- Email: [email protected]
Middle cranial fossa facial nerve decompression before two years of age
International Journal of Pediatric Otorhinolaryngology 77 (2013) 570–572
Contents lists available at SciVerse ScienceDirect
International Journal of Pediatric Otorhinolaryngology journal homepage: www.elsevier.com/locate/ijporl
Case Report
Middle cranial fossa facial nerve decompression before two years of age Robert S. Kang a,1,*, Jay T. Rubinstein b,c,d,2 a
City of Hope National Medical Center, Division of Otolaryngology – Head & Neck Surgery, Department of Surgery, 1500 East Duarte Road, Duarte, CA 91010-3000, United States University of Washington, Department of Otolaryngology – Head & Neck Surgery, Box 357923, CHDD Building, CD176, Seattle, WA 98195-7923, United States c Virginia Merrill Bloedel Hearing Research Center, United States d Seattle Children’s Hospital, United States b
A R T I C L E I N F O
A B S T R A C T
Article history: Received 17 September 2012 Received in revised form 5 December 2012 Accepted 10 December 2012 Available online 5 January 2013
We present a case report of a 17-month old patient who underwent serial bilateral total facial nerve decompression procedures for complete bilateral facial paralysis in the setting of craniometaphyseal dysplasia via combined middle cranial fossa and transmastoid approaches. The surgical decision-making process and procedures were reviewed. The patient recovered without complications from the staged surgical procedures, and developed partial return of function of both facial nerves postoperatively, with symmetric House-Brackmann grades of II–III. Despite its technical difficulty in the setting of this particular disorder, facial nerve decompression may be appropriate in the setting of acute facial palsy in craniometaphyseal dysplasia, with the potential for return of function. Given the extremely rare nature of the disease process, variable surgical experience and the clinical condition of the patient remain our best guides for management. ß 2012 Elsevier Ireland Ltd. All rights reserved.
Keywords: Craniometaphyseal dysplasia Facial paralysis
1. Case report
2. Physical exam
Approval by the University of Washington Institutional Review Board was obtained prior to conducting this study. A 4-month-old female was diagnosed with autosomal dominant craniometaphyseal dysplasia based on ANK gene identification. The patient developed sudden-onset, complete right-sided facial paralysis with middle ear effusions four week prior to our evaluation, and was treated with bilateral tympanostomy tubes, amoxicillinclavulanate, acyclovir, high-dose prednisone, and lubricating ophthalmic ointment. One week preceding her facial paralysis she had brief symptoms of nausea, emesis and fevers that spontaneously resolved. One week prior to our evaluation, however, the patient developed new, left-sided facial paralysis, progressing gradually from the perioral to the periorbital area. The patient’s mother and sibling also had a history of craniometaphyseal dysplasia requiring middle ear surgery and craniofacial recontouring and the mother had long-standing unilateral House-Brackmann grade IV facial palsy and bilateral maximal conductive hearing loss.
Tympanic membranes contained patent tympanostomy tubes bilaterally with well-aerated middle ears. House-Brackmann grade VI facial paralysis was evident bilaterally. The remainder of the cranial nerve exam was unremarkable. Pure tone audiometry revealed moderate hearing loss rising to borderline-normal hearing bilaterally, slightly worse on the right. Computed tomography images were obtained, demonstrating diffuse skull base bony overgrowth, with severe stenosis of multiple cranial nerve foramina and the middle ears bilaterally (Fig. 1).
