Total pulmonary arterial reconstruction in a patient with arterial tortuosity syndrome affecting the pulmonary artery: a case report and review of the literature.

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. CASE PRESENTATION: 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. CONCLUSION: 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.

Association between cervicocerebral artery dissection and tortuosity - a review on quantitative and qualitative assessment.

Cervicocerebral artery dissection stands out as a significant contributor to ischemic stroke in young adults. Several studies have shown that arterial tortuosity is associated with dissection. We searched Pubmed and Embase to identify studies on the association between arterial tortuosity and cervicocerebral artery dissection, and to perform a review on the epidemiology of cervicocerebral artery tortuosity and dissection, pathophysiology, measurement of vessels tortuosity, strength of association between tortuosity and dissection, clinical manifestation and management strategies. The prevalence of tortuosity in dissected cervical arteries was reported to be around 22%-65% while it is only around 8%-22% in non-dissected arteries. In tortuous cervical arteries elastin and tunica media degradation, increased wall stiffness, changes in hemodynamics as well as arterial wall inflammation might be associated with dissection. Arterial tortuosity index and vertebrobasilar artery deviation is used to measure the level of vessel tortuosity. Studies have shown an independent association between these two measurements and cervicocerebral artery dissection. Different anatomical variants of tortuosity such as loops, coils and kinks may have a different level of association with cervicocerebral artery dissection. Symptomatic patients with extracranial cervical artery dissection are often treated with anticoagulant or antiplatelet agents, while patients with intracranial arterial dissection were often treated with antiplatelets only due to concerns of developing subarachnoid hemorrhage. Patients with recurrent ischemia, compromised cerebral blood flow or contraindications for antithrombotic agents are usually treated with open surgery or endovascular technique. Those with subarachnoid hemorrhage and intracranial artery dissection are often managed with surgical intervention due to high risk of re-hemorrhage.

[Functional assessment of internal carotid artery tortuosity in patients with multifocal atherosclerosis]. / Vazhnost' dinamicheskoi funktsional'noi otsenki patologicheskoi izvitosti vnutrennikh sonnykh arterii u bol'nykh s mul'tifokal'nym aterosklerozom.

The review is devoted to diagnosis and treatment of internal carotid artery tortuosity. The authors consider modern classification, epidemiology and diagnostic options using neuroimaging or ultrasound-assisted functional stress tests depending on medical history and complaints. In addition to standard Doppler ultrasound, rotational and orthostatic tests are advisable due to possible changes of local shape and hemodynamic parameters following body position changes, especially in patients with concomitant atherosclerotic stenosis. Thus, a personalized approach is especially important for treatment and diagnostics of internal carotid artery tortuosity.

[Dynamic functional assessment of internal carotid artery tortuosity in patients with multifocal atherosclerosis]. / Dinamicheskaya funktsional'naya otsenka patologicheskikh izvitostei vnutrennikh sonnykh arterii u bol'nykh s mul'tifokal'nym aterosklerozom. Klinicheskii sluchai.

A personalized approach with attention to anamnesis and specific symptoms is necessary in patients with internal carotid artery tortuosity. Neuroimaging (especially before elective surgery) or functional stress tests following ultrasound of supra-aortic vessels may be necessary depending on medical history and complaints. In addition to standard Doppler ultrasound, these patients should undergo rotational and orthostatic transformation tests. We analyze changes in shape and hemodynamic parameters within the tortuosity area in various body positions. This is especially valuable for patients with concomitant carotid artery stenosis. The article presents a clinical case illustrating the importance of such approach.

Global intracranial arterial tortuosity is associated with intracranial atherosclerotic burden.

The effect of arterial tortuosity on intracranial atherosclerosis (ICAS) is not well understood. This study aimed to evaluate the effect of global intracranial arterial tortuosity on intracranial atherosclerotic burden in patients with ischemic stroke. We included patients with acute ischemic stroke who underwent magnetic resonance angiography (MRA) and classified them into three groups according to the ICAS burden. Global tortuosity index (GTI) was defined as the standardized mean curvature of the entire intracranial arteries, measured by in-house vessel analysis software. Of the 516 patients included, 274 patients had no ICAS, 140 patients had a low ICAS burden, and 102 patients had a high ICAS burden. GTI increased with higher ICAS burden. After adjustment for age, sex, vascular risk factors, and standardized mean arterial area, GTI was independently associated with ICAS burden (adjusted odds ratio [adjusted OR] 1.33; 95% confidence interval [CI] 1.09-1.62). The degree of association increased when the arterial tortuosity was analyzed limited to the basal arteries (adjusted OR 1.48; 95% CI 1.22-1.81). We demonstrated that GTI is associated with ICAS burden in patients with ischemic stroke, suggesting a role for global arterial tortuosity in ICAS.

