Objective:To update the classification of extremity primary lymphedema (PLE) based on age of onset, lymphatic anomalies and genetics.Methods:A prospective research method was adopted. Patients with lower and upper limb PLE who visited Department of Plastic & Reconstructive Surgery, Shanghai Ninth People’s Hospital, Shanghai Jiao Tong University School of Medicine from January 207 to December 2021 were selected. Sex, age of onset, location, family history and morbidity were documented. The lymphatic imaging findings of magnetic resonance lymphography (MRL), indocyanine green lymphography (ICGL) and lymphoscintigraphy (LSG), skin tissue histology and whole exome sequencing were evaluated. Descriptive statistical method was used for data statistics.Results:A total of 1 046 patients were included, among whom 1 013 had lower extremity involvement and 33 had upper extremity involvement. Divided by the age of onset, there were 237 cases of congenital (<1 year old) and 809 of late-onset (≥1 year old), with a ratio of about 1∶4. Among the late-onset patients, most patients began to suffer the disease during adolescence (11-20 years old), with a total of 276 cases. Among patients with congenital lower limb PLE, 12.7% (26/204) had a family history. Among those with late-onset of PLE, 6.6% (53/809) had a family history. A total of 225 patients underwent whole exome sequencing (208 cases in the lower limbs and 17 cases in the upper limbs), showing that 37 patients (17.8%, 37/208) with lower limb PLE were found to carry 38 pathogenic variants in FLT4, GJC2, CELSR1, PTPN14, FOXC2 and GATA2, only 1 patient (5.9%, 1/17) with upper limb PLE was found to carry a PIEZO1 compound heterozygote variant. Three major lymphatic anomalies were identified, in which segmental lymphatic dysfunction, characterized by delayed or partial demonstration of lymph vessels, was the most common type and associated with FLT4, GJC2, CELSR1, and PTPN14 mutations. The second most common type was lymphatic hyperplasia, which was associated with FOXC2 and GATA2 variants, followed by initial lymphatic aplasia or dysfunction and associated with FLT4 and PIEZO1 mutation.Conclusion:A classification of extremity PLE is proposed based on age of onset, lymphatic anomalies and genetics, which are segmental lymphatic dysfunction, lymphatic hyperplasia, and initial lymphatic aplasia or dysfunction.

Objective:To update the classification of extremity primary lymphedema (PLE) based on age of onset, lymphatic anomalies and genetics.Methods:A prospective research method was adopted. Patients with lower and upper limb PLE who visited Department of Plastic & Reconstructive Surgery, Shanghai Ninth People’s Hospital, Shanghai Jiao Tong University School of Medicine from January 207 to December 2021 were selected. Sex, age of onset, location, family history and morbidity were documented. The lymphatic imaging findings of magnetic resonance lymphography (MRL), indocyanine green lymphography (ICGL) and lymphoscintigraphy (LSG), skin tissue histology and whole exome sequencing were evaluated. Descriptive statistical method was used for data statistics.Results:A total of 1 046 patients were included, among whom 1 013 had lower extremity involvement and 33 had upper extremity involvement. Divided by the age of onset, there were 237 cases of congenital (<1 year old) and 809 of late-onset (≥1 year old), with a ratio of about 1∶4. Among the late-onset patients, most patients began to suffer the disease during adolescence (11-20 years old), with a total of 276 cases. Among patients with congenital lower limb PLE, 12.7% (26/204) had a family history. Among those with late-onset of PLE, 6.6% (53/809) had a family history. A total of 225 patients underwent whole exome sequencing (208 cases in the lower limbs and 17 cases in the upper limbs), showing that 37 patients (17.8%, 37/208) with lower limb PLE were found to carry 38 pathogenic variants in FLT4, GJC2, CELSR1, PTPN14, FOXC2 and GATA2, only 1 patient (5.9%, 1/17) with upper limb PLE was found to carry a PIEZO1 compound heterozygote variant. Three major lymphatic anomalies were identified, in which segmental lymphatic dysfunction, characterized by delayed or partial demonstration of lymph vessels, was the most common type and associated with FLT4, GJC2, CELSR1, and PTPN14 mutations. The second most common type was lymphatic hyperplasia, which was associated with FOXC2 and GATA2 variants, followed by initial lymphatic aplasia or dysfunction and associated with FLT4 and PIEZO1 mutation.Conclusion:A classification of extremity PLE is proposed based on age of onset, lymphatic anomalies and genetics, which are segmental lymphatic dysfunction, lymphatic hyperplasia, and initial lymphatic aplasia or dysfunction.

