Patients with multi-organ disorders may present with a plethora of confusing symptoms and signs. Often early diagnosis of significant disease is essential and can be difficult with standard radiological techniques. This case report presents the use of two radioisotopic techniques to assess brain and lung perfusion in a patient with such an acute-multi-organ disorder-the HELLP syndrome

Buscombe, John R.

Hilson, Andrew J.W.

Lass, Piotr

English

Via Medica Journals

73Shortcommu-nicationUse of lung and brain perfusionimaging in the HELLP syndromenant (first trimester) with left calf tenderness. This was found oncontrast venography to be secondary to a deep venous thrombo-sis and the patient treated with heparin. Following pregnancy thepatient was given Warfarin and remained anti-coagulated. How-ever her requirement for Warfarin continued to rise over the sub-sequent 12 months.An auto-antibody screen was performed which showed a pos-itive anti-DNA antibody titre at a titre of greater than 100. Therewere no double-stranded DNA antibodies though anticardiolipinantibody titre was greater than 1 in 120 rising to a titre of 1 in 274over 12 months.A family history revealed a maternal great aunt who had diedof pulmonary embolism during pregnancy and a female maternalcousin who had also presented with a deep venous thrombosisduring pregnancy.At the end of her pregnancy the patients blood pressure be-gan to rise and she appeared to be developing pre-eclampsia.Delivery was induced and four days after being delivered of ahealthy infant, the patient suffered a series of epileptiform convul-sions. An MRI was performed but this was unremarkable, howev-er a Tc-99m HMPAO study (Figure 1) was performed which showedmultiple defects in perfusion throughout the cerebral hemispheresconsistent with multiple small infarcts.Her circulating platelet count began to fall, and she devel-oped both liver and renal impairment consistent with a HELLPsyndrome. To reduce the level of anti-cardiolipin antibodies plas-ma exchange was initiated, but during treatment the patient de-veloped chest pain and dyspnoea. Ventilation/perfusion lung scan-ning revealed multiple perfusion defects on the Tc-99m MAA scanbut a normal Kr-81m ventilation scan consistent with multiple pul-monary emboli (Figure 2).DiscussionThis case illustrates the flexibility of nuclear medicine techniquesin being able to identify different sites of vascular occlusion. Te-99m HMPAO brain perfusion SPECT can be used to identify thesmall infarcts which occur within the brains of patients with HELLPsyndrome -the use of anatomical methodologies such as CT hasbeen described (6). However in our patient, defects consistentwith multiple small brain infarcts were only seen on Tc-99m HM-PAO imaging but not with MRI.The condition itself is rare and affects about 1 in 15,000 livebirths, it is less common in Caucasians than other racial groupsJohn R. Buscombe, Piotr Lass1, Andrew J.W. HilsonDepartment of Nuclear Medicine, Royal Free Hospital and School ofMedicine, London, NW3 2QG, UK1Department of Nuclear Medicine, Medical University of Gdaæsk, Gdaæsk,PolandAbstractPatients with multi-organ disorders may present with a plethoraof confusing symptoms and signs. Often early diagnosis of sig-nificant disease is essential and can be difficult with standardradiological techniques. This case report presents the use oftwo radioisotopic techniques to assess brain and lung perfu-sion in a patient with such an acute-multi-organ disorder - theHELLP syndrome.Key words: Tc-99m HMPAO, brain perfusion, ventilation per-fusion lung scintigraphy, HELLP syndromeIntroductionBrain perfusion imaging using Tc-99m hexamethylpropyleneam-ine-oxine (HMPAO) has been used in a variety of clinical condi-tions (1-3). It is of particular use when infarcts are small and maybe missed on X-ray computed tomography (CT). Such diffusemicro vascular disease may be seen in patients with multi-infarctdementia (4) but is rarely seen in younger people unless they havean underlying cause for microangiopathy. In some cases this maybe combined with a syndrome which results in widespread em-boli. Such a condition, normally occurring at the end of first preg-nancy, is the recently described Haemolysis-Elevated Liver enzyme-Low Platelet