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Background/Aims@#Enteroenteric intussusception in Peutz-Jeghers syndrome (EI-PJS) is traditionally treated by surgery. However, enteroscopic treatment is a minimally invasive approach worth attempting. We aimed to develop a risk scoring system to facilitate decision-making in the treatment of EI-PJS. @*Methods@#This was a single-center case-control study, including 80 patients diagnosed with PJS and coexisting intussusception between January 2015 and January 2021 in Air Force Medical Center. We performed logistic regression analysis to identify independent risk factors and allocated different points to each subcategory of risk factors; the total score of individuals ranged from 0 to 9 points. Then, we constructed a risk stratification system based on the possibility of requiring surgery: 0–3 points for “low-risk,” 4–6 points for “moderate-risk,” and 7–9 points for “high-risk.” @*Results@#Sixty-one patients (76.25%) were successfully treated with enteroscopy. Sixteen patients (20.0%) failed enteroscopic treatment and subsequently underwent surgery, and three patients (3.75%) received surgery directly. Abdominal pain, the diameter of the responsible polyp, and the length of intussusception were independent risk factors for predicting the possibility of requiring surgery. According to the risk scoring system, the incidence rates of surgery were 4.44% in the low-risk tier, 30.43% in the moderate-risk tier, and 83.33% in the high-risk tier. From low- to high-risk tiers, the trend of increasing risk was significant (p<0.001). @*Conclusions@#We developed a risk scoring system based on abdominal pain, diameter of the responsible polyps, and length of intussusception. It can preoperatively stratify patients according to the risk of requiring surgery for EI-PJS to facilitate treatment decision-making.
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Data of 7 patients with complex benign esophageal strictures (CBESs) who underwent endoscopic longitudinal incision combined with local injection of bleomycin were retrospectively reviewed at Air Force Medical Center from August 2018 to November 2019.The length of preoperative esophageal stenosis was 4-14 cm and the minimum diameter of esophageal stenosis was 0.2-0.4 cm in 7 cases. The procedure was successful for all 7 patients.No adverse events occurred during 5-14 months of follow-up period.Restenosis and dysphagia occurred in 5 cases.The interval between the first endoscopic treatment and the recurrence of esophageal stenosis was 30-120 days.Among the 5 cases of recurrence, 4 cases remained unobstructed after 2 treatments and 1 case remained unobstructed after 4 treatments. The dysphagia scores of 7 patients were graded from 0 to 1 by the end of follow-up. Endoscopic longitudinal incision combined with bleomycin therapy in treatment of CBESs is safe and effective.
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Objective:To evaluate the efficacy and safety of balloon-assisted enteroscopy (BAE) in the follow-up treatment of small intestinal polyps in patients with Peutz-Jeghers syndrome (PJS).Methods:From February 2005 to September 2019, the clinical data of patients with PJS who visited Air Force Medical Center many times and underwent BAE for small intestinal polyps surveillance and treatment were collected. The number of follow-up treatment times under BAE, operation-related complications and conservative theragy or surgical treatment were observed. Spearman rank correlation analysis was used to analyze the correlation between follow-up treatment times under BAE and the number and maximum diameter of small intestinal polyps.Results:A total of 165 PJS patients were enrolled, including 98 males and 67 females, and the mean age of first visit was (22.5±9.4) years. A total of 664 BAE operations were performed in the 165 PJS patients. The number of BAE operations and the number of follow-up treatments for each patient were 4.0±1.8 and 2.8±1.1, respectively. There were 26, 52, 35, 28, 12, 4, 2, 2, 2 and 2 patients who underwent 2, 3, 4, 5, 6, 7, 8, 9, 10 and 11 BAE operations, respectively. There were 165, 76, 30, 12, 4 and 4 patients who received 2, 3, 4, 5, 6 and 7 follow-up treatments, respectively. During the first to the fifth