* Corresponding author. Tel.: +1 626 471 7100/206 697 2434; fax: +1 626 301 8855. E-mail address: [email protected] (R.S. Kang). 1 This author was affiliated with the University of Washington, Department of Otolaryngology – Head and Neck Surgery, and has since changed affiliation to the institution listed above. 2 Tel.: +1 206 221 0704; fax: +1 206 616 1828. 0165-5876/$ – see front matter ß 2012 Elsevier Ireland Ltd. All rights reserved. http://dx.doi.org/10.1016/j.ijporl.2012.12.013
3. Surgical procedure The patient’s age was near 18 months, and we elected to proceed with a left-sided total facial nerve decompression including the internal auditory canal (IAC), as this side was acute in presentation whereas the right side had been paralyzed for over a month. This was performed via a combined middle fossa and transmastoid approach. There was extensive bone growth as expected, and the bone was dense, vascular, and ivory in shade such that the labyrinthine bone was extremely difficult to distinguish from the surrounding bone. Tedious dissection was undertaken, and the superior semicircular canal was carefully blue-lined. The IAC was decompressed from the porus acousticus to the meatal foramen. The labyrinthine segment of the facial nerve was completely decompressed, exposing the geniculate and greater superficial petrosal nerve. The tympanic segment was
R.S. Kang, J.T. Rubinstein / International Journal of Pediatric Otorhinolaryngology 77 (2013) 570–572
571
4. Results
Fig. 1. Computed tomography images of the temporal bones in coronal (top) and axial (bottom) sections demonstrate diffuse skull thickening and stenosis of cranial nerve foramina including the internal auditory canals (white arrows) and fallopian canals (black arrows).
The patient showed evidence of movement in the nasolabial fold region as well as improvement in her left eye closure in the immediate postoperative period, prior to her discharge on postoperative day three. A three-week postoperative evaluation demonstrated normal inner ear function by Brainstem AuditoryEvoked Response (BAER) testing. She had recovered the ability to close her left eye, and had some movement of her left orbicularis oris muscle. Her right eye still did not close but she began to demonstrate movement of the orbicularis oris on the un-operated, right side as well. In light of this spontaneous recovery, her planned, staged right-sided facial nerve decompression procedure was canceled. By her 3-month evaluation, the patient had recovered substantial function of her right facial nerve, with more limited improvement in the operative, left side. At 8 months, her facial nerve exam was essentially symmetric. Postoperative computed tomography images were obtained (Fig. 2). Nearly 11 months postoperatively, however, she again developed sudden onset, complete, right-sided facial paralysis, and the decision was made to proceed with right-sided total facial nerve decompression via a combined middle fossa and transmastoid approach as was previously performed on her left side (Figs. 3 and 4). She was discharged on postoperative day three without
decompressed from the middle fossa to the cochleariform process. It was then decompressed through the mastoid from the cochleariform process to the stylomastoid foramen. A small ring of bone was left encasing the nerve at the level of the cochleariform process in the middle ear so as to prevent free flow of cerebrospinal fluid from the IAC into the middle ear. The facial nerve could not be stimulated with either the Prass probe or the Parsons-McCabe nerve stimulator at maximal settings (2 mA and 5 V, respectively).
Fig. 3. Intraoperative view of the right-sided middle cranial fossa approach. FN = facial nerve; SSC = superior semicircular canal.
Fig. 2. Postoperative view following combined middle cranial fossa and transmastoid approach. Top: the left temporoparietal bone flap is visualized in the 3-dimensional image. Bottom: coronal computed tomography image demonstrates the decompressed left IAC (white arrow) and the mastoidectomy (black arrow).
Fig. 4. Intraoperative view of the right-sided transmastoid approach. FR = facial recess; FN = facial nerve.
572
R.S. Kang, J.T. Rubinstein / International Journal of Pediatric Otorhinolaryngology 77 (2013) 570–572
