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A fatal case of Gradenigo's syndrome in Zimbabwe and the Danish-Zimbabwean ENT collaboration
International Journal of Pediatric Otorhinolaryngology 97 (2017) 181e184
Contents lists available at ScienceDirect
International Journal of Pediatric Otorhinolaryngology journal homepage: http://www.ijporlonline.com/
Case Report
A fatal case of Gradenigo's syndrome in Zimbabwe and the DanishZimbabwean ENT collaboration P.V.F. Jensen, M.D. a, *, M.B. Avnstorp a, T. Dzongodza b, C. Chidziva b, C. von Buchwald a a Dept. of Otorhinolaryngology, Head and Neck Surgery and Audiology, Rigshospitalet, University of Copenhagen, Blegdamsvej 9, DK-2100, Copenhagen Ø, Denmark b Dept. of Otorhinolaryngology, Parirenyatwa Hospital, Mazove Street, Harare, Zimbabwe
a r t i c l e i n f o
a b s t r a c t
Article history: Received 29 January 2017 Received in revised form 7 April 2017 Accepted 10 April 2017 Available online 11 April 2017
As a part of a bilateral educational exchange program two Danish ENT residents were invited to Zimbabwe in 2015. During this exchange a 9-year-old girl was admitted due to complications to acute otitis media (AOM). She developed Gradenigo's syndrome and later on a brain abscess leading to a fatal outcome. Life threatening complications to AOM are rare in developed countries today but are still a challenge in developing countries. We put forward this case from a developing country to bring focus to the fact that a fatal outcome is the consequence if specialist treatment is not accessible. © 2017 Elsevier B.V. All rights reserved.
Keywords: Gradenigo's syndrome Acute otitis media Abducens nerve palsy Trigeminal nerve palsy Pediatric otorhinolaryngology Educational international exchange
1. Introduction Gradenigo's syndrome (GS) is a rare and potentially fatal condition defined as the clinical triad of ipsilateral acute otitis media (AOM), abducens nerve palsy and pain in the distribution of the ophthalmic and maxillary branches of the trigeminal nerve. It was first described in 1907 by the Italian otolaryngologist, Giuseppe Gradenigo [1]. GS is a complication to AOM where the infection spreads to the petrous apex in the temporal bone resulting in petrositis affecting the nearby trigeminal and abducens nerves at the level of the Gasserian Ganglion. After the introduction of penicillin in the Western world, the incidence of GS and other complications to AOM have diminished [2]. Studies conducted in developed countries reveal that 80% of children will have experienced at least one episode of AOM by the age of three years [3,4]. Furthermore, 40% will have had seven or more episodes of AOM before the age of seven [5]. WHO estimates
* Corresponding author. Dept. of Otorhinolaryngology, Head & Neck Surgery and Audiology, F2071, Rigshospitalet, University of Copenhagen, Blegdamsvej 9, DK2100, Copenhagen Ø, Denmark. E-mail address: [email protected] (P.V.F. Jensen). http://dx.doi.org/10.1016/j.ijporl.2017.04.014 0165-5876/© 2017 Elsevier B.V. All rights reserved.
that between 65 and 330 million people worldwide suffer from chronic suppurative otitis media (CSOM) [6]. AOM can lead to death, mainly through intracranial complications such as meningitis and brain abscess. A study has estimated that 21.000 people die from complications to AOM and CSOM each year with the highest incidences in the developing countries, as there is limited access to specialist healthcare and antibiotics [7]. Complications to AOM are, beside meningitis and brain abscess, mastoiditis, labyrinthitis, facial nerve paralysis, petrositis and intracranial venous sinus thrombosis [8]. Though GS in developed countries is very rare today, cases still appear in literature. Published cases are in general from the Western parts of the world, including several European countries, where neuroimaging and inter disciplinary aggressive treatment is readily available and the outcome not yet reported fatal [9]. In Zimbabwe there are three ENT departments, two situated in the capital Harare, and the third in Bulawayo. A total of eight ENT consultants and eight ENT residents serve a population of approximately 15 million people. In rural hospitals, where no ENT expertise is present, the health care staff may therefore not be confident with diagnosis and capable of treatment of complications to AOM. Combined with a long distance to the ENT departments, untreated cases of AOM may evolve with severe complications.
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In Zimbabwe there is free access to health care until the age of five years. When above this age, the patient or the family has to pay for treatment, which is often impossible due to poverty. In some limited and severe cases government funds can be provided to cover the expenses. In 2011, a collaboration between the ENT departments in Harare, Zimbabwe and Rigshospitalet, Denmark was initiated. The concept comprises bilateral exchange of ENT residents, a monthly videotele-journal club and joint research projects aiming to increase bilateral academic and practical medical experience [10]. In March 2015 two Danish ENT residents visited the ENT departments in Harare, Zimbabwe for a month. During this period a child was admitted with complications to AOM and developed GS. To our knowledge, this report represents the second case of GS from a developing country in Africa [11]. The fatal outcome in a case of a child developing complications to AOM supports the importance of establishing a collaboration between ENT departments in developing and developed countries.