The dominance of dorsal scapular artery as the blood supply to muscles of the back in the absence of two primary vessels: a cadaveric case report.

PURPOSE: Understanding of rare or unknown anatomical variations of the vasculature of the neck is critical to reduce the risk of complications during surgeries and other invasive procedures in the neck and shoulder regions. METHODS: Bilateral dissection of the neck and muscles of the back of an 87-year-old Caucasian male donor was performed to demonstrate the origin, course and termination of the arteries that arise in the neck. RESULTS: Several anatomical variations were noted on the right side of the neck of the donor body - (i) only inferior thyroid and ascending cervical arteries originated from the thyrocervical trunk (TCT), from the first part of the subclavian artery (SA), whereas the transverse cervical (TCA) and suprascapular (SSA) arteries were entirely absent, (ii) Dorsal scapular artery (DSA) emerged normally from the third part of the SA. However, after supplying the rhomboids and levator scapulae muscles, DSA provided two additional branches to the trapezius muscle and a branch to the supraspinatus muscle. Interestingly, the branches to the trapezius muscle from the DSA were the only sources of blood supply to the muscle. CONCLUSION: We report a unique anatomical variation involving the absence of the TCA and SSA from the TCT. The unilateral absence of these major vessels and the branches of DSA supplying the trapezius and supraspinatus muscles have not been reported previously in the literature in a single case report. This case study may provide useful information for head and neck reconstruction and shoulder repair surgeries.

Superior laryngeal artery originating from the lingual artery.

In most cases, the superior laryngeal artery (SLA) branches from the superior thyroid artery, which, in turn, leaves the external carotid artery. Few dissection studies found previously that the SLA could originate from the lingual artery. We report here probably the first evidence of such a rare anatomical variation found unilaterally in a retrospectively evaluated by computed tomography angiography adult male case. The left SLA left a suprahyoid coil of the lingual artery and continued over the greater hyoid horn to enter the larynx through the thyrohyoid membrane. On both sides, thyroid foramina were found, but only the right one used for the entry of the right SLA. Therefore, the rare SLA origin from the lingual artery can be documented on computed tomography angiograms, which could help during preoperative evaluations and prevent unwanted surgical complications.

The presence and the anatomical variations of the accessory appendicular artery: A systematic review of 604 cases.

INTRODUCTION: The accessory appendicular artery (AAA) is an accessory source of blood supply to the appendix. Its existence and potential point of origin are seldom addressed in the literature. METHODS: To fill this knowledge gap, we performed a systematic review of all available studies involving both cadaveric and surgical specimens and documented the presence and the origin of the AAA, following the guidelines outlined in the preferred reporting items for systematic reviews and meta-analysis (PRISMA) statement. RESULTS: Eleven studies, with an overall of 604 specimens were included. Our research revealed that the AAA is notably absent in most cases (83.6%). When present, it most commonly originates from the posterior cecal artery (12.4%), followed by the descending branch of the ileocolic artery (2%), and the ileal branch of the ileocolic artery (0.7%). Instances of origin from the anterior cecal, common cecal, or the ileocolic trunk were even more infrequent. Based on our observations, we introduced a new simplified classification system. DISCUSSION: The effect of an accessory artery on the process of appendicitis remains to be clarified. We firmly recommend that surgeons should consider the possible presence and various origins of the AAA during appendectomy procedures to avoid serious complications.

Arterial Embolization and Cone-Beam Computed Tomography-Guided Lung Resection for Anomalous Systemic Arterial Blood Supply to Normal Lung: Two Case Reports.

Systemic arterial blood supply to a normal lung is a rare anatomical abnormality. Surgery is usually indicated because this abnormality leads to pulmonary hypertension. Herein, we report our experience and ideas for safe vessel dissection. Case 1 was a woman in her 50s. We performed a left lower lobectomy following percutaneous coil embolization. The aberrant artery with emboli was confirmed intraoperatively by cone-beam computed tomography (CBCT) to safely dissect under thoracoscopic surgery (TS). Case 2 was a man in his 40s. Following percutaneous endovascular plug occlusion, we performed a left partial resection using indocyanine green fluorescence navigation. Intraoperatively, CBCT imaging demonstrated the aberrant artery and exact position of the emboli. This combination technique of interventional radiology and TS with CBCT imaging was considered safe and more secure for the treatment of anomalous systemic arterial blood supply to a normal lung.

