are stagaation thrombosis in arteriosclerosis, exterial compresain and pimar ... normal platelets, others showed a hyaline cyto- plasm, often with vacuoles and ...
910 Proc. roy. Soc. Med. Volume 62 September1969
ing became unacceptable to both patiet and parnts, and blood s W 0sWuley refusd. The scheme had to be abandoned aftWr four weeks. l pbetw increased The recognized platet sicness ad raised plasma homocystine forms the basis of dietary therpy in the adolscent, which aims to prrtthe Iliromboeznbolic features of the di. The extent to whieh increased platelet st is tXh sole cause of these episodes remains largy speculative, but the role of the a ie vauclar chage, so frequently observed at autopy in t. Tis these individuals, cannot be progressive structural damage is not reversd, by dietary tratment. While the value of dietary therapy in the affected newborn is not in dispute, the b s for the adolscent would not sem ca. iTe added practical difficulties of dietry r ion d are consderin these mentally retarded able, while from the humanitarian point of i, this.dietary policy seems hard to justify. REFERENCES Bar.G w & Spah G L (1967) Laneet i, 337 Carey M C, Doae_w D E, FitzGerwId 0 & McAuley F D (1968) Amer. J. Med. 45, 26 Perry T L, Hams S, Love D L, Crawford L E & Tischler B (1968) Lancet ii, 474
Retil Vein Tlombosis wit Pe_riphal Venous Thrombosis and S C Kalhan MB DCH (for J Lorber mD FRcp) (Children's Hospital, Sheffield)
~ ~ ~ ~ ~ .si . |.s
26
Fig 1 Veogram of right leg showing a block dt the level of the feoroAl1 vein. The i-liac veins have been filled by the collaterals
ment, and while still on prednisolone 10 mg/day, he still had- fresh episodes of thrombosis in the n right leg. Detailed- huatologic e did not reveal any clotting defect, the only persistent abnormality being thrombocytopenia, prolonged partial thromboplastin timne and decreaingly elevated ESR. No LE cells or antinuclear factors demoinstrated at any stage. Discussion
A B, boy aged 9+ years Presented in April 1968 with a six-week history of pain followed by swelling of the whole right leg, which showed signs of deep venous thrombosis, confirmed by venogram (Fig 1). No systemic symptoms or signs. Investigation showed high ESR and platelet counts of around 6 on various occasions. Bone marrow suggestive -of subacute or chronic idiopathic thrombocytopenic purpura. Partial thromboplastin time prolonged, hence anticoagulants were not given. Early in May 1968 developed pain in both eyes, diplopia, impaired vision and slight restriction of visual fields. Bilateral retinal vein thrombosis found. The diagnosis of a diffuse vasculitis involving retinal and peripheral veins was made. Treated with prednisolone 40 mg/day. His vision and fundi returned to normal within four weeks. Ten weeks later, and again on subsequent occasions, he developed superficial thromboses of various veins in the right leg and thigh. Biopsy was not helpful. Six months after starting treat-
The association of peripheral venous thrombosis with retinal venous occlusion has not been described before., In the,presece of the bilateral retinal fnig,tepossibility of an intracranial space-ocupying lsion was considered but ex-cluded. Embolism of the retinal veins fromn the leg veins could not have occurred in t-he absence of a congbnital heart disease; as the lesions were too symm tiscal for this possibility. The usual cause of retinal, vein thrombosis are stagaation thrombosis in arteriosclerosis, exterial compresain and pimar vascumtis. The first two are usalmy seen in aduts and there was no clinical evidenme for these in this child: by
exclusion, vasculitis was favoured. The VI nere palsy responsible for his diplopia renuined u lain and, it lasted about six to eight days with coiplete recovery. The other unusual feature was the prewsnce of low platelets with prolonged partial thromboplastin time and prothrombin time. The platelets were particularly low during acute episodes of
27
Section ofPwdiatrics
911
