BACKGROUND: Arterial tortuosity syndrome is a rare Autosomal recessive disease that leads to a loss of function of the connective tissues of the body, this happens due to a mutation in the solute carrier family 2 member 10 (SLC2A10) gene. ATS is more likely to occur in Large and medium-sized arteries including the aorta and pulmonary arteries. This syndrome causes the arteries to be elongated and tortuous, This tortuosity disturbs the blood circulation resulting in stenosis and lack of blood flow to organs and this chronic turbulent flow increases the risk of aneurysm development, dissection and ischemic events.
METHODS: A 2 years old Arabian female child was diagnosed with ATS affecting the pulmonary arteries as a newborn, underwent a pulmonary arterial surgical reconstruction at the age of 2 years old due to the development of pulmonary artery stenosis with left pulmonary artery having a peak gradient of 73 mmHg with a peak velocity of 4.3 m/s and the right pulmonary artery having a peak gradient of 46 mmHg with a peak velocity of 3.4 m/s causing right ventricular hypertension. After surgical repair the left pulmonary artery has a peak pressure gradient of 20 mmHg, with the right pulmonary artery having a peak pressure gradient of 20 mmHg.
CONCLUSIONS: ATS is a rare genetic condition that affects the great arteries especially the pulmonary arteries causing stenotic and tortuous vessels that may be central branches or distal peripheral branches that leads to severe right ventricular dysfunction and hypertension. We believe that surgical treatment provides the optimum outcomes when compared to transcather approaches especially when the peripheral arteries are involved. Some challenges and hiccups might occur, especially lung reperfusion injury that needs to be diagnosed and treated accordingly.

BACKGROUND: The current study presents a novel and precise surgical technique for complete reconstruction of the aortic valve using glutaraldehyde-treated autologous pericardium in a patient with aortic valve disease and endocarditis. The technique aims to provide a more effective and reproducible method for aortic valve repair, with the goal of improving outcomes and quality of life for patients with aortic valve disease.
METHODS: A 35-year-old Iranian male with aortic valve disease and endocarditis underwent aortic valve reconstruction surgery. Preoperative echocardiography showed a degenerative aortic valve with severe regurgitation, reduced left ventricular ejection fraction, and specific aortic root dimensions. The surgical technique involved precise measurements and calculations to design the size and shape of the new aortic valve cusps using autologous pericardium, with the goal of optimizing coaptation and function. The surgeon calculated the intercommissural distance based on the aortic annulus diameter to determine cusp size and shape. He tailored the pericardial cusps to have a height equal to 80% of the coaptation margin length. Detailed suturing techniques were used to ensure proper alignment and coaptation of the new cusps. Intraoperative evaluation of the valve function using suction and transesophageal echocardiography showed good coaptation and minimal residual regurgitation. At the 3-year follow-up, the patient had a well-functioning aortic valve with only trivial leak and was in satisfactory clinical condition.
CONCLUSIONS: Glutaraldehyde-treated autologous pericardium is a validated leaflet alternative, and the causes of its failure are late annular dilatation and other technique breakdowns. Current evidence reveals that aortic valve reconstruction with glutaraldehyde-treated autologous pericardium is associated with many advantages with the potential to improve patient outcomes and quality of life. Further clinical studies are warranted to evaluate the long-term durability and efficacy of this approach.

OBJECTIVE: Oronasal fistulas are common sequelae following cleft lip and palate surgery and can significantly impact a patient\'s quality of life. They result from various factors, including surgical techniques, tissue management, and patient-specific factors. This case report explores the modern approach to oronasal fistula closure using periodontal plastic surgery principles.
METHODS: The report presents two cases of patients with oronasal fistulas due to previous maxillofacial surgical intervention. These patients underwent microsurgical procedures that involved partial flap thickness preparation of the fistula areas, the use of connective tissue grafts from the palate, and meticulous suturing techniques to ensure graft integrity. The procedures were performed in stages, and postoperative care was provided.
RESULTS: Both cases demonstrated successful fistula closure and graft survival. The patients reported improvements in breathing, speech, aesthetics, and quality of life. The second case also included guided bone regeneration and implant placement.
CONCLUSIONS: Oronasal fistulas resulting from maxillofacial surgery can be effectively treated using periodontal plastic surgery techniques, significantly improving patients\' quality of life and aesthetic outcomes. This approach represents a valuable addition to the existing repertoire of oronasal fistula closure methods.

