescue)
105 52 12 12
Complications Early (b 30 days)
Late (N 30 days)
18% (n = 19) 19% (n = 10) 0% 0%
11% (n = 12) 23% (n = 12) 25% (n = 3) 8% (n = 1)
Note: Re-operation is for early or late complication.
Re-operation
Mortality related to TOGD
Mortality unrelated to TOGD
12% (n = 13) 11.5% (n = 6) 8% (n = 1) 8% (n = 1)
3% (n = 3) 4% (n = 2) 8% (n = 1) 0%
9% (n = 9) 27% (n = 14) 50% (n = 6) 0%
710 Table 3
R.T. Peters et al. Early and late complications by group.
Group
Complications
Children with NI (primary)
Early(b 30 days)
Late(N 30 days)
18%, requiring re-operation: Enterocutaneous fistula (n = 1)
11%, requiring re-operation: Strangulation of transhiatal herniation of stomach fundus requiring partial gastrectomy (n = 1) Small bowel herniation under roux loop with extensive necrosis (n = 2) Paraesophageal hernia (n = 1) Gastrostomy dislodgement with incorrect replacement (n = 1) Small bowel obstruction (n = 1)
Cecal volvulus (n = 1)
Children with NI (rescue)
Children no NI (primary) Children no NI (rescue)
Iatrogenic diaphragmatic hernia (n = 1) Esophagojejunal leak and requirement for revision pyloroplasty (n = 1) Slow gastric emptying (n = 2)(1 required pyloroplasty) Iatrogenic esophagojejunal anastomic injury (n = 1) Subphrenic collection (n = 1) Small bowel obstruction (n = 1) Esophagojejunal leak and intra-abdominal collection (n = 1) Minor complications (n = 9) 19%, requiring re-operation: Esophagojejunal leak with subphrenic collection and wound dehiscence (n = 1) Necrosis of Roux-en-Y loop (n = 1) Small bowel obstruction (n = 1) Esophagojejunal leak (n = 1) Minor complications (n = 6) 0%
0%
dependent on tube feeds. The Liverpool group further defined severe NI as children having a gross motor function score of 5 [14]. Ultimately it is the parents or care givers that will decide, with appropriate counseling from their surgeon, which antireflux procedure to opt for as a first procedure. TOGD offers them a procedure that eliminates GOR (although there is a 2.2% risk of jejunal reflux) and allows bolus gastrostomy feeding. When performed as a primary procedure, the difficulty caused by adhesions from previous anti-reflux procedures is avoided as is the need for redo anti-reflux procedures. Table 4
Esophagojejunal stricture (n = 2) Small bowel obstruction (n = 3) Minor complications (n = 1) 23%, requiring re-operation: Esophagojejunal stricture (n = 3)(2 required dilatation) Paraesophageal hernia (n = 2) Bile reflux (n = 3) Minor complications (n = 4) 25%, requiring re-operation: Small bowel herniation into chest (n = 1) Chronic diarrhea (n = 2) 8%, requiring re-operation: Paraesophageal hernia (n = 1)
The two major groups represented in the study are NI children and neurologically normal (NN) children with congenital or acquired esophageal disorders and the indications almost always reflux. As a result, it is reasonable to compare the outcomes here with those reported for fundoplication in similar patients. Series of fundoplication in children report vastly different failure rates and differ in their definitions of failure which are either recurrent GOR and/or the need for reoperative surgery. In a study of 198 profound NI children who underwent open fundoplication, 49 had documented recurrent GOR or a
Indications for TOGD in neurologically normal children.
