Prevention of Postsurgical Lymphedema by 9-cis Retinoic Acid.

OBJECTIVE: To determine the effect of 9-cis retinoic acid (9-cis RA) on postsurgical lymphedema. BACKGROUND: 9-cis RA promotes lymphangiogenesis in vitro and in vivo and has promise as a therapeutic agent to limit the development of postsurgical lymphedema. METHODS: Lymphedema was induced in the right hind limb after a single fraction of 20 Gy radiation, popliteal lymphadenectomy, and lymphatic vessel ablation. Postoperatively, mice were randomly divided in to 2 groups that received daily intraperitoneal injections of either (1) an oil-based vehicle solution (control) or (2) 0.08 mg/kg of 9-cis RA dissolved in a vehicle solution. Outcome measures included paw thickness, lymphatic drainage, and lymphatic vessel density as measured by podoplanin immunohistochemistry and whole mount skin analysis. RESULTS: Using our combined injury protocol, postsurgical lymphedema was observed 89% of the time. 9-cis RA-treated animals had less early postsurgical edema and significantly less paw lymphedema compared with vehicle-treated animals at all time-points (P < 0.001). 9-cis RA-treated animals had significantly faster lymphatic drainage as measured by indocyanine green clearance and increased lymphatic vessel density as measured by podoplanin immunohistochemistry (P < 0.001) and whole mount skin analysis (P < 0.05). CONCLUSIONS: We have developed a highly reproducible model of secondary lymphedema and have demonstrated that 9-cis RA significantly prevents postsurgical lymphedema. Treatment with 9-cis RA is associated with increased lymphatic clearance and lymphangiogenesis. Because 9-cis RA (alitretinoin) is already approved for clinical use by the US Food and Drug Administration for other conditions, it has the potential to be repurposed as a preventative agent for postsurgical lymphedema in humans.

Cutting Edge: memory regulatory t cells require IL-7 and not IL-2 for their maintenance in peripheral tissues.

Thymic Foxp3-expressing regulatory T cells are activated by peripheral self-antigen to increase their suppressive function, and a fraction of these cells survive as memory regulatory T cells (mTregs). mTregs persist in nonlymphoid tissue after cessation of Ag expression and have enhanced capacity to suppress tissue-specific autoimmunity. In this study, we show that murine mTregs express specific effector memory T cell markers and localize preferentially to hair follicles in skin. Memory Tregs express high levels of both IL-2Rα and IL-7Rα. Using a genetic-deletion approach, we show that IL-2 is required to generate mTregs from naive CD4(+) T cell precursors in vivo. However, IL-2 is not required to maintain these cells in the skin and skin-draining lymph nodes. Conversely, IL-7 is essential for maintaining mTregs in skin in the steady state. These results elucidate the fundamental biology of mTregs and show that IL-7 plays an important role in their survival in skin.

CXCR2 inhibition enhances sulindac-mediated suppression of colon cancer development.

Small chemical compound sulindac has been approved as a preventive approach against colon cancer for its effectiveness in treatment of precancerous adenoma. Due to its severe toxicities in the cardiovascular, gastrointestinal and renal systems, however, a combination of low-dose sulindac with other chemopreventive agents has been sought after as an alternative therapeutic strategy that could increase its effectiveness, while minimizing its adverse effects. To identify the promising alternative approach, we investigated the therapeutic potential of targeting the interleukin (IL)-8/CXCR2 pathway in colon cancer treatment using both loss-of-function (CXCR2 knockout) and gain-of-function (IL-8 overexpression) mouse models, as the IL-8/CXCR2 pathway has been shown to be activated in intestinal tumors of both human and experimental animals. We found that deletion of CXCR2 gene and ectopic expression of IL-8 suppresses and enhances, respectively, intestinal tumor development caused by a mutation in the APC gene. Moreover, a single copy deletion of CXCR2 gene resulted in abrogation of COX-2 and Gro-α upregulation in intestinal tumors caused by the APC mutation. Moreover, a single copy (heterozygote) deletion of CXCR2 gene was sufficient to synergize with a low-dose sulindac treatment in suppressing APCmin-induced intestinal polyposis. Together, our study provides a therapeutic justification of combined inhibition of CXCR2 and sulindac treatment in colon cancer prevention.

Angiomatous kaposi sarcoma: a variant that mimics hemangiomas.