Fig. 1. CT scan on day seven after admittance to Parirenyatwa Hospital, Harare. She was diagnosed with meningitis and treated with i.v. antibiotics. The coronal section displays a fluid filled right middle ear cleft and mastoid. CT scan with 0.5 mm slices.
2. Case story A 9-year-old girl was admitted to the pediatric ward at Parirenyatwa Hospital, Harare, due to a discharging right ear, headache and fever. She had a year-long history of CSOM, and was recommended a mastoidectomy three years earlier by an ENT specialist. The family could not obtain the necessary funds for the surgery, which was therefore not performed. On admission the child complained of reduced hearing on the right ear. On examination there was tenderness but no swelling of the right mastoid and the external ears were symmetrical. A hearing test could not be performed. She was meningeal with positive Kernig's sign i.e. stretching the leg with the hip flexed to 90 resulting in back pain. Human Immunodeficiency Virus, tuberculosis and malaria status screening were all negative. Vaccination status of the child was not mentioned in the patient record. Intravenous ceftriaxone 1 g twice daily was initiated. In the following days the child's state deteriorated with vomiting and an Ear, Nose and Throat consultation was performed. Otomicroscopic examination showed a pinhole perforation on the tympanic membrane and pus in the right ear canal was found. The child was diagnosed with meningitis from an otogenic focus and was booked for wide myringotomy and grommet insertion. Lumbar puncture was declined by the parents as they were not willing to have the procedure done. No evaluation or treatment for suspicion of raised intracranial pressure was done due to lack of funds. On day seven after admittance a CT scan showed a fluid filled middle ear cleft and mastoid (Fig. 1). Bone destruction with an intraosseous collection within the petrous temporal bone was seen as well as bone breeches with an intracranial extension (Fig. 2) with a resultant shallow collection overlying the anterior aspect of the right cerebellar hemisphere and middle cerebellar peduncle. At this point, the child was clinically doing better with signs of meningitis dissolving and fever was no longer present. Intravenous ceftriaxone was continued. On day 14 after admittance she was seen for a follow-up ENT evaluation. At this point she had developed abducens and facial nerve palsies and complained of facial pain and dizziness. Otalgia and ear discharge were still present. I.v. ceftriaxone was continued in combination with floxacine ear drops, twice daily. Grommet insertion was eventually performed on day 17, but the following day the patient presented spiking temperatures. A second CT scan was recommended as an abscess was suspected, but was declined due to lack of funds. The following days the child deteriorated with new episodes of vomiting and shortness of breath. On day 21 after
Fig. 2. CT scan on day seven displaying petrous temporal bone destruction confirming it was an otogenic meningitis. CT scan with 0.5 mm slices.
admittance she fell in level of consciousness, (from GCS 15/15 to GCS 7/15). Funds were raised for an urgent CT mastoid and brain, which showed a 4 5 cm large intracranial abscess in the posterior cranial fossa (Fig. 3). The child was booked on the emergency theatre list for a mastoidectomy and evacuation of the intracranial
Fig. 3. CT scan from patient on day 21 after admittance. The sagittal section demonstrates an intracranial abscess in the posterior fossa. CT scan with 0.5 mm slices.
P.V.F. Jensen et al. / International Journal of Pediatric Otorhinolaryngology 97 (2017) 181e184
abscess. Thirteen millilitres of pus was drained from the abscess. Puss was sent to microbiological examination but cancelled due to patient outcome. Due to lack of funds no microbiology had been done prior to this point. After surgery the patient was admitted in the Intensive Care Unit. On day 22 (the day after surgery) she did not gain consciousness with pupils not reacting to light. On day 23 and 25 respectively, brain dead tests were performed and the child was declared brain death. 3. Discussion
183
it is readily available with a low false-positive rate [13]. The ideal investigation is therefore a combination of CT and MRI. However, in countries such as Zimbabwe the accessibility to CT and MRI scans are impeded by the fact that the majority of the population have to pay for the scans themselves. MRI is not available in state run institutions and very expensive in private setting. This time delay before funds were raised can result in deterioration of illness leading to a life-threatening or even fatal state before diagnostic neuroimaging is possible, which our case presentation is an example of.
This case presents our second mutual Zimbabwean and Danish report [10]. To our knowledge, it is the second published case on GS from a developing country in Africa. It demonstrates some of the feared complications of AOM, which are rarely seen in the developed parts of the world today. However, most published cases are from developed countries and include patients diagnosed with GS that survived due to relevant medical and surgical treatment commenced in time [8,9,12e30]. As illustrated in this case from Zimbabwe, with limited access to specialist treatment as well as shortage of resources people still die from complications to AOM. According to a study published in 2012, it is estimated that 21.000 people die of complications to AOM and CSOM every year [7]. Many of these deaths could be prevented if diagnosis was established in due time followed by immediate and relevant medical care. Educating more doctors as well as investing in specialty training to increase the number of e.g. ENT specialists is one way to reduce mortality in diseases like AOM. Furthermore, vaccination programmes are a proven tool to control and eliminate life-threatening infectious diseases. In Zimbabwe, there is a free vaccination programme including Haemophilus Influenzae type B and since 2012 pneumococcus amongst others [31]. In 2005 to 2009 immunisation coverage fell to only 60% due to lack of staff. This let to a severe measles outbreak claiming the lives of more than 630 children. In 2010 more ressources were allocated and it is estimated that 95% of children in Zimababwe follow the vaccination programme today. This illustrates that more ressources need to be uninterruptedly channeled to vaccination programmes in the developing countries [32].
