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Gradenigo's Syndrome

Gradenigo’s syndrome is a complication of an otitis media infection and mastoiditis, involving three distinct manifestations: suppurative otitis media infection, facial pain compatible with the distribution of the trigeminal nerve and abducens nerve palsy.


Presentation

Gradenigo's syndrome, otherwise referred to as Gradenigo-Lannois syndrome and petrous apicitis, primarily arises as a complication following a case of otitis media infection and inflammation of the mastoid air cells of the temporal bone, that has extended to the petrous temporal bone [1] [2]. Its typical presentation involves pain that follows the distribution of the trigeminal nerve (5th cranial nerve), alongside palsy of the abducens nerve (6th cranial nerve) and otorrhea, the latter resulting from an otitis media infection [3]. Even though the aforementioned triad is considered to be the classical presentation of Gradenigo's syndrome, clinical characteristics of the infection are often diverse; photophobia, fever, excessive secretion of tears and a diminished corneal sensitivity may further complicate the clinical picture. Patients may also present with persistent headaches and some studies even report symptomatology consistent with facial and vestibulocochlear nerve pathology [4]. If left misdiagnosed or untreated, Gradenigo's syndrome can lead to serious neurological complications and death [5].

Given that Gradenigo's syndrome occurs as a complication of an original otitis media infection that has extended to involve the apex of the petrous temporal bone, the syndrome is currently considered a rare medical entity, since antibiotics started to be used in daily clinical practice.

Chronic Infection
  • In adults, it is more often due to chronic infection. Gradenigo's syndrome is acute otomastoiditis, trigeminal (CN 5) nerve neuritis, and Abducens (CN 6) palsy (CN 6 controls the lateral rectus muscle).[neuroradiologyonthenet.blogspot.com]
Pathologist
  • 他の表記法 使用例(英語) 使用例(日本語) 備考 Aarskog アースコグ Aarskog's syndrome アースコグ症候群 DagfinnAarskog (1928- ) Norwegianpediatrician and human geneticist Adamkiewicz アダムキーヴィッツ Adamkiewicz' artery アダムキーヴィッツ動脈 AlbertWojciech Adamkiewicz (1850-1921) Polish physician and pathologist[jams.med.or.jp]
Abdominal Mass
  • We report a case of a non-Hodgkin's lymphoma in a young woman presenting with an abdominal mass and an unusual instance of cranial nerve palsies mimicking Gradenigo's syndrome.[ncbi.nlm.nih.gov]
  • mass and an unusual instance of cranial nerve palsies mimicking Gradenigo’s syndrome.[doi.org]
Abdominal Pain
  • Haug Browse recently published Learning/CME Learning/CME View all learning/CME CME Partial Oral versus Intravenous Antibiotic Treatment of Endocarditis Case 4-2019: An 18-Year-Old Man with Abdominal Pain and Hematochezia Bridging the Gap Challenge Yourself[nejm.org]
Lacrimation
  • […] the syndrome include: retroorbital pain due to pain in the area supplied by the ophthalmic branch of the trigeminal nerve (fifth cranial nerve), abducens nerve palsy (sixth cranial nerve) otitis mediaOther symptoms can include photophobia, excessive lacrimation[en.wikipedia.org]
  • Other principal symptoms are photphobia, excessive lacrimation, fever, and reduced corneal sensitivity. The Gradenigo triad consists of otitis, abducens paralysis, and deep pain.[whonamedit.com]
  • Other symptoms can include photophobia, excessive lacrimation, fever, and reduced corneal sensitivity. The syndrome is usually caused by the spread of an infection into the petrous apex of the temporal bone.[ipfs.io]
  • […] supplied by the ophthalmic branch of the trigeminal nerve (fifth cranial nerve), Diplopia - ipsilateral paralysis of the abducens nerve (sixth cranial nerve), and Persistent Ear Discharge - otitis media Other symptoms can include photophobia, excessive lacrimation[wikidoc.org]
Facial Pain
  • A 60-year-old woman presented to the emergency department with 7 days of right-sided headache, facial pain, and diplopia. She awoke with the headache and facial pain 7 days earlier.[ncbi.nlm.nih.gov]
  • BACKGROUND Gradenigo's syndrome includes the triad of suppurative otitis media, ipsilateral sixth (abducens) cranial nerve palsy and facial pain in the distribution of the fifth (trigeminal) cranial nerve.[ncbi.nlm.nih.gov]
  • This condition is characterized by a triad of otorrhea, facial pain and diplopia, related to otitis media in the pre-antibiotic era.[ncbi.nlm.nih.gov]
  • Gradenigo's syndrome is a rare but life threatening complication of acute otitis media (AOM), which includes a classic triad of otitis media, deep facial pain and ipsilateral abducens nerve paralysis.[ncbi.nlm.nih.gov]
  • We report a case of an 8-year-old that developed Gradenigo's syndrome, a condition characterized by the triad of otitis media, facial pain in the distribution of the trigeminal nerve, and abducens nerve palsy (Yeung and Lustig, 2016; Janjua et al., 2016[ncbi.nlm.nih.gov]