The initial treatment of open laryngeal trauma must be implemented immediately, with the primary focus on saving lives. However, in the later stages, various factors may cause changes in the structure and function of the larynx, which requires special attention. This article reports on the treatment process of a patient with depression who suffered from laryngeal trauma. Due to the late stage of laryngeal infection causing laryngeal defects, a hyoid epiglottis combined with sternocleidomastoid muscle clavicular flap repair was performed. Additionally, personalized functional exercise was performed, ultimately resulting in recovery.
摘要: 开放性喉外伤的初始救治需争分夺秒，以抢救生命为主，但后期可能会因各种因素导致喉结构及功能的改变，需要特别重视。本文报道1例抑郁症患者喉外伤的救治过程，因为后期伴有喉感染引起喉缺损行舌骨会厌联合胸锁乳突肌锁骨瓣修复，并行个体化的功能锻炼，最后痊愈。.

BACKGROUND: Alveolar echinococcosis (AE) primarily affects the liver and potentially spreads to other organs. Managing recurrent AE poses significant challenges, especially when it involves critical structures and multiple major organs.
METHODS: We present a case of a 59-year-old female with recurrent AE affecting the liver, heart, and lungs following two previous hepatectomies, the hepatic lesions persisted, adhering to major veins, and imaging revealed additional diaphragmatic, cardiac, and pulmonary involvement. The ex vivo liver resection and autotransplantation (ELRA), first in human combined with right atrium (RA) reconstruction were performed utilizing cardiopulmonary bypass, and repairs of the pericardium and diaphragm. This approach aimed to offer a potentially curative solution for lesions previously considered inoperable without requiring a donor organ or immunosuppressants. The patient encountered multiple serious complications, including atrial fibrillation, deteriorated liver function, severe pulmonary infection, respiratory failure, and acute kidney injury (AKI). These complications necessitated intensive intraoperative and postoperative care, emphasizing the need for a comprehensive management strategy in such complicated high-risk surgeries.
CONCLUSIONS: The multidisciplinary collaboration in this case proved effective and yielded significant therapeutic outcomes for a rare case of advanced hepatic, cardiac, and pulmonary AE. The combined approach of ELRA and RA reconstruction under extracorporeal circulation demonstrated distinct advantages of ELRA in treating complex HAE. Meanwhile, assessing diaphragm function during the perioperative period, especially in patients at high risk of developing pulmonary complications and undergoing diaphragmectomy is vital to promote optimal postoperative recovery. For multi-resistant infection, it is imperative to take all possible measures to mitigate the risk of AKI if vancomycin administration is deemed necessary.

The bladder exstrophy-epispadias complex includes some of the most challenging conditions treated by pediatric urologists. They are associated with the need for multiple intricate reconstructive procedures, aimed at restoring the anatomy and function of the bladder, urethra and external genitalia. These patients often endure multiple redo reconstructive procedures to improve urinary function, sexual function and cosmesis throughout the first two decades of life. In this article, we present the 30-year experience of a single surgeon performing redo surgery for males born with epispadias and bladder exstrophy. Through detailed documentation of 6 clinical cases, we highlight technical aspects that may contribute to a successful surgical reconstruction in these patients. The article is focused specifically on patients undergoing redo epispadias repair with or without concomitant continence procedures. We make the case for complete penile disassembly with external rotation of the corpora to correct recurrent dorsal curvature; this approach also allows the surgeon to have access to the proximal urethra and bladder neck after opening the intersymphiseal scar/band. This is useful when additional procedures on the bladder, such as bladder neck tailoring, are necessary. We also highlight the importance of avoiding reverse Byars\' flaps when performing skin closure, due to the resulting midline scar. Besides being associated with a poor cosmetic outcome, it can also contribute to recurrent dorsal curvature. The authors advocate for rotational skin flaps to cover the penile shaft. Correction of dorsal curvature and improved cosmesis obtained with complete penile disassembly sometimes comes at the expense of the urethra being left as a hypospadias (figure). This will require further surgeries (usually a 2-stage buccal mucosa graft), much like the treatment of proximal hypospadias. Redo epispadias surgery in males remains a challenge. The systematic approach offered by the case scenarios may help guide surgeons dealing with this difficult condition. Patient with complications after repair of classic bladder exstrophy. A) Stone retrieved from posterior urethra after complete penile disassembly. B) After opening the inter-symphiseal scar, the bladder has been opened and the bladder neck tailored. C) Complete penile disassembly has been completed with corporal bodies and urethra individualized. D,E,F) Final appearance of the repair; abdominal wall was closed with anterior rectus sheath flaps, penile skin was closed with rotational flaps and urethra ended up as a hypospadias.

