THE WESTERN JOURNAL OF MEDICINE
THE
WESTERN
JOURNAL
OF
MEDICINE
9 *
JUNE 1991
JUNE
1991
9 *
154
154
* *
6
Previously established barriers to complete resection, such as anterior cranial floor invasion, extension to the orbital apex, sphenoid sinus involvement, cavernous sinus and internal carotid artery involvement, and intracranial extension, can all be handled in carefully selected patients. For lesions involving the anterior cranial fossa, a transfacial incision with an approach through the maxillary and ethmoid sinuses is combined with a low anterior craniotomy. Total tumor excision can be accomplished even when dura and brain are involved. On occasion, an approach to the middle fossa through a subcranial route can achieve exposure of the cavernous sinus, eustachian tube, and internal carotid artery.
Middle fossa involvement is more difficult to manage and usually requires an infratemporal fossa and middle cranial fossa combined approach. Temporal lobe involvement and invasion of Meckel's cave, the cavernous sinus, and the petrous and cavernous carotid artery can all be safely managed through this approach. The postoperative course may be stormy, and the availability of a dedicated or a neurosurgical intensive care unit with specially trained nurses is invaluable. Three- to five-year tumor-free survival figures are being compiled by many centers doing skull base surgical procedures. Results are encouraging, especially in light of the hopeless prognosis otherwise facing these patients. PAUL DONALD. MD Sacramento, California
REFERENCES
Al-Mefty 0: Surgery of the Cranial Base. Boston, Mass, Kluwer, 1987 Donald P: Cranial facial surgery for head and neck cancer, chap 18, In Johnson JT (Ed): American Academy of Otolaryngology: Head and Neck Surgery-Vol 2, Instruction Course. St Louis, Mo, Mosby, 1988 Sekhar L, Schramm VL: Tumors of the Cranial Base: Diagnosis and Treatment. Mt Kisco, NY, Futura, 1987
Functional Endoscopic Sinus Surgery in Children FUNCTIONAL ENDOSCOPIC SINUS SURGERY is an operative approach to the paranasal sinuses in which the objective is to reestablish adequate sinus ventilation and mucociliary clearance in patients with chronic sinus disease. Narrow mucosallined channels exist in the middle meatus where there is a high potential for mucosal layers to contact each other. The various ostia of the anterior ethmoid, frontal, and maxillary sinuses drain in proximity to this area. Because of mucosal hyperplasia and inflammation following an infection or allergy, these channels (and subsequently the ostia) may be blocked with resultant sinus obstruction. The effect on the sinuses may be either reversible (acute sinusitis) or irreversible (chronic sinusitis), depending on the source and time course of the original insult to the middle meatus-anterior ethmoid complex. This middle meatus-anterior ethmoid complex has been called the ostiomeatal unit or complex. In the United States, endoscopic sinus surgical techniques have replaced open techniques in many instances. Many children treated with endoscopic sinus operations have considerable atopy, allergy, asthma, and primary and secondary immunodeficiencies. Other clinical groups include children with cystic fibrosis and immotile cilia or Kartagener's syndrome. Children with chronic sinus disease,
as
717
717
6
confirmed by the
history, physical examination, and repeated plain sinus films, may be treated with an endoscopic sinus operation. An ade-
quate allergic and immunologic workup should be done before referral to an otolaryngologist-head and neck surgeon. It should be emphasized that an adequate trial of medical therapy is always completed before any consideration of operative intervention. The initial diagnostic evaluation always includes nasal endoscopy under topical anesthesia. This may be difficult, if not impossible, depending on the age and cooperation of the child. The middle meatus is carefully examined for evidence of chronic infection. Depending on the history and extent of the disease, medical therapy is often continued or altered and the patient reevaluated at a later date. Children who continue to have persistent disease after adequate medical therapy undergo direct coronal computed tomographic evaluation of the ostiomeatal complex and paranasal sinuses. If the computed tomographic scan shows mucosal disease involving the ostiomeatal complex and ethmoid and maxillary sinuses, an endoscopic sinus operation may be considered. In children, the procedure requires a general anesthetic and is done on an outpatient basis under endoscopic visualization. The key to the operation is opening