Spastic hemiparesis and presumed prenatal embolisation.

Abstract

SUMMARY An infant with clinical evidence of prenatal right subclavian arterial occlusion subsequently developed left hemiparesis with cystic infarction of the territory of the right middle cerebral artery. Perinatal limb but elsewhere they were normal. The arm and forearm circumferences, measured at midpoints, were 1-0 cm smaller on the right than on the left. The rest of the physical examination was unremarkable.Plain radiographs of the cervical spine, chest, and right shoulder were normal. A full blood count on day 1 yielded the following results: haemoglobin concentration 223 g/l; packed cell volume 63%; white cell count 20-2x109/l and platelet count 80X109/l. Viral studies were all negative. A splint was applied to the affected limb.On the second day of life the tip of the right ring finger had become dark blue, though the other fingers were viable. Doppler ultrasound scan of the affected limb detected pulsation in the right subclavian artery to the outer border of the first rib, but not beyond. Over the next 72 hours the Doppler pulsation became audible in the axillary artery down to the radial artery and pulses could be felt. The infant remained otherwise well.On discharge at the age of 2 weeks movements in the affected limb had considerably improved and all the pulses were palpable. The range of active movements of the left limbs were within normal limits. Because the improvement was so rapid no further investigations were carried out. The child subsequently failed to attend the follow up clinic.At the age of 7 months the infant was brought back to the clinic the mother complaining that since the age of 4 months the child had had limited use of her left limbs; she had otherwise been well. A full neurological examination showed a left spastic hemiparesis and left hemianopia. The head circumference had dropped to the 10th centile having been on the 25th centile at birth. Her right upper limb was normal, as was her social behaviour. Computed tomography brain scan at the age of 7 months showed a cystic lesion in the area of the right middle cerebral artery with shrinkage of the right hemisphere ( figure). When reviewed at 17 months of age, she could only move about by shuffling on her bottom but she appeared cognitively normal and no seizures had been observed. Discussion

Two Children With Both Arm Ischemia and Arterial Ischemic Stroke During the Perinatal Period

“…A review of the literature revealed reports of 8 other children who appeared to have had emboli to both an extremity and the brain during the perinatal period [3][4][5][6][7][8][9] (Table 1). Each of these children presented with limb ischemia within 10 minutes of…”

“…Two had malformation and decreased muscle mass in the affected ischemic limbs, suggesting the occlusion took place in utero. 3,9 Both of the 2 newly presented cases also had abnormal limb appearance at birth.…”

“…Color duplex ultrasound is the recommended imaging test for identifying occluded vessels in ischemic limbs, 1 and 5 of the cases were evaluated with ultrasound. 3,5,6,8,9 Radiographs were performed in 5 cases, 3,4,6,7 including case 2, and were all normal, suggesting that radiography may not contribute much to these evaluations.…”

“…Placentas were examined in only 4 cases; 2 were normal, 4,8 and 2 were believed to be possible sources of emboli. 3,7 Almost all of the previously and currently described cases had at least 1 possible risk factor identified. The 1 exception was from 1989, when less was known about perinatal thromboembolism, and fewer tests for thrombophilia were available.…”

“…6,8 Three children received conservative management of limb ischemia, such as splinting, local heat, and frequent dressing changes, and had spontaneous restoration of blood flow. 3,6 One child underwent thrombectomy, 8 and 4 children required debridement, skin grafting, and/or amputation. 7,9 Some physicians prefer conservative management of limb ischemia if the limb is viable, but thrombolytics may be considered.…”