Moyamoya arteriopathy and middle aortic syndrome (MAS) are each rare, often progressive vascular diseases; their comorbid intersection increases their complexity because of the independent and contradictory demands on blood pressure. The management of pediatric patients with these comorbid conditions requires multidisciplinary consultation given the high risk involved. In the current study, the authors aim to describe the clinical and surgical history of pediatric patients with comorbid moyamoya arteriopathy and MAS and describe a multidisciplinary approach to care for these patients. This study is a retrospective review of the clinical and radiological records of patients at Boston Children's Hospital who were treated with cerebral revascularization surgery for comorbid moyamoya arteriopathy and MAS from January 2004 to January 2024. Analysis of collected perioperative, surgical, inpatient, and follow-up data was conducted using R version 4.4.2 (R Foundation for Statistical Computing). Eleven patients, 5 male (45.5%), were included in the study. At presentation, 9 patients (81.8%) had symptoms from brain ischemia. Nine patients were on antihypertensive medications (median 2, IQR 1-2). All patients underwent surgery for moyamoya arteriopathy, and 6 patients (54.5%) also underwent surgical treatment for MAS. After moyamoya surgery, 4 patients (36.4%) required an intensified blood pressure regimen to achieve the same goal, whereas 3 patients (27.3%) had a reduced need for blood pressure medications. Four (66.7%) of the 6 patients who underwent MAS surgical treatment did so after moyamoya treatment. Perioperative stroke occurred after renal stent angioplasty before moyamoya surgery but also after aortic bypass following moyamoya surgery. Managing comorbid moyamoya arteriopathy and MAS is challenging, as patients have a higher risk of ischemic perioperative complications. These patients may have hypertension secondary to their aortic disease, and treating the aortic disease may correct the hypertension but with possible new relative hypotension and relative cerebral hypoperfusion. Consequently, moyamoya revascularization prior to aortic repair may be a strategy to reduce the risk of stroke.

Middle aortic syndrome is a rare vascular disorder characterised by segmental narrowing of the distal thoracic aorta and/or proximal abdominal aorta, often accompanied by renal artery involvement. The condition may arise from genetic abnormalities, acquired factors, or idiopathic causes. In this study, we report the case of a 9-year-old boy who was found to have hypertension during a routine school health examination. Computed tomography angiography confirmed the diagnosis of middle aortic syndrome, revealing long-segment stenosis of the distal thoracic aorta. Following a comprehensive assessment, the patient underwent descending aortic reconstruction with a 14 mm Intergard knitted vascular graft. At the 6-month follow-up, imaging demonstrated satisfactory graft patency, and the patient's blood pressure remained well-controlled without the need for antihypertensive medications.

This article reports the case of a male patient in his 70s with middle aortic syndrome (MAS). The patient was admitted with abdominal pain, abdominal distension, vomiting, and cessation of defecation for 2 days as chief complaints and was diagnosed with intestinal obstruction. Enhanced computed tomography and vascular reconstruction revealed distal occlusion of the abdominal aorta. The celiac trunk, superior mesenteric artery, and inferior mesenteric artery were not visualized, whereas bilateral renal arteries maintained blood flow. Given the patient's history of intermittent claudication and uncontrolled hypertension, the condition was ultimately diagnosed as MAS secondary to Takayasu's arteritis, with bowel obstruction identified as a secondary manifestation of MAS. After insertion of a nasointestinal obstruction catheter, the patient's symptoms of intestinal obstruction were relieved. However, the obstruction recurred, and the patient subsequently improved following vascular reconstruction. During postoperative follow-up to date, intestinal obstruction has recurred only once due to improper diet and resolved after dietary modifications. MAS is relatively rare in clinical practice, particularly among older patients. We hope that this case study provides clinicians with an opportunity for learning and knowledge exchange, thereby contributing to improved diagnostic and therapeutic approaches for patients with similar conditions.

Mid-aortic syndrome is a condition characterized by obstructive lesions of the thoracoabdominal aorta, occurring in approximately 0.5-2% of cases. This report presents the long-term outcomes of a clinical case involving transcatheter treatment of mid-aortic syndrome in a child. At an early age, the patient underwent recanalization of an occluded thoracoabdominal segment, followed by staged balloon angioplasty and implantation of an Advanta V12 OTW 9×59 mm stent-graft (Getinge Group, Sweden). After the initial procedure, the patient was followed up over a 3-year period. During a routine hospitalization, an increase in the peak pressure gradient between the upper and lower extremities up to 30 mmHg was noted, along with a stenosis of the mid-portion of the stent-graft to 3.3 mm in diameter. Due to the evolving clinical presentation, a decision was made to perform repeat endovascular intervention. A Visi-Pro stent (Medtronic Corp., USA), 9×17 mm in size was implanted at the site of maximum stenosis followed by sequential balloon angioplasty. Control angiography revealed moderate residual stenosis with a luminal diameter of 7.5 mm and a peak gradient of 10 mmHg. Antiplatelet therapy (acetylsalicylic acid) and antihypertensive therapy (enalapril) were prescribed. The patient was discharged in satisfactory condition on postoperative day 5. Transcatheter techniques do not always achieve complete elimination of the stenotic area and require close follow-up due to the risk of neointimal hyperplasia. Nevertheless, the endovascular approach to palliative correction of mid-aortic syndrome allows the child to reach an optimal age for definitive surgical repair while reducing the need for repeated open procedures.

Coarctation (CoA) of the abdominal aorta, better classified as midaortic syndrome (MAS), represents a rare and complex vascular anomaly that results in narrowing of the descending distal thoracic and/or abdominal aorta. Clinical presentation in pediatric patients is complex and sometimes undetected; consequently, prognosis is grim, especially in preterm infants. Most common indications are hypertension, which mostly remains unresolved with high doses and/or combinations of antihypertensive medications, claudication of lower limbs and/or feeble or absent femoral pulses. Surgery appears to be the only preferred choice of treatment in pediatric patients for lifelong disease management. In this case presentation, a 12-year-old male child exhibited severe headache and myalgia over the past six months. Physical examination revealed resting blood pressure of 146/80 mmHg despite being on three antihypertensive medications. A Doppler test and CT angiogram confirmed the presence of long-segment coarctation of the distal thoracic and proximal abdominal aorta. Other reports, such as blood tests, ECG and 2D echocardiogram, were normal. The patient thereafter underwent successful thoraco-abdominal aorto-aortic bypass employing a Dacron tube graft (14 mm x 60 cm), which resulted in an immediate drop in the brachio-femoral gradient (radial-160/85 mmHg, femoral-120/80 mmHg, mean gradient 16.6 mmHg). The patient was discharged on postoperative day (POD) 12, and the antihypertensive medications were readjusted. In subsequent follow-up periods for up to two years, all antihypertensive medications were stopped. At five years POD, the blood pressure rebounded to 170/94 mmHg. The CT angiograms post-surgery at one-month and five-year POD were normal. The patient did not report any adverse event (AE) and remained asymptomatic for five years.