Workup

The first step towards a precise diagnosis of Gradenigo's syndrome is a complete medical history, involving symptomatology and duration thereof and a confirmation of a recent or chronic otitis media infection. Manifestations that include an abducens nerve palsy, pain along the distribution of the trigeminal nerve and an otitis media infection that also involves the discharge of pus greatly direct suspicion towards Gradenigo's syndrome.

Imaging modalities that are used to diagnose the syndrome encompass a computerized tomography scan (CT scan) and a magnetic resonance imaging scan (MRI scan) of the temporal bone. The former usually illustrates hypodensity in the region of the petrous apex; the latter is expected to display hyperintensity on T2-weighted images and hypointensity on T1-weighted images. The infected region is enhanced in both the CT and the MRI scan. An MRI scan can also exhibit the pathology that underlies Gradenigo's syndrome and can range between cholesteatomas, malignant tumors, inflammatory granulomas and osteomyelitis [6] [7]. A single study also implemented diffusion-weighted MRI to aid in the depiction of abscesses and cholesteatomas in regions where diffusion is limited [6].

The differential diagnosis of Gradenigo's syndrome also entails various other pathologies that can assume the same symptomatology. Nasopharyngeal carcinoma and lymphoma of the petrous bone are two conditions whose symptomatology can greatly resemble that of Gradenigo's syndrome. Attention should be brought to the possibility of afebrile, non-otological manifestations after infectious petrositis, which could be misdiagnosed as a malignancy, with the consequence of antimicrobial treatment postponement influencing the prognosis [8].

Turbid Cerebrospinal Fluid
  • Turbid cerebrospinal fluid could be drained. Follow-up could not be done as the patient refused treatment and was discharged against medical advice.[ncbi.nlm.nih.gov]

Treatment

  • Although there is little evidence to support the use of medical therapy in the treatment of Gradenigo's syndrome resulting from chronic ear disease, we here demonstrate successful conservative treatment of Gradenigo's syndrome following chronic otitis[ncbi.nlm.nih.gov]
  • This type of presentation was common prior to development of antibiotic treatments, and is now a rare complication.[en.wikipedia.org]
  • Appropriate management requires antibiotic treatment and possible surgical intervention.[ncbi.nlm.nih.gov]
  • With sensitive diagnostic acumen this rare cause of Gradenigo's like syndrome was identified and correct treatment initiated.[ncbi.nlm.nih.gov]
  • With the invasion of the disease, the symptoms are manifested in different ways so that it may be confusion and delay in diagnosis and treatment in this situation.[ncbi.nlm.nih.gov]

Prognosis

  • Attention should be brought to the possibility of afebrile, non-otological manifestations after infectious petrositis, which could be misdiagnosed as a malignancy, with the consequence of antimicrobial treatment postponement influencing the prognosis.[symptoma.com]
  • Improved prognosis with intensive treatment of children with cranial soft tissue sarcomas arising in nonorbital parameningeal sites. A report from the Intergroup Rhabdomyosarcoma Study. Cancer. 1987, 59:1846-1902. 12.[arquivosdeorl.org.br]
  • Prognosis Formerly, the prognosis was very poor with death a common occurrence.[patient.info]