return of function on the operative, right side. Two months following her second surgery, however, her facial movement again appeared to be symmetric with movement in all branches. Her exam was stable at 8 months, 19 months after her initial surgery, with a House-Brackmann grade of II–III bilaterally. 5. Discussion Craniometaphyseal dysplasia is a rare condition that is commonly transmitted in an autosomal dominant fashion related to a mutation in the ANK gene, but may also present sporadically and in a more severe autosomal recessive form [1,2]. When manifested in the head and neck, characteristic facial dysmorphisms may be present, such as frontal bone bossing, prognathism, nasal bridge widening, and ocular hypertelorism. Compression of cranial nerve foramina can present at an early age, causing symptoms of facial paresis, strabismus, blindness, and hearing loss [3]. Surgical treatment involves decompression of cranial structures that become encroached by the pathologic bony overgrowth of the skull and skull base. Singhal and Cochrane describe a patient with increased intracranial pressure and impaired visual acuity treated surgically with expansile cranioplasty and optic nerve sheath fenestration. The patient’s preoperative headaches and papilledema resolved by 6 months, and her seizure control and visual acuity also improved [4]. Sun et al. report an endaural canalplasty and ossiculoplasty in a patient with bilateral mixed hearing loss with fixed ossicles [5]. Conductive hearing loss has been effectively treated in similar fashion employing ossicular reconstruction prostheses and stapedotomy procedures [6,7]. Various decompressive and craniofacial remodeling procedures have been described, with varying degrees of success [8–11]. Authors have recommended careful ophthalmologic screening for evidence of visual deterioration and signs of increased intracranial pressure. It was also noted that children with progressive disease demonstrated a high rate of failure when treated with early craniofacial surgery [12]. In this case presentation, complete facial paralysis and radiologic evidence of cranial foramina stenosis led to surgical intervention that has thus far resulted in partial return of function of bilateral facial nerves. Our surgical intervention included complete bilateral facial nerve decompression from a combined middle cranial fossa and transmastoid approach. The internal auditory canals were also decompressed bilaterally to proactively
protect hearing and vestibular function as they were already exposed in decompressing the facial nerves. While cases of conservative treatment of facial palsy with such medical interventions as steroids, calcitriol, and low calcium diet have been described, in the setting of acute complete facial paralysis, the surgeon is presented with a potentially irreversible condition if spontaneous improvement is not observed in a timely fashion [13]. Recurrence of facial palsy is possible as bone re-growth is expected, and symptoms must continue to be monitored. Such intervention is extremely challenging even for a neurotologist experienced in middle fossa procedures. It remains to be seen whether long-term facial nerve function will be preserved.
Conflict of interest There was no grant or other support for this study. References [1] P. Prontera, D. Rogaia, C. Sobacchi, V.L. Tavares, G. Mazzotta, M.R. Passos-Bueno, et al., Craniometaphyseal dysplasia with severe craniofacial involvement shows homozygosity at 6q21-22.1 locus, Am. J. Med. Genet. A 155A (2011) 1106–1108. [2] Y.H. Kim, D.H. Roh, B.Y. Choi, S.H. Oh, Craniometaphyseal dysplasia, Acta Otolaryngol. 125 (2005) 797–800. [3] G. Kietzer, M.M. Paparella, Otolaryngologic disorder in craniometaphyseal dysplasia, Laryngoscope 79 (1969) 921–941. [4] A. Singhal, D.D. Cochrane, Optic nerve sheath fenestration and bilateral expansile cranioplasty for raised intracranial pressure in craniometaphyseal dysplasia: a case report, Childs Nerv. Syst. 24 (2008) 521–524. [5] G.H. Sun, R.N. Samy, B.T. Tinkle, R.S. Cornelius, D.K. Brown, Craniometaphyseal dysplasia-induced hearing loss, Otol. Neurotol. 32 (2011) e9–e10. [6] J. Shea, R. Gerbe, N. Ayani, Craniometaphyseal dysplasia: the first successful surgical treatment for associated hearing loss, Laryngoscope 91 (1981) 1369– 1374. [7] D.C. Franz, K.L. Horn, J. Aase, Craniometaphyseal dysplasia: operative findings and treatment, Am. J. Otol. 17 (1996) 283–287. [8] D.R. Millard Jr., D.O. Maisels, J.H. Batstone, B.W. Yates, Craniofacial surgery in craniometaphyseal dysplasia, Am. J. Surg. 113 (1967) 615–621. [9] N. Laurian, Y. Zohar, Correction of maxillary retrognathia in a case of craniometaphyseal dysplasia, J. Oral Surg. 38 (1980) 609–612. [10] K. Satoh, T. Iwata, H. Ikeda, Unsuccessful consequence of optic canal decompression for a case of craniometaphyseal dysplasia, Plast. Reconstr. Surg. 94 (1994) 705–708. [11] M. Feingold, 28-year follow-up of the craniofacial findings in a patient with craniometaphyseal dysplasia, Am. J. Med. Genet. 86 (1999) 501–502. [12] W.M. Sheppard, R.J. Shprintzen, S.A. Tatum, C.I. Woods, Craniometaphyseal dysplasia: a case report and review of medical and surgical management, Int. J. Pediatr. Otorhinolaryngol. 67 (2003) 687–693. [13] L.L. Key Jr., F. Volberg, R. Baron, C.S. Anast, Treatment of craniometaphyseal dysplasia with calcitriol, J. Pediatr. 112 (1988) 583–587.
