Experience With Vertical Sleeve Gastrectomy in Adolescent and Young Adult Ehlers-Danlos Syndrome Patients: a Case Series and Review of the Literature

Abstract

Ehlers-Danlos syndromes (EDSs) are a diverse and rare group of inherited disorders affecting connective tissue, characterized by joint hypermobility, skin and tissue hyperextensibility, and friable vessels [1, 2]. These disorders are classified into thirteen subtypes based on phenotypic variations, inheritance pattern, and known genetic mutations [3]. EDSmutations lead to defective collagen fibers, which reduce connective tissue structural integrity and place these patients at risk for spontaneous rupture of organs and vessels, recurrent hernias, bowel dysmotility (especially gastroparesis), and surgical complications [4, 5]. Patients with EDS can also have significant gastrointestinal symptomatology, including gastroesophageal reflux disease, dysphagia, and chronic constipation among others [6]. Pediatric severe obesity prevalence is a worsening trend in the United States, affecting approximately 9% of adolescents [7, 8]. Youth who are overweight or have obesity are at risk for developing medical, social, and mental health consequences which persist into adulthood and increase their likelihood for early morbidity and mortality [9]. Metabolic and bariatric surgery (MBS) has been determined to be the most effective treatment option for adults with severe obesity and is now recognized by the American Academy of Pediatrics (AAP) as the treatment of choice for children with class III obesity (BMI ≥ 140% of the 95th percentile for age) or with class II obesity (BMI ≥ 120% of the 95th percentile for age) and an associated comorbid condition [10]. While adolescents with EDS are not shown in the literature to be at higher risk of developing severe obesity than their peers, there is evidence that the pro-inflammatory effects of obesity can increase the severity of their chronic disease [11, 12].