times of BAE follow-up treatment, the numbers of small bowel polyps resected under BAE of each patient were 7.0 (2.0, 11.0), 4.0 (2.0, 10.0), 3.0 (1.0, 8.5), 5.5 (1.8 , 10.3) and 3.0 (2.0, 6.8), respectively; and the maximum diameters of resected small intestinal polyps were 4.5 cm(3.0 cm, 6.0 cm), 3.0 cm(1.5 cm, 4.0 cm), 2.0 cm (1.5 cm, 3.0 cm), 3.0 cm(2.5 cm, 4.0 cm) and 2.5 cm(1.5 cm, 4.0) cm, respectively. The results of Spearman rank correlation analysis indicated that the number of follow-up treatment under BAE was negatively correlated with the number and maximum diameter of resected small intestinal polyps ( r s=-0.141, P=0.003; r s=-0.400, P<0.01). The total incidence of operation-related complications of small intestinal polyps resection under BAE was 3.2% (21/664), and 61.9%(13/21)of the complications were cured by conservative therapy. During the follow-up period, there were 26 cases of PJS complicated with intestinal intussusception, of which 20 cases (76.9%, 20/26) disappeared after BAE resection of small intestinal polyps. A total of 13 patients received surgery for intussusception (6 cases) or large polyps blocking the intestinal lumen (7cases). Conclusions:BAE is safe and effective in the follow-up treatment of small intestinal polyps in PJS. It can reduce the number and maximum diameter of small intestinal polyps, prevent and treat the complications related to small intestinal polyps, and effectively reduced surgical treatments.
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Objective:To analyze the relationship between STK11 mutation and the cumulative risk of intussusception in patients with Peutz-Jeghers syndrome (PJS). Methods:From December 2017 to June 2019, the clinical data of 167 patients with PJS in Air Force Medical Center were collected, including gender, age, family history, age of first intussusception and results of gene test. Kaplan-Meier was used to analyze the cumulative risk of intussusception in patients with different mutation types. Wilcoxon rank sum test and log-rank test were used for statistical analysis.Results:Among 167 patients, 89.8% patients (150/167) had S TK11 mutation, and 50.7% of the mutation sites were found in exons 1, exon 4 and exon 5. And 70.6% patients (118/167) developed intussusception. The median age of patients with intussusception for the first time was 15 years (ranged from 2 to 52 years). Among 118 PJS patients with intussusception, 53 patients had family history and 65 patients had no family history; 70 cases were male and 48 cases were female. There were no statistically significant differences in the cumulative risk of intussusception between PJS patients with and without family history, and between male and female PJS patients (both P>0.05). There were 107 cases (90.7%) with STK11 mutation ( STK11 mutation group) and 11 cases (9.3%) without STK11 mutation ( STK11 non-mutation group). The median age of first intussusception of STK11 gene mutation group was younger than that of STK11 non-mutation group, and the difference was statistically significant ( Z=-2.108, P=0.035). In STK11 mutation group, 29 cases (27.1%) were nonsense mutations (nonsense mutation group), 23 cases (21.5%) were frameshift mutations (frameshift mutation group), 21 cases (19.6%) were missense mutations (missense mutation group), 26 cases (24.3%) were splicing mutations (splicing mutation group) and 8 cases (7.5%) of other mutations. There were statistically significant differences in the cumulative risk of intussusception between the STK11 mutation group and the STK11 non-mutation group, the STK11 splicing mutation group and the STK11 non-mutation group, STK11 missense mutation group and the STK11 non-mutation group ( χ2=5.570, 10.167 and 6.653, all P<0.05). There were no statistically significant differences in the cumulative risk of intussusception between STK11 nonsense mutation group and STK11 non-mutation group, between STK11 frame-shift mutation group and STK11 non-mutation group, among groups with different mutation types of STK11 gene (all P>0.05). Conclusions:The younger the age of first intussusception in PJS patients with STK11 mutation, the higher the cumulative risk. The mutation type of STK11 gene has potential value for the risk assessment of intussusception in PJS patients.