Paraparesis in Coarctation of Aorta: Diagnosis in Disguise.

Abstract

To the Editor: A 55-year-old woman presented with sudden onset symmetric weakness of both lower limbs along with truncal weakness and urinary retention for 7 days. However, she denied any history of pain, trauma, loss of consciousness, or any evidence of upper limb weakness, breathlessness, or cranial nerve involvement. According to history, a provisional diagnosis of acute transverse myelitis was made, for which she was being treated before coming to the Institute of Medical Sciences, Banaras Hindu University. On examination, the pulse of lower limbs was feeble as compared to upper limb pulse. Blood pressure of upper limb was 210/100 mmHg, while it was not recordable in the lower limbs. Prominent superficial blood vessels were visible on her back in the interscapular region, and bruits could be palpated along her intercostal spaces. Neurological examination revealed complete loss of power (grade 0/5) in lower limbs with hypoesthesia below C7 vertebra. Deep tendon reflexes were absent, and plantar reflexes were not elicitable. Examination of the upper limbs and cranial nerves were normal. There was no spinal tenderness or deformity. Systolic murmur prominent at aortic area was noted on auscultation. The rest of the systemic examination was within normal limit. Basic blood investigation including hemocoagulation tests was within normal limit. A chest X-ray showed notching of inferior margins of ribs with a normal cardiac shadow and lung parenchyma. Electrocardiograph revealed sinus rhythm with left ventricular enlargement. Two-dimensional echocardiography showed the presence of moderate left ventricular hypertrophy with decreased flow in the descending thoracic aorta beyond the origin of left subclavian artery, with no other valvular or congenital cardiac malformations. Computed tomography of aorta revealed a circumferential constriction of the descending aorta of length 1.5 mm and distance 2.1 cm from the origin of left subclavian artery, with 82% stenosis of the lumen, which suggested that the patient had coarctation of aorta (CoA). A large number of intercostal and phrenic collaterals were also seen, along with tortuous bilateral internal mammary arteries and inferior epigastric arteries. A contrast enhanced magnetic resonance imaging of the spine showed the presence of multiple tortuous engorged vessels in epidural and intradural spaces along mixed signal intensity lesion with convex posterior margin noted in epidural space of C7, D1, and D2 suggestive of epidural hematoma at C7–D2 level adhered to the dura causing spinal cord compression [Figure 1]. This compression of the spine cord was severe enough to cause myelopathy below the level of C7 in the form of sudden onset of flaccid weakness of truncal and lower limb muscles due to spinal shock. Because of the limited options of surgical management at our center, we referred the patient to higher center for further treatment. Figure 1 Multiple tortuous engorged vessels in epidural and intradural spaces along with an epidural hematoma at C7–D2 level adhered to the dura causing cord compression (a-c). CoA has an incidence of 0.3–0.4/1000 live births and accounts for 5–7% of adults with congenital heart disease. Usually associated complications with it are aortic dissection, infective endocarditis, and rupture of aneurysms in the circle of Willis leading to intracranial hemorrhage. Spinal cord complications of CoA are rare, and only a few cases have been reported due to subarachnoid hemorrhage following rupture of an aneurysm of abnormally dilated spinal artery.[1,2] Compressive myelopathy due to collaterals in the spinal cord is a very unusual presentation, and few reports have been reported worldwide.[3] The development of an acute, nontraumatic epidural hematoma is even rarer.[4] Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.