Indian J Pediatr DOI 10.1007/s12098-014-1438-z
SCIENTIFIC LETTER
Bilateral Central Retinal Artery Occlusion: An Unusual Presentation of Polyarteritis Nodosa Arpita Dev Thakker & Mona Gajre & Raju Khubchandani & Rupesh Mendadkar & Ashish Doshi & Alka Jadhav
Received: 15 September 2013 / Accepted: 24 March 2014 # Dr. K C Chaudhuri Foundation 2014
To the Editor: Polyarteritis Nodosa (PAN) is a disease of medium sized arteries with typical involvement of the abdominal viscera, skin and kidneys. Sudden visual loss due to bilateral central- retinal- artery-obstruction (CRAO) is an unusual presentation of childhood classic PAN. A three-year-old boy presented to us with one month history of intermittent high grade fever and tender erythematous nodules over both extremities. He also had malena, abdominal pain and sudden decrease in vision since 4 d. He had a blood pressure of 150/90 mmHg (more than 99th percentile for age and height). Fundus revealed cherry red spot, dot-blot-hemorrhages surrounding the macula and optic disc edema suggestive of bilateral CRAO (Figs. 1 and 2). Significant investigations were as follows: Hb-8.3 g/dL, WBC-16,300/cumm (neutrophils 70 %), platelet count6,97,000/dL, CRP-20 mg/dL (elevated), elevated ESR75 mm at end of 1 h. Various markers i.e., HIV, HBsAg, HCV, Anti-ds DNA, Anti-neutrophillic cytoplasmic antibody, ASLO titre were negative . Urine analysis, renal and liver function tests were normal. Chest radiograph, echocardiography, carotid and renal doppler studies were normal. Skin biopsy showed arterial wall inflammation and perivascular infiltrate of neutrophils and lymphocytes. In view of histological evidence of necrotizing vasculitis in medium artery, skin involvement, hypertension and A. D. Thakker (*) : M. Gajre : R. Mendadkar : A. Jadhav
bilateral CRAO, a diagnosis of PAN with bilateral CRAO was made. Computed-tomography-angiogram of the abdomen and brain were normal. The child was treated with injection methylprednisolone (30 mg/kg) for 3 d followed by oral prednisolone. He also received cyclophosphamide injection (500 mg/m2) once a month for 6 mo followed by oral azathioprine. Antihypertensives were given during the acute episode and then tapered. The new criteria for PAN are: histopathological evidence of necrotizing vasculitis in medium or small arteries or angiographic abnormality (aneurysm, stenosis or occlusion) as a mandatory criterion; plus one of the following five: skin involvement, myalgia, hypertension, peripheral neuropathy, renal involvement [1–3]. Permanent remission can be achieved unlike other vasculitides [4]. Relapses can also occur if treatment is delayed or inadequate.
Pale optic nerve
Cherry red spot
Department of Pediatrics, Lokmanya Tilak Municipal Medical College and General Hospital, Sion, Mumbai, Maharashtra, India e-mail: [email protected] R. Khubchandani Department of Pediatrics, Jaslok Hospital and Medical Research Centre, Mumbai, Maharashtra, India A. Doshi Department of Ophthalmology, KBH Bachooali Eye and ENT Hospital, Mumbai, India
Dot blot hemorrhages
Fig. 1 Right eye showing dot blot hemorrhages, cherry red spot and pale optic nerve
Indian J Pediatr
blindness and or unexplained hypertension to enable early diagnosis and proper management which can lead to reduced morbidity and mortality. Conflict of Interest None.
Cherry red spot Role of Funding Source None.
Dot blot hemorrhages
Fig. 2 Left eye showing dot blot hemorrhages and cherry red spot
Despite being a rare disease, PAN should be included as a differential diagnosis in children with bilateral sudden onset
References 1. Ozen S, Ruperto N, Dillon MJ, Bagga A, Barron K, Davin JC, et al. EULAR/PReS endorsed consensus criteria for the classification of childhood vasculitides. Ann Rheum Dis. 2006;65:936–41. 2. Dillon MJ, Ozen S. A new international classification of childhood vasculitis. Pediatr Nephrol. 2006;21:1219–22. 3. Ozen S, Pistoric A, Iusan M, Ravelli A, Cuttica R, Lovell D, et al, for the Paediatric Rheumatology International Trials Organisation (PRINTO) The EULAR/PRINTO/PRES criteria for childhood polyarteritis. Ankara 2008. Abstract presentation at EULAR, June 2009. Ann Rheum Dis. 2009;68:713. 4. Dillon MJ, Eleftheriou D, Brogan PA. Medium-size-vessel vasculitis. Pediatr Nephrol. 2010;25:1641–52.
