Critical Care |

Decompressive Hemicraniectomy: A Novel Therapy for Sneddon Syndrome Presenting as a Massive Stroke FREE TO VIEW

Mohsin Ijaz, MD; Sara Qasim Bughio, MD; Dmitry Lvovsky, MD; Daniel Miller, MD
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Bronx Lebanon Hospital Center, Bronx, NY

Chest. 2014;146(4_MeetingAbstracts):275A. doi:10.1378/chest.1995009
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SESSION TITLE: Critical Care Cases II

SESSION TYPE: Affiliate Case Report Slide

PRESENTED ON: Tuesday, October 28, 2014 at 07:30 AM - 08:30 AM

INTRODUCTION: Sneddon’s syndrome (SS) is a rare hypercoaguable state associated with ischemic strokes and livedo reticularis that occurs primarily in young females with incidence of four new cases per million.

CASE PRESENTATION: A 33 year old female presented with complains of abdominal pain, nausea and vomiting. She had 4 normal vaginal deliveries and one spontaneous abortion. She denied smoking, alcohol or drug use. She was not taking any medications. On examination she had lacy, vascular, non-blanching reticular pattern of purplish discoloration over the entire abdomen. Later she was found confused with dysarthria, left sided facial droop and right sided hemiplegia. Underlying mass lesion was suspected and thrombolysis was not done. MRI/ MRA and echocardiogram were obtained immediately (See table 1). She soon became critically ill with signs of impending brain herniation. She was started on mechanical ventilation and eventually underwent decompressive hemicraniectomy (DH). She developed DVTs in bilateral lower extremities for which inferior vena cava filter was placed. Biopsy of the abdominal lesion was consistent with livedo reticularis. She subsequently improved clinically, was successfully extubated, and later discharged to a rehabilitation center.

DISCUSSION: SS is part of the seronegative antiphospholipid group of diseases. It manifests with diverse presentations, such as; secondary headaches, seizures, dementia, vasculitis, hypercoaguable states, Raynaud’s phenomenon, cardiac thrombosis, atrial myxoma, recurrent miscarriages, cognitive impairment, renal microvascular obstruction leading to hypertension and renal failure. It mainly occurs sporadically, although a few familial cases have been reported. Our differentials included Systemic Lupus Erythematosus, Antiphospholipid syndrome, Polyarteritis Nodosa, Microscopic Polyangitis, Syphilis, Polycythemia Vera and SS. Our patient did not meet criteria for any of the diseases listed above. Conclusively, by exclusion, we arrived to the diagnosis of Sneddon’s syndrome. (See table 2). Traditional treatment with corticosteroids and Azathioprine has not shown promising results. Antiplatelet agents may be of some benefit.

CONCLUSIONS: DH in a young patient can be life saving and may improve neurologic outcome. In cases of large ischemic strokes thrombolysis should be avoided to prevent hemorrhagic conversion.

Reference #1: Zelger B, Sepp N, Stockhammer G, et al. Sneddon's syndrome: A long-term follow-up of 21 patients. Archives of Dermatology. 1993;129:437-447

DISCLOSURE: The following authors have nothing to disclose: Mohsin Ijaz, Sara Qasim Bughio, Dmitry Lvovsky, Daniel Miller

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