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Show Clinical Correspondence Section Editors: Robert Avery, DO Karl C. Golnik, MD Caroline Froment, MD, PhD An-Guor Wang, MD Incomplete Gradenigo Syndrome in a Patient With Mastoiditis and Lateral Sinus Thrombosis Si-Bo Li, MD, Xiu-Chuan Jia, PhD, Jia-Xi Song, MD, Shi-Cong Zhao, MD, Jun-Shu Gao, PhD, Wei Jin, PhD G radenigo syndrome (GS) is a rare, but life-threatening complication of middle ear infection that consists of pain in the trigeminal territory, ipsilateral abducens nerve palsy, and petrous apicitis. In the antibiotic era, the symptoms of GS have not always been atypical, which makes it more difficult for physicians to make the diagnosis. We present one rare adult case of incomplete GS associated with ipsilateral lateral sinus thrombosis secondary to mastoiditis. A 28-year-old man presented to the emergency room with a 10-day history of left-sided headache with aggravation for the last 4 days. His past medical history was unremarkable except for an episode of left-sided headache accompanied by intermittent fever and constant tinnitus in the left ear 1 month before admission, which subsided after treatment with oral antibiotics for 1 week. The new-onset headache located in the left-sided temporal region was persistent, throbbing and insufferable; the headache was accompanied by nausea and vomiting and had worsened over the last 4 days. The temperature, blood laboratory results, and physical examination results were normal. Brain CT demonstrated high-density areas in the left transverse and sigmoid sinuses (Fig. 1A). After taking gabapentin, his headache was obviously alleviated. However, 6 days later, the patient suffered unbearable pain in his left-sided frontal and retro-orbital areas, with diplopia when looking to the left. Physical examination revealed slight paralysis of the left lateral rectus muscle. MRI/venography (MRI/MRV) with and without contrast demonstrated left transverse and sigDepartment of Neurology (S-BL, J-XS, S-CZ, J-SG, WJ), Hebei General Hospital, Shijiazhuang 050051, China; Department of Medical Image (X-CJ), Hebei General Hospital, Shijiazhuang 050051, China; Graduate School (S-BL), Hebei North University, Zhangjiakou 075000, China; and Graduate School (S-CZ), Hebei Medical University, Shijiazhuang 050017, China. This study was funded by Hebei clinical specialty capacity building and specialty leader project in 2018. The authors report no conflicts of interest. Address Correspondence to Wei Jin, PhD, Department of Neurology, Hebei General Hospital, Shijiazhuang, Hebei; E-mail: jinwei8626@ 126.com This is an open access article distributed under the terms of the Creative Commons Attribution-Non Commercial-No Derivatives License 4.0 (CCBY-NC-ND), where it is permissible to download and share the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal. e76 moid sinus thrombosis with partial obliteration, ipsilateral mastoiditis and abnormal enhancement in the left petrous apex and adjacent dura (Fig. 1B–H). Moreover, cerebral venous thrombosis extending from the left transverse sinus was clearly visible (Fig. 1I). Laboratory tests showed a white blood cell count of 8.56 · 109/L, with 92.4% neutrophils. The intracranial pressure was slightly increased (210 mmH2O). The cerebrospinal fluid examination yielded a normal result. Based on the above findings, the diagnosis of incomplete GS with otogenic lateral sinus thrombosis (OLST) caused by mastoiditis was made. He was treated with intravenous (IV) piperacillin sodium and tazobactam sodium 2.5 g every 8 hours and with a subcutaneous injection of low-molecule-weight heparin. After just 1 day, the diplopia had completely abated, and the headache was noticeably relieved. One week later, lumbar puncture revealed a normal intracranial pressure (155 mmH2O). Contrast-enhanced MRI showed narrowing of the thrombosis in the left transverse and sigmoid sinuses and resolution of the inflammation in the left mastoid, ipsilateral petrous apex, and adjacent dura (Fig. 1J, K). Cerebral angiography confirmed slow blood flow in the left transverse and sigmoid sinuses accompanied by local filling defects. After 2 weeks of IV antibiotics, the patient achieved full recovery and was discharged; the patient was prescribed oral amoxicillin and clavulanate potassium for another 2 weeks. Anticoagulant therapy with oral rivaroxaban was also continued in the following 3 months. Although a followup MRV examination approximately 2 months after discharge did not show recanalization of the left transverse and sigmoid sinuses (Fig. 1L), the patient remained in good health without any sign of complications over the course of the 3-month follow-up period. GS, which is a rare but life-threatening complication of otitis media (OM) or mastoiditis, consists of OM, deep facial or retro-orbital pain in the territory of the first and second branches of the trigeminal nerve, and ipsilateral abducens nerve palsy (1). The underlying cause of GS is petrous apicitis secondary to otologic infections. Because of the close anatomic relationship, the trigeminal nerve ganglion and the abducens nerve are most likely to be affected and manifest symptoms of GS. OLST is another uncommon, but high-risk complication of infectious middle ear Li et al: J Neuro-Ophthalmol 2023; 43: e76-e78 Clinical Correspondence FIG. 1. Brain CT and brain MRI/MRV. CT showed high-density areas in the left transverse and sigmoid sinuses (A). T1 MRI (B), T2 MRI (C) and fluid attenuated inversion recovery (FLAIR) (D) sequences revealed hyperintense signals in the left transverse and sigmoid sinuses. MRV demonstrated diminished perfusion of the left transverse