Abstract
BackgroundLoeys-Dietz syndrome (LDS) is a systemic connective tissue disease (CTD) associated with a predisposition for intestinal inflammation, food allergy, and failure to thrive, often necessitating nutritional supplementation via gastrostomy tube. Poor wound healing has also been observed in in some patients with CTD, potentially increasing the risk of surgical interventions. We undertook to determine the safety and efficacy of gastrostomy tube placement in this population.MethodsWe performed a retrospective cohort study of 10 LDS patients who had a total of 12 gastrostomy tubes placed.ResultsNo procedural complications occurred, although one patient developed buried bumper syndrome in the near post-procedural time period and one patient had a small abscess at a surgical stitch. Most patients exhibited improvements in growth, with a median immediate improvement in BMI Z-score of 0.2 per month following the institution of gastrostomy tube feedings. Those with uncontrolled inflammation due to inflammatory bowel disease or eosinophilic gastrointestinal disease showed the least benefit and in some cases failed to demonstrate significant weight gain despite nutritional supplementation.ConclusionsGastrostomy tube placement (surgical or endoscopic) is a generally safe and a reasonable therapeutic option for patients with LDS despite their underlying CTD.
Highlights
Loeys-Dietz syndrome (LDS) is a systemic connective tissue disease (CTD) associated with a predisposition for intestinal inflammation, food allergy, and failure to thrive, often necessitating nutritional supplementation via gastrostomy tube
Original records regarding gastrostomy tube placement were not available for one patient who has been excluded from further discussion
Patients 2 and 5 had gastrostomy tubes placed twice Body mass index (BMI) Z-score from Centers for Disease Control (CDC) growth chart; at ages less than 2 years old, the World Health Organization (WHO) weight/length Z-score was used †Food Allergy (FA) food allergy; eosinophilic gastrointestinal disease (EGID) eosinophilic GI disease; Ulcerative colitis (UC) ulcerative colitis *PEG Percutaneous endoscopic gastrostomy; Lap Laproscopic; Bilateral inguinal hernia repair (BIH) bilateral inguinal hernia repair; LIH Left inguinal hernia repair §Age at placement rounded to nearest year **in the immediate post-operative period ‡Most recent BMI Z-score or last BMI Z score prior to discontinuation of gastrostomy tube feeds years follow up
Summary
The records of 182 Type 1 and 2 LDS patients were evaluated. Original records regarding gastrostomy tube placement were not available for one patient who has been excluded from further discussion. Two of the 10 patients underwent a second gastrostomy tube placement after the originally placed tube was removed (in both cases as a result of patient/family decision). Patient 2 had the second gastrostomy placed endoscopically at the original site. Patient 5 had the second gastrostomy placed at a new site. Half of the patients had Type 1 LDS and half were male. The indication for 10/12 gastrostomy tube placements was failure to thrive. Three patients were less than 1 year of age at the time of placement. Patient LDS Sex Comorbidities† Technique* Type age at Indication BMI Z-score at BMI Δ Z-
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