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Enterostomy tube placement in children with spinal muscular atrophy type 1
ENTEROSTOMY TUBE PLACEMENT IN CHILDREN WITH SPINAL MUSCULAR ATROPHY TYPE 1 K. SY, BSC, S. MAHANT, MD, N. TABACK, PHD, J. VAJSAR, MD, P. G. CHAIT, MBBCH,
AND
J. N. FRIEDMAN, MBCHB
Objective To determine the major complication rate in the first 30 days after enterostomy tube insertion in infants with spinal muscular atrophy (SMA) type 1. Study design A retrospective case review of all children with SMA type 1 who had a gastrostomy or gastrojejunostomy tube placed by the image-guided technique at the Hospital for Sick Children from 1994 –2004. Major complications were classified as peritonitis, aspiration pneumonia, respiratory failure, nonelective admission to the pediatric intensive care unit, and death. Results Twelve children were identified as having SMA type 1 with an enterostomy tube insertion. The median age at tube insertion was 6.1 months (range 2.2 to 15.8 months). Major complications in the first 30 days after the procedure included aspiration pneumonia (5/12 patients [41.6%]), respiratory failure requiring admission to the pediatric intensive care unit (4/12 [33%]), and death (2/12 [16.7%]). Children with development of aspiration pneumonia were significantly older at time of tube insertion (P < .05) than those with no aspiration. Conclusions Major complications including death are seen in children with SMA type 1 in the first 30 days after enterostomy tube insertion. (J Pediatr 2006;149:837-9) pinal muscular atrophy (SMA) is an autosomal recessive disease that is characterized by the progressive degeneration of the lower motor neurons in the spinal cord. Type 1 SMA is the most severe form, and the incidence is estimated between 1 in 10,000 to 1 in 25,000 live births.1 Apart from severe generalized limb muscle weakness and hypotonia, children have development of bulbar and respiratory muscle weakness leading to progressive respiratory difficulties and dysphagia. For SMA type 1, survival probability at 2 years of age of 18% was initially reported.2 The usual cause of death is from respiratory failure.3 Survival can be prolonged with ventilatory support.4-6 Tube feeding may be considered for infants with SMA type 1 to maintain an adequate nutritional state, decrease the risk of aspiration pneumonia and improve quality of life. The image-guided percutaneous technique for enterostomy tube placement is highly successful, has minimal anesthetic requirements, and is suitable for the medically fragile infant.7,8 However, in the early post-procedure period, respiratory complications related to anesthesia and weakness may result in aspiration of oropharyngeal contents, atelectasis, and respiratory failure. Given the natural history of SMA type 1 and the lack of disease-stabilizing treatments or cure, the application of technology and interventions in this population From the Division of Paediatric Medicine needs to be thoughtfully approached.9,10 Few studies have examined outcomes of enterand Paediatric Outcomes Research Team, ostomy tube insertion in infants with SMA type 1.11,12 The objective of this study was to the Department of Paediatrics, the Department of Diagnostic Imaging, and the Dividetermine the major complication rate in the first 30 days after enterostomy tube insertion sion of Neurology, The Hospital for Sick in children with SMA type 1. Children, University of Toronto, and St.
S
METHODS This study was conducted at the Hospital for Sick Children (SickKids), a tertiary care pediatric hospital in Toronto, Ontario, Canada. All patients who were SMA type 1 and who had enterostomy tubes placed at the SickKids from January 1994 to July 2004 were included in the study. Patients were identified through the Esh IGT database (Esh, Thornhill, Ontario, Canada). The study was approved by the SickKids Research Ethics Board. Patients were classified as having SMA type 1 if they presented with severe weakness in the first 6 months of life and were found to have a homozygous deletion of the SMN gene. Before the gene discovery the diagnosis was made on muscle biopsy. GERD HSC
Gastroesophageal reflux disease Hospital for Sick Children
PICU SMA
Pediatric intensive care unit Spinal muscular atrophy
Michael’s Hospital, Department of Public Health Sciences, Toronto, Ontario, Canada. Submitted for publication Mar 8, 2006; last revision received Jun 1, 2006; accepted Aug 21, 2006. Reprint requests: Sanjay Mahant, MD, FRCPC, Division of Paediatric Medicine, Paediatric Outcomes Research Team, The Hospital for Sick Children, The University of Toronto, Toronto, Ontario, Canada M5G1X8. E-mail: sanjay.mahant@sickkids. ca. 0022-3476/$ - see front matter Copyright © 2006 Mosby Inc. All rights reserved. 10.1016/j.jpeds.2006.08.048
837
A list of possible complications of enterostomy tube insertion were collected a priori.13 Early complications were defined as those occurring within 30 days of the procedure. Pneumonia, respiratory failure, unplanned intensive care admission, peritonitis, and death were classified as major complications. The percutaneous image-guided gastrostomy and gastrojejunostomy tube insertion technique has been well described.7,8 Children are observed in a postanesthetic care unit for 6 hours after the procedure. When stable, they are transferred to the pediatric ward for monitoring. Admission to the intensive care unit is not routine practice.