Objective To evaluate the effectiveness of autologous platelet-rich gel (APG)in the repair of diabetic foot ulcers.Methods This study was a single-center,prospective,randomized controlled trial.A total of 60 patients with diabetic foot ulcers (Wagner grade 2 - 3 )were randomly divided into autologous APG group (treatment group)and recombinant bovine basic fibroblast growth factor gel group (control group).After 8 weeks, we compared wound healing rate and wound healing time at five levels (overall ulcer,superficial ulcer,sinus ulcer, Wagner 2 and Wagner 3)in the two groups.Results In APG treatment group and control group,the healing rate of overall sample ulcer (93.33% vs .63.33%,P =0.005),sinus ulcer (84.62% vs .36.36%,P =0.033),Wagner 3 (81.82% vs .30%,P =0.030)differed significantly,but did not significantly differ in superficial ulcer (100%vs .78.95%,P =0.106)or Wagner 2 (100% vs .80%,P =0.106).Ulcer healing time was 31 d vs .41.5 d,23 d vs .32 d,32 d vs .56 d,25 d vs .32 d,38 d vs .56 d,with significant difference between the two groups (P <0.05). Conclusion Autologous platelet-rich gel can effectively increase the curative rate of diabetic foot and shorten healing time.

OBJECTIVETo demonstrate the feasibility of extralevator abdominoperineal excision (ELAPE) for locally advanced low cancer in China.

METHODSA prospective multicenter clinical trial was carried out by 7 general hospitals across China from August 2008 to October 2011. A total of 102 patients underwent ELAPE for primary locally advanced low rectal cancer. There were 60 male and 42 female patients. The patients' characteristics, complications and prognosis were recorded.

RESULTSAll patients underwent the ELAPE procedure successfully. The median operating time was 180 minutes (range 110-495 minutes) and median intraoperative blood loss was 200 ml (range 50-1000 ml). The rates of sexual dysfunction, perineal complications, urinary retention, and chronic perineal pain were 40.5%, 23.5%, 18.6% and 13.7%, respectively. Chronic perineal pain was associated with coccygectomy (12 months postoperatively, t = 8.06, P < 0.01), and the pain might gradually ease over time. Reconstruction of pelvic floor with biologic mesh was associated with lower rate of perineal dehiscence (χ(2) = 13.502, P = 0.006) and overall perineal wound complications (χ(2) = 5.836, P = 0.016) compared with primary closure. A positive circumferential margin (CRM) was demonstrated in 6 (5.9%) patients, and intraoperative perforations occurred in 4 (3.9%) patients. All CRM involvement and intraoperative perforation located at anteriorly and anterolaterally. The local recurrence was 4.9% at a median follow-up of 35 months (range, 18-58 months).

CONCLUSIONSELAPE performed in the prone position for low rectal cancer leads to a reduction in CRM involvement, intraoperative perforations, and local recurrence, but it might result in a little high rate of perineal wound related complications. Reconstruction of pelvic floor with biologic mesh might lower the rate of perineal wound complications.

Adult ; Aged ; Digestive System Surgical Procedures ; methods ; Female ; Humans ; Male ; Middle Aged ; Perineum ; surgery ; Postoperative Complications ; Prognosis ; Prospective Studies ; Rectal Neoplasms ; surgery ; Treatment Outcome

Objective To report the preliminary result of cylindrical abdominoperineal resection (cylindrical APR) and pelvic reconstruction with human acellular dermal matrix (HADM). Methods Cylindrical APR was performed in 13 consecutive patients with advanced very low rectal cancer between January 2008 and April 2009. The mesorectum was not dissected off the levator muscles at abdominal part of the operation, the perineal part of the operation was done in the prone position. The levator muscles were exposed circumferentiaUy. The coccyx and part of the 5th sacrum were dissected and Waldeyer" s fascia divided. The levator muscles were divided laterally on both sides from posterior to anterior. The remaining pelvic floor muscle fibers were divided just posterior to the transverse perineal muscles and the levator muscles were resected en bloc with the anus and lower rectum. The specimen was cylindrical. The pelvic defects were reconstructed with HADM. Results There was no bowel perforation, and all specimens were proved CMR negative by pathology. Perineal wounds were healed uneventfully. After 8 months" follow-up, no patient developed perineal wound breakdown, bulge or hernia. There was 1 patient developed perineal wound infection; one patient developed seroma and three with perineal pain. Five patients with short-term urinary retention recovered within 10 days. Conclusion Clinical APR and HADM pelvic reconstruction can reduce circumferential margin positive and rectum perforation rate, and made the procedure easier and safer without increasing complications.