Underlying condition
Indication for TOGD
Esophageal atresia (n = 15)
Severe GOR (n = 10) Severe colo-esophageal reflux (n = 3) Severe microgastria Necrotic stomach post-fundoplication Severe GOR (n = 3) Severe GOR (n = 2) Congenital short esophagus Esophageal obstruction following repeated repairs/fundoplication (n = 2) Insufficient stomach capacity
Tracheoesophageal cleft (n = 3) Esophageal caustic injury with esophagocoloplasty (n = 2) Congenital diaphragmatic hernia (n = 1) Congenital esophageal stenosis (n = 2) Gastric remnant following subtotal gastrectomy for bleeding (n = 1)
Morbidity and mortality in TOGD mechanical problem with the fundoplication, resulting in an operative failure rate of 25% [15]. A large series of 7467 children with over 95% undergoing open fundoplication noted a re-operation rate of 11.8% among NI children and 3.6% among NN children [16]. A number of series have reported children undergoing either open or laparoscopic fundoplication including 20 severe NI children where 30% experienced recurrent gastresophageal reflux [17]. In a further series of 456 children, where 33% were NI, re-operation rates at 2 years were 13.4% in the laparoscopy group and 6.7% in the open group [18]. They did not find an increased risk for reoperation among the NI group. Following laparoscopic fundoplication in NI children, documented recurrent GOR or need for re-operation ranges from 12% to 14.2% [19,20]. A large series of 1050 patients had a low wrap failure rate of 4% but only 10% of the subjects were NI [21]. The length of follow-up is important when interpreting studies as reflux may recur a variable length of time following original operation [18]. A systematic review of anti-reflux surgery in children with GOR identified 17 studies concerning efficacy and of these only 3 reported a success rate with follow-up beyond 6 months defined as complete resolution of GOR symptoms [22]. The median success rate was 72% (70%–96%) and two of the studies are discussed previously [17,19]. Seven of the TOGD studies quoted an average length of follow-up and these ranged from 13 months to 4 years. Fundoplication has a higher failure rate in esophageal atresia patients and when undertaken as a redo operation. In patients with esophageal atresia undergoing fundoplication for GOR, wrap disruption and recurrent GOR occurred in 33% in one study [23]. In a recent study of 81 redo fundoplications for recurrent GOR, failure rates with recurrent vomiting were 48% in neurologically impaired children and 32% in neurologically normal children [24]. The re-operation rate for TOGD (11.6%) is therefore broadly comparable to the re-operation rates/operative failure rates following failed primary fundoplication in the series discussed previously and markedly better than those for esophageal atresia patients or following redo fundoplication. Complication rates following fundoplication are difficult to compare between studies as it is not always apparent what they have included as a complication. Open fundoplication does appear to carry a higher complication rate as does fundoplication when undertaken in NI children. Among 198 NI children undergoing open fundoplication, 29% had early complications, 10% had one or more late complications and interestingly there was an 8% incidence of early and late small bowel obstruction [15]. A study of 7083 fundoplication admissions where 56% underwent laparoscopic fundoplication and the rest an open procedure found a 12% surgical complication rate in the laparoscopic group and a 25% rate for open surgery [25]. Of 7467 children undergoing mostly open fundoplication, major complication rates were 4.2% among NN children but
711 12.8% among NI children [16]. Lower rates are reported including a 4% post-operative complication rate after 1048 laparoscopic fundoplication although as mentioned earlier, only approximately 10% were in NI children [21]. We found a 16% early complication rate following TOGD procedure (Table 3). One third of these required re-operation and complications related to leak from the esophagojejunal anastomosis were the most frequent indication and are therefore the major early complications in this procedure. Conversely, two esophagojejunal leaks were managed conservatively without return to operating theater. Some series reported slow gastric emptying post-operatively although they did not state how this presented. We do not routinely perform pyloroplasty but its use should be considered where delayed gastric emptying is considered a problem pre-operatively. The necessary gastrostomy associated with TOGD is used to decompress any gastric distension if slow gastric emptying were to be an issue. Late complications occurred in 15.5% of patients following TOGD. Serious complications requiring reoperation included transhiatal herniation of either the stomach or small bowel into the chest or internal herniation of small bowel related to the Roux loop. As with open fundoplication, there is a risk of small bowel obstruction following TOGD (3.3%). Bile or alkaline reflux occurred in 2.2% and although none underwent operative intervention, there has been interest in the use of intussusception valves to eliminate this problem. The nature of the TOGD procedure means that GOR is eliminated in all cases. Early in-hospital deaths following fundoplication range from 0 to 1.3% [16,20,25,26], and one study reported an 11% 30-day mortality rate for children with cerebral palsy undergoing fundoplication [27]. The overall in-hospital mortality rate of 9987 children who underwent an anti-reflux procedure in the United Stated between 1996 and 2003 was 1.3%. Forty-five percent of the study population were NI and they were more likely to die in-hospital with an adjusted odds ratio of 1.77 [26]. Although the overall mortality related to TOGD was 3.3% (n = 6), some of these occurred months or even years following TOGD. The comparable in-hospital mortality from the TOGD procedure was 0.6% (n = 1) with no deaths within the first 30 days. There were 35 deaths in total during the study periods. This reflects the natural history of the underlying conditions leading to these children requiring an anti-reflux procedure. The mean time to death following TOGD where reported was 21.2 months (range 1–79 months). Long-term mortality rates following fundoplication range from 18.6% to 32.9% with the highest rates found among NI children and a median length of follow-up of 22 to 27 months where reported [19,20,27]. The 5-year survival rate after fundoplication for children with cerebral palsy and gastrostomy was 59% in one of these studies [27]. Quality of life assessment in neurologically impaired children who have undergone TOGD revealed statistically significant improvements in ease of feeding, vomiting and
712 retching symptoms, child's enjoyment of life and their overall comfort [28]. Similarly, fundoplication achieves a significant improvement in quality of life for children and their parents [29]. Review of all available cases of TOGD has revealed an acceptable complication and mortality rate. Rates of reoperation are comparable to and lower than some reoperation rates following fundoplication. They are considerably lower when compared with recurrent GOR rates following recurrent fundoplication. TOGD is a useful procedure in managing severe GOR in NI children, in NN children with specific conditions including esophageal atresia and as a rescue procedure following failed fundoplication.