Contents lists available at SciVerse ScienceDirect
International Journal of Pediatric Otorhinolaryngology journal homepage: www.elsevier.com/locate/ijporl
Case Report
Middle cranial fossa facial nerve decompression before two years of age Robert S. Kang a,1,*, Jay T. Rubinstein b,c,d,2 a
City of Hope National Medical Center, Division of Otolaryngology – Head & Neck Surgery, Department of Surgery, 1500 East Duarte Road, Duarte, CA 91010-3000, United States University of Washington, Department of Otolaryngology – Head & Neck Surgery, Box 357923, CHDD Building, CD176, Seattle, WA 98195-7923, United States c Virginia Merrill Bloedel Hearing Research Center, United States d Seattle Children’s Hospital, United States b
A R T I C L E I N F O
A B S T R A C T
Article history: Received 17 September 2012 Received in revised form 5 December 2012 Accepted 10 December 2012 Available online 5 January 2013
We present a case report of a 17-month old patient who underwent serial bilateral total facial nerve decompression procedures for complete bilateral facial paralysis in the setting of craniometaphyseal dysplasia via combined middle cranial fossa and transmastoid approaches. The surgical decision-making process and procedures were reviewed. The patient recovered without complications from the staged surgical procedures, and developed partial return of function of both facial nerves postoperatively, with symmetric House-Brackmann grades of II–III. Despite its technical difficulty in the setting of this particular disorder, facial nerve decompression may be appropriate in the setting of acute facial palsy in craniometaphyseal dysplasia, with the potential for return of function. Given the extremely rare nature of the disease process, variable surgical experience and the clinical condition of the patient remain our best guides for management. ß 2012 Elsevier Ireland Ltd. All rights reserved.
Keywords: Craniometaphyseal dysplasia Facial paralysis
1. Case report
2. Physical exam
Approval by the University of Washington Institutional Review Board was obtained prior to conducting this study. A 4-month-old female was diagnosed with autosomal dominant craniometaphyseal dysplasia based on ANK gene identification. The patient developed sudden-onset, complete right-sided facial paralysis with middle ear effusions four week prior to our evaluation, and was treated with bilateral tympanostomy tubes, amoxicillinclavulanate, acyclovir, high-dose prednisone, and lubricating ophthalmic ointment. One week preceding her facial paralysis she had brief symptoms of nausea, emesis and fevers that spontaneously resolved. One week prior to our evaluation, however, the patient developed new, left-sided facial paralysis, progressing gradually from the perioral to the periorbital area. The patient’s mother and sibling also had a history of craniometaphyseal dysplasia requiring middle ear surgery and craniofacial recontouring and the mother had long-standing unilateral House-Brackmann grade IV facial palsy and bilateral maximal conductive hearing loss.
Tympanic membranes contained patent tympanostomy tubes bilaterally with well-aerated middle ears. House-Brackmann grade VI facial paralysis was evident bilaterally. The remainder of the cranial nerve exam was unremarkable. Pure tone audiometry revealed moderate hearing loss rising to borderline-normal hearing bilaterally, slightly worse on the right. Computed tomography images were obtained, demonstrating diffuse skull base bony overgrowth, with severe stenosis of multiple cranial nerve foramina and the middle ears bilaterally (Fig. 1).
* Corresponding author. Tel.: +1 626 471 7100/206 697 2434; fax: +1 626 301 8855. E-mail address: [email protected] (R.S. Kang). 1 This author was affiliated with the University of Washington, Department of Otolaryngology – Head and Neck Surgery, and has since changed affiliation to the institution listed above. 2 Tel.: +1 206 221 0704; fax: +1 206 616 1828. 0165-5876/$ – see front matter ß 2012 Elsevier Ireland Ltd. All rights reserved. http://dx.doi.org/10.1016/j.ijporl.2012.12.013
3. Surgical procedure The patient’s age was near 18 months, and we elected to proceed with a left-sided total facial nerve decompression including the internal auditory canal (IAC), as this side was acute in presentation whereas the right side had been paralyzed for over a month. This was performed via a combined middle fossa and transmastoid approach. There was extensive bone growth as expected, and the bone was dense, vascular, and ivory in shade such that the labyrinthine bone was extremely difficult to distinguish from the surrounding bone. Tedious dissection was undertaken, and the superior semicircular canal was carefully blue-lined. The IAC was decompressed from the porus acousticus to the meatal foramen. The labyrinthine segment of the facial nerve was completely decompressed, exposing the geniculate and greater superficial petrosal nerve. The tympanic segment was
R.S. Kang, J.T. Rubinstein / International Journal of Pediatric Otorhinolaryngology 77 (2013) 570–572
571
4. Results
Fig. 1. Computed tomography images of the temporal bones in coronal (top) and axial (bottom) sections demonstrate diffuse skull thickening and stenosis of cranial nerve foramina including the internal auditory canals (white arrows) and fallopian canals (black arrows).
