Long-term Result of Treatment of Prader-Willi Syndrome by Scopinaro's Bilio-pancreatic Diversion. Study of Three Cases and the Effect of Dextrofenfluramine on the Postoperative Evolution

Abstract

The Prader-Willi Syndrome shortens the life of patients due to the morbid obesity which it entails. The compulsive hyperphagia associated with it makes a dietetic treatment or a gastroplasty difficult. This study presents the case histories of three patients suffering from the Prader-Willi syndrome who were operated on by means of a Scopinaro's bilio-pancreatic diversion. Following a marked reduction the first year, the weight loss stabilized and then tended to diminish. The observation of three cases which continued for two and a half to six years did not reveal any considerable metabolic problems. The deficiency of iron, vitamins D and B12 as well as folic acid had to be made up by supplementation. These results are comparable with the most favorable ones in the literature. Even if the effect on the weight loss is not spectacular, the operation manages to hold off the development of the obesity, inexorable for those with the Prader-Willi syndrome, and prevents lethal complications, without having notable side effects. Lifting coercive dietary measures improves the quality of life.

BioEnterics Intragastric Balloon for Treatment of Morbid Obesity in Prader–Willi Syndrome: Specific Risks and Benefits

“…Yet, in these patients, a drastic body weight reduction is mandatory to reduce the risk of cardio-respiratory and metabolic complications and to improve life expectancy. In the last three decades, several bariatric surgical techniques were applied to treat morbid obesity in PWS patients, such as trunkal vagotomy [13], jejunoileal bypass [14,15], vertical banded gastroplasty [16,17], adjustable silicon gastric banding [18], gastric bypass [19], and biliopancreatic diversion (BPD) [20][21][22][23][24][25][26]. However, a higher morbidity and mortality rate in these patients, compared to those with simple obesity, has been reported [27].…”

Prader-Willi syndrome–associated obesity treated by biliopancreatic diversion with duodenal switch. Case report and literature review

“…Two other reports [26,27] claim that Scopinaro BPD offers the best long-term outcome in patients with PWS. On the contrary, Laurent-Jacard et al [14] and Grugni et al [25] found the weight loss after BPD in their patients with PWS to be insignificant, but that was probably caused by the large gastric remnant (250 mL). Dumping syndrome was occasionally a problem after Scopinaro BPD, causing lifedisturbing diarrhea.…”

“…As far as age is concerned, even if the literature presents surgical treatment of obese PWS patients between the age of 11 and 40, most of them are between the age of 18 and 25 years [13][14][15]. The main reason for avoiding bariatric surgery in children and adolescents is that such an action might impair the normal growth and development of the patient.…”

“…Table 3, 42 patients (including our case) with PWS-associated obesity treated with bariatric surgery were found in the English literature. Several surgical techniques have been reported on the matter, such as trunkal vagotomy [17], jejunoileal bypass [13,18,19], vertical banded gastroplasty (VBG) [20,21], adjustable silicon gastric banding [22], gastric bypass [23,24], and BPD [14,15,[25][26][27]. However, the optimal procedure is far from settled.…”

Bariatric surgery in individuals with severe cognitive impairment: report of two cases

“…6,7,[20][21][22] With the sole exception of the study by Miyata et al, 14 in which an individual with Prader-Willi syndrome presented an initial improvement of metabolic and weight conditions, followed by progressive worsening, the vast majority of the studies have consistently observed significant improvements following a variety of techniques, regarding both metabolic features and weight loss, albeit to a lesser extent than what is observed in the general population with obesity when bariatric surgery is implemented. [6][7][8][9][10][11][12][13][15][16][17][18][19] More studies are necessary, in order to provide evidence of higher quality that could lead to possible algorithms for this heterogeneous group of individuals. There is recent evidence that even mild cognitive impairment may play a role in the outcomes from bariatric surgery, such that it may lead to worse results and, especially, poor adherence to long-term follow-up.…”