Acknowledgments The authors thank Dr. Paul D Danielson, Dr. Saleem Islam and Mr. Adrian Bianchi for providing details of cases performed since their publications.
References [1] Bianchi A. Total esophagogastric dissociation: an alternative approach. J Pediatr Surg 1997;32:1291-4. [2] de Lagausie P, Bonnard A, Schultz A, et al. Reflux in esophageal atresia, tracheoesophageal cleft, and esophagocoloplasty: Bianchi's procedure as an alternative approach. J Pediatr Surg 2005;40:666-9. [3] Danielson PD, Emmens RW. Esophagogastric disconnection for gastroesophageal reflux in children with severe neurological impairment. J Pediatr Surg 1999;34:84-6 [discussion 87]. [4] Islam S, Teitelbaum DH, Buntain WL, et al. Esophagogastric separation for failed fundoplication in neurologically impaired children. J Pediatr Surg 2004;39:287-90 [discussion 290-91]. [5] Lall A, Morabito A, Dall'Oglio L, et al. Total oesophagogastric dissociation: experience in 2 centres. J Pediatr Surg 2006;41:342-6. [6] Alberti D, Zaranko E, Cheli M, et al. Total oesophagogastric dissociation in neurologically impaired patients: long-term results. Dig Liver Dis 2006;38:A105. [7] Hazebroek EJ, Hazebroek FWJ, Leibman S, et al. Total esophagogastric dissociation in adult neurologically impaired patients with severe gastroesophageal reflux: an alternative approach. Dis Esophagus 2008;21:742-5. [8] Morabito A, Lall A, Piccolo R, et al. Total esophagogastric dissociation: 10 years' review. J Pediatr Surg 2006;41:919-22. [9] Boubnova J, Hery G, Ughetto F, et al. Laparoscopic total esophagogastric dissociation. J Pediatr Surg 2009;44:e1-3. [10] Lall A, Morabito A, Bianchi A. “Total gastric dissociation (TGD)” in difficult clinical situations. Eur J Pediatr Surg 2006;16:396-8. [11] Goyal A, Khalil B, Choo K, et al. Esophagogastric dissociation in the neurologically impaired: an alternative to fundoplication? J Pediatr Surg 2005;40:915-9.