Statistical analysis Descriptive statistics are used to present baseline characteristics. Exact 95% confidence intervals were calculated for the proportion of subjects where the outcome was aspiration pneumonia, respiratory failure, an unplanned PICU admission, and death. Mean age of subjects with development of aspiration pneumonia or respiratory failure were compared by use of an exact Wilcoxon 2-sample test. Fisher’s exact test was used to evaluate the associations between a history of pneumonia before enterostomy tube placement and (1) respiratory failure and (2) aspiration pneumonia after enterostomy tube placement. All analyses were done with SAS 8.02 (SAS Institute, Inc, Cary, NC).
RESULTS Fourteen patients were identified through the Image Guided Therapy database as having SMA type 1 and enterostomy tube insertion. Two patients were excluded because their genetic testing did not show a deletion of the SMN gene, and muscle biopsy specimens were inconclusive. The median age at time of tube placement was 6.1 months (range 2.2 to 15.8 months), with a mean weight of 6.9 kg (range 2.7 to 14.6 kg) for the 12 patients. The primary indication for tube insertion in all patients was oral motor feeding dysfunction resulting in an inability to maintain adequate nutrition and hydration. All 7 patients who had a videofluoroscopic feeding study had evidence of aspiration of oral contents. All other children had clinical evidence of oral motor feeding dysfunction. No patients had a tracheostomy before or at the time of tube insertion. The tubes were placed under local anesthetic in 4 patients and sedation in 6 patients. General anesthetic was only required in the remaining 2 patients, both of whom were already intubated because of respiratory complications. There was a 100% success rate for enterostomy tube placement. Seven of the 12 patients had no major complications in the first 30 days. No patients had development of peritonitis or sepsis. For 2 patients, the gastrostomy tube was changed to a gastrojejunostomy tube because of gastroesophageal reflux disease. The 30-day mortality rate after the procedure was 16.7% (2/12) (95% CI 2.1%-48.4%). The first patient who died had a tube inserted at 5.5 months of age. This child had 838
Sy et al
2 aspiration pneumonias and 1 respiratory arrest before tube placement. After discharge from hospital post gastrostomy tube placement, the infant required PICU admission with presumed aspiration pneumonia. After discussions with the family, a palliative care plan was adopted, and the infant died 10 days after gastrostomy tube placement. The second patient who died had a tube placed at 11.7 months of age. This child had 1 aspiration pneumonia requiring PICU admission before tube placement and had development of aspiration pneumonia 6 days after gastrostomy tube placement. The tube was changed to a gastrojejunostomy tube to prevent reflux-induced aspiration. A palliative care plan was adopted, and the child died 29 days after the initial tube placement. Aspiration pneumonia occurred in 5/12 (41.6%) patients within 30 days of their procedure (95% CI 15.2%72.3%). Children with development of aspiration pneumonia were significantly older at time of tube insertion compared with those who did not have aspiration pneumonia (mean age 356 days vs 158 days, P ⫽ .03). Four patients (33%) (95 CI 10.0%-65.1%) required PICU admission for respiratory failure. They were older at time of tube insertion compared with those who did not have respiratory failure (mean age 365 days vs 183 days, P ⫽ .07), but this was not statistically significant. One of the 4 was managed with noninvasive ventilation, and the other 3 required intubation and ventilation. Seven patients had a history of pneumonia before tube insertion. Four of these children had development of aspiration pneumonia within 30 days after the procedure, and 4 had at least 1 episode of respiratory failure. Patients without a history of pneumonia before tube insertion (n ⫽ 5) did not have respiratory failure, and only 1 had aspiration pneumonia within 30 days of the procedure. The associations between a history of pneumonia before enterostomy tube placement and (1) respiratory failure and (2) pneumonia after enterostomy tube insertion had P values of .06 and .24, respectively.