We describe 14 cases of angiomatous Kaposi sarcoma (KS), a distinct histological variant of KS first mentioned by Gottlieb and Ackerman in 1988 that can easily be mistaken for a hemangioma. Intriguingly, this variant of KS has not attracted much attention and has not been studied in detail. Immunohistochemistry showed prominent staining of podoplanin (D2-40) of the neoplastic vasculature but not the preexisting vessels, suggesting lymphatic differentiation, despite the erythrocyte-filled round lumens. To test whether D2-40 staining of round vessels with erythrocytes was distinctive, we stained sinusoidal hemangiomas and cellular angiolipomas, both of which have these structures. In contrast to angiomatous KS, the vessels in both entities were podoplanin (D2-40) negative. The finding of round erythrocyte-filled vessels with podoplanin (D2-40) positivity may be distinctive for this form of KS.

Supraorbital cutaneous fetal rhabdomyoma of intermediate type: a case report.

A 7-year-old boy was presented with a long-standing slowly growing mass of the left supraorbital area. A biopsy specimen revealed a bland spindle cell proliferation with scattered polygonal cells with acidophilic cytoplasm and cross-striations. Our differential diagnosis included rhabdomyoma of fetal type, leiomyoma with trapping of regenerating skeletal muscle elements, and rhabdomyomatous mesenchymal hamartoma of the skin. Immunohistochemistry demonstrated strong positivity of myoglobin and desmin as well as negativity of caldesmon, suggesting skeletal muscle lineage. The excisional specimen confirmed our diagnosis of cutaneous fetal rhabdomyoma of intermediate type. Additional immunostaining performed on the excisional specimen showed strong Wilms Tumor 1 but only a very faint and focal p63 expression.

Recurrent Zenker's Traction Diverticulum After Cervical Spine Surgery: Lessons Learned.

This is a report of our institutional experience regarding pharyngoesophageal diverticula formation following anterior cervical spine surgery (ACSS). It is a retrospective chart review of institutional patients from January 2008 to May 2020. Patients at our institution were identified by our two senior authors. Inclusion criteria included patients > 18 years old, a history of prior ACSS, and a confirmed diagnosis of pharyngoesophageal diverticulum with radiographic imaging. Three patients were identified to have an ACSS-related diverticulum. The case presentations describe surgical management and the subsequent postoperative course. One patient had a particularly complicated course with recurrent diverticulum formation despite prior excision. The patient continued to have dense scar tissue adhering the posterior esophageal wall to the nearby cervical spine plates, despite prior excision and rotation of nearby tissue. This difficult case demonstrated the need for an open and aggressive approach. ACSS-related diverticula that form in patients with a history of prior anterior cervical spine surgery appear to be a form of traction diverticulum due to dense scar tissue that adheres the pharyngoesophageal mucosa to the adjacent cervical spinal plate. This type of diverticulum differs from Zenker's diverticulum. Surgical management is recommended to resolve patients' symptoms.

Long-Term Plate Complications in Patient-Specific Plates Utilizing Computer-Aided Design.

OBJECTIVE: Assess the long-term plate complications with patient-specific plates (PSPs) created with computer-aided design (CAD) and computer-aided manufacturing (CAM) for fibula free flap reconstructions for mandibular defects. METHODS: Retrospective chart review from January 2010 to July 2022 of patients who underwent mandibular reconstruction with a fibula free flap and PSP. Primary outcome was plating-related complications, defined as plate exposure, fracture, loose screws, and plate removal. RESULTS: A total of 221 patients underwent PSP fibula reconstruction. Average age was 59.8 + 14.3 years old with male to female ratio of 2:1. Squamous cell carcinoma of the mandible was the most common reason for resection, 47.5%, n = 105. Plate removal occurred in 11% of patients (n = 25) about 17.4 months after the initial surgery. Plates were removed due to exposure (76%, n = 19) or screw loosening (24%, n = 6). Malignancy was associated with an increased risk of plate complications when compared to benign tumor (odds ratio [OR] 9.04, confidence interval [CI] 1.36-3.85), osteonecrosis (OR 1.38, CI 0.59-3.48), and trauma (OR 1.26, CI 0.23-12.8). Postoperative radiation therapy (OR 2.27, CI 1.07-4.82, p = 0.026) and surgical site infection (OR 9.22, CI 4.11-21.88, p = 0.001) were associated with more plate complications. CONCLUSIONS: CAD creates PSPs that remain stable in the majority of patients over the long term. Plate removal is less compared to non-PSP reconstruction. Consideration of the soft tissue envelope over the plate and management of perioperative infection at the time of surgery should be entertained. LEVEL OF EVIDENCE: Level 4 Laryngoscope, 2024.