the ostiomeatal complex, but it may include a complete ethmoidectomy ifthe disease is extensive. Middle meatal antrostomies are created to open the natural ostia of the maxillary sinuses. Our experience thus far has been with 38 children who have undergone an endoscopic sinus operation. Of this number, most have been children with chronic sinus disease who are otherwise normal. As of this writing, an overall success rate of about 85% has been obtained in this group in terms of resolving symptoms of chronic sinus disease. Children with chronic sinus disease and concomitant hematologic disorders who are also receiving chemotherapeutic agents have responded similarly. Children who are atopic, with significant allergy and asthma, have responded well initially, including notable abatement of their asthma. Approximately half have had a relapse oftheir sinus and pulmonary symptoms six months to a year following the first procedure. Further follow-up and experience are necessary. DENNIS M. CROCKETT, MD Los Angeles, California
REFERENCES Gross CW, Gurucharri MJ, Lazar RH, Tong TE: Functional endonasal sinus surgery in the pediatric age group. Laryngoscope 1989; 99:272-275 Kennedy DW, Zinreich SJ: The functional approach to inflammatory sinus disease: Current perspectives and technique modifications. Am J Rhinol 1988; 2:89-96 Kennedy DW, Zinreich SJ, Rosenbaum AE, Johns ME: Functional endoscopic sinus surgery: Theory and diagnostic evaluation. Arch Otolaryngol 1985; 1 1 1:576-582 Messerklinger W: Endoscopy of the Nose. Munich, Gennany, Urban and Schwartzberg, 1978
Cochlear Implants in Children THE COCHLEAR IMPLANT is a medical device, part of which is placed surgically, that uses electrical stimulation to provide hearing. In recent years, the device has become an accepted method for providing auditory stimulation to the profoundly deaf. Two devices, the 3M/House single-channel cochlear implant and the Nucleus multichannel cochlear implant, have been formally approved by the United States Food and Drug Administration (FDA) for commercial marketing to deaf adults. More than 600 children ages 2 through 17 years have been implanted with either a single- or a multielectrode device. In September 1988, the FDXs Ear, Nose, and Throat Advisory Panel voted to recommend marketing approval of
EPITOMES-OTOLARYNGOLOGY/HEAD AND NECK SURGERY
718
the 3M/House implant for children, mirroring the widespread professional acceptance of such devices in children. The 3M device is no longer available, but the Nucleus multichannel cochlear implant received FDA marketing approval for use in children in June 1990. Extensive auditory, speech, educational, and psychological testing is done before and after implantation for children. Results show that cochlear implants provide auditory detection over much of the speech signal. Compared with before implantation, there is considerable improvement in auditory discrimination and speech production skills. Limited openset word and sentence recognition is possible for at least some children, perhaps as many as 30%. Complications with the device have been minimal but include inappropriate placement of the electrode array, postauricular flap infection, facial paresis, transient dizziness or pain, and partial device extrusion. About 1% of patients in the Nucleus clinical trials had unresolved complications. Selection criteria include bilateral profound sensorineural deafness, a lack of substantial benefit from hearing aids, psychological suitability, and medical suitability. The primary criterion for selection is confirmation of profound sensorineural hearing loss, usually with an average unaided threshold for the speech frequencies of poorer than 1 10 dB or an aided speech detection threshold of poorer than about 53 dB. Patients who are candidates show poorer performance on selected auditory discrimination tasks while using appropriately fit hearing aids than those using an implant. Families and, if possible, candidates should be well motivated and possess appropriate expectations. Medical contraindications include deafness caused by lesions of the acoustic nerve or central auditory pathway, active middle ear infections, cochlear ossification that prevents electrode insertion, an absence of cochlear development, and tympanic membrane perforation. The cochlear implant can provide sound to deaf adults and children unable to benefit from hearing aids. The complex assessment, rehabilitation, and counseling should be done by centers with the multidisciplinary staff necessary to provide effective care for patients with this special auditory prosthesis.