DISCUSSION This study suggests that although the insertion of an enterostomy tube with the image-guided technique is technically successful with minimal anesthesia in children with SMA type 1, it may be associated with a high rate of early major respiratory complications, as well as death. Older age at time of tube insertion was associated with aspiration pneumonia, and this association approached significance with regard to development of respiratory failure. Older age, which may be a reflection of advanced neuromuscular weakness and bulbar dysfunction, may be an important predictor of early complications associated with tube insertion. Further studies are required to explore the relationship between age, advanced neuromuscular disease, and outcomes to determine the optimal timing of the procedure if it is to be undertaken. Friedman et al13 reported a mortality rate of 0.4% (with the only death related to peritonitis and sepsis) and a major complication rate of 5% in a study of complications associated with image-guided gastrostomy and gastrojejunostomy tubes in a heterogeneous group of children at our institution. The The Journal of Pediatrics • December 2006
30-day mortality rate (16.7%) and early major complication rate (41.6%) found in children with SMA type 1 in our study compares very unfavorably with these results. Few studies have specifically examined the impact or complications of enterostomy tube placement in children with SMA type 1 or neuromuscular disease.5,11,12 Birnkrant et al12 reviewed the course of 4 children with SMA type 1 treated with noninvasive ventilation and gastrostomy feeding. All had gastrostomy tubes placed between 2 and 9 months of age. Survival was only 1 to 3.5 months after presenting with severe aspiration. No patients died of tube placement. The impact of gastrostomy for these patients was unclear. Based on a review of 15 children with neuromuscular disease (which included 1 child with SMA type 1), Seguy et al11 questioned the use of gastrostomy feeding in children with severe congenital myopathy, severe respiratory disease, or severe gastroesophageal reflux disease (GERD) because of the potential respiratory complications and short term prognosis. Another study reported the use of gastrostomy in infants with SMA type 1 and briefly described 2 children with development of respiratory complications after gastrostomy tube insertion; however, outcome related to gastrostomy tube insertion was not the focus of the article.5 GERD that results in significant pulmonary complications may contribute to morbidity in children with SMA type 1 and neuromuscular disease.11 However, the management of GERD that is not responsive to maximal medical therapy has not been well studied in this population. Bach et al5 found no differences in hospitalization or mortality rates in patients with SMA type 1 after fundoplication. Because of the potential risks of surgery and anesthesia, patients in our study with significant GERD had their gastrostomy tube advanced to a gastrojejunostomy tube, which required no anesthetic. Given the retrospective study design and lack of control group, there are limitations to this study. Our findings imply an association but not necessarily causation. Given the natural history of the disease, it is difficult to know whether the deaths and early major complications are related purely to the underlying disease or are affected by the intervention. Prospective studies are required to determine the impact of enterostomy tube placement on mortality rate, respi-
ratory complications, quality of life, and the optimal timing of the procedure if it is to be undertaken. Parental perceptions of the impact of enterostomy tube feeding in their children with SMA type I are of paramount importance and require further study. It is imperative that the goals of tube feeding, natural history of the underlying neuromuscular disease, potential complications, and issues of quality of life are fully explored with the family before undertaking this intervention.
