An 18-year-old patient with Prader-Willi syndrome: a case report on dental management under sedation and general anesthesia

Abstract

Prader-Willi syndrome (PWS) is a rare genetic disorder reported rarely in dentistry. Dental practitioners should know the features of PWS because affected patients have a variety of dental symptoms. The current report describes a case of PWS. An 18-year-old male patient presented with traumatic injuries. Initial emergency treatments were performed under sedation, and further treatments were conducted under general anesthesia. After adequate healing, periodic follow-up and dietary management according to the patient's age and nutritional phase were recommended. Dental management of PWS patients consists of active preventive measures in addition to dietary consultation according to age and nutritional phase.

<p>Oral health in patients with Prader-Willi syndrome: current perspectives</p>

“…A variety of oral diseases are found in patients with PWS (Table 2) such as caries,1,17,21,25–31 hypoplastic enamel,1,17,25,28,31 viscous saliva,1,17,21,25,30–32 periodontal disease,25–28 tooth wear,1,26,27,31,32 dental erosion,33 rampant caries,1,29,31 oral microsomia,25,31 dental malocclusion,1,17,21,26–29 candidiasis,25,27 erythematous lesions of the oral mucosa,27 increased biofilm accumulation,1,21,25,31,32 gingivitis,1,25,27,31,32 angular cheilitis, dry mouth,28 demineralization,21,25,31 dentoalveolar abscess,29,31 hypotonic tongue,21,31 mouth breathing,21,31,32 root stumps30 and a delay in tooth eruption 28Table 2Oral manifestation, referenceOral manifestation Júnior et al, 2005Scardina; Fuca; Messina, 2007Bailleuil-Forestier et al, 2008Yanagita et al, 2011Witt et al, 2011Setti et al, 2012Gardens et al, 2014Song et al, 2015Hurren, Flack, 2016 (review)Tayal et al, 2016Roman-Torres et al, 2017Olczak-Kowalczyk et al, 2018*…”

“…The cases reported cooperation by the patient with PWS 26,27. However, poor cooperation encourages conscious sedation29 or general anesthesia 29,31. Stubbornness, aggression, temper tantrums, and impulsivity were observed in an 18-year-old PWS patient in the first oral intervention.…”

“…Emergency treatment of the traumatic injury, a crown fracture of the maxillary central incisors caused by a fall with pulp involvement were planned. Other treatments were made under general anesthesia 29…”

“…PWS patients have a higher anesthesiologic risk 5,29. Airway management is difficult due to scoliosis, a high palatal vault, and micrognathia.…”

Special Care Dentistry in a Patient with Prader–Willi Syndrome through the Use of Atraumatic Restorative Treatment under General Anesthesia

“…The PWS needs to be well known and worked in the dental office through a multidisciplinary perspective, aiming at an accurate and effective assistance and determining a reestablishment of the patient's oral health [ 3 ]. The scientific literature mentions that there is a scarcity of studies about PWS patients' oral conditions [ 12 , 13 ]; however, there are reports of ambulatory services, even with patients with aggressive behavior [ 11 ]. Other studies do not report intervention under general anesthesia [ 10 , 14 ].…”