Bowel Perforation in Vascular Ehlers-Danlos Syndrome: Case Report and Comprehensive Review.

INTRODUCTION: Ehlers-Danlos syndromes (EDS) comprise a rare variety of genetic disorders, affecting all types of collagen. Herein, we describe a case of the vascular type of EDS, with coexisting segmental absence of intestinal musculature, while simultaneously performing a narrative review of the existing literature. CASE PRESENTATION: A 23-year-old male patient with a history of multiple abdominal operations due to recurrent bowel perforations and the presence of a high-output enterocutaneous fistula was admitted to our surgical department for further evaluation and treatment. After detailed diagnostic testing, the diagnosis of vascular-type EDS (vEDS) was made and a conservative therapeutic approach was adopted. In addition, a comprehensive review of the international literature was carried out by applying the appropriate search terms. RESULTS: The diagnosis of vEDS was molecularly confirmed by means of genetic testing. The patient was treated conservatively, with parenteral nutrition and supportive methods. Thirty-four cases of bowel perforation in vEDS have been reported so far. Interestingly, this case is the second one ever to report co-existence of vEDS with Segmental Absence of Intestinal Musculature. CONCLUSIONS: Establishing the diagnosis of vEDS promptly is of vital significance in order to ensure that patients receive appropriate treatment. Due to initial non-specific clinical presentation, EDS should always be included in the differential diagnoses of young patients with unexplained perforations of the gastrointestinal tract.

Long-Term Outcomes of Lymph Node Transfer in Secondary Lymphedema and Its Correlation with Flap Characteristics.

BACKGROUND: This retrospective study aimed to assess the impact of certain flap characteristics on long-term outcomes following microsurgical treatment in Breast Cancer-Related Lymphedema (BCRL) patients. METHODS: Sixty-four out of 65 BCRL patients, guided by the "Selected Lymph Node" ("SeLyN") technique, underwent Vascularized Lymph Node Transfer (VLNT) between 2012 and 2018. According to their surface size, flaps were divided into small (<25 cm2, n = 32) and large (>25 cm2, n = 32). Twelve large and six small flaps were combined with free abdominally based breast reconstruction procedures. Lymphedema stage, flap size, vascular pedicle and number of lymph nodes (LNs) were analyzed in correlation with long-term Volume Differential Reduction (VDR). RESULTS: At 36-month follow-up, no major complication was recorded in 64 cases; one flap failure was excluded from the study. Mean flap size was 27.4 cm2, mean LNs/flap 3.3 and mean VDR 55.7%. Small and large flaps had 2.8 vs. 3.8 LNs/flap (p = 0.001), resulting in 49.6% vs. 61.8% VDR (p = 0.032), respectively. Lymphedema stage and vascular pedicle (SIEA or SCIA/SCIP) had no significant impact on VDR. CONCLUSION: In our series, larger flaps included a higher number of functional LNs, directly associated with better outcomes as quantified by improved VDR.

Functional lymphatic reconstruction with the "Selected Lymph Node" technique guided by a SPECT-CT lymphoscintigraphy.

BACKGROUND: A new technique named "Selected Lymph Node" ("SeLyN") was evaluated, aiming to identify the most functional groin lymph nodes (LNs) for an effective LN transplantation. METHODS: Bilateral lower-limb SPECT-CT was performed in the upper-limb lymphedema patients, to select the most radioactive inguinal LN. Recorded data included demographics, stage, etiology of lymphedema, flap consistency in accordance to preoperative findings, flap size, number of LN, and harvesting time. Infection episodes per year and volume changes of the upper limbs were documented. Donor-site complications were recorded and lower-limb evaluation was performed through clinical examination, volume analysis, and lymphoscintigraphy. RESULTS: A total of 41 patients underwent a "SeLyN" transfer technique. The mean flap size was 28.34 cm2 containing a mean of 3.4 LNs. The mean time spent on flap harvest was 39 minutes. A mean 56.5% volume reduction (P < .001) and a mean 1.41 to 0.29 infection episodes per patient per year (P < .001) were recorded. Clinical evaluation and lymphography of the donor site advocated no major complications for a mean follow-up period of 42.5 months. CONCLUSIONS: "SeLyN" is a safe and effective technique in selecting the most suitable LNs, minimizing the donor-site morbidity, and decreasing the overall operating time.

A rectovaginal fistula after treatment with bevacizumab. A dangerous side effect needing emergency treatment.

Despite its effectiveness in the treatment of malignant tumors, bevacizumab is associated with a variety of side effects such as the formation of fistulas (i.e., tracheoesophageal, colovaginal, or rectovaginal). It is important to recognize immediately the emergence of this entity in order to discontinue bevacizumab permanently and treat the fistula.

A randomized control study of treating secondary stage II breast cancer-related lymphoedema with free lymph node transfer.

Microsurgical techniques are increasingly used for treating severe lymphoedema cases. The purpose of this study was to evaluate the effectiveness of free vascularized lymph node transfer (LNT) in stage II breast cancer-related lymphoedema patients in comparison with non-surgical management. During the last 3 years, 83 female patients were examined at our lymphoedema clinic. Finally, 36 cases were included in this study and randomly divided in two groups: group A patients (n = 18, mean age 47 years) underwent microsurgical LNT; followed by 6 months of physiotherapy and compression, while group B patients (n = 18, mean age 49 years) were managed by physiotherapy and compression alone for 6 months. Patients of both groups removed their elastic garments after 6 months and were re-examined 1 year later. All the 36 patients had detailed evaluation of the affected extremity including limb volume measurement, infection episodes and scale scoring of pain, feeling of heaviness and functional status both at baseline and 18 month. Limb volume reduction was observed in both groups; mean reduction was greater in group A (57 %) than in group B (18 %). Infection episodes in group A were significantly reduced compared to those in group B patients. All group A patients reported painless and feeling of heaviness-free extremities with overall functional improvement, while the corresponding changes in group B patients were no more than marginal. Moreover, the LNT procedure was estimated as cost effective compared to conservative treatment alone. LNT represents an effective therapeutic approach for stage II lymphoedema patients; it significantly reduces limb volume, decreases recurrent infections and improves the overall function.