Etiology

  • The majority of published cases involve children, and the most common etiology is otitis media. We report a case of a diabetic man who presented with repeating Gradenigo's syndrome symptoms due to NPC.[ncbi.nlm.nih.gov]
  • The last case was a Gradenigo-Lannois syndrome, and its etiology, either chronic otitis or sphenoidal sinusitis, is discussed.[ncbi.nlm.nih.gov]
  • […] to the trigeminal nerve References: [1] [2] Foster-Kennedy syndrome Orbital apex syndrome Cavernous sinus syndrome Etiology Cavernous sinus thrombosis (e.g., due to sinusitis ) Carotid-cavernous fistula Cavernous sinus tumors Carotid artery aneurysms[amboss.com]
  • The etiology of the sixth nerve palsy is understood anatomically, given the course of the sixth nerve as it passes the petroclinoid ligament. As such, the Otolaryngology Service was consulted.[reviewofophthalmology.com]
  • ) suggestive of cholesterol granuloma. 1 , 2 Gradenigo syndrome is traditionally characterized by the triad of otorrhea, diplopia, and pain in the trigeminal territory due to suppurative otitis media, but it may be associated with other less common etiologies[neurology.org]

Epidemiology

  • An awareness of its existence and appropriate levels of suspicion of the condition are necessary to prevent severe damage or death in those affected. [ 2, 3 ] Epidemiology There are no reliable recent figures.[patient.info]
  • 壊死性胆嚢炎 necrotizing cholecystitis 壊死性腸炎 necrotizing enteritis(enterocolitis) 壊死性リンパ節炎 necrotizing lymphadenitis 壊疽 gangrene ガス壊疽 gas gangrene 糖尿病性壊疽 diabetic gangrene フルニエール壊疽 Fournier's gangrene 壊疽性口内炎 gangrenous stomatitis 衛星現象 satellite phenomenon 疫学 epidemiology[docsplayer.net]
Sex distribution
Age distribution

Pathophysiology

  • The pathophysiology, therapy and differential diagnosis of this syndrome, which has become rare since the introduction of antibiotics, are discussed.[ncbi.nlm.nih.gov]
  • The potential role of hypocortisolism in the pathophysiology of stress-related bodily disorders. Psychoneuroendocrinology, 25(1), 1-35. ‎ Pagina 52 - In: Wall PD, Melzack R (eds), Textbook of pain. Churchill Livingstone, Edinburgh... ‎[books.google.it]

Prevention

  • There is little evidence to support increased use of antibiotics in acute otitis media to prevent this complication.[ncbi.nlm.nih.gov]
  • Timely management with intravenous antibiotics ( surgery) is needed to prevent intra-cranial complications.[ncbi.nlm.nih.gov]
  • Timely management with intravenous antibiotics ( surgery) is needed to prevent intra-cranial complications. Publication Journal of the Pakistan Medical Association[ecommons.aku.edu]
  • Early recognition of condition is important to prevent intracranial complications like Meningitis, Intracranial abscess, Spread to skull base and involvement of IX, X, XI cranial nerves (Vernet’s syndrome), Prevertebral/parapharyngeal abscess, Spread[neuroradiologycases.com]
  • […] antibiotics in cases of acute otitis media ( where they are felt to be indicated) may help to prevent chronic presentations due to partially treated mastoiditis.[patient.info]

References

Article

  1. Devic M, Boucher M, Raveau M. Some cases of Gradenigo-Lannois syndrome. Journal de médecine de Lyon. 1996;47 (96):537–547.
  2. Bléry M, Chagnon S, Picard A, Babin C. Cranial osteitis: a report on four cases, including a Gradenigo-Lannois syndrome (author's transl). Journal de radiologie. 1980;61(11):677–681.
  3. Motamed M, Kalan A. Gradenigo's syndrome. Postgraduate medical journal.2000;76(899):559-560.
  4. Sherman SC, Buchanan A. Gradenigo syndrome: a case report and review of a rare complication of otitis media. J Emerg Med. 2004;27:253–256.
  5. Valles JM, Fekete R. Gradenigo Syndrome: Unusual Consequence of Otitis Media. Case Rep Neurol. 2014;6(2):197–201.
  6. Ibrahim M, Shah G, Parmar H. Diffusion-weighted MRI identifies petrous apex abscess in Gradenigo syndrome. J Neuroophthalmol. 2010;30:34–36.
  7. Pedroso JL, de Aquino CC, Abrahão A, et al. Gradenigo's Syndrome: Beyond the Classical Triad of Diplopia, Facial Pain and Otorrhea. Case Rep Neurol. 2011;3:45–47.
  8. Yoong HS, Kiaang TK. Gradenigo's syndrome presenting as a tumor. Otolaryngol Head Neck Surg. 2006;135:821–822

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Last updated: 2019-07-11 21:13