Outcomes Following Treatment for Carotid Blowout in Head and Neck Cancer Patients.

OBJECTIVE: The aim of the study was to determine outcomes after interventional radiology treatment of carotid blowout. METHODS: Patients with head and neck cancer and who received interventional radiology treatment for carotid blowout between 2000 and 2022 were included. Pre-treatment, treatment, and post-treatment variables were evaluated. RESULTS: Fourteen patients met inclusion criteria. Eleven patients (78.6%) had a history of radiation. Twelve (85.7%) blowouts occurred within 6 months of recent intervention. Initial treatment was with stenting (n = 9, 64.3%), coil embolization (n = 4, 28.6%), or both (n = 1, 7.1%). Six patients (42.9%) underwent subsequent carotid bypass. Morbidity following treatment included stroke (n = 1) and rebleeding (n = 4). Six-month survival was 57.1%. Of the patients who survived past six months, 5/8 were treated with carotid bypass and coverage. Four patients died of cancer progression, three of rebleeding, and three of medical complications. CONCLUSION: The majority of carotid blowout occurs within 6 months of surgery or radiation. Many who survive will die of cancer progression or medical illness. Carotid bypass with flap coverage may be a worthwhile treatment for carotid blowout and should be considered as an adjunct to endovascular treatment. LEVEL OF EVIDENCE: 4 Laryngoscope, 134:695-700, 2024.

Microvascular reconstruction of midface osteoradionecrosis.

BACKGROUND: Head and neck osteoradionecrosis (ORN) of the midface requiring free flap (FF) reconstruction is uncommon. This multi-institutional study was designed to review outcomes for this rare patient population. METHODS: Retrospective multi-institutional review of FF reconstruction for midface ORN (2005-2022; n = 54). RESULTS: The FF survival rate was 87% (n = 54). Patients were less likely to be tolerating a regular diet at 3 months postoperative if they had a preoperative history of prior head and surgery (80% vs. 95%; p = 0.02), a pathologic fracture (50% vs. 90%; p = 0.04), exposed bone intraorally (43% vs. 94%; p = 0.002), or a fistula (67% vs. 96%; p = 0.03). Mean albumin was higher in patients whose FF survived (3.6 ± 0.5 vs. 2.7 ± 1.4; p = 0.03). Patients with low prealbumin were more likely to undergo a hematoma evacuation (27% vs. 0%; p = 0.02). CONCLUSION: In this series of midface ORN requiring FF reconstruction preoperative nutritional status impacted postoperative complications. Preoperative occurrence of a fistula, pathologic fracture, and intraoral bone exposure correlated with decreased tolerance of a regular diet following reconstruction.

A Review of Atomic-Force Microscopy in Skin Barrier Function Assessment.

Skin barrier function (SBF) disorders are a class of pathologies that affect a significant portion of the world population. These disorders cause skin lesions with intense itch, impacting patients' physical and psychological well-being as well as their social functioning. It is in the interest of patients that their disorder be monitored closely while under treatment to evaluate the effectiveness of the ongoing therapy and any potential adverse reactions. Symptom-based assessment techniques are widely used by clinicians; however, they carry some limitations. Techniques to assess skin barrier impairment are critical for understanding the nature of the disease and for helping personalize treatment. This review recalls the anatomy of the skin barrier and describes an atomic-force microscopy approach to quantitatively monitor its disorders and their response to treatment. We review a panel of studies that show that this technique is highly relevant for SBF disorder research, and we aim to motivate its adoption into clinical settings.

Fibula Free Flap Reconstruction of Cervical Spine Defects: A Multi-Institutional Study.