REFERENCES 1. Siddique N, Sufit R, Siddique T. Degenerative motor, sensory, and autonomic disorders. In: Goetz C, editor. Textbook of clinical neurology. 2nd ed. Philadelphia: WB Saunders; 2003. p 759-66. 2. Zerres K, Rudnik-Schoneborn S. Natural history in proximal spinal muscular atrophy: clinical analysis of 445 patients and suggestions for a modification of existing classifications. Arch Neurol 1995;52:518-23. 3. Ioos C, Leclairr-Richard D, Mrad S, Barois A, Estournet-Mathiaud B. Respiratory capacity course in patients with infantile spinal muscular atrophy. Chest 2004;126:831-7. 4. Chung BHY, Wong VCN, Ip P. Spinal muscular atrophy: Survival pattern and functional status. Pediatrics 2004;114:548-53. 5. Bach JR, Baird SJ, Plosky D, Navado J, Weaver B. Spinal muscular atrophy type 1: management and outcomes. Pediatr Pulmonol 2002;34:16-22. 6. Bach JR, Niranjan V. Spinal muscular atrophy type 1: a noninvasive respiratory management approach. Chest 2000;117:1100-5. 7. Chait PG, Baskin KM, Temple M, Connolly B. Pediatric gastrointestinal interventions. In: Stringer DA, Babyn PS, editors. Pediatric gastrointestinal imaging and intervention. 2nd ed. Hamilton: BC Decker; 2000. p. 97-160. 8. Chait PG, Weinberg J, Connolly BL, Pencharz P, Richards H, Clift JE, et al. Retrograde percutaneous gastrostomy and gastrojejunostomy in 505 children: a 4 1/2-year experience. Radiology 1996;201:691-5. 9. Hardart MKM, Truong RD. Spinal muscular atrophy type 1. The challenge of defining a child’s best interest. Arch Dis Child 2003;88:848-50. 10. Bush A, Fraser J, Jardine E, Paton J, Simonds A, Wallis C. Respiratory management of the infant with type 1 spinal muscular atrophy. Arch Dis Child 2005;90:709-11. 11. Seguy D, Michaud L, Guimber D, Cuisset JM, Devos P, Turck D, et al. Efficacy and tolerance of gastrostomy feeding in pediatric forms of neuromuscular diseases. J Parenter Enteral Nutr 2002;26:298-304. 12. Birnkrant DJ, Pope JF, Martin JE, Repucci AH, Eiben RM. Treatment of type I spinal muscular atrophy with noninvasive ventilation and gastrostomy feeding. Pediatr Neurol 1998;18:407-10. 13. Friedman JN, Ahmed S, Connolly B, Chait P, Mahant S. Complications associated with image-guided gastrostomy and gastrojejunostomy tubes in children. Pediatrics 2004;114:458-61.
Enterostomy Tube Placement In Children With Spinal Muscular Atrophy Type 1
839
AND
J. N. FRIEDMAN, MBCHB
Objective To determine the major complication rate in the first 30 days after enterostomy tube insertion in infants with spinal muscular atrophy (SMA) type 1. Study design A retrospective case review of all children with SMA type 1 who had a gastrostomy or gastrojejunostomy tube placed by the image-guided technique at the Hospital for Sick Children from 1994 –2004. Major complications were classified as peritonitis, aspiration pneumonia, respiratory failure, nonelective admission to the pediatric intensive care unit, and death. Results Twelve children were identified as having SMA type 1 with an enterostomy tube insertion. The median age at tube insertion was 6.1 months (range 2.2 to 15.8 months). Major complications in the first 30 days after the procedure included aspiration pneumonia (5/12 patients [41.6%]), respiratory failure requiring admission to the pediatric intensive care unit (4/12 [33%]), and death (2/12 [16.7%]). Children with development of aspiration pneumonia were significantly older at time of tube insertion (P < .05) than those with no aspiration. Conclusions Major complications including death are seen in children with SMA type 1 in the first 30 days after enterostomy tube insertion. (J Pediatr 2006;149:837-9) pinal muscular atrophy (SMA) is an autosomal recessive disease that is characterized by the progressive degeneration of the lower motor neurons in the spinal cord. Type 1 SMA is the most severe form, and the incidence is estimated between 1 in 10,000 to 1 in 25,000 live births.1 Apart from severe generalized limb muscle weakness and hypotonia, children have development of bulbar and respiratory muscle weakness leading to progressive respiratory difficulties and dysphagia. For SMA type 1, survival probability at 2 years of age of 18% was initially reported.2 The usual cause of death is from respiratory failure.3 Survival can be prolonged with ventilatory support.4-6 Tube feeding may be considered for infants with SMA type 1 to maintain an adequate nutritional state, decrease the risk of aspiration pneumonia and improve quality of life. The image-guided percutaneous technique for enterostomy tube placement is highly successful, has minimal anesthetic requirements, and is suitable for the medically fragile infant.7,8 However, in the early post-procedure period, respiratory complications related to anesthesia and weakness may result in aspiration of oropharyngeal contents, atelectasis, and respiratory failure. Given the natural history of SMA type 1 and the lack of disease-stabilizing treatments or cure, the application of technology and interventions in this population From the Division of Paediatric Medicine needs to be thoughtfully approached.9,10 Few studies have examined outcomes of enterand Paediatric Outcomes Research Team, ostomy tube insertion in infants with SMA type 1.11,12 The objective of this study was to the Department of Paediatrics, the Department of Diagnostic Imaging, and the Dividetermine the major complication rate in the first 30 days after enterostomy tube insertion sion of Neurology, The Hospital for Sick in children with SMA type 1. Children, University of Toronto, and St.