UNASSIGNED: The aim of this case report is to present a case of chronic cervical ligament tear and instability, which occurred by an unusual work injury with an eversion/hyper-pronation mechanism in contrast to the usual mechanism of inversion. The ligament was reconstructed using an allograft with satisfactory results up to 30 months after surgery. A new magnetic resonance imaging protocol (MRI) was developed to better evaluate the cervical ligament/graft.
UNASSIGNED: In diagnosis of foot sprains, a specific ligament injury should always be sought. In this case, physical examination producing tenderness at the location of the cervical ligament and correlating this with an oblique intercolumn stress test that reproduced pain with apprehension and gross instability supported the diagnosis. Retrospectively applying anatomic knowledge to the earlier MRI findings of bone marrow edema at the insertion points of the cervical ligament on the talus and calcaneus was important in confirming the diagnosis. To better evaluate the cervical ligament allograft tendon reconstruction, a novel volumetric MRI sequence was developed which may prove helpful to also diagnose cervical ligament injuries in future cases. Anatomic reconstruction of the cervical ligament provided satisfactory clinical and radiographic results at 30-month follow-up.Level of Evidence: V.

Four patients with rectal cancer required reconstruction of a defect of the posterior vaginal wall. All patients received neoadjuvant (chemo)radiotherapy, followed by an en bloc (abdomino)perineal resection of the rectum and posterior vaginal wall. The extent of the vaginal defect necessitated closure using a tissue flap with skin island. The gluteal turnover flap was used for this purpose as an alternative to conventional more invasive myocutaneous flaps (gracilis, gluteus, or rectus abdominis). The gluteal turnover flap was created through a curved incision at a maximum width of 2.5 cm from the edge of the perineal wound, thereby creating a half-moon shape skin island. The subcutaneous fat was dissected toward the gluteal muscle, and the gluteal fascia was incised. Thereafter, the flap was rotated into the defect and the skin island was sutured into the vaginal wall defect. The contralateral subcutaneous fat was mobilized for perineal closure in the midline, after which no donor site was visible.The duration of surgery varied from 77 to 392 min, and the hospital stay ranged between 3 and 16 days. A perineal wound dehiscence occurred in two patients, requiring an additional VY gluteal plasty in one patient. Complete vaginal and perineal wound healing was achieved in all patients. The gluteal turnover flap is a promising least invasive technique to reconstruct posterior vaginal wall defects after abdominoperineal resection for rectal cancer.

Epidermolysis bullosa (EB) encompasses a range of rare genetic dermatological conditions characterized by mucocutaneous fragility and a predisposition to blister formation, often triggered by minimal trauma. Blisters in the pharynx and esophagus are well-documented, particularly in dystrophic EB (DEB). However, there have been few reports of mucocutaneous squamous cell carcinoma (SCC) in the head and neck region, for which surgery is usually avoided. This report presents the first case of free jejunal flap reconstruction after total pharyngolaryngoesophagectomy for hypopharyngeal cancer in a 57-year-old patient with DEB. The patient with a known diagnosis of DEB had a history of SCC of the left hand and esophageal dilatation for esophageal stricture. PET-CT imaging during examination of systemic metastases associated with the left-hand SCC revealed abnormal accumulation in the hypopharynx, which was confirmed as SCC by biopsy. Total pharyngolaryngoesophagectomy was performed, followed by reconstruction of the defect using a free jejunal flap. A segment of the jejunum, approximately 15 cm in length, was transplanted with multiple vascular pedicles. The patient made an uneventful recovery postoperatively and was able to continue oral intake 15 months later with no complications and no recurrence of SCC in the head and neck region. While cutaneous SCC is common in DEB, extracutaneous SCC is relatively rare. In most previous cases, non-surgical approaches with radiotherapy and chemotherapy were chosen due to skin fragility and multimorbidity. In the present case, vascular fragility and mucosal damage of the intestinal tract were not observed, and routine vascular and enteric anastomoses could be performed, with an uneventful postoperative course. Our findings suggest that highly invasive surgery, including free tissue transplantation such as with a free jejunal flap, can be performed in patients with DEB.

Soft-tissue sarcomas represent a cohort of rare and heterogeneous malignant tumors that could affect various body parts, with a higher incidence in the lower extremity. When these tumors are surgically removed, both the superficial and deep lymphatic pathways could also be damaged and might require immediate reconstruction to prevent lymphatic complications. In the present report, we describe a case of a patient affected by a high-grade (G3) spindle cell pleomorphic rhabdomyosarcoma of the upper medial thigh. A 22 × 20 cm mass was removed with exposure of the deep femoral vessels and the great saphenous vein. After intraoperative indocyanine green lymphography, it was determined that the superficial lymphatic vessels were intact, but the deep lymphatic system was unavoidably damaged. As a reconstructive procedure, we performed a pedicled SCIP-based vascularized lymphatic vessel transfer and vascularized lymph node transfer to restore the deep lymphatic system and dead space obliteration. The procedure was successful, and no signs of lymphatic impairment were observed during the two-year follow-up period. We believe that this novel approach might be helpful in cases of large and profound defects that involve the deep lymphatic system. The combination of these two techniques could help restore deep lymph drainage, minimizing the risk of superficial system overload and lymphatic dysfunction. No other cases have been described so far employing the same approach. Considering the obtained results, this procedure might be worth further investigation.