3.3. Treatment
3.1. Anatomy and pathophysiology
4. Conclusion
The petrous portion of the temporal bone is a pyramidal structure. The infectious process in GS spreads from the base (mastoid and middle ear) of the petrous bone to the apex located in the most central part of the bone. A fold of dura mater at the petrous apex forms Dorello's canal in which the abducens nerve runs towards the cavernous sinus [13]. The proximity of these structures is the reason why an abducens nerve palsy arises. In the vicinity is also the Gasserian ganglion from which the ophthalmic and maxillary branches of the trigeminal nerve arise close to the petrous apex explaining the facial pain as well as pain located to or behind the eye that also characterizes GS. The facial nerve palsy occurring in this presented case may be due to the facial nerve's relation to the middle ear.
We presented this fatal case of a 9-year-old otherwise healthy Zimbabwean girl with AOM leading to GS and intracranial complications. It is our aim that this mutual Zimbabwean and Danish case report will raise awareness of the fatal complications of AOM in the developing world as well as stimulating a fruitful bridging between developing and developed countries.
3.2. Neuroimaging CT scans are preferred to describe bone destruction, which can be seen due to infection. Magnetic Resonance Imaging (MRI) is valuable in describing Central Nervous System (CNS) pathology and meningeal enhancement and holds information on the composition of the process, which cannot be obtained by a CT scan [13,15]. A CT scan is often the primary investigation when GS is suspected, as
The literature describes a tendency towards conservative management of GS with intravenous antibiotics (10e64 days) and ventilation tube insertion instead of mastoidectomy [8,9,12e30]. A CT scan is recommended, and if the patient presents with an intracranial abscess or deteriorates during conservative treatment, immediate surgery is warranted [9,17e19,21,26]. Petrositis is equated with osteomyelitis and therefore prolonged and aggressive i.v. antibiotic treatment is warranted to avoid relapse. Close and continuous monitoring of the patient's condition is crucial and in case of deterioration acute neuro-imaging is of paramount importance [9]. 3.4. Microbiology The child received i.v. ceftriaxone and later on the treatment was supplemented with floxacine ear drops. Ceftriaxone is used to treat acute bacterial AOM which often is caused haemophilus influenzae or streptococcus pneumonia. Floxacine is effective against bacteria such as pseudomonas aeruginosa and staphylococci which are often seen in CSOM. The patient suffered from AOM but had a history with CSOM. Therefore, it would have been preferable that floxacine ear drops had been administered in combination with i.v. ceftriaxone when the child was admitted. Ideally, there should have been sent material to microbiology early so the treatment could have been changed or supplemented.
Informed consent Informed consent was obtained from the parents of the child included in this study. Conflict of interest None. Acknowledgements Dr. Peter Sand Myschetzky for his guidance in selecting the images to augment this case presentation. The exchange was supported by the non-profit Mouritzen la Fontaine Foundation.
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References [1] G. Gradenigo, Uber die Paralyse des N. Abduzens bei Otitis, Arch. F. Ohrenheilk. 74 (1907) 149e158. [2] R.A. Chole, P.J. Donald, Petrous apicitis - clinical considerations, Ann. Otol. Rhinol. Laryngol. 92 (1983) 544e551. [3] D.W. Teele, J.O. Klein, B. Rosner, Epidemiology of otitis media during the first seven years of life in children in greater Boston: a prospective, cohort study, J. Infect. Dis. 160 (1989) 83e94. [4] A. Vergison, R. Dagan, A. Arguedas, J. Bonhoeffer, R. Cohen, I. Dhooge, et al., Otitis media and its consequences: beyond the earache, Lancet Infect. Dis. 10 (2010) 195e203. [5] M.L. Casselbrant, E.M. Mandel, Epidemiology, in: R.M. Rosenfeld, C.D. Bluestone (Eds.), Evidence-based Otitis Media, BC Decker Inc, Hamilton, 2003, pp. 147e162. [6] J. Acuin, Chronic Suppurative Otitis Media - Burden of Illness and Management Options, World Health Organization, Geneva, 2004. http://www.who. int/pbd/publications/Chronicsuppurativeotitis_media.pdf. [7] L. Monasta, L. Ronfani, F. Marchetti, M. Montico, L.V. Brumatti, A. Bavcar, et al., Burden of disease caused by otitis media: systematic review and global estimates, PLoS One 7 (4) (2012). http://journals.plos.org/plosone/article?id¼10. 