INTRODUCTION: Cervical spine defects result in spinal instability, putting the spinal cord and vertebral arteries at risk of damage and possibly devastating neurological injuries. The fibula free flap can span the spinal defects for stability. There is a paucity of literature on this technique. METHOD: Multi-institutional retrospective case series reviewing patients who underwent cervical spine reconstruction with a fibula free flap. Patient demographic information, comorbidities, characteristics of cervical spine defects, and free flap complications were collected. RESULTS: A total of 1187 fibula free flaps across 10 different institutions were reviewed. Thirteen patients (1.09%) underwent cervical spine reconstruction with a fibula free flap. Average age was 52.3 years old with an age range of 12-79 years. There were six males (46.1%) and seven females (53.8%). The most common defect etiology was infection (n = 6, 46.1%). Most commonly involved cervical spine level of the defect was C5 (n = 10) followed by C6 (n = 9) and C4 (n = 8). The majority of reconstructed defects spanned three or more cervical levels, (n = 9, 69.2%). Facial artery was the most common arterial anastomosis (n = 8). Eight patients (61.5%) required a tracheostomy during their postoperative course. None of the patients had symptomatic or radiographic nonunion. CONCLUSION: This case series demonstrates that a vascularized fibula flap is a potential reconstructive option for cervical spine defects, especially in defects greater than three cervical levels, in the setting of infection, and previously radiated patients. LEVEL OF EVIDENCE: Level 4 Laryngoscope, 2024.

Microvascular reconstruction of medication related osteonecrosis of the head and neck.

BACKGROUND: Medication related osteonecrosis of the jaw (MRONJ) requiring free flap (FF) reconstruction is uncommon with limited reported findings. METHODS: Multicenter, retrospective case series of 49 consecutive adult patients presenting with advanced MRONJ requiring FF reconstruction from 2010 to 2022. Perioperative complications and outcomes were analyzed. RESULTS: Eighty-two percent (n = 40) of cases were of the mandible and 18% (n = 9) were of the maxilla. The mean follow-up was 15 months (±19.6). The majority of FF survived (96%, n = 47). FF reconstructions of the maxilla were more likely to require postoperative debridement (56%, 95% CI [27, 81%] vs. 15%, 95% CI [7, 25%], p = 0.008) or develop intraoral bone exposure (56%, 95% CI [27, 81%] vs. 18%, 95% CI [9, 27%], p = 0.02). Most patients (71%, n = 35) received preoperative antibiotics which was associated with a higher rate of FF survival (100% vs. 86%, 95% CI [60, 96%], p = 0.02) and fewer complications. CONCLUSIONS: Patients undergoing FF reconstruction for MRONJ do well with high rates of FF success. MRONJ of the maxilla have a higher rate of some complications. Preoperative antibiotics correlated with higher FF survival and fewer postoperative complications.

Microvascular Free Flap Outcomes in Maxillectomy Defects from Invasive Fungal Sinusitis.

OBJECTIVES: Microvascular free tissue transfer is routinely used for reconstructing midface defects in patients with malignancy, however, studies regarding reconstructive outcomes in invasive fungal sinusitis (IFS) are lacking. We aim to describe outcomes of free flap reconstruction for IFS defects, determine the optimal time to perform reconstruction, and if anti-fungal medications or other risk factors of an immunocompromised patient population affect reconstructive outcomes. METHODS: Retrospective review of reconstruction for IFS (2010-2022). Age, BMI, hemoglobin A1c, number of surgical debridements, and interval from the last debridement to reconstruction were compared between patients with delayed wound healing versus those without. Predictor variables for delayed wound healing and the effect of time on free flap reconstruction were analyzed. RESULTS: Twenty-seven patients underwent free flap reconstruction for IFS. Three patients were immunocompromised from leukemia and 21 had diabetes mellitus (DM). Patients underwent an average of four surgical debridements for treatment of IFS. The interval from the last IFS debridement to flap reconstruction was 5.58 months (±5.5). Seven flaps (25.9%) had delayed wound healing. A shorter interval of less than 2 months between the last debridement for IFS and reconstructive free flap procedure was associated with delayed wound healing (Fisher Exact Test p = 0.0062). Other factors including DM, BMI, HgA1c, and bone reconstruction were not associated with delayed wound healing. CONCLUSION: Patients with maxillectomy defects from IFS can undergo microvascular-free flap reconstruction with good outcomes while on anti-fungal medication. Early reconstruction in the first 2 months after the last IFS debridement is associated with delayed wound healing. LEVEL OF EVIDENCE: 4 Laryngoscope, 134:1642-1647, 2024.

A Rare Manifestation of Infectious Mononucleosis Tonsillitis.