R.T. Peters et al. [12] Ishimaru Y, Kisaki Y, Fujino J. Experience with esophagogastric dissociation for gastroesophageal reflux disease in patients with severe neurological impairment. Nihon ShoÅni Geka Gakkai zasshi 2005;41: 226-31. [13] 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. [14] Palisano R, Rosenbaum P, Walter S, et al. Development and reliability of a system to classify gross motor function in children with cerebral palsy. Dev Med Child Neurol 1997;39:214-23. [15] Martinez DA, Ginn-Pease ME, Caniano DA. Sequelae of antireflux surgery in profoundly disabled children. J Pediatr Surg 1992;27: 267-71 [discussion 271-3]. [16] Fonkalsrud EW, Ashcraft KW, Coran AG, et al. Surgical treatment of gastroesophageal reflux in children: a combined hospital study of 7467 patients. Pediatrics 1998;101:419-22. [17] Cheung KM, Tse HW, Tse PWT, et al. Nissen fundoplication and gastrostomy in severely neurologically impaired children with gastroesophageal reflux. Hong Kong Med J 2006;12:282-8. [18] Diaz DM, Gibbons TE, Heiss K, et al. Antireflux surgery outcomes in pediatric gastroesophageal reflux disease. Am J Gastroenterol 2005; 100:1844-52. [19] Capito C, Leclair M-D, Piloquet H, et al. Long-term outcome of laparoscopic Nissen–Rossetti fundoplication for neurologically impaired and normal children. Surg Endosc 2008;22:875-80. [20] Kubiak R, Andrews J, Grant HW. Long-term outcome of laparoscopic Nissen fundoplication compared with laparoscopic thal fundoplication in children: a prospective, randomized study. Ann Surg 2011;253: 44-9. [21] Rothenberg SS. The first decade's experience with laparoscopic Nissen fundoplication in infants and children. J Pediatr Surg 2005;40: 142-6 [discussion 147]. [22] Siddiqui MRS, Abdulaal Y, Nisar A, et al. A meta-analysis of outcomes after open and laparoscopic Nissen's fundoplication for gastro-oesophageal reflux disease in children. Pediatr Surg Int 2011; 27:359-66. [23] Wheatley MJ, Coran AG, Wesley JR. Efficacy of the Nissen fundoplication in the management of gastroesophageal reflux following esophageal atresia repair. J Pediatr Surg 1993;28:53-5. [24] Pacilli M, Eaton S, Maritsi D, et al. Factors predicting failure of redo Nissen fundoplication in children. Pediatr Surg Int 2007;23:499-503. [25] Fox D, Morrato E, Campagna EJ, et al. Outcomes of laparoscopic versus open fundoplication in children's hospitals: 2005–2008. Pediatrics 2011;127:872-80. [26] Lasser MS, Liao JG, Burd RS. National trends in the use of antireflux procedures for children. Pediatrics 2006;118:1828-35. [27] Wockenforth R, Gillespie CS, Jaffray B. Survival of children following Nissen fundoplication. Br J Surg 2011;98:680-5. [28] Zaidi T, Sudall C, Kauffmann L, et al. Physical outcome and quality of life after total esophagogastric dissociation in children with severe neurodisability and gastroesophageal reflux, from the caregiver's perspective. J Pediatr Surg 2010;45:1772-6. [29] Engelmann C, Gritsa S, Ure BM. Impact of laparoscopic anterior 270 degrees fundoplication on the quality of life and symptoms profile of neurodevelopmentally delayed versus neurologically unimpaired children and their parents. Surg Endosc 2010;24:1287-95.
www.elsevier.com/locate/jpedsurg
Original articles
Morbidity and mortality in total esophagogastric dissociation: A systematic review☆ Robert T. Peters a , Yan Li Goh b , Jessica Maria Veitch b , Basem A. Khalil a , Antonino Morabito a,⁎ a
Department of Paediatric Surgery, Royal Manchester Children's Hospital, Manchester, M13 9WL, United Kingdom School of Medicine, The University of Manchester, Oxford Road, Manchester, M13 9PT, United Kingdom
b
Received 9 March 2012; revised 14 October 2012; accepted 13 November 2012
Key words: Child; Infant; Gastroesophageal reflux; Fundoplication; Esophagogastric dissociation
Abstract Background/Purpose: Total esophagogastric dissociation has been described as both a primary and a rescue procedure for patients with severe gastroesophageal reflux. Although most commonly used in the neurologically impaired, it has also been used in those with no neurological impairment. The main objective of this study was to determine morbidity and mortality for this procedure. Methods: All published cases of esophagogastric dissociation in children were identified. Series were updated where possible by personal communication with the author. Patient characteristics, indications, morbidity, and mortality were analyzed. Results: One hundred eighty-one cases were identified. One hundred seventeen were primary operations and 64 were rescue procedures. There were 29 (16.0%) early complications and 28 (15.5%) late complications with 6 (3.3%) deaths related to the procedure of a total of 35 deaths. Twenty-one patients (11.6%) required re-operation in the study periods. Conclusions: Esophagogastric dissociation has an acceptable morbidity and mortality. It is useful as both a primary and a rescue procedure. © 2013 Elsevier Inc. All rights reserved.
Total esophagogastric dissociation (TOGD) is an antireflux procedure originally described as a “rescue” procedure for use in children with neurological impairment (NI) by Bianchi [1] in 1997. Its use has subsequently been broadened to include neurologically normal children and adults with NI [2]. TOGD is now used as both a primary surgical procedure and as “rescue” therapy following failed fundoplication.
