Nissen fundoplication in the treatment of children with familial dysautonomia

Perioperative management of familial dysautonomia

“…Postoperative complications included primarily cyclic vomiting and pulmonary complications such as pneumonia, atelectasis, excessive secretions and respiratory arrest requiring reintubation on two occasions [6,7,9,12,16,[19][20][21]. Haemodynamic lability was reported in the postoperative period [6,7,9,12,16,[19][20][21], but less frequently than in the intraoperative period [6,7,[9][10][11][12][13][16][17][18][19]. One patient experienced a postoperative seizure [9] and one patient intraoperative confusion [15].…”

Laparoscopic Nissen fundoplication in children under 2 years of age

“…Operative risk is especially high in children with familial dysautonomia [1,12]. Concomitant impairment of gastrointestinal motility is also unique to the pediatric population.…”

“…It has been widely accepted in recent years that even small children with severe symptomatic GER, including those with neurological impairment, may benefit from an antireflux operation such as Nissen fundoplication [3,6,10]. The very same conclusion was drawn for children with familial dysautonomia [12].…”

A world without pain or tears*

“…Initially medical management with prokinetic agents, H 2 -antagonists, thickening of feeds, and positioning can be tried. However, if medical management is not successful, using the criteria of persistence of poor weight gain and growth, pneumonia, hematemesis, or apnea, then surgical intervention (fundoplication) is recommended [8,13,17,22,75].…”

“…However liquids tend to remain problematic and an increasing number of children with familial dysautonomia are being treated with Nissen fundoplication with gastrostomy [5,8,13,17,22,75]. …”