S
METHODS This study was conducted at the Hospital for Sick Children (SickKids), a tertiary care pediatric hospital in Toronto, Ontario, Canada. All patients who were SMA type 1 and who had enterostomy tubes placed at the SickKids from January 1994 to July 2004 were included in the study. Patients were identified through the Esh IGT database (Esh, Thornhill, Ontario, Canada). The study was approved by the SickKids Research Ethics Board. Patients were classified as having SMA type 1 if they presented with severe weakness in the first 6 months of life and were found to have a homozygous deletion of the SMN gene. Before the gene discovery the diagnosis was made on muscle biopsy. GERD HSC
Gastroesophageal reflux disease Hospital for Sick Children
PICU SMA
Pediatric intensive care unit Spinal muscular atrophy
Michael’s Hospital, Department of Public Health Sciences, Toronto, Ontario, Canada. Submitted for publication Mar 8, 2006; last revision received Jun 1, 2006; accepted Aug 21, 2006. Reprint requests: Sanjay Mahant, MD, FRCPC, Division of Paediatric Medicine, Paediatric Outcomes Research Team, The Hospital for Sick Children, The University of Toronto, Toronto, Ontario, Canada M5G1X8. E-mail: sanjay.mahant@sickkids. ca. 0022-3476/$ - see front matter Copyright © 2006 Mosby Inc. All rights reserved. 10.1016/j.jpeds.2006.08.048
837
A list of possible complications of enterostomy tube insertion were collected a priori.13 Early complications were defined as those occurring within 30 days of the procedure. Pneumonia, respiratory failure, unplanned intensive care admission, peritonitis, and death were classified as major complications. The percutaneous image-guided gastrostomy and gastrojejunostomy tube insertion technique has been well described.7,8 Children are observed in a postanesthetic care unit for 6 hours after the procedure. When stable, they are transferred to the pediatric ward for monitoring. Admission to the intensive care unit is not routine practice.
Statistical analysis Descriptive statistics are used to present baseline characteristics. Exact 95% confidence intervals were calculated for the proportion of subjects where the outcome was aspiration pneumonia, respiratory failure, an unplanned PICU admission, and death. Mean age of subjects with development of aspiration pneumonia or respiratory failure were compared by use of an exact Wilcoxon 2-sample test. Fisher’s exact test was used to evaluate the associations between a history of pneumonia before enterostomy tube placement and (1) respiratory failure and (2) aspiration pneumonia after enterostomy tube placement. All analyses were done with SAS 8.02 (SAS Institute, Inc, Cary, NC).