count (HELLP) syndrome (5). In this, we demonstratethe use of radioisotopic techniques to investigate both micro-an-giopathic infarcts in the brain and major vessel emboli in the lungsusing standard lung and brain perfusion imaging methods.Case report31-year-old white Caucasian patient with an initial clinical diagno-sis of system lupus erythematosis presented aged 31 when preg-Correspondence to: Dr J.R. BuscombeDepartment of Nuclear Medicine, Royal Free Hospital,Pond Street, London NW3 2QGTel/fax: (+44 171) 830246974 Nuclear Medicine Review Vol. 3, No. 1, 2000Shortcommu-nicationAB75Shortcommu-nicationFigure 1. With brain imaging the normal MRI (Figure 1a) contrasts with the small defects seen in the cortex on Tc-99m HMPAO brainperfusion SPECT displayed as transaxial slices (Figure 1b) and as a 3D surface rendered image (Figure 1c).Figure 2. Tc-99m MAA and Kr-81m lung scintigraphy showing a significant ventilation/perfusion mismatch in the right lung base (ventilation films markedvent and perfusion films marked pert, anterior images are in upper row and posterior images in the lower row).C76 Nuclear Medicine Review Vol. 3, No. 1, 2000Shortcommu-nication(7). Treatment in these patients often proves difficult with repeat-ed emboli occurring (8). As in the patient reported here, there isevidence of a familial predisposition to the HELLP syndrome witheither the frank syndrome appearing within families or other familymembers having a history of multiple emboli. At present it is un-clear whether this family link operates through the expression ofanti-phospholipid antibodies or the anti-cardiolipin antibodiespresent in our patient (9).As this condition is a multi-organ disorder it is surprising thatthere have not been other reports of the use of imaging. The onlyreported use of nuclear medicine has been to assess the degree ofhepatic impairment using hepatobiliary scintigraphy and Tc-99msulphur colloid imaging (1011).The distinct features of this case are not only the first reporteduse of both Tc-99m HMPAO and radionuclide ventilation perfusionlung imaging in a patient with the HELLP syndrome but also toemphasise the enhanced sensitivity and versatility of radionuclidetechniques in patients such as this who have acute multi-organdisease.Referencesl. Sharp PF, Smith FW, Gemmell HG. Tc-99m HMPAO steroisiomers aspotential agents for imaging cerebral blood flow: human volunteer stud-ies. J Nucl Med 1986; 21: 171177.2. Ell PJ. Mapping cerebral blood flow. J Nucl Med 1992; 33: 18431845.3. Ell PJ, Jarritt PH, Costa DC, Cullum ID, Lui D. Functional imaging of thebrain. Semin Nucl Med 17: 214229.4. Gemmell HG, Sharp PF, Besson JA, et al. Differential diagnosis in de-mentia using the cerebral blood flow agent Tc-99m HM-POA: a SPECTstudy. J Comput Assist Tomogr 1897; 11: 398402.5. Weinstein L. Syndrome of hemolysis, elevated liver enzymes, and lowplatelets: A severe consequence of hypertension in pregnancy. Am JObstet Gynecol 1982; 142: 159167.6. Stefanowicz M, Lipinska J, Stefanowicz E, Malarkewicz CJ. Zmiany wtkance mózgowej w przebytym zespole HELLP potwierdzone bada-niem CT. Ginekologia Polska 1995; 66: 185187.7. Sebban E, Benfila JL, Pennehowat G, et al. Predisposition ethniquedu HELLP syndrome. Etude retrospective de 12 cas dont 5 serveresen post partum. J de Gynecologie, Obstetrics et Biologique de laReproduction 1994; 23: 181187.8. Omstein MH, Rand JH. An association between refractory HELLP syn-drome and anti-phospholipid antibodies during pregnancy, a reportof 2 cases. J Rheumatol 1994; 21: 13601361.9. Berti P, Contino L, Presondo P, et al. Is the HELLP syndrome due to inher-ited factors? Report of two cases. Haematologica 1994; 79: 170172.10. Rosen JM, Luhman KC, Tank RA. Hepatobiliary scintigraphy inthe evaluation of preeclampsi and HELLP syndrome. Clin NuclMed 1994; 19: 740741.11. Davidson RM, Barron BJ, White PA, et al. Diagnosis by radiocol-loid imaging of post partum hepatic necrosis in the syndromeof hemolysis, elevated liver enzymes, and low platelets. Clin NuclMed 1992; 17: 322324.

Use of lung and brain perfusion imaging in the HELLP syndrome

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Use of lung and brain perfusion imaging in the HELLP syndrome

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