Anomaly of Haughton type D left cervical aortic arch in combination with type B dissection: case report and literature review

BACKGROUND: The anomaly of cervical aortic arch is a rare phenomenon first described by Reid in 1914 and categorized by Haughton in 1975. The left cervical aortic arch Type D consisting of an ipsilateral descending aorta and coarctation or aneurysmatic formation of the arch demonstrates a complicated form requiring surgical management. Because of its rarity and unspecific symptoms only few cases are documented with the focus on surgical management. CASE PRESENTATION: A 43-year old, asymptomatic woman presented with a mediastinal mass overlapping the aortic arch region in a routine x-ray. For verification, a computed tomography was performed and revealed incidentally a type B dissection originating from an aneurysm of a left cervical arch with a maximum diameter of 6 cm. Because of the huge diameter and the potential risk of rupture, an urgent surgical repair was planned. Surgical access was performed through median sternotomy and an additional left lateral thoracic incision through the fourth intercostal space. Simultaneously to the preparation, partial cardiopulmonary bypass was installed in the left groin. After preparation of the recurrent and phrenic nerve and the supraaortic branches, the descending aorta was clamped. Before the distal anastomosis to a straight graft, we performed a fenestration of the dissection membrane about a length of 5 cm to preserve the perfusion of both lumina. Then, the straight graft was sutured to the proximal part of descending aorta. The left axillary artery originated directly from the aneurysm and was dissected and reimplanted with a separate 8 mm sidegraft to the straight graft between the distal arch and proximal descending aorta. The patient was extubated on first postoperative day and recovered well. CONCLUSION: The left cervical aortic arch type D is a rare disease, which is prone to aneurysm formation due to abnormal flow patterns and tortuosity of the aorta. The difficulty lays in the identification of the pathology, especially in the physical examination, since a pulsating mass or cervical murmur seem to be the most specific symptoms in the majority of young, female patients. If diagnosed, surgical therapy with resection of the aneurysm and reimplantation of the axillary artery under cardiopulmonary bypass demonstrates the treatment of choice.