F. HOUSE, MD Newport Beach, Californica WILLIAM
REFERENCES Berliner KI, Luxford WM, House WF, Tonokawa LL: Cochlear implants in children, In Myers EN, Bluestone CD, Brackmann DE, Krause CJ (Eds): Advances in Otolaryngology: Head and Neck Surgery, Vol 4. St Louis, Mo, Mosby Year Book, 1990, pp 61-79 House WF, Berliner KI, Luxford WM: Cochlear implants in deaf children. Curr Probl Pediatr 1987; 17:347-388 National Institutes of Health: Cochlear Implants. Consensus Development Conference Statement 1988; 7:1-25
Anticytoplasmic Autoantibody Test for Wegener's Granulomatosis WEGENER'S GRANULOMATOSIS is a systemic disease characterized by necrotizing laryngeal granulomas with vasculitis of the upper and lower respiratory tract, systemic vasculitis, and focal necrotizing glomerulitis. The disease can be limited or generalized. When there are nasal, pulmonary, and renal findings, a clinical diagnosis and histologic confirmation by nasal or kidney biopsy are relatively simple. In Wegener's disease with limited and localized findings, such as isolated laryngeal subglottic involvement, diagnosis
may be difficult. Not every nasal or laryngeal biopsy specimen will demonstrate characteristic histologic findings. The finding of a nonspecific inflammatory infiltrate is more likely. The anticytoplasmic autoantibody test has been shown to have high specificity for Wegener's granulomatosis. The test was also useful to monitor reactivation and remissions of the disease. The absence of the autoantibody does not rule out Wegener's disease. The test is useful in diagnosing the disease in patients in whom histologic confirmation is not always possible-such as those with isolated subglottic stenosis. The test is done with a standard immunofluorescence technique. The cytopreparation is considered positive for anticytoplasmic autoantibody if the characteristic focal, centrally accentuated, fine granular cytoplasmic staining pattern is present in most neutrophils. Experience with pattern recognition and strict adherence to the methodologic protocol are essential for obtaining reproducible and reliable results. LAWRENCE W. DE SANTO, MD Scottsdale, Arizona REFERENCES DeRemee RA, McDonald TJ, Weiland LH: Wegener's granulomatosis: Observations on treatment with antimicrobial agents. Mayo Clin Proc 1985; 60:27-32 Specks U, DeRemee RA: Significance of antibodies to cytoplasmic components of neutrophils. Thorax 1989; 44:369-370 Van der Woude FJ, Rasmussen N, Lobatto S, et al: Autoantibodies against neutrophils and monocytes: Tool for diagnosis and marker of disease activity in Wegener's granulomatosis. Lancet 1985; 1:425-429
Gadolinium Magnetic Resonance Imaging in Bell's Palsy IT HAS BEEN DISCOVERED that the facial nerve in Bell's palsy has a characteristic appearance on gadolinium-enhanced magnetic resonance imaging (Gd-MRI) scans. Despite this important advance in imaging technology, the clinical presentation remains the mainstay of diagnosis. The diagnosis of Bell's palsy in the past has been one of exclusion, arrived at when a rapid onset of facial weakness is unaccompanied by signs or symptoms suggestive of disease involving the central nervous system, temporal bone, or parotid gland. More than 80 causes have been identified that result in facial paralysis.
Many patients with serious underlying diseases affecting the facial nerve, such as tumors and infections, have been inappropriately given the diagnosis of Bell's palsy. Such a misdiagnosis may delay needed therapy and diminish the probability of eventual nerve recovery. When a facial palsy has atypical features, the diagnosis of Bell's palsy should be questioned, and a Gd-MRI scan of the nerve obtained. The most common reason for doing a Gd-MRI scan is weakness that persists for a longer period than would be expected in an uncomplicated Bell's palsy. When paralysis persists beyond two months, the diagnosis of idiopathic paralysis should be questioned. Other atypical features include slowly progressive palsy, facial palsy accompanied by spasm, recurrent palsy, unusual degrees of pain, and the presence of multiple cranial neuropathies or other neurologic symptoms. On Gd-MRI scans, the facial nerve in Bell's palsy appears bright because of gadolinium uptake in the inflamed segment. This is usually most pronounced in the region of the geniculate ganglion at the lateral end of the internal auditory canal, but more diffuse enhancement of the intratemporal portion may be seen. This enhancement typically persists for several months and may not resolve entirely, even after paral-
THE
WESTERN
JOURNAL
OF
MEDICINE
9 *
JUNE 1991
JUNE
1991
9 *
154
154
* *
6
Previously established barriers to complete resection, such as anterior cranial floor invasion, extension to the orbital apex, sphenoid sinus involvement, cavernous sinus and internal carotid artery involvement, and intracranial extension, can all be handled in carefully selected patients. For lesions involving the anterior cranial fossa, a transfacial incision with an approach through the maxillary and ethmoid sinuses is combined with a low anterior craniotomy. Total tumor excision can be accomplished even when dura and brain are involved. On occasion, an approach to the middle fossa through a subcranial route can achieve exposure of the cavernous sinus, eustachian tube, and internal carotid artery.