SCIENTIFIC LETTER
Bilateral Central Retinal Artery Occlusion: An Unusual Presentation of Polyarteritis Nodosa Arpita Dev Thakker & Mona Gajre & Raju Khubchandani & Rupesh Mendadkar & Ashish Doshi & Alka Jadhav
Received: 15 September 2013 / Accepted: 24 March 2014 # Dr. K C Chaudhuri Foundation 2014
To the Editor: Polyarteritis Nodosa (PAN) is a disease of medium sized arteries with typical involvement of the abdominal viscera, skin and kidneys. Sudden visual loss due to bilateral central- retinal- artery-obstruction (CRAO) is an unusual presentation of childhood classic PAN. A three-year-old boy presented to us with one month history of intermittent high grade fever and tender erythematous nodules over both extremities. He also had malena, abdominal pain and sudden decrease in vision since 4 d. He had a blood pressure of 150/90 mmHg (more than 99th percentile for age and height). Fundus revealed cherry red spot, dot-blot-hemorrhages surrounding the macula and optic disc edema suggestive of bilateral CRAO (Figs. 1 and 2). Significant investigations were as follows: Hb-8.3 g/dL, WBC-16,300/cumm (neutrophils 70 %), platelet count6,97,000/dL, CRP-20 mg/dL (elevated), elevated ESR75 mm at end of 1 h. Various markers i.e., HIV, HBsAg, HCV, Anti-ds DNA, Anti-neutrophillic cytoplasmic antibody, ASLO titre were negative . Urine analysis, renal and liver function tests were normal. Chest radiograph, echocardiography, carotid and renal doppler studies were normal. Skin biopsy showed arterial wall inflammation and perivascular infiltrate of neutrophils and lymphocytes. In view of histological evidence of necrotizing vasculitis in medium artery, skin involvement, hypertension and A. D. Thakker (*) : M. Gajre : R. Mendadkar : A. Jadhav
bilateral CRAO, a diagnosis of PAN with bilateral CRAO was made. Computed-tomography-angiogram of the abdomen and brain were normal. The child was treated with injection methylprednisolone (30 mg/kg) for 3 d followed by oral prednisolone. He also received cyclophosphamide injection (500 mg/m2) once a month for 6 mo followed by oral azathioprine. Antihypertensives were given during the acute episode and then tapered. The new criteria for PAN are: histopathological evidence of necrotizing vasculitis in medium or small arteries or angiographic abnormality (aneurysm, stenosis or occlusion) as a mandatory criterion; plus one of the following five: skin involvement, myalgia, hypertension, peripheral neuropathy, renal involvement [1–3]. Permanent remission can be achieved unlike other vasculitides [4]. Relapses can also occur if treatment is delayed or inadequate.
Pale optic nerve
Cherry red spot
Department of Pediatrics, Lokmanya Tilak Municipal Medical College and General Hospital, Sion, Mumbai, Maharashtra, India e-mail: [email protected] R. Khubchandani Department of Pediatrics, Jaslok Hospital and Medical Research Centre, Mumbai, Maharashtra, India A. Doshi Department of Ophthalmology, KBH Bachooali Eye and ENT Hospital, Mumbai, India
Dot blot hemorrhages
Fig. 1 Right eye showing dot blot hemorrhages, cherry red spot and pale optic nerve
Indian J Pediatr
blindness and or unexplained hypertension to enable early diagnosis and proper management which can lead to reduced morbidity and mortality. Conflict of Interest None.
Cherry red spot Role of Funding Source None.
Dot blot hemorrhages
Fig. 2 Left eye showing dot blot hemorrhages and cherry red spot
Despite being a rare disease, PAN should be included as a differential diagnosis in children with bilateral sudden onset
References 1. Ozen S, Ruperto N, Dillon MJ, Bagga A, Barron K, Davin JC, et al. EULAR/PReS endorsed consensus criteria for the classification of childhood vasculitides. Ann Rheum Dis. 2006;65:936–41. 2. Dillon MJ, Ozen S. A new international classification of childhood vasculitis. Pediatr Nephrol. 2006;21:1219–22. 3. Ozen S, Pistoric A, Iusan M, Ravelli A, Cuttica R, Lovell D, et al, for the Paediatric Rheumatology International Trials Organisation (PRINTO) The EULAR/PRINTO/PRES criteria for childhood polyarteritis. Ankara 2008. Abstract presentation at EULAR, June 2009. Ann Rheum Dis. 2009;68:713. 4. Dillon MJ, Eleftheriou D, Brogan PA. Medium-size-vessel vasculitis. Pediatr Nephrol. 2010;25:1641–52.