1371/journal.pone.0036226. [8] K. Piron, T. Gordts, R. Herzeel, Gradenigo syndrome: a case-report, Bull. Soc. Belge. Ophthalmol. 290 (2003) 43e47. [9] P.V.F. Jensen, M.S. Hansen, M.N. Møller, J.P. Saunte, The forgotten syndrome? Four cases of Gradenigo's Syndrome and a review of the literature, Strabismus 24 (1) (2016) 21e27. [10] M.B. Avnstorp, P.V.F. Jensen, T. Dzongodza, N. Matinhira, C. Chidziva, J. Melchiors, et al., The introduction of emergency cricothyroidotomy simulation training contributed to the saving of two lives, J. Laryngol. Otol. 130 (10) (2016) 923e927. [11] D.P.O. Oyatsi, Multiple cranial nerve palsies complicating tympanomastoiditis: case report, E Afr. Med. J. 79 (12) (2002) 665e666. ^, T.A.F. Rocha, M.G.F. Pereira, [12] A.R.S. Neto, M.J. Bezerra, A.R. Galvao V.F.A. Ferreira, et al., Gradenigo syndrome: case report and review of literature, Neurobiologia 72 (2009) 143e147. [13] S.A. Lutter, J.E. Kerschner, M.J. Chusid, Gradenigo syndrome a rare but serious complication of otitis media, Pediatr. Emerg. Care 21 (2005) 384e386. [14] J.L. Pedroso, C.C.H. Aquino, A. Abrahao, R.A. de Oliveira, L.F. Pinto, M.l. Bezerra, et al., Gradenigo's syndrome: beyond the classical triad of diplopia, facial pain and otorrhea, Case Rep. Neurol. 3 (2011) 45e47. [15] S. Tornabene, G.M. Vilke, Gradenigo's syndrome, J. Emerg. Med. 38 (4) (2007) 449e451. [16] K.Y. Choi, S. Park, Petrositis with bilateral abducens nerve palsies complicated
by acute otitis media, Clin. Exp. Otorhinolaryngol. 7 (2014) 59e62. [17] M. Heshin-Bekenstein, O. Megged, U. Peleg, S. Shahroor-Karni, R. Bass, M. Benifla, et al., Gradenigo's syndrome: is fusobacterium different? Two cases and review of the literature, Int. J. Pediatr. Otorhinolaryngol. 78 (2014) 166e169. [18] H.N. Humayun, S. Akhtar, S. Ahmed, Gradenigo's Syndrome e surgical management in a child, J. Pak Med. Ass. 61 (2011) 393e394. [19] C.L. Jacobsen, M.A. Bruhn, Y. Yavarian, M.L. Gaihede, Mastoiditis and Gradenigo's Syndrome with anaerobic bacteria, BMC Ear Nose Throat Disord. 14 (2012), 12:10. [20] O.E. Jegede, W. Abuhammour, R. Hasan, Stenotrophomonas maltophilia causing Gradenigo's syndrome, J. Pediatr. Infect. Dis. 5 (2010) 285e288. [21] I. Kantas, A. Papadopoulou, D. Balatsouras, A. Aspris, N. Marangos, Therapeutic approach to Gradenigo's syndrome: a case report, J. Med. Case Rep. 4 (2010) 151. [22] S.K. Kong, I.W. Lee, E.K. Goh, S.E. Park, Acute otitis media-induced petrous apicits presenting as the Gradenigo Syndrome: successfully treated by ventilation tube insertion, Am. J. Otolaryngol. 32 (2011) 445e447. [23] R. Marianowski, S. Rocton, J.L. Ait-Amer, M.P. Morisseau-Durand, Y. Manach, Conservative management of Gradenigo syndrome in a child, Int. J. Pediatr. Otorhinolaryngol. 57 (2001) 79e83. [24] Y. Plodpai, S. Hirunpat, W. Kiddee, Gradenigo's Syndrome secondary to chronic otitis media on a background of previous radical mastoidectomy: a case report, J. Med. Case Rep. 23 (2014), 8 217. [25] T.E. Rossor, Y.C. Anderson, N.B. Steventon, L.M. Voss, Conservative management of Gradenigo's syndrome in a child, BMJ Case Rep. (2011) doi:1136/ bcr.03.2011.3978. [26] M.R.G. Santiago, N.A. Almazan, Gradenigo syndrome, Philipp J. Otolaryngol. Head. Neck Surg. 23 (2) (2008) 46e48. [27] A. Scardapane, M.D. Torto, M. Nozzi, C. Elio, L. Breda, F. Chiarelli, Gradenigo's syndrome with lateral venous sinus thrombosis: successful conservative treatment, Eur. J. Pediatr. 169 (2010) 437e440. [28] B. Ulkumen, Y. Kaplan, Conservative treatment of Gradenigo's syndrome triggered by acute otitis media, Pak J. Med. Sci. 28 (2012) 735e737. [29] J.M. Valles, R. Fekete, Gradenigo syndrome: unusual consequence of otitis media, Case Rep. Neurol. 6 (2014) 197e201. [30] Y. Finkelstein, N. Marcus, R. Mosseri, Z. Bar-Sever, B.Z. Garty, Streptococcus acidominimus infection in a child causing Gradenigo's syndrome, Int. J. Pediatr. Otorhinolaryngol. 67 (2003) 815e817. [31] http://www.zimbabas.co.zw/new-vaccination-schedule-starting-20th-june2012/#.WOfrgxiqG1s (Accessed 7 April 2017). [32] https://www.unicef.org/zimbabwe/media_15272.html (Accessed 7 April 2017).