The patient showed evidence of movement in the nasolabial fold region as well as improvement in her left eye closure in the immediate postoperative period, prior to her discharge on postoperative day three. A three-week postoperative evaluation demonstrated normal inner ear function by Brainstem AuditoryEvoked Response (BAER) testing. She had recovered the ability to close her left eye, and had some movement of her left orbicularis oris muscle. Her right eye still did not close but she began to demonstrate movement of the orbicularis oris on the un-operated, right side as well. In light of this spontaneous recovery, her planned, staged right-sided facial nerve decompression procedure was canceled. By her 3-month evaluation, the patient had recovered substantial function of her right facial nerve, with more limited improvement in the operative, left side. At 8 months, her facial nerve exam was essentially symmetric. Postoperative computed tomography images were obtained (Fig. 2). Nearly 11 months postoperatively, however, she again developed sudden onset, complete, right-sided facial paralysis, and the decision was made to proceed with right-sided total facial nerve decompression via a combined middle fossa and transmastoid approach as was previously performed on her left side (Figs. 3 and 4). She was discharged on postoperative day three without
decompressed from the middle fossa to the cochleariform process. It was then decompressed through the mastoid from the cochleariform process to the stylomastoid foramen. A small ring of bone was left encasing the nerve at the level of the cochleariform process in the middle ear so as to prevent free flow of cerebrospinal fluid from the IAC into the middle ear. The facial nerve could not be stimulated with either the Prass probe or the Parsons-McCabe nerve stimulator at maximal settings (2 mA and 5 V, respectively).
Fig. 3. Intraoperative view of the right-sided middle cranial fossa approach. FN = facial nerve; SSC = superior semicircular canal.
Fig. 2. Postoperative view following combined middle cranial fossa and transmastoid approach. Top: the left temporoparietal bone flap is visualized in the 3-dimensional image. Bottom: coronal computed tomography image demonstrates the decompressed left IAC (white arrow) and the mastoidectomy (black arrow).
Fig. 4. Intraoperative view of the right-sided transmastoid approach. FR = facial recess; FN = facial nerve.
572
R.S. Kang, J.T. Rubinstein / International Journal of Pediatric Otorhinolaryngology 77 (2013) 570–572
return of function on the operative, right side. Two months following her second surgery, however, her facial movement again appeared to be symmetric with movement in all branches. Her exam was stable at 8 months, 19 months after her initial surgery, with a House-Brackmann grade of II–III bilaterally. 5. Discussion Craniometaphyseal dysplasia is a rare condition that is commonly transmitted in an autosomal dominant fashion related to a mutation in the ANK gene, but may also present sporadically and in a more severe autosomal recessive form [1,2]. When manifested in the head and neck, characteristic facial dysmorphisms may be present, such as frontal bone bossing, prognathism, nasal bridge widening, and ocular hypertelorism. Compression of cranial nerve foramina can present at an early age, causing symptoms of facial paresis, strabismus, blindness, and hearing loss [3]. Surgical treatment involves decompression of cranial structures that become encroached by the pathologic bony overgrowth of the skull and skull base. Singhal and Cochrane describe a patient with increased intracranial pressure and impaired visual acuity treated surgically with expansile cranioplasty and optic nerve sheath fenestration. The patient’s preoperative headaches and papilledema resolved by 6 months, and her seizure control and visual acuity also improved [4]. Sun et al. report an endaural canalplasty and ossiculoplasty in a patient with