☆ Conflicts of interest: None. ⁎ Corresponding author. Tel.: +44 161 7012194; fax: +44 161 7012767. E-mail address: [email protected] (A. Morabito).
0022-3468/$ – see front matter © 2013 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.jpedsurg.2012.11.049
The procedure involves separation of the esophagus from the stomach just superior to the lower esophageal sphincter with closure of the gastrotomy. An iso-peristaltic Roux-en-Y esophagojejunostomy is performed and, if not already present, a gastrostomy is placed. The patient is then fed enterally via the gastrostomy although they can swallow saliva and some may also be able to take oral nutrition. [1]. The aim of this study was to analyze data from all published cases of TOGD in children with particular attention to patient characteristics, indications, complications and mortality. Where available, unpublished cases were added by personal communication with authors of published
708
R.T. Peters et al.
series. There have been nine reported cases of its use in adults but these have not been included in this review [2–7].
1. Methods Medline, PubMed, National Library of Health and Google Scholarly articles from year 1997 to August 2011 were searched. Search terms used were [total] ([o]esophago-gastric/ gastro-[o]esophageal) (dissociation/disconnection/separation). A primary procedure is defined as the first operation performed (eg for gastroesophageal reflux) and a rescue procedure as a further operation following failed earlier operation(s). Early complications are those occurring in the first 30 days following the procedure or those described as early within analyzed articles. Late complications are those occurring later than 30 days following TOGD or those described as late. Authors of published studies were contacted by e-mail and information on any TOGD performed since their publications was requested. Where available, information on these cases was added to the current study. Raw data were available from Manchester for neurologically impaired children and this was used rather than published series although 26 of these cases had previously been published [8].
2. Results One hundred eighty-one patients who had undergone TOGD were identified from 10 articles, personal communication with authors of these articles and data held at our institution (Table 1, Fig. 1) [2–6,9–13]. A two-center study combining data from Manchester and Rome was analyzed to Table 1 Summary of studies and their contribution by number of patients to this analysis. Study
Neurologically Neurologically impaired children normal children
Danielson and Emmens 26 [3] (+ personal communication) Buratti et al. [13] 7 Islam et al. [4] (+ personal 11 communication) Goyal et al. [11] 20 Ishimaru et al. [12] 2 Lagausie et al. [2] Alberti et al. [6] 6 Lall et al. [5] (Rome) 23 Lall et al. [10] (difficult situations) 1 Boubnova et al. [9] (laparoscopic) Manchester (data from 61 center) Total 157
12
11 1
24
gain the Rome data [5]. Only the most recent publication from a center was analyzed where this previous case series was updated. Seventy-two of the children (39.8%) in the study were treated in Manchester. The majority of identified cases of TOGD were for children with neurological impairment (n = 157, 86.7%). Twenty-four (13.3%) were performed in neurologically normal children. The percentage of each group where TOGD was performed as a primary procedure rather than a rescue procedure was 67% and 50% respectively (Fig. 1). Overall there were 29 (16.0%) early complications and 28 (15.5%) late complications. Of these patients, 21 (11.6%) required re-operation. In the study periods, there were 35 (193%) deaths but only 6 (3.3%) were attributable to the TOGD procedure. The complications, re-operations and mortality are discussed by patient group below and summarized in Tables 2 and 3.
2.1. Children with severe neurological impairment The largest group in the identified cases was children with neurological impairment, compromising 157 children (86.7%). One hundred five (66.9%) of these patients underwent TOGD as a primary procedure. When stated, the indications were almost always for gastroesophageal reflux (GOR) with vomiting, failure to thrive and recurrent aspiration pneumonia being the most commonly stated clinical features. The group that underwent TOGD as a rescue procedure (n = 52) had mostly undergone one or more fundoplications but two had a surgical jejunostomy formed. Mean ages at time of operation are given in Fig. 1. The children undergoing TOGD as a rescue procedure were on average 2 years older than those having a primary procedure. Complications with those requiring re-operation are highlighted and mortality are shown in Tables 2 and 3. There were nine early complications requiring re-operation. Three of these were for leak from the esophagojejunal anastomosis (one also had leak from the pyloroplasty), although others with such leaks were able to be managed conservatively. Slow gastric emptying was noted in four patients and one required re-operation and pyloroplasty. The other early reoperations were for iatrogenic diaphragmatic hernia, cecal volvulus, necrosis of the Roux-en-Y loop, enterocutaneous fistula and drainage of a subphrenic collection. There were 10 late complications requiring re-operation. There were four paraesophageal hernias with one leading to extensive gastric necrosis. Two children had small bowel herniation under the Roux-en-Y loop, one of these occurring several years following TOGD. There were further late small bowel obstructions, one of which required operative intervention. Two esophagojejunal strictures required balloon dilatation and in one child a displaced gastrostomy was reinserted intra-abdominally leading to their death. Highlighting the underlying conditions of this group of children, 23 (14.6%) died of causes unrelated to the TOGD within the study periods. There were 5 (3.2%) deaths that
Morbidity and mortality in TOGD
709 TOGD (181)
Children with NI (157)
Fig. 1
Children with no NI (24)
Primary (105)
Rescue (52)
Primary (12)
Rescue (12)
[4.6y (0.1-15)]
[6.6y (1-16)]
[2.6y (0.1-4.6)]
[3.5y (1.8-6)]
Breakdown of patients by group and type of procedure (number of patients) [mean age years (range)].
