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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.

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]
  • 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]
  • We report a case of typical Gradenigo's syndrome, including left abducens nerve palsy, left facial pain and paresthesia in V1 and V2 distribution of trigeminal nerve caused by solitary osseous plasmacytoma of the left petrous apex.[ncbi.nlm.nih.gov]
Excitement
  • Gradenigo’s Syndrome and Otitis Media We have discussed how exciting the management of otitis media can be already (see Acute Otitis Media and AOM and Cochlear Implants ).[carolinascoreconcepts.com]
  • […] by · Published January 12, 2012 · Updated October 5, 2012 We have discussed how exciting the management of otitis media can be already (see Acute Otitis Media and AOM and Cochlear Implants ).[pedemmorsels.com]

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].

Helicobacter Pylori
  • Browse recently published Learning Learning View all learning CME Autoimmune Polyendocrine Syndromes Case 9-2018: A 55-Year-Old Man with HIV Infection and a Mass on the Right Side of the Face Helicobacter pylori Therapy for the Prevention of Metachronous[nejm.org]

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]
  • See full list of 12 symptoms of Gradenigo's syndrome Treatments for Gradenigo's syndrome Antibiotics, surgery on the infected mastoid bone is also sometimes needed more treatments...»[rightdiagnosis.com]
  • The treatment of choice is: (LQ) A Myrigotomy with penicillin B MYringotomy with grommet C On Iy anti biotics D Wait and watch Procedure for serous otitis media is: (DNB June 2010) A Trmpanoplasty B Mastoidectomy C Myringotomy D Medical treatment Grommet[gradestack.com]
  • Appropriate management requires antibiotic treatment and possible surgical intervention.[ncbi.nlm.nih.gov]
  • With changing medical technology, a review of the diagnostic and treatment options for this rare but serious condition, is timely.[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

  • Inside for each eye disorder: Etiology Classic signs Symptoms Differential Diagnosis Work-up Risk Factors Management Pharmacology Future Considerations[books.google.com]
  • 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]
  • Superior orbital fissure syndrome Etiology : sphenoid wing meningioma ; and rarely, trauma Clinical features Upper eyelid ptosis Proptosis of the eye Ophthalmoplegia Dilated and fixed pupil Loss of sensation of the upper eyelid and forehead due to damage[amboss.com]
  • The original reference is provided for historical interest, and review articles are included to show recent advances in etiology and treatment.[books.google.de]

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]
  • Epidemiology Frequency Petrous apicitis was commonly encountered before the introduction of antibiotics. It now appears quite rarely.[emedicine.medscape.com]
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]
  • Gradenigo classically described the following as the classic features of this syndrome: Discharging ear Retro orbital pain Adbucent nerve paralysis causing diplopia Causative factors: Uncontrolled mastoiditis Epidural abscess following mastoiditis Pathophysiology[drtbalu.co.in]
  • Pathophysiology Because of the extensive pneumatization and presence of rich bone marrow within the petrous apex, it is susceptible to infection or inflammation, typically in combination with mastoiditis.[emedicine.medscape.com]
  • Pathophysiology (Ref. Hari. 18 th ed., Pg- 3362) In acute Bell's palsy there is inflammation of the facial nerve with mononuclear cells, consistent with an infectious or immune cause.[gradestack.com]
  • While it is thought that airway inflammation and hypersensitivity are related to nonacid reflux, its pathophysiology can be explained by two major mechanisms.[e-ceo.org]

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 DOWNLOADS Since September 27, 2012 COinS[ecommons.aku.edu]
  • Timely management with intravenous antibiotics ( surgery) is needed to prevent intra-cranial complications. Keywords: Gradenigo’s syndrome, Earache, Retro-orbital pain. Introduction Otitis media is a common disease involving children.[jpma.org.pk]
  • Browse recently published Learning Learning View all learning CME Autoimmune Polyendocrine Syndromes Case 9-2018: A 55-Year-Old Man with HIV Infection and a Mass on the Right Side of the Face Helicobacter pylori Therapy for the Prevention of Metachronous[nejm.org]

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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