Laparoscopic Nissen Fundoplication During Gastrostomy Tube Placement and Noninvasive Ventilation May Improve Survival in Type I and Severe Type II Spinal Muscular Atrophy

Abstract

Progressive respiratory muscle weakness with bulbar involvement is the main cause of morbidity and mortality in type I and severe type II spinal muscular atrophy. Noninvasive positive pressure ventilation techniques coupled with laparoscopic gastrointestinal procedures may allow for improved morbidity and mortality. The authors present a series of 7 spinal muscular atrophy patients (6 type I and 1 severe type II) who successfully underwent laparoscopic gastrostomy tube insertion coupled with Nissen fundoplication and early postoperative extubation using noninvasive positive pressure ventilation techniques. The authors measured the length of survival and the frequencies of pneumonia and hospitalization before and after surgery as outcomes of these new surgical and medical interventions. All 7 patients had respiratory symptoms (unmanageable oropharyngeal secretions, cough, pneumonia), difficulty feeding, and weight loss. Six patients had documented reflux via diagnostic testing preoperatively. Five patients were on noninvasive positive pressure ventilation and other supportive respiratory therapies prior to surgery. All 7 patients survived the procedures. By August 2006, 5 patients with type I and 1 with severe type II spinal muscular atrophy were alive and medically stable at home 1.5 months to 41 months post-op. One patient with type I expired approximately 5 months post-op due to obstructive apnea. This case series demonstrates that laparoscopic gastrostomy tube placement coupled with Nissen fundoplication and noninvasive positive pressure ventilation can be successfully used as a treatment option to allow for early postoperative extubation and to optimize quality of life in type I and severe type II spinal muscular atrophy patients.

Severe SMA mice show organ impairment that cannot be rescued by therapy with the HDACi JNJ-26481585

“…Diarrhea or fluid in the abdomen are not commonly described features of SMA in human, but reflux esophagitis is a known feature. 60 Occasional case reports of constipation later developing into diarrhea as well as diminished haustration of the colon and small intestine distension exist. 61,62 Acute heart failure explains sudden death.…”

Early laparoscopic fundoplication and gastrostomy in infants with spinal muscular atrophy type I

“…Only 1 study to date has evaluated the outcome of patients with SMA undergoing antireflux procedures [12]. Unfortunately, this study did not evaluate type I patients, specifically those who are theoretically subject to the highest perioperative risks.…”

Dislodgement of Laparoscopic Gastrostomy During Early Postoperative Period: an unusual complication of non-invasive ventilation

“…7 Although recent esophageal surgery is considered as a contraindication for NIV by some of the authors 7 , there are studies reporting the successful and safety use of NIV in the immediate early postoperative periods of laparoscopic Nissen fundoplication, Roux-en-Y gastric bypass, and even oesophagectomy. [8][9][10] So we decided to perform NIV to our patient early after procedure.…”