Contents lists available at ScienceDirect
International Journal of Pediatric Otorhinolaryngology journal homepage: http://www.ijporlonline.com/
Case Report
A fatal case of Gradenigo's syndrome in Zimbabwe and the DanishZimbabwean ENT collaboration P.V.F. Jensen, M.D. a, *, M.B. Avnstorp a, T. Dzongodza b, C. Chidziva b, C. von Buchwald a a Dept. of Otorhinolaryngology, Head and Neck Surgery and Audiology, Rigshospitalet, University of Copenhagen, Blegdamsvej 9, DK-2100, Copenhagen Ø, Denmark b Dept. of Otorhinolaryngology, Parirenyatwa Hospital, Mazove Street, Harare, Zimbabwe
a r t i c l e i n f o
a b s t r a c t
Article history: Received 29 January 2017 Received in revised form 7 April 2017 Accepted 10 April 2017 Available online 11 April 2017
As a part of a bilateral educational exchange program two Danish ENT residents were invited to Zimbabwe in 2015. During this exchange a 9-year-old girl was admitted due to complications to acute otitis media (AOM). She developed Gradenigo's syndrome and later on a brain abscess leading to a fatal outcome. Life threatening complications to AOM are rare in developed countries today but are still a challenge in developing countries. We put forward this case from a developing country to bring focus to the fact that a fatal outcome is the consequence if specialist treatment is not accessible. © 2017 Elsevier B.V. All rights reserved.
Keywords: Gradenigo's syndrome Acute otitis media Abducens nerve palsy Trigeminal nerve palsy Pediatric otorhinolaryngology Educational international exchange
1. Introduction Gradenigo's syndrome (GS) is a rare and potentially fatal condition defined as the clinical triad of ipsilateral acute otitis media (AOM), abducens nerve palsy and pain in the distribution of the ophthalmic and maxillary branches of the trigeminal nerve. It was first described in 1907 by the Italian otolaryngologist, Giuseppe Gradenigo [1]. GS is a complication to AOM where the infection spreads to the petrous apex in the temporal bone resulting in petrositis affecting the nearby trigeminal and abducens nerves at the level of the Gasserian Ganglion. After the introduction of penicillin in the Western world, the incidence of GS and other complications to AOM have diminished [2]. Studies conducted in developed countries reveal that 80% of children will have experienced at least one episode of AOM by the age of three years [3,4]. Furthermore, 40% will have had seven or more episodes of AOM before the age of seven [5]. WHO estimates
* Corresponding author. Dept. of Otorhinolaryngology, Head & Neck Surgery and Audiology, F2071, Rigshospitalet, University of Copenhagen, Blegdamsvej 9, DK2100, Copenhagen Ø, Denmark. E-mail address: [email protected] (P.V.F. Jensen). http://dx.doi.org/10.1016/j.ijporl.2017.04.014 0165-5876/© 2017 Elsevier B.V. All rights reserved.
that between 65 and 330 million people worldwide suffer from chronic suppurative otitis media (CSOM) [6]. AOM can lead to death, mainly through intracranial complications such as meningitis and brain abscess. A study has estimated that 21.000 people die from complications to AOM and CSOM each year with the highest incidences in the developing countries, as there is limited access to specialist healthcare and antibiotics [7]. Complications to AOM are, beside meningitis and brain abscess, mastoiditis, labyrinthitis, facial nerve paralysis, petrositis and intracranial venous sinus thrombosis [8]. Though GS in developed countries is very rare today, cases still appear in literature. Published cases are in general from the Western parts of the world, including several European countries, where neuroimaging and inter disciplinary aggressive treatment is readily available and the outcome not yet reported fatal [9]. In Zimbabwe there are three ENT departments, two situated in the capital Harare, and the third in Bulawayo. A total of eight ENT consultants and eight ENT residents serve a population of approximately 15 million people. In rural hospitals, where no ENT expertise is present, the health care staff may therefore not be confident with diagnosis and capable of treatment of complications to AOM. Combined with a long distance to the ENT departments, untreated cases of AOM may evolve with severe complications.
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P.V.F. Jensen et al. / International Journal of Pediatric Otorhinolaryngology 97 (2017) 181e184
In Zimbabwe there is free access to health care until the age of five years. When above this age, the patient or the family has to pay for treatment, which is often impossible due to poverty. In some limited and severe cases government funds can be provided to cover the expenses. In 2011, a collaboration between the ENT departments in Harare, Zimbabwe and Rigshospitalet, Denmark was initiated. The concept comprises bilateral exchange of ENT residents, a monthly videotele-journal club and joint research projects aiming to increase bilateral academic and practical medical experience [10]. In March 2015 two Danish ENT residents visited the ENT departments in Harare, Zimbabwe for a month. During this period a child was admitted with complications to AOM and developed GS. To our knowledge, this report represents the second case of GS from a developing country in Africa [11]. The fatal outcome in a case of a child developing complications to AOM supports the importance of establishing a collaboration between ENT departments in developing and developed countries.