and sigmoid sinuses (E). T1 fast-spoiled gradient recalled (FSPGR) imaging revealed abnormal enhancement of the left transverse and sigmoid sinuses, petrous apex and adjacent dura (F, G). CUBE imaging showed abnormal enhancement of ipsilateral mastoid air cells (H). T1 FSPGR imaging clearly showed cerebral venous thrombosis extending from the left transverse sinus (I). After 1 week of treatment, T1 FSPGR and CUBE imaging revealed narrowing of the thrombosis and resolution of the inflammation in the left mastoid, ipsilateral petrous apex and adjacent dura (J, K). Follow-up MRV 2 months after discharge did not show recanalization of the left transverse and sigmoid sinuses (L). disease that can occur via the direct extension of infections or hematogenous dissemination (2). In the present case, the sudden onset of slight palsy of the left abducens nerve, pain Li et al: J Neuro-Ophthalmol 2023; 43: e76-e78 in the frontal and retro-orbital areas, and imaging characteristics prompted us to consider the diagnosis of GS accompanied by OLST. Because of the lack of OM, e77 Clinical Correspondence according to the definitions proposed by McLaren et al (3), incomplete GS was a more accurate diagnosis. GS and OLST are rare complications of otologic infections. Case reports of GS accompanied by OLST are particularly scarce (4). Because the patient in the present case showed slight signs of mastoiditis on MRI, which did not seem to account for the multiple intracranial lesions, we were cautious in making the diagnosis. It is noteworthy that 1 month before admission, the patient had been treated with a 1-week course of oral antibiotics for fever, headache, and constant tinnitus in the ipsilateral ear. Despite the paucity of further proof, we speculate that the patient had suffered from acute mastoiditis. Unfortunately, inadequate antibiotic treatment did not prevent the occurrence of complications, although it alleviated the mastoid inflammatory process. In this regard, because the use of antibiotics can mask the typical imaging features of infectious middle ear diseases, we must remain vigilant to avoid delays in diagnosis. Another interesting “mismatch” in the present case is the seemingly benign process and extensive thrombosis in the lateral sinus accompanied by ipsilateral cerebral venous thrombosis, which can most likely be attributed to early antibiotic application. The early application of potent antibiotics for middle ear infections has substantially altered the patterns and progression of OLST, and patients with OLST may have no typical clinical manifestations (2). This means that there may be more cases of OLST than are clinically diagnosed, and clinicians should maintain a high level of awareness to avoid missing the diagnosis of this sometimes inconspicuous, but life-threatening complication of middle ear infection. Regarding treatment, the long-term use of broadspectrum antibiotics is uncontroversial for GS or OLST. However, because of a lack of definitive evidence, the role of anticoagulation therapy in OLST is debatable (5). Given the multiple intracranial complications, the patient in the present case was treated with anticoagulation therapy. Over the 3-month follow-up period, the patient remained symptom-free, although brain MRV did not show recanalization of the left transverse and sigmoid sinuses; the patient probably benefited from the intercommunication of intracranial veins. Data from 8 cases of OLST showed e78 that whether receiving anticoagulation therapy or not, all the patients who underwent mastoidectomy and were treated with broad-spectrum antibiotics achieved satisfactory outcomes (6). Whether anticoagulation therapy is necessary for OLST when associated with an adequately controlled middle ear infection remains to be determined; more data need to be collected before drawing a more definitive conclusion. Therefore, it is important for clinicians to weigh the benefits and risks of anticoagulation therapy to make an appropriate choice. In the antibiotic era, GS accompanied by OLST is a rare, but dangerous complication of middle ear infectious diseases. Because of its rarity and subtle presentation, in the face of any atypical symptoms, a higher degree of suspicion is needed for clinicians to make an early diagnosis and administer appropriate treatment. STATEMENT OF AUTHORSHIP Conception and design: S.-B. Li; Acquisition of data: X.-C. Jia, S.-C. Zhao; Analysis and interpretation of data: X.-C. Jia. Drafting the manuscript: S.-B. Li; Revising it for intellectual content: J.-X. Song, J.S. Gao. Final approval of the completed manuscript: W. Jin. REFERENCES 1. Matis GK, de A Silva DO, Chrysou OI, Karanikas MA, Birbilis TA. Giuseppe Gradenigo: much more than a syndrome! Historical vignette. Surg Neurol Int. 2012;3:122. 2. Funamura JL, Nguyen AT, Diaz RC. Otogenic lateral sinus thrombosis: case series and controversies. Int J Pediatr Otorhinolaryngol. 2014;78:866–870. 3. McLaren J, Cohen MS, El Saleeby CM. How well do we know Gradenigo? A comprehensive literature review and proposal for novel diagnostic categories of Gradenigo’s syndrome. Int J Pediatr Otorhinolaryngol. 2020;132:109942. 4. Özkaçmaz S. Acute otitis media associated with Gradenigo syndrome and transverse sinus thrombosis: a case report. J Int Med Res. 2019;47:1348–1352. 5. Au JK, Adam SI, Michaelides EM. Contemporary management of pediatric lateral sinus thrombosis: a twenty year review. Am J Otolaryngol. 2013;34:145–150. 6. de Oliveira Penido N, Testa JR, Inoue DP, Cruz OL. Presentation, treatment, and clinical course of otogenic lateral sinus thrombosis. Acta Otolaryngol. 2009;129:729–734. Li et al: J Neuro-Ophthalmol 2023; 43: e76-e78 |
