Headache in late pregnancy: a symptom for Vogt–Koyanagi–Harada disease
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- Matsubara, S., Kuwata, T., Ohkawara, Y. et al. Arch Gynecol Obstet (2011) 283: 1423. doi:10.1007/s00404-010-1748-y
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Headache in late pregnancy is warning of the presence of severe and/or lethal disorders. Here, we present a case of Vogt–Koyanagi–Harada disease (VKH), in which headache was the preceding and predominant symptom. At 37 weeks of pregnancy, a Japanese 1-para woman with an uneventful pregnancy complained of severe headache and then blurred vision. Although we initially diagnosed this condition as pregnancy-associated retinal detachment, headache and blurred vision persisted after delivery. Eye anterior segment lesions appeared and VKH disease was diagnosed, with systemic steroid administration completely ameliorating both the headache and visual disturbance. Obstetricians must be aware that headache may be the first sign of VKH disease.
Headache during pregnancy, especially in late pregnancy, sometimes indicates severe and/or lethal diseases, including cerebral vascular attack, hemolysis elevated liver enzymes and low platelets syndrome (HELLP), impending eclampsia, etc. We present a case of Vogt–Koyanagi–Harada disease (VKH), in which headache preceded the appearance of eye symptoms, nearly evading diagnosis.
We expected spontaneous resolution of the detachment, and thus no treatment, but at 381/7 the headache worsened and she was admitted. She had blood pressure of 100/67 mmHg without proteinuria, pretibial edema, or blood data indicative of HELLP. Acetaminophen did not ameliorate the headache. Headache and visual disturbance led her to become depressed. Considering the immature cervix and strong wishes of the patient, a cesarean section was performed at 383/7. The headache persisted postpartum. Ophthalmological examination revealed deteriorating visual acuity (best-corrected visual acuity of 0.2 bilaterally), deterioration of the retinal detachment, and inflammation of the eye anterior segment characteristic of VKH. HLA-D4 was positive, also compatible with VKH. We diagnosed this condition as VKH and steroid pulse treatment was performed; 500 mg intravenous methylprednisolone for 3 days followed by oral 50 mg prednisolone with gradual tapering for 1 month. This led to complete disappearance of the headache, resumption of visual acuity, and resolution of the retinal detachment (Fig. 1b). The patient agreed to reporting and patient anonymity was preserved. The ophthalmological aspect of this case was previously described in Japanese .
VKH is characterized by bilateral posterior uveitis. In the prodromal stage of VKH, extraocular symptoms (headache, tinnitus, dizziness, or nausea, etc.) may be present . These symptoms may persist into the acute uveitic stage, which is characterized by diffuse choroiditis and visual blurring . Fluid may accumulate beneath the retina; this may frequently lead to retinal detachment, which can be revealed by optical coherence tomogram . Patients with VKH may frequently develop sterile meningitis . A lumbar puncture will reveal lymphocytic pleocytosis of the cerebrospinal fluid .
Ophthalmologically, there are some differences between pregnancy-associated detachment versus detachment observed in a patient with VKH. Subretinal fluid is thinner and the retina is mobile in pregnancy-associated detachment, whereas subretinal fluid is thicker and the retina elevates in a lobulated pattern in detachment in VKH . We first diagnosed this condition as pregnancy-associated retinal detachment. It cannot be rejected that more detailed and more frequent ophthalmological examination may have led to the earlier diagnosis of VKH in this patient.
Previous reports [5, 6] have described the course of VKH during pregnancy; however, almost all dealt with cases in the first or second trimester, focusing on the relationship between the safety of steroid therapy and the pregnancy outcome: the present case is, to our knowledge, the latest occurrence ever in the English literature. Importantly, headache was the first and visual disturbance was the second in terms of both the patient’s complaint priority and their occurrence. As described, in some cases of VKH, headache preceded the development of characteristic ophthalmologic findings and led to diagnostic confusion , which held true in the present case. No reports have highlighted the significance of headache in pregnant patients with VKH. The concomitant appearance of headache and blurred vision may alert a clinician to the diagnosis of temporal arteritis, which is an ophthalmological emergent situation. Temporal arteritis usually occur in elderly people and is devoid of uveitis.
Although obstetricians may well be trained to consider severe and/or lethal diseases in the face of a pregnant woman with a headache, few may consider VKH, for which immediate steroid treatment is required. We must be aware that headache may be the first sign of VKH.
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