Arterial tortuosity in pediatric Loeys-Dietz syndrome patients.

Loeys-Dietz syndrome (LDS) is an autosomal connective tissue disorder commonly presenting with hypertelorism, bifid uvula, aortic aneurysms, and arterial tortuosity. The aim of the present study was to investigate differences in tortuosity index (TI) between genotypes of LDS, possible progression over time and its use as an adjunctive prognostic tool alongside aortic dimensions to aid timely surgical planning in pediatric patients. A retrospective observational study of pediatric LDS patients referred to our center (November 2012-February 2021) was conducted. Using magnetic resonance angiography (MRA) with 3D maximum intensity projection volume-rendered angiogram, arterial TI was measured. Twenty three patients had genetically confirmed LDS with at least one head and neck MRA and 19 had no less than one follow-up MRA available. All patients presented arterial tortuosity. Patients with TGFBR2 variants had greater values of TI compared to patients with TGFB2 variants (p = 0.041). For patients who did not undergo surgery (n = 18), z-scores at the level of the sinus of Valsalva showed a significant correlation with vertebral TI (rs = 0.547). There was one death during follow-up. This study demonstrates that patients with LDS and TGFBR2 variants have greater values of TI than patients with TGFB2 variants and that greatest values of TI are associated with increased aortic root z-scores. Furthermore, as TI decreases over time, less frequent neuroimaging follow-up can be considered. Nevertheless, additional studies are needed to better define more accurate risk stratification and long-term surveillance in these patients.

Persistent Stapedial Artery, Oval Window Atresia and Congenital Stapes Agenesis-Case Report.

Background: The persistent stapedial artery (PSA) is a rare congenital vascular malformation involving the middle ear. It is usually associated with pulsatile tinnitus and/or conductive hearing loss and can account for multiple risks during middle ear surgery. Case Report: we present a case of a 9-year-old male child with conductive hearing loss and persistent stapedial artery in his right ear, who was admitted to our ENT Department for hearing loss. During surgery, we discovered PSA along with congenital stapes agenesis and oval window atresia, as well as an abnormal trajectory of the mastoid segment of the facial nerve. After ossicular reconstruction (transcanal total ossicular replacement prosthesis) with cochleostomy, no surgical complications were recorded and hearing improvement was monitored by pre- and postoperative audiometry. Conclusion: Stapedial artery is a rare anatomical middle ear abnormality that can prevent proper surgical hearing restoration and can be associated with other simultaneous temporal bone malformations.

Abnormal obturator artery with ipsilateral inferior epigastric artery passed behind the femoral vein / Arteria obturatriz anómala con arteria epigástrica inferior ipsilateral que cruza posterior a la vena femoral

SUMMARY: Clear awareness of the vascular variations is critical in surgeries, which may cause massive hemorrhage during surgical procedures. During educational dissection of a male cadaver, we encountered a combined variation of the left obturator artery and ipsilateral aberrant inferior epigastric artery. The left obturator artery originated from the external iliac artery, then coursed inward, adherent to the superior pubic ramus. The left inferior epigastric artery originated from the femoral artery, and coursed behind the femoral vein. These anatomical variations shown in one person were extremely rare. This is particularly true with regard to these variations while performing pelvic and inguinal region surgeries.

El conocimiento claro de las variaciones vasculares es fundamental en las cirugías, ya que pueden causar una hemorragia masiva durante los procedimientos quirúrgicos. Durante la disección educativa de un cadáver de sexo masculino, encontramos una variación combinada de la arteria obturatriz izquierda y la arteria epigástrica inferior ipsilateral aberrante. La arteria obturatriz izquierda se originaba en la arteria ilíaca externa, luego discurrió hacia medial, adhiriéndose a la rama púbica superior. La arteria epigástrica inferior izquierda se originaba en la arteria femoral y discurría por detrás de la vena femoral. Estas variaciones anatómicas mostradas en una sola persona son extremadamente raras. Esto es importante de conocer estas variaciones cuando se realizan cirugías de las regiones pélvica e inguinal.

Incidental Finding of a Persistent Stapedial Artery in a Patient Presenting With Conductive Hearing Loss.

A persistent stapedial artery is a congenital vascular malformation that can cause tinnitus and/or conductive hearing loss. Although rare, this case highlights the importance of recognizing aberrant anatomy as a potential cause of patients' symptoms. It also demonstrates how to recognize and treat patients with a symptomatic persistent stapedial artery.