Fig. 1. CT scan on day seven after admittance to Parirenyatwa Hospital, Harare. She was diagnosed with meningitis and treated with i.v. antibiotics. The coronal section displays a fluid filled right middle ear cleft and mastoid. CT scan with 0.5 mm slices.
2. Case story A 9-year-old girl was admitted to the pediatric ward at Parirenyatwa Hospital, Harare, due to a discharging right ear, headache and fever. She had a year-long history of CSOM, and was recommended a mastoidectomy three years earlier by an ENT specialist. The family could not obtain the necessary funds for the surgery, which was therefore not performed. On admission the child complained of reduced hearing on the right ear. On examination there was tenderness but no swelling of the right mastoid and the external ears were symmetrical. A hearing test could not be performed. She was meningeal with positive Kernig's sign i.e. stretching the leg with the hip flexed to 90 resulting in back pain. Human Immunodeficiency Virus, tuberculosis and malaria status screening were all negative. Vaccination status of the child was not mentioned in the patient record. Intravenous ceftriaxone 1 g twice daily was initiated. In the following days the child's state deteriorated with vomiting and an Ear, Nose and Throat consultation was performed. Otomicroscopic examination showed a pinhole perforation on the tympanic membrane and pus in the right ear canal was found. The child was diagnosed with meningitis from an otogenic focus and was booked for wide myringotomy and grommet insertion. Lumbar puncture was declined by the parents as they were not willing to have the procedure done. No evaluation or treatment for suspicion of raised intracranial pressure was done due to lack of funds. On day seven after admittance a CT scan showed a fluid filled middle ear cleft and mastoid (Fig. 1). Bone destruction with an intraosseous collection within the petrous temporal bone was seen as well as bone breeches with an intracranial extension (Fig. 2) with a resultant shallow collection overlying the anterior aspect of the right cerebellar hemisphere and middle cerebellar peduncle. At this point, the child was clinically doing better with signs of meningitis dissolving and fever was no longer present. Intravenous ceftriaxone was continued. On day 14 after admittance she was seen for a follow-up ENT evaluation. At this point she had developed abducens and facial nerve palsies and complained of facial pain and dizziness. Otalgia and ear discharge were still present. I.v. ceftriaxone was continued in combination with floxacine ear drops, twice daily. Grommet insertion was eventually performed on day 17, but the following day the patient presented spiking temperatures. A second CT scan was recommended as an abscess was suspected, but was declined due to lack of funds. The following days the child deteriorated with new episodes of vomiting and shortness of breath. On day 21 after
Fig. 2. CT scan on day seven displaying petrous temporal bone destruction confirming it was an otogenic meningitis. CT scan with 0.5 mm slices.
admittance she fell in level of consciousness, (from GCS 15/15 to GCS 7/15). Funds were raised for an urgent CT mastoid and brain, which showed a 4 5 cm large intracranial abscess in the posterior cranial fossa (Fig. 3). The child was booked on the emergency theatre list for a mastoidectomy and evacuation of the intracranial
Fig. 3. CT scan from patient on day 21 after admittance. The sagittal section demonstrates an intracranial abscess in the posterior fossa. CT scan with 0.5 mm slices.
P.V.F. Jensen et al. / International Journal of Pediatric Otorhinolaryngology 97 (2017) 181e184
abscess. Thirteen millilitres of pus was drained from the abscess. Puss was sent to microbiological examination but cancelled due to patient outcome. Due to lack of funds no microbiology had been done prior to this point. After surgery the patient was admitted in the Intensive Care Unit. On day 22 (the day after surgery) she did not gain consciousness with pupils not reacting to light. On day 23 and 25 respectively, brain dead tests were performed and the child was declared brain death. 3. Discussion
183
it is readily available with a low false-positive rate [13]. The ideal investigation is therefore a combination of CT and MRI. However, in countries such as Zimbabwe the accessibility to CT and MRI scans are impeded by the fact that the majority of the population have to pay for the scans themselves. MRI is not available in state run institutions and very expensive in private setting. This time delay before funds were raised can result in deterioration of illness leading to a life-threatening or even fatal state before diagnostic neuroimaging is possible, which our case presentation is an example of.
This case presents our second mutual Zimbabwean and Danish report [10]. To our knowledge, it is the second published case on GS from a developing country in Africa. It demonstrates some of the feared complications of AOM, which are rarely seen in the developed parts of the world today. However, most published cases are from developed countries and include patients diagnosed with GS that survived due to relevant medical and surgical treatment commenced in time [8,9,12e30]. As illustrated in this case from Zimbabwe, with limited access to specialist treatment as well as shortage of resources people still die from complications to AOM. According to a study published in 2012, it is estimated that 21.000 people die of complications to AOM and CSOM every year [7]. Many of these deaths could be prevented if diagnosis was established in due time followed by immediate and relevant medical care. Educating more doctors as well as investing in specialty training to increase the number of e.g. ENT specialists is one way to reduce mortality in diseases like AOM. Furthermore, vaccination programmes are a proven tool to control and eliminate life-threatening infectious diseases. In Zimbabwe, there is a free vaccination programme including Haemophilus Influenzae type B and since 2012 pneumococcus amongst others [31]. In 2005 to 2009 immunisation coverage fell to only 60% due to lack of staff. This let to a severe measles outbreak claiming the lives of more than 630 children. In 2010 more ressources were allocated and it is estimated that 95% of children in Zimababwe follow the vaccination programme today. This illustrates that more ressources need to be uninterruptedly channeled to vaccination programmes in the developing countries [32].