bilateral mixed hearing loss with fixed ossicles [5]. Conductive hearing loss has been effectively treated in similar fashion employing ossicular reconstruction prostheses and stapedotomy procedures [6,7]. Various decompressive and craniofacial remodeling procedures have been described, with varying degrees of success [8–11]. Authors have recommended careful ophthalmologic screening for evidence of visual deterioration and signs of increased intracranial pressure. It was also noted that children with progressive disease demonstrated a high rate of failure when treated with early craniofacial surgery [12]. In this case presentation, complete facial paralysis and radiologic evidence of cranial foramina stenosis led to surgical intervention that has thus far resulted in partial return of function of bilateral facial nerves. Our surgical intervention included complete bilateral facial nerve decompression from a combined middle cranial fossa and transmastoid approach. The internal auditory canals were also decompressed bilaterally to proactively
protect hearing and vestibular function as they were already exposed in decompressing the facial nerves. While cases of conservative treatment of facial palsy with such medical interventions as steroids, calcitriol, and low calcium diet have been described, in the setting of acute complete facial paralysis, the surgeon is presented with a potentially irreversible condition if spontaneous improvement is not observed in a timely fashion [13]. Recurrence of facial palsy is possible as bone re-growth is expected, and symptoms must continue to be monitored. Such intervention is extremely challenging even for a neurotologist experienced in middle fossa procedures. It remains to be seen whether long-term facial nerve function will be preserved.
Conflict of interest There was no grant or other support for this study. References [1] P. Prontera, D. Rogaia, C. Sobacchi, V.L. Tavares, G. Mazzotta, M.R. Passos-Bueno, et al., Craniometaphyseal dysplasia with severe craniofacial involvement shows homozygosity at 6q21-22.1 locus, Am. J. Med. Genet. A 155A (2011) 1106–1108. [2] Y.H. Kim, D.H. Roh, B.Y. Choi, S.H. Oh, Craniometaphyseal dysplasia, Acta Otolaryngol. 125 (2005) 797–800. [3] G. Kietzer, M.M. Paparella, Otolaryngologic disorder in craniometaphyseal dysplasia, Laryngoscope 79 (1969) 921–941. [4] A. Singhal, D.D. Cochrane, Optic nerve sheath fenestration and bilateral expansile cranioplasty for raised intracranial pressure in craniometaphyseal dysplasia: a case report, Childs Nerv. Syst. 24 (2008) 521–524. [5] G.H. Sun, R.N. Samy, B.T. Tinkle, R.S. Cornelius, D.K. Brown, Craniometaphyseal dysplasia-induced hearing loss, Otol. Neurotol. 32 (2011) e9–e10. [6] J. Shea, R. Gerbe, N. Ayani, Craniometaphyseal dysplasia: the first successful surgical treatment for associated hearing loss, Laryngoscope 91 (1981) 1369– 1374. [7] D.C. Franz, K.L. Horn, J. Aase, Craniometaphyseal dysplasia: operative findings and treatment, Am. J. Otol. 17 (1996) 283–287. [8] D.R. Millard Jr., D.O. Maisels, J.H. Batstone, B.W. Yates, Craniofacial surgery in craniometaphyseal dysplasia, Am. J. Surg. 113 (1967) 615–621. [9] N. Laurian, Y. Zohar, Correction of maxillary retrognathia in a case of craniometaphyseal dysplasia, J. Oral Surg. 38 (1980) 609–612. [10] K. Satoh, T. Iwata, H. Ikeda, Unsuccessful consequence of optic canal decompression for a case of craniometaphyseal dysplasia, Plast. Reconstr. Surg. 94 (1994) 705–708. [11] M. Feingold, 28-year follow-up of the craniofacial findings in a patient with craniometaphyseal dysplasia, Am. J. Med. Genet. 86 (1999) 501–502. [12] W.M. Sheppard, R.J. Shprintzen, S.A. Tatum, C.I. Woods, Craniometaphyseal dysplasia: a case report and review of medical and surgical management, Int. J. Pediatr. Otorhinolaryngol. 67 (2003) 687–693. [13] L.L. Key Jr., F. Volberg, R. Baron, C.S. Anast, Treatment of craniometaphyseal dysplasia with calcitriol, J. Pediatr. 112 (1988) 583–587.