Subarachnoid Hemorrhage from Ruptured Internal Carotid Artery Aneurysm: Association with Arterial Tortuosity.

OBJECTIVE: Many researchers have found a correlation between tortuous arteries and development of aneurysms in cerebral arteries. We decided to determine whether tortuosity of the internal carotid artery can be related to its aneurysm rupture. METHODS: We retrospectively analyzed the internal carotid artery anatomy of 149 patients with internal carotid artery aneurysms. For each patient, we calculated relative length (RL), sum of angle metrics (SOAM), triangular index (TI), product of angle distance (PAD), and inflection count metrics (ICM). RESULTS: A total of 33 patients (22.15%) had subarachnoid hemorrhage. These patients had significantly lower SOAM (0.31 ± 0.17 vs. 0.42 ± 0.21; P < 0.01), TI (0.27 ± 0.09 vs. 0.31 ± 0.11; P = 0.03) and ICM (0.25 ± 0.11 vs. 0.31 ± 0.17; P = 0.04). In multivariate logistic regression analysis, higher SOAM (odds ratio, 0.780; 95% confidence interval, 0.619-0.961; P = 0.025) remained independently associated with lower risk of internal carotid artery aneurysm rupture. In addition, we found significant positive correlation of aneurysm dome size with SOAM (R = 0.224; P = 0.013) and PAD (0.269; P < 0.01). Our study also showed that age (R = 0.252; P = 0.036), Glasgow Coma Scale score (R = -0.706; P < 0.01), and TI (R = -0.249; P = 0.042) were independently correlated with modified Rankin Scale score on discharge. CONCLUSIONS: Lower tortuosity might be a protective factor against internal carotid artery aneurysm rupture and poor outcome after subarachnoid hemorrhage. Higher tortuosity is correlated with internal carotid artery aneurysm growth.

Anesthetic Management of a Patient With Arterial Tortuosity Syndrome Undergoing Cerebral Revascularization Surgery-A Case Report.

Arterial tortuosity syndrome is a rare hereditary autosomal recessive connective tissue disorder characterized by elongation and tortuosity of the large- and medium-sized arteries. We present the case of a 13-year old child with arterial tortuosity syndrome who underwent occipital encephaloduroarteriomyosynangiosis for posterior circulation insufficiency. The constellation of clinical features in our patient portended significant anesthetic concerns, including difficult airway due to craniofacial abnormalities, risk of stroke, and myocardial infarction due to intracerebral and coronary arterial tortuosity and stenosis. The pertinent anesthetic implications are summarized, and we describe the anesthetic technique and use of multimodal neuromonitoring relevant for the case.

Arterial Tortuosity and Its Correlation with White Matter Hyperintensities in Acute Ischemic Stroke.

Introduction: The association between arterial tortuosity and acute ischemic stroke (AIS) has been reported, but showing inconsistent results. We hypothesized that tortuosity of extra- and intracranial large arteries might be higher in AIS patients. Furthermore, we explored the correlation between artery tortuosity and white matter hyperintensity (WMH) severity in AIS patients. Methods: 166 AIS patients identified as large artery atherosclerosis, and 83 control subjects were enrolled. All subjects received three-dimensional computed tomography angiography (CTA). Arterial tortuosity was evaluated using the tortuosity index. WMHs were evaluated using magnetic resonance imaging in all AIS patients. Results: AIS patients showed significantly increased arterial tortuosity index relative to controls, including left carotid artery (CA) (p = 0.001), right CA (p < 0.001), left common carotid artery (CCA) (p < 0.001), right CCA (p < 0.001), left internal carotid artery (p = 0.001), right internal carotid artery (p = 0.01), left extracranial internal carotid artery (EICA) (p < 0.001), right EICA (p = 0.01), and vertebral artery dominance (VAD) (p = 0.001). The tortuosity of all above arteries was associated with the presence of AIS. AIS patients with moderate or severe WMHs had a higher tortuosity index in left CA (p = 0.005), left CCA (p = 0.003), left EICA (p = 0.07), and VAD (p = 0.001). In addition, the tortuosity of left EICA and VAD was associated with WMH severity in AIS patients. Conclusions: Increased extra- and intracranial large arteries tortuosity is associated with AIS. The tortuosity of left carotid artery system and vertebral artery may be the independent risk factors for WMH severity in AIS patients. Clinical Trial Registration. This trial is registered with NCT03122002 (http://www.clinicaltrials.gov).