3.3. Treatment
3.1. Anatomy and pathophysiology
4. Conclusion
The petrous portion of the temporal bone is a pyramidal structure. The infectious process in GS spreads from the base (mastoid and middle ear) of the petrous bone to the apex located in the most central part of the bone. A fold of dura mater at the petrous apex forms Dorello's canal in which the abducens nerve runs towards the cavernous sinus [13]. The proximity of these structures is the reason why an abducens nerve palsy arises. In the vicinity is also the Gasserian ganglion from which the ophthalmic and maxillary branches of the trigeminal nerve arise close to the petrous apex explaining the facial pain as well as pain located to or behind the eye that also characterizes GS. The facial nerve palsy occurring in this presented case may be due to the facial nerve's relation to the middle ear.
We presented this fatal case of a 9-year-old otherwise healthy Zimbabwean girl with AOM leading to GS and intracranial complications. It is our aim that this mutual Zimbabwean and Danish case report will raise awareness of the fatal complications of AOM in the developing world as well as stimulating a fruitful bridging between developing and developed countries.
3.2. Neuroimaging CT scans are preferred to describe bone destruction, which can be seen due to infection. Magnetic Resonance Imaging (MRI) is valuable in describing Central Nervous System (CNS) pathology and meningeal enhancement and holds information on the composition of the process, which cannot be obtained by a CT scan [13,15]. A CT scan is often the primary investigation when GS is suspected, as
The literature describes a tendency towards conservative management of GS with intravenous antibiotics (10e64 days) and ventilation tube insertion instead of mastoidectomy [8,9,12e30]. A CT scan is recommended, and if the patient presents with an intracranial abscess or deteriorates during conservative treatment, immediate surgery is warranted [9,17e19,21,26]. Petrositis is equated with osteomyelitis and therefore prolonged and aggressive i.v. antibiotic treatment is warranted to avoid relapse. Close and continuous monitoring of the patient's condition is crucial and in case of deterioration acute neuro-imaging is of paramount importance [9]. 3.4. Microbiology The child received i.v. ceftriaxone and later on the treatment was supplemented with floxacine ear drops. Ceftriaxone is used to treat acute bacterial AOM which often is caused haemophilus influenzae or streptococcus pneumonia. Floxacine is effective against bacteria such as pseudomonas aeruginosa and staphylococci which are often seen in CSOM. The patient suffered from AOM but had a history with CSOM. Therefore, it would have been preferable that floxacine ear drops had been administered in combination with i.v. ceftriaxone when the child was admitted. Ideally, there should have been sent material to microbiology early so the treatment could have been changed or supplemented.
Informed consent Informed consent was obtained from the parents of the child included in this study. Conflict of interest None. Acknowledgements Dr. Peter Sand Myschetzky for his guidance in selecting the images to augment this case presentation. The exchange was supported by the non-profit Mouritzen la Fontaine Foundation.
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References [1] G. Gradenigo, Uber die Paralyse des N. Abduzens bei Otitis, Arch. F. Ohrenheilk. 74 (1907) 149e158. [2] R.A. Chole, P.J. Donald, Petrous apicitis - clinical considerations, Ann. Otol. Rhinol. Laryngol. 92 (1983) 544e551. [3] D.W. Teele, J.O. Klein, B. Rosner, Epidemiology of otitis media during the first seven years of life in children in greater Boston: a prospective, cohort study, J. Infect. Dis. 160 (1989) 83e94. [4] A. Vergison, R. Dagan, A. Arguedas, J. Bonhoeffer, R. Cohen, I. Dhooge, et al., Otitis media and its consequences: beyond the earache, Lancet Infect. Dis. 10 (2010) 195e203. [5] M.L. Casselbrant, E.M. Mandel, Epidemiology, in: R.M. Rosenfeld, C.D. Bluestone (Eds.), Evidence-based Otitis Media, BC Decker Inc, Hamilton, 2003, pp. 147e162. [6] J. Acuin, Chronic Suppurative Otitis Media - Burden of Illness and Management Options, World Health Organization, Geneva, 2004. http://www.who. int/pbd/publications/Chronicsuppurativeotitis_media.pdf. [7] L. Monasta, L. Ronfani, F. Marchetti, M. Montico, L.V. Brumatti, A. Bavcar, et al., Burden of disease caused by otitis media: systematic review and global estimates, PLoS One 7 (4) (2012). http://journals.plos.org/plosone/article?id¼10. 