the rescue group a child presented with paraesophageal hernia 6 months following TOGD. This was repaired and he subsequently did well. Prior to TOGD he had undergone two failed fundoplications and had recurrent aspiration pneumonias. One patient died 2 years following TOGD as a result of the procedure as described previously. A further six died of unrelated causes. Four of these died of progressive respiratory deterioration and respiratory arrest and two died of pneumococcal infection.
could be directly attributed to the procedure. Three children died of small bowel obstruction. One of these had undergone re-operation for a paraesophageal hernia but died during the second episode of obstruction before reaching the hospital. One child who experienced necrosis of the Roux-en-Y loop died following repeated episodes of abdominal sepsis. As described previously there was a death following incorrect re-insertion of gastrostomy.
2.2. Children with no neurological impairment There were 24 (13.3%) neurologically normal children who underwent TOGD. Underlying conditions and indications for procedure are shown in Table 4. The most common indication was severe GOR in children born with esophageal atresia. In those who had previously undergone colonic interposition, the procedure performed was cologastric dissociation rather than TOGD but results are included in this study. Five of the esophageal patients had previously undergone between one and four fundoplications each. There were no reported early complications. In the primary group, two children experienced chronic diarrhea which resolved with dietary modification. Two years following the original operation a child who had TOGD for congenital short esophagus secondary to congenital diaphragmatic hernia presented with herniation of small bowel into the chest. He died of the resulting peritonitis. In
Table 2
We have identified 181 cases of TOGD in the literature and from our own experience. There are nine cases reported of TOGD use in adults but they have not been included here as the focus is on children. The original description of the use of TOGD was in five children as a rescue procedure [1]. Encouraged by these results, Bianchi proposed its evaluation as a primary procedure in children in whom enteral feeding was not expected to be established. Several of the studies summarized in this review did just that [3,5,6,8,11]. All of these papers stated that they considered TOGD as a primary procedure in severely NI children with severe GOR. Most will have no ability to swallow and will be completely
Complications, re-operation rates and mortality by group.
Group
Children Children Children Children
3. Discussion
Patients
with NI (primary) with NI (rescue) no NI (primary) no NI (rescue)
105 52 12 12
Complications Early (b 30 days)
Late (N 30 days)
18% (n = 19) 19% (n = 10) 0% 0%
11% (n = 12) 23% (n = 12) 25% (n = 3) 8% (n = 1)
Note: Re-operation is for early or late complication.
Re-operation
Mortality related to TOGD
Mortality unrelated to TOGD
12% (n = 13) 11.5% (n = 6) 8% (n = 1) 8% (n = 1)
3% (n = 3) 4% (n = 2) 8% (n = 1) 0%
9% (n = 9) 27% (n = 14) 50% (n = 6) 0%
710 Table 3
R.T. Peters et al. Early and late complications by group.