RESULTS Fourteen patients were identified through the Image Guided Therapy database as having SMA type 1 and enterostomy tube insertion. Two patients were excluded because their genetic testing did not show a deletion of the SMN gene, and muscle biopsy specimens were inconclusive. The median age at time of tube placement was 6.1 months (range 2.2 to 15.8 months), with a mean weight of 6.9 kg (range 2.7 to 14.6 kg) for the 12 patients. The primary indication for tube insertion in all patients was oral motor feeding dysfunction resulting in an inability to maintain adequate nutrition and hydration. All 7 patients who had a videofluoroscopic feeding study had evidence of aspiration of oral contents. All other children had clinical evidence of oral motor feeding dysfunction. No patients had a tracheostomy before or at the time of tube insertion. The tubes were placed under local anesthetic in 4 patients and sedation in 6 patients. General anesthetic was only required in the remaining 2 patients, both of whom were already intubated because of respiratory complications. There was a 100% success rate for enterostomy tube placement. Seven of the 12 patients had no major complications in the first 30 days. No patients had development of peritonitis or sepsis. For 2 patients, the gastrostomy tube was changed to a gastrojejunostomy tube because of gastroesophageal reflux disease. The 30-day mortality rate after the procedure was 16.7% (2/12) (95% CI 2.1%-48.4%). The first patient who died had a tube inserted at 5.5 months of age. This child had 838
Sy et al
2 aspiration pneumonias and 1 respiratory arrest before tube placement. After discharge from hospital post gastrostomy tube placement, the infant required PICU admission with presumed aspiration pneumonia. After discussions with the family, a palliative care plan was adopted, and the infant died 10 days after gastrostomy tube placement. The second patient who died had a tube placed at 11.7 months of age. This child had 1 aspiration pneumonia requiring PICU admission before tube placement and had development of aspiration pneumonia 6 days after gastrostomy tube placement. The tube was changed to a gastrojejunostomy tube to prevent reflux-induced aspiration. A palliative care plan was adopted, and the child died 29 days after the initial tube placement. Aspiration pneumonia occurred in 5/12 (41.6%) patients within 30 days of their procedure (95% CI 15.2%72.3%). Children with development of aspiration pneumonia were significantly older at time of tube insertion compared with those who did not have aspiration pneumonia (mean age 356 days vs 158 days, P ⫽ .03). Four patients (33%) (95 CI 10.0%-65.1%) required PICU admission for respiratory failure. They were older at time of tube insertion compared with those who did not have respiratory failure (mean age 365 days vs 183 days, P ⫽ .07), but this was not statistically significant. One of the 4 was managed with noninvasive ventilation, and the other 3 required intubation and ventilation. Seven patients had a history of pneumonia before tube insertion. Four of these children had development of aspiration pneumonia within 30 days after the procedure, and 4 had at least 1 episode of respiratory failure. Patients without a history of pneumonia before tube insertion (n ⫽ 5) did not have respiratory failure, and only 1 had aspiration pneumonia within 30 days of the procedure. The associations between a history of pneumonia before enterostomy tube placement and (1) respiratory failure and (2) pneumonia after enterostomy tube insertion had P values of .06 and .24, respectively.
DISCUSSION This study suggests that although the insertion of an enterostomy tube with the image-guided technique is technically successful with minimal anesthesia in children with SMA type 1, it may be associated with a high rate of early major respiratory complications, as well as death. Older age at time of tube insertion was associated with aspiration pneumonia, and this association approached significance with regard to development of respiratory failure. Older age, which may be a reflection of advanced neuromuscular weakness and bulbar dysfunction, may be an important predictor of early complications associated with tube insertion. Further studies are required to explore the relationship between age, advanced neuromuscular disease, and outcomes to determine the optimal timing of the procedure if it is to be undertaken. Friedman et al13 reported a mortality rate of 0.4% (with the only death related to peritonitis and sepsis) and a major complication rate of 5% in a study of complications associated with image-guided gastrostomy and gastrojejunostomy tubes in a heterogeneous group of children at our institution. The The Journal of Pediatrics • December 2006
