4/21/2018 Presenting complaint Blurred vision Dizzyness & - - PowerPoint PPT Presentation

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4 21 2018
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4/21/2018 Presenting complaint Blurred vision Dizzyness & - - PowerPoint PPT Presentation

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4/21/2018 Presenting complaint Blurred vision Dizzyness & headaches Finger numbness Case Presentation Dysarthria Dr Sadia Quyam Evelina London Childrens Hospital Investigations History of Presenting complaint Experienced the symptoms

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4/21/2018 1

Case Presentation

Dr Sadia Quyam

Evelina London Childrens Hospital

Presenting complaint Blurred vision Dizzyness & headaches Finger numbness Dysarthria History of Presenting complaint Experienced the symptoms whilst walking to school Attended ED: 2-3 similar episodes over the last yr

PMH Asthma Severe heamophilia A Small PFO Aspergers syndrome

Progressive effort intolerance 18m Investigations

Head CT and MRI

Normal

Echo

small PFO, raised pulmonary pressures

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Investigations Investigations Hb 141 g/dl. VO2 assumed 146mls/min/m2.

FiO2 0.21, TPG 45mmHg Qpi 3.8 L/min/m2 PVRI 11.8 WU.m2 FiO2 1.0 TPG 44mmHg Qpi 3.8 L/min/m2 PVRI 13.3 WU.m2 FiO2 1.0 + iNO 20ppm TPG 43mmHg Qpi 3.4 L/min/m2 PVRI 12.6WU.m2

Summary

11 year old boy with severe haemophilia A treated with recombinant factor VIII via porto-cath. Well controlled with only one bleeding complication in history. Progressive effort limitation for 18 months (WHO III) Presented acutely with neurological symptoms consistent with TIA, haemorrhage excluded on neuro-imaging. During the acute assessment he was found to have a murmur and subsequent echo showed evidence of severe PH, confirmed on catheterisation. Further work up included a CT which was reported locally and did not reveal a cause for his PH - referred to National PH service

MRI angio

Repeat Echo showed central venous catheter deep in the RA prolapsing through the TCV

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Review

CT initially reported as normally twice but on further and in the context f the history felt to be abnormal Relative paucity of the vasculature noted in the RLL. The regional variation in calibre signifies thromboembolic disease, es Conclusion: Severe pulmonary hypertension, no features of PVOD Concern that CTEPH is a cause. The PFO may explain the cerebral events.

MRI angio MRI angio

Review

Although felt to be operable, significant challenge and risk of doing so in a child with heamophillia

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PEA

Cath