1371/journal.pone.0036226. [8] K. Piron, T. Gordts, R. Herzeel, Gradenigo syndrome: a case-report, Bull. Soc. Belge. Ophthalmol. 290 (2003) 43e47. [9] P.V.F. Jensen, M.S. Hansen, M.N. Møller, J.P. Saunte, The forgotten syndrome? Four cases of Gradenigo's Syndrome and a review of the literature, Strabismus 24 (1) (2016) 21e27. [10] M.B. Avnstorp, P.V.F. Jensen, T. Dzongodza, N. Matinhira, C. Chidziva, J. Melchiors, et al., The introduction of emergency cricothyroidotomy simulation training contributed to the saving of two lives, J. Laryngol. Otol. 130 (10) (2016) 923e927. [11] D.P.O. Oyatsi, Multiple cranial nerve palsies complicating tympanomastoiditis: case report, E Afr. Med. J. 79 (12) (2002) 665e666. ^, T.A.F. Rocha, M.G.F. Pereira, [12] A.R.S. Neto, M.J. Bezerra, A.R. Galvao V.F.A. Ferreira, et al., Gradenigo syndrome: case report and review of literature, Neurobiologia 72 (2009) 143e147. [13] S.A. Lutter, J.E. Kerschner, M.J. Chusid, Gradenigo syndrome a rare but serious complication of otitis media, Pediatr. Emerg. Care 21 (2005) 384e386. [14] J.L. Pedroso, C.C.H. Aquino, A. Abrahao, R.A. de Oliveira, L.F. Pinto, M.l. Bezerra, et al., Gradenigo's syndrome: beyond the classical triad of diplopia, facial pain and otorrhea, Case Rep. Neurol. 3 (2011) 45e47. [15] S. Tornabene, G.M. Vilke, Gradenigo's syndrome, J. Emerg. Med. 38 (4) (2007) 449e451. [16] K.Y. Choi, S. Park, Petrositis with bilateral abducens nerve palsies complicated
by acute otitis media, Clin. Exp. Otorhinolaryngol. 7 (2014) 59e62. [17] M. Heshin-Bekenstein, O. Megged, U. Peleg, S. Shahroor-Karni, R. Bass, M. Benifla, et al., Gradenigo's syndrome: is fusobacterium different? Two cases and review of the literature, Int. J. Pediatr. Otorhinolaryngol. 78 (2014) 166e169. [18] H.N. Humayun, S. Akhtar, S. Ahmed, Gradenigo's Syndrome e surgical management in a child, J. Pak Med. Ass. 61 (2011) 393e394. [19] C.L. Jacobsen, M.A. Bruhn, Y. Yavarian, M.L. Gaihede, Mastoiditis and Gradenigo's Syndrome with anaerobic bacteria, BMC Ear Nose Throat Disord. 14 (2012), 12:10. [20] O.E. Jegede, W. Abuhammour, R. Hasan, Stenotrophomonas maltophilia causing Gradenigo's syndrome, J. Pediatr. Infect. Dis. 5 (2010) 285e288. [21] I. Kantas, A. Papadopoulou, D. Balatsouras, A. Aspris, N. Marangos, Therapeutic approach to Gradenigo's syndrome: a case report, J. Med. Case Rep. 4 (2010) 151. [22] S.K. Kong, I.W. Lee, E.K. Goh, S.E. Park, Acute otitis media-induced petrous apicits presenting as the Gradenigo Syndrome: successfully treated by ventilation tube insertion, Am. J. Otolaryngol. 32 (2011) 445e447. [23] R. Marianowski, S. Rocton, J.L. Ait-Amer, M.P. Morisseau-Durand, Y. Manach, Conservative management of Gradenigo syndrome in a child, Int. J. Pediatr. Otorhinolaryngol. 57 (2001) 79e83. [24] Y. Plodpai, S. Hirunpat, W. Kiddee, Gradenigo's Syndrome secondary to chronic otitis media on a background of previous radical mastoidectomy: a case report, J. Med. Case Rep. 23 (2014), 8 217. [25] T.E. Rossor, Y.C. Anderson, N.B. Steventon, L.M. Voss, Conservative management of Gradenigo's syndrome in a child, BMJ Case Rep. (2011) doi:1136/ bcr.03.2011.3978. [26] M.R.G. Santiago, N.A. Almazan, Gradenigo syndrome, Philipp J. Otolaryngol. Head. Neck Surg. 23 (2) (2008) 46e48. [27] A. Scardapane, M.D. Torto, M. Nozzi, C. Elio, L. Breda, F. Chiarelli, Gradenigo's syndrome with lateral venous sinus thrombosis: successful conservative treatment, Eur. J. Pediatr. 169 (2010) 437e440. [28] B. Ulkumen, Y. Kaplan, Conservative treatment of Gradenigo's syndrome triggered by acute otitis media, Pak J. Med. Sci. 28 (2012) 735e737. [29] J.M. Valles, R. Fekete, Gradenigo syndrome: unusual consequence of otitis media, Case Rep. Neurol. 6 (2014) 197e201. [30] Y. Finkelstein, N. Marcus, R. Mosseri, Z. Bar-Sever, B.Z. Garty, Streptococcus acidominimus infection in a child causing Gradenigo's syndrome, Int. J. Pediatr. Otorhinolaryngol. 67 (2003) 815e817. [31] http://www.zimbabas.co.zw/new-vaccination-schedule-starting-20th-june2012/#.WOfrgxiqG1s (Accessed 7 April 2017). [32] https://www.unicef.org/zimbabwe/media_15272.html (Accessed 7 April 2017).