thrombosis. Bone marrow showed increased number of megakaryocytes - some normal with normal platelets, others showed a hyaline cytoplasm, often with vacuoles and devoid of platelets. This was suggestive of subacute or chronic idiopathic thrombocytopenic purpura. The association of low platelets with massive intravascular thrombosis has been described before, but these cases also have increased coagulability of blood. We considered in this case the consumption of platelets to be one of the factors. He never developed purpura, hypertension or high blood urea, and thrombotic thrombocytopenic purpura is thus excluded. In the presence of these features, we considered this to be a diffuse vasculitis and treated him with prednisolone to which he responded well. His eyes recovered but he continues to have superFig l Chest X-ray, November 1961, showing globular ficial and deep femoral thromboses. REFERENCES Haurani F 1, Regan J, Carroll R T & Erslev A J (1964) Amer. J. med. Sci. 248, 260 Klien B A & Olwi0 J H (1956) Arch. Ophthal. 56, 207 Kimura S J, Carriker F R & Hogan M J (1956) Arch. Ophthat. 56, 361
Cerebral Emboli from Aneurysm of Left Atrial Appendage Judith Hall MB MRCPEd (for R H Dobbs MD FRCP) (London Hospital, London)
enlargement of left cardiac border
Excision of aneurysm of left atrial appendage (Mr E Aberdeen). Anticoagulants discontinued post-operatively. May 1968: Palatopharyngoplasty (Mr J Watson, East Grmnstead). Comment There are three main features of interest: The cerebral incidents in retrospect were compatible wsith emboli arising directly from the aneurysm of the left atrial appendage Nature of the cardiac lesiont Aneurysms of the left atrium are uncommon except in association with mitral valve disease Taussig (1960) mentions a case but the appendage was not involved. Three children with aneurysms of the left atrial appendage were reported (Parmley 1962, Pitts & Potts 1962), the X-rays were all similar to the present case; one had cerebral and other emboli. Thoracotomy was postponed in S A because of the hazards. Anticoagulant therapy successfully prevented further embolic episodes. At operation the aneurysm contained no clot (clots were found in 2 of the other reported cases). Eight adult cases have been reported (Williams 1963, Godwm et al 1968), 2 with cerebral emboli. The speech defect in S A has two components: executive dysphasia, and dysarthria. The former (and his hemiplegia) has improved steadily. The dysarthria remains severe. This and the residual dysphagia (with drooling) are expressions of a suprabulbar palsy due to multiple emboli.
S A, boy born 15.12.60 History: November 1961, sudden onset transient weakness of right arm; fifteen generalized convulsions during next two days, each lasting one minute, preceded by fifteen minutes of unusual quietness. On examination: Pyrexial. 'Dreamy and far away'. No other abnormalities. Investigations: CSF, skull X-ray and ECG normal. Chest X-ray showed globular enlargement of left cardiac border (Fig 1). Progress: No further fits. Mental state normal and apyrexial within two days. Provisional diagnosis: 'viral encephalitis'. September 1962: Left-sided convulsive movements with development of coma for one day and left hemiplegia which improved over three days. Two weeks later, similar episode affecting right side and resulting in dysphasia, dysarthria, dysphagia and right hemiplegia which have persisted ever since. Chest X-ray unchanged. Diagnosis of cerebral emboli considered. Angiography suggested lesion REFERENCES Godwin T F, Auger P, Key J A & Wigle E D involving left ventricle. Circulation 37, 397 Subsequent treatment: December 1962-November (1968) Parmley L F (1962) Circulaticn 25, 553 1966: Anticoagulant therapy with phenindione. Pitts R MII & Potts W (1962) Arch. Surg. 84, 334 B (1960) Congenital Malformations of the Heart. No further cerebral incidents occurred. Physio- Taussig Cambridge, Mass. & London; 2, 930 therapy and speech therapy. November 1966: Williams W G (1963) Brit. Heart J. 25, 637