Middle fossa involvement is more difficult to manage and usually requires an infratemporal fossa and middle cranial fossa combined approach. Temporal lobe involvement and invasion of Meckel's cave, the cavernous sinus, and the petrous and cavernous carotid artery can all be safely managed through this approach. The postoperative course may be stormy, and the availability of a dedicated or a neurosurgical intensive care unit with specially trained nurses is invaluable. Three- to five-year tumor-free survival figures are being compiled by many centers doing skull base surgical procedures. Results are encouraging, especially in light of the hopeless prognosis otherwise facing these patients. PAUL DONALD. MD Sacramento, California
REFERENCES
Al-Mefty 0: Surgery of the Cranial Base. Boston, Mass, Kluwer, 1987 Donald P: Cranial facial surgery for head and neck cancer, chap 18, In Johnson JT (Ed): American Academy of Otolaryngology: Head and Neck Surgery-Vol 2, Instruction Course. St Louis, Mo, Mosby, 1988 Sekhar L, Schramm VL: Tumors of the Cranial Base: Diagnosis and Treatment. Mt Kisco, NY, Futura, 1987
Functional Endoscopic Sinus Surgery in Children FUNCTIONAL ENDOSCOPIC SINUS SURGERY is an operative approach to the paranasal sinuses in which the objective is to reestablish adequate sinus ventilation and mucociliary clearance in patients with chronic sinus disease. Narrow mucosallined channels exist in the middle meatus where there is a high potential for mucosal layers to contact each other. The various ostia of the anterior ethmoid, frontal, and maxillary sinuses drain in proximity to this area. Because of mucosal hyperplasia and inflammation following an infection or allergy, these channels (and subsequently the ostia) may be blocked with resultant sinus obstruction. The effect on the sinuses may be either reversible (acute sinusitis) or irreversible (chronic sinusitis), depending on the source and time course of the original insult to the middle meatus-anterior ethmoid complex. This middle meatus-anterior ethmoid complex has been called the ostiomeatal unit or complex. In the United States, endoscopic sinus surgical techniques have replaced open techniques in many instances. Many children treated with endoscopic sinus operations have considerable atopy, allergy, asthma, and primary and secondary immunodeficiencies. Other clinical groups include children with cystic fibrosis and immotile cilia or Kartagener's syndrome. Children with chronic sinus disease,
as
717
717
6
confirmed by the
history, physical examination, and repeated plain sinus films, may be treated with an endoscopic sinus operation. An ade-
quate allergic and immunologic workup should be done before referral to an otolaryngologist-head and neck surgeon. It should be emphasized that an adequate trial of medical therapy is always completed before any consideration of operative intervention. The initial diagnostic evaluation always includes nasal endoscopy under topical anesthesia. This may be difficult, if not impossible, depending on the age and cooperation of the child. The middle meatus is carefully examined for evidence of chronic infection. Depending on the history and extent of the disease, medical therapy is often continued or altered and the patient reevaluated at a later date. Children who continue to have persistent disease after adequate medical therapy undergo direct coronal computed tomographic evaluation of the ostiomeatal complex and paranasal sinuses. If the computed tomographic scan shows mucosal disease involving the ostiomeatal complex and ethmoid and maxillary sinuses, an endoscopic sinus operation may be considered. In children, the procedure requires a general anesthetic and is done on an outpatient basis under endoscopic visualization. The key to the operation is opening the ostiomeatal complex, but it may include a complete ethmoidectomy ifthe disease is extensive. Middle meatal antrostomies are created to open the natural ostia of the maxillary sinuses. Our experience thus far has been with 38 children who have undergone an endoscopic sinus operation. Of this number, most have been children with chronic sinus disease who are otherwise normal. As of this writing, an overall success rate of about 85% has been obtained in this group in terms of resolving symptoms of chronic sinus disease. Children with chronic sinus disease and concomitant hematologic disorders who are also receiving chemotherapeutic agents have responded similarly. Children who are atopic, with significant allergy and asthma, have responded well initially, including notable abatement of their asthma. Approximately half have had a relapse oftheir sinus and pulmonary symptoms six months to a year following the first procedure. Further follow-up and experience are necessary. DENNIS M. CROCKETT, MD Los Angeles, California