30-day mortality rate (16.7%) and early major complication rate (41.6%) found in children with SMA type 1 in our study compares very unfavorably with these results. Few studies have specifically examined the impact or complications of enterostomy tube placement in children with SMA type 1 or neuromuscular disease.5,11,12 Birnkrant et al12 reviewed the course of 4 children with SMA type 1 treated with noninvasive ventilation and gastrostomy feeding. All had gastrostomy tubes placed between 2 and 9 months of age. Survival was only 1 to 3.5 months after presenting with severe aspiration. No patients died of tube placement. The impact of gastrostomy for these patients was unclear. Based on a review of 15 children with neuromuscular disease (which included 1 child with SMA type 1), Seguy et al11 questioned the use of gastrostomy feeding in children with severe congenital myopathy, severe respiratory disease, or severe gastroesophageal reflux disease (GERD) because of the potential respiratory complications and short term prognosis. Another study reported the use of gastrostomy in infants with SMA type 1 and briefly described 2 children with development of respiratory complications after gastrostomy tube insertion; however, outcome related to gastrostomy tube insertion was not the focus of the article.5 GERD that results in significant pulmonary complications may contribute to morbidity in children with SMA type 1 and neuromuscular disease.11 However, the management of GERD that is not responsive to maximal medical therapy has not been well studied in this population. Bach et al5 found no differences in hospitalization or mortality rates in patients with SMA type 1 after fundoplication. Because of the potential risks of surgery and anesthesia, patients in our study with significant GERD had their gastrostomy tube advanced to a gastrojejunostomy tube, which required no anesthetic. Given the retrospective study design and lack of control group, there are limitations to this study. Our findings imply an association but not necessarily causation. Given the natural history of the disease, it is difficult to know whether the deaths and early major complications are related purely to the underlying disease or are affected by the intervention. Prospective studies are required to determine the impact of enterostomy tube placement on mortality rate, respi-
ratory complications, quality of life, and the optimal timing of the procedure if it is to be undertaken. Parental perceptions of the impact of enterostomy tube feeding in their children with SMA type I are of paramount importance and require further study. It is imperative that the goals of tube feeding, natural history of the underlying neuromuscular disease, potential complications, and issues of quality of life are fully explored with the family before undertaking this intervention.
REFERENCES 1. Siddique N, Sufit R, Siddique T. Degenerative motor, sensory, and autonomic disorders. In: Goetz C, editor. Textbook of clinical neurology. 2nd ed. Philadelphia: WB Saunders; 2003. p 759-66. 2. Zerres K, Rudnik-Schoneborn S. Natural history in proximal spinal muscular atrophy: clinical analysis of 445 patients and suggestions for a modification of existing classifications. Arch Neurol 1995;52:518-23. 3. Ioos C, Leclairr-Richard D, Mrad S, Barois A, Estournet-Mathiaud B. Respiratory capacity course in patients with infantile spinal muscular atrophy. Chest 2004;126:831-7. 4. Chung BHY, Wong VCN, Ip P. Spinal muscular atrophy: Survival pattern and functional status. Pediatrics 2004;114:548-53. 5. Bach JR, Baird SJ, Plosky D, Navado J, Weaver B. Spinal muscular atrophy type 1: management and outcomes. Pediatr Pulmonol 2002;34:16-22. 6. Bach JR, Niranjan V. Spinal muscular atrophy type 1: a noninvasive respiratory management approach. Chest 2000;117:1100-5. 7. Chait PG, Baskin KM, Temple M, Connolly B. Pediatric gastrointestinal interventions. In: Stringer DA, Babyn PS, editors. Pediatric gastrointestinal imaging and intervention. 2nd ed. Hamilton: BC Decker; 2000. p. 97-160. 8. Chait PG, Weinberg J, Connolly BL, Pencharz P, Richards H, Clift JE, et al. Retrograde percutaneous gastrostomy and gastrojejunostomy in 505 children: a 4 1/2-year experience. Radiology 1996;201:691-5. 9. Hardart MKM, Truong RD. Spinal muscular atrophy type 1. The challenge of defining a child’s best interest. Arch Dis Child 2003;88:848-50. 10. Bush A, Fraser J, Jardine E, Paton J, Simonds A, Wallis C. Respiratory management of the infant with type 1 spinal muscular atrophy. Arch Dis Child 2005;90:709-11. 11. Seguy D, Michaud L, Guimber D, Cuisset JM, Devos P, Turck D, et al. Efficacy and tolerance of gastrostomy feeding in pediatric forms of neuromuscular diseases. J Parenter Enteral Nutr 2002;26:298-304. 12. Birnkrant DJ, Pope JF, Martin JE, Repucci AH, Eiben RM. Treatment of type I spinal muscular atrophy with noninvasive ventilation and gastrostomy feeding. Pediatr Neurol 1998;18:407-10. 13. Friedman JN, Ahmed S, Connolly B, Chait P, Mahant S. Complications associated with image-guided gastrostomy and gastrojejunostomy tubes in children. Pediatrics 2004;114:458-61.
Enterostomy Tube Placement In Children With Spinal Muscular Atrophy Type 1
839