Group
Complications
Children with NI (primary)
Early(b 30 days)
Late(N 30 days)
18%, requiring re-operation: Enterocutaneous fistula (n = 1)
11%, requiring re-operation: Strangulation of transhiatal herniation of stomach fundus requiring partial gastrectomy (n = 1) Small bowel herniation under roux loop with extensive necrosis (n = 2) Paraesophageal hernia (n = 1) Gastrostomy dislodgement with incorrect replacement (n = 1) Small bowel obstruction (n = 1)
Cecal volvulus (n = 1)
Children with NI (rescue)
Children no NI (primary) Children no NI (rescue)
Iatrogenic diaphragmatic hernia (n = 1) Esophagojejunal leak and requirement for revision pyloroplasty (n = 1) Slow gastric emptying (n = 2)(1 required pyloroplasty) Iatrogenic esophagojejunal anastomic injury (n = 1) Subphrenic collection (n = 1) Small bowel obstruction (n = 1) Esophagojejunal leak and intra-abdominal collection (n = 1) Minor complications (n = 9) 19%, requiring re-operation: Esophagojejunal leak with subphrenic collection and wound dehiscence (n = 1) Necrosis of Roux-en-Y loop (n = 1) Small bowel obstruction (n = 1) Esophagojejunal leak (n = 1) Minor complications (n = 6) 0%
0%
dependent on tube feeds. The Liverpool group further defined severe NI as children having a gross motor function score of 5 [14]. Ultimately it is the parents or care givers that will decide, with appropriate counseling from their surgeon, which antireflux procedure to opt for as a first procedure. TOGD offers them a procedure that eliminates GOR (although there is a 2.2% risk of jejunal reflux) and allows bolus gastrostomy feeding. When performed as a primary procedure, the difficulty caused by adhesions from previous anti-reflux procedures is avoided as is the need for redo anti-reflux procedures. Table 4
Esophagojejunal stricture (n = 2) Small bowel obstruction (n = 3) Minor complications (n = 1) 23%, requiring re-operation: Esophagojejunal stricture (n = 3)(2 required dilatation) Paraesophageal hernia (n = 2) Bile reflux (n = 3) Minor complications (n = 4) 25%, requiring re-operation: Small bowel herniation into chest (n = 1) Chronic diarrhea (n = 2) 8%, requiring re-operation: Paraesophageal hernia (n = 1)
The two major groups represented in the study are NI children and neurologically normal (NN) children with congenital or acquired esophageal disorders and the indications almost always reflux. As a result, it is reasonable to compare the outcomes here with those reported for fundoplication in similar patients. Series of fundoplication in children report vastly different failure rates and differ in their definitions of failure which are either recurrent GOR and/or the need for reoperative surgery. In a study of 198 profound NI children who underwent open fundoplication, 49 had documented recurrent GOR or a
Indications for TOGD in neurologically normal children.
Underlying condition
Indication for TOGD
Esophageal atresia (n = 15)
Severe GOR (n = 10) Severe colo-esophageal reflux (n = 3) Severe microgastria Necrotic stomach post-fundoplication Severe GOR (n = 3) Severe GOR (n = 2) Congenital short esophagus Esophageal obstruction following repeated repairs/fundoplication (n = 2) Insufficient stomach capacity
Tracheoesophageal cleft (n = 3) Esophageal caustic injury with esophagocoloplasty (n = 2) Congenital diaphragmatic hernia (n = 1) Congenital esophageal stenosis (n = 2) Gastric remnant following subtotal gastrectomy for bleeding (n = 1)
Morbidity and mortality in TOGD mechanical problem with the fundoplication, resulting in an operative failure rate of 25% [15]. A large series of 7467 children with over 95% undergoing open fundoplication noted a re-operation rate of 11.8% among NI children and 3.6% among NN children [16]. A number of series have reported children undergoing either open or laparoscopic fundoplication including 20 severe NI children where 30% experienced recurrent gastresophageal reflux [17]. In a further series of 456 children, where 33% were NI, re-operation rates at 2 years were 13.4% in the laparoscopy group and 6.7% in the open group [18]. They did not find an increased risk for reoperation among the NI group. Following laparoscopic fundoplication in NI children, documented recurrent GOR or need for re-operation ranges from 12% to 14.2% [19,20]. A large series of 1050 patients had a low wrap failure rate of 4% but only 10% of the subjects were NI [21]. The length of follow-up is important when interpreting studies as reflux may recur a variable length of time following original operation [18]. A systematic review of anti-reflux surgery in children with GOR identified 17 studies concerning efficacy and of these only 3 reported a success rate with follow-up beyond 6 months defined as complete resolution of GOR symptoms [22]. The median success rate was 72% (70%–96%) and two of the studies are discussed previously [17,19]. Seven of the TOGD studies quoted an average length of follow-up and these ranged from 13 months to 4 years. Fundoplication has a higher failure rate in esophageal atresia patients and when undertaken as a redo operation. In patients with esophageal atresia undergoing fundoplication for GOR, wrap disruption and recurrent GOR occurred in 33% in one study [23]. In a recent study of 81 redo fundoplications for recurrent