The purpose of this case report is to describe a rare case of infectious mononucleosis (IM) hemorrhagic tonsillitis. Our patient presented with acute tonsillitis complicated by spontaneous tonsillar hemorrhage. This is a single case report with a literature review. A 19-year-old male presented to the emergency department with a 10-day history of worsening sore throat, recurrent fevers, malaise, and dysphagia to solids and liquids, as well as a three-day history of epistaxis and hemoptysis. He tested positive for Epstein-Barr virus and rhinovirus. On exam, a "hot potato" voice was noted along with bilateral tonsillar edema, erythema, and hypertrophy. Both tonsils with dry blood coating and no exudates were visualized. Computed tomography (CT) imaging of the neck demonstrated subcutaneous emphysema isolated to the tonsils. Treatment consisted of intravenous antibiotics and steroids, followed by an oral antibiotic, with subsequent full resolution of symptoms. This case illustrates a rare, severe manifestation of IM tonsillitis that radiographically can mimic other more severe soft-tissue neck infections on imaging, such as cervical necrotizing fasciitis. In patients presenting with hematemesis, hemoptysis, and/or epistaxis, along with tonsillar edema, erythema, and hypertrophy, a diagnosis of spontaneous hemorrhagic tonsillitis should be considered. The radiographic findings of soft-tissue emphysema in the deep spaces of the head and neck region may be seen in the setting of IM, mimicking other soft-tissue infections of the deep neck spaces.

The Dynamic Tracheoesophageal Prosthesis Length.

OBJECTIVE: In a postlaryngectomy patient, tracheoesophageal (TE) speech is considered to be the most effective and preferred method of communication. Previous research has demonstrated that despite an appropriately sized TE prosthesis placement at the time of puncture, there are a portion of patients that require resizing postoperatively. The purpose of this study was to report on the variability of the tracheoesophageal prosthesis length. STUDY DESIGN: Retrospective chart review. SETTING: Tertiary care academic medical center. METHODS: This was a retrospective chart review of 62 patients who underwent secondary tracheoesophageal puncture (TEP) at a tertiary care academic medical center from January 2008 to November 2019. Patient demographic information, average changes in prosthesis length, number of prosthesis adjustments, and timing of prosthesis exchanges were collected. RESULTS: 62 patients met criteria for study inclusion. Mean age was 61.96 years old with 49 being male (79%) and 13 (21%) females. Overall change in prosthesis length was - 3.85 mm ± 3.58 with time to first prosthesis change at 2.29 months ± 2.73. There was an average of 4.37 changes ± 3.43 before reaching a stable length. Twenty-six patients (41.9%) had increases in their prosthesis length resulting in closure of the tracheoesophageal fistula requiring seven patients (11.3%) to return to the operating room for repuncturing. History of smoking (P = 0.02), Blom-Singer prosthesis type (P = 0.03), and larger diameter (P = 0.01) appeared to be predisposing factors for a fluctuating prosthesis length. CONCLUSION: Tracheoesophageal prosthesis length decreases over time for secondary punctures, requiring adjustments with a speech language pathologist. There are a clinically significant portion that have fluctuations in prosthesis length resulting in an increased risk for requiring re-puncturing.

Management of the Failing Flap.

Free tissue transfer has become the reconstructive modality of choice for replacing composite tissue defects. While the success rate in high-volume centers is reported to be greater than 95%, up to 10% of patients will require revision of their vascular anastomosis secondary to thrombosis or compromise to flow. In the intraoperative setting, immediate revision is successful in the majority of cases. Rarely, the flap cannot be revascularized and a secondary option must be used. In the perioperative setting revision is successful if the patient can be brought back to the operating room in a timely fashion. Revision rates up to 70% are reported. A small number of these patients may then suffer a second episode of compromise where revision is less successful at 30%. In these cases, consideration should be given to secondary reconstruction rather than attempting salvage. Finally, there are a small number of patients whose flaps will fail following discharge from the hospital. These patients can rarely be salvaged and secondary reconstructive options should be explored.

Free Flap Outcomes for Head and Neck Surgery in Patients with COVID-19.