GOR, failure rates with recurrent vomiting were 48% in neurologically impaired children and 32% in neurologically normal children [24]. The re-operation rate for TOGD (11.6%) is therefore broadly comparable to the re-operation rates/operative failure rates following failed primary fundoplication in the series discussed previously and markedly better than those for esophageal atresia patients or following redo fundoplication. Complication rates following fundoplication are difficult to compare between studies as it is not always apparent what they have included as a complication. Open fundoplication does appear to carry a higher complication rate as does fundoplication when undertaken in NI children. Among 198 NI children undergoing open fundoplication, 29% had early complications, 10% had one or more late complications and interestingly there was an 8% incidence of early and late small bowel obstruction [15]. A study of 7083 fundoplication admissions where 56% underwent laparoscopic fundoplication and the rest an open procedure found a 12% surgical complication rate in the laparoscopic group and a 25% rate for open surgery [25]. Of 7467 children undergoing mostly open fundoplication, major complication rates were 4.2% among NN children but
711 12.8% among NI children [16]. Lower rates are reported including a 4% post-operative complication rate after 1048 laparoscopic fundoplication although as mentioned earlier, only approximately 10% were in NI children [21]. We found a 16% early complication rate following TOGD procedure (Table 3). One third of these required re-operation and complications related to leak from the esophagojejunal anastomosis were the most frequent indication and are therefore the major early complications in this procedure. Conversely, two esophagojejunal leaks were managed conservatively without return to operating theater. Some series reported slow gastric emptying post-operatively although they did not state how this presented. We do not routinely perform pyloroplasty but its use should be considered where delayed gastric emptying is considered a problem pre-operatively. The necessary gastrostomy associated with TOGD is used to decompress any gastric distension if slow gastric emptying were to be an issue. Late complications occurred in 15.5% of patients following TOGD. Serious complications requiring reoperation included transhiatal herniation of either the stomach or small bowel into the chest or internal herniation of small bowel related to the Roux loop. As with open fundoplication, there is a risk of small bowel obstruction following TOGD (3.3%). Bile or alkaline reflux occurred in 2.2% and although none underwent operative intervention, there has been interest in the use of intussusception valves to eliminate this problem. The nature of the TOGD procedure means that GOR is eliminated in all cases. Early in-hospital deaths following fundoplication range from 0 to 1.3% [16,20,25,26], and one study reported an 11% 30-day mortality rate for children with cerebral palsy undergoing fundoplication [27]. The overall in-hospital mortality rate of 9987 children who underwent an anti-reflux procedure in the United Stated between 1996 and 2003 was 1.3%. Forty-five percent of the study population were NI and they were more likely to die in-hospital with an adjusted odds ratio of 1.77 [26]. Although the overall mortality related to TOGD was 3.3% (n = 6), some of these occurred months or even years following TOGD. The comparable in-hospital mortality from the TOGD procedure was 0.6% (n = 1) with no deaths within the first 30 days. There were 35 deaths in total during the study periods. This reflects the natural history of the underlying conditions leading to these children requiring an anti-reflux procedure. The mean time to death following TOGD where reported was 21.2 months (range 1–79 months). Long-term mortality rates following fundoplication range from 18.6% to 32.9% with the highest rates found among NI children and a median length of follow-up of 22 to 27 months where reported [19,20,27]. The 5-year survival rate after fundoplication for children with cerebral palsy and gastrostomy was 59% in one of these studies [27]. Quality of life assessment in neurologically impaired children who have undergone TOGD revealed statistically significant improvements in ease of feeding, vomiting and
712 retching symptoms, child's enjoyment of life and their overall comfort [28]. Similarly, fundoplication achieves a significant improvement in quality of life for children and their parents [29]. Review of all available cases of TOGD has revealed an acceptable complication and mortality rate. Rates of reoperation are comparable to and lower than some reoperation rates following fundoplication. They are considerably lower when compared with recurrent GOR rates following recurrent fundoplication. TOGD is a useful procedure in managing severe GOR in NI children, in NN children with specific conditions including esophageal atresia and as a rescue procedure following failed fundoplication.
Acknowledgments The authors thank Dr. Paul D Danielson, Dr. Saleem Islam and Mr. Adrian Bianchi for providing details of cases performed since their publications.
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