INTRODUCTION: Coronavirus disease 2019 (COVID-19) affects the vascular system, subjecting patients to a hypercoagulable state. This is of particular concern for the success of microvascular free flap reconstruction. This study aims to report head and neck free flap complications in patients with COVID-19 during the perioperative period. We believe these patients are more likely to experience flap complications given the hypercoagulable state. METHODS: This is a multi-institutional retrospective case series of patients infected with COVID-19 during the perioperative period for head and neck free flap reconstruction from March 2020 to January 2022. RESULTS: Data was collected on 40 patients from 14 institutions. Twenty-one patients (52.5%) had a positive COVID-19 test within 10 days before surgery and 7 days after surgery. The remaining patients had a positive test earlier than 10 days before surgery. A positive test caused a delay in surgery for 16 patients (40.0%) with an average delay of 44.7 days (9-198 days). Two free flap complications (5.0%) occurred with no free flap deaths. Four patients (10.0%) had surgical complications and 10 patients had medical complications (25.0%). Five patients (12.5%) suffered from postoperative COVID-19 pneumonia. Three deaths were COVID-19-related and one from cancer recurrence during the study period. CONCLUSION: Despite the heightened risk of coagulopathy in COVID-19 patients, head and neck free flap reconstructions in patients with COVID-19 are not at higher risk for free flap complications. However, these patients are at increased risk of medical complications. LEVEL OF EVIDENCE: 4 Laryngoscope, 2023.

Head and neck cancer readmission reduction (HANCARRE) project: Reducing 30-day readmissions.

Objective: Unplanned 30-day readmissions result in increased costs and decreased patient satisfaction. The objective of this study was to compare readmission rates before and after a multidisciplinary quality improvement initiative that focuses on patient and staff education, use of targeted skilled nursing facilities, and appropriate use of patient observation status. Methods: This was a quality improvement study of all unplanned admissions to the Head and Neck Oncology service at a tertiary care facility during a 3-year period between October 2015 and September 2018. In October 2016, when the Head and Neck Oncology service revised its discharge practices for patients undergoing extirpative and/or reconstructive surgery. These changes included enhancing patient education, increasing the use of a skilled nursing facility with directed staff education and patient handoffs by advanced practice nurses, and appropriate utilization of 23-h observation status for returning patients. The readmission rate from the pre-intervention era (October 2015 through September 2016) was compared to the readmission rate from the post-intervention era (October 2016 through September 2018). Secondary outcomes were the rates of 23-h observation within 30 days of the discharge as well as emergency room visits within 30 days of discharge. Results: In this sample of 449 patients, 161 (35.9%) were observed before the change-in-practice (before October 2016), and 288 (64.1%) were observed following the change-in-practice (after September 2016). On univariable analysis, the risk of readmission declined by approximately 41.4% from the pre-intervention era, though this conclusion was not statistically significant (P = 0.06). On multivariable analysis, patients at moderate or high risk of death were 2.31 times more likely than those at minor risk of death to readmit within 30 days (P = 0.03). Similarly, those with recurrent or persistent cancer were 3.33 times more likely than those undergoing initial curative surgical management of cancer to readmit within 30 days (P = 0.001). No patient characteristics were associated with a 23-h observation following discharge (all P > 0.05). Conclusions were similar for emergency room visits following discharge. Conclusions: A three-part quality improvement strategy resulted in a clinically important decrease in 30-day readmissions, though the decline was not statistically significant. There were no significant changes in 23-h observation within 30 days of discharge or emergency room visits within 30 days of discharge.

MR angiographic findings of aberrant right subclavian artery with agenesis of the left common carotid artery and anomalous origin of the vertebral arteries.

INTRODUCTION: Congenital aortic arch anomalies and variants have been extensively characterized in the medical literature. Proper identification of these anomalies is important when surgical or percutaneous interventions are indicated. CASE PRESENTATION: We present a case of a 48-year old male who presented to the emergency department with altered mental status. Magnetic resonance angiography (MRA) findings revealed an aberrant right subclavian artery (ARSA), early bifurcation of the right common carotid artery (CCA) with anomalous origin of the right vertebral artery (VA) from the right common carotid artery bifurcation, anomalous left vertebral artery originating from the aortic arch, and absent left common carotid artery with independent origins of the left external carotid artery (ECA) and internal carotid artery (ICA). No other abnormalities were identified, and the patient demonstrated no symptoms attributable to his vascular anomalies. CONCLUSION: To our knowledge, this unique combination of anomalies has never been reported in the literature. With an understanding of embryological pathways, even exceedingly rare anomalies like this one can be explained.