REFERENCES Gross CW, Gurucharri MJ, Lazar RH, Tong TE: Functional endonasal sinus surgery in the pediatric age group. Laryngoscope 1989; 99:272-275 Kennedy DW, Zinreich SJ: The functional approach to inflammatory sinus disease: Current perspectives and technique modifications. Am J Rhinol 1988; 2:89-96 Kennedy DW, Zinreich SJ, Rosenbaum AE, Johns ME: Functional endoscopic sinus surgery: Theory and diagnostic evaluation. Arch Otolaryngol 1985; 1 1 1:576-582 Messerklinger W: Endoscopy of the Nose. Munich, Gennany, Urban and Schwartzberg, 1978
Cochlear Implants in Children THE COCHLEAR IMPLANT is a medical device, part of which is placed surgically, that uses electrical stimulation to provide hearing. In recent years, the device has become an accepted method for providing auditory stimulation to the profoundly deaf. Two devices, the 3M/House single-channel cochlear implant and the Nucleus multichannel cochlear implant, have been formally approved by the United States Food and Drug Administration (FDA) for commercial marketing to deaf adults. More than 600 children ages 2 through 17 years have been implanted with either a single- or a multielectrode device. In September 1988, the FDXs Ear, Nose, and Throat Advisory Panel voted to recommend marketing approval of
EPITOMES-OTOLARYNGOLOGY/HEAD AND NECK SURGERY
718
the 3M/House implant for children, mirroring the widespread professional acceptance of such devices in children. The 3M device is no longer available, but the Nucleus multichannel cochlear implant received FDA marketing approval for use in children in June 1990. Extensive auditory, speech, educational, and psychological testing is done before and after implantation for children. Results show that cochlear implants provide auditory detection over much of the speech signal. Compared with before implantation, there is considerable improvement in auditory discrimination and speech production skills. Limited openset word and sentence recognition is possible for at least some children, perhaps as many as 30%. Complications with the device have been minimal but include inappropriate placement of the electrode array, postauricular flap infection, facial paresis, transient dizziness or pain, and partial device extrusion. About 1% of patients in the Nucleus clinical trials had unresolved complications. Selection criteria include bilateral profound sensorineural deafness, a lack of substantial benefit from hearing aids, psychological suitability, and medical suitability. The primary criterion for selection is confirmation of profound sensorineural hearing loss, usually with an average unaided threshold for the speech frequencies of poorer than 1 10 dB or an aided speech detection threshold of poorer than about 53 dB. Patients who are candidates show poorer performance on selected auditory discrimination tasks while using appropriately fit hearing aids than those using an implant. Families and, if possible, candidates should be well motivated and possess appropriate expectations. Medical contraindications include deafness caused by lesions of the acoustic nerve or central auditory pathway, active middle ear infections, cochlear ossification that prevents electrode insertion, an absence of cochlear development, and tympanic membrane perforation. The cochlear implant can provide sound to deaf adults and children unable to benefit from hearing aids. The complex assessment, rehabilitation, and counseling should be done by centers with the multidisciplinary staff necessary to provide effective care for patients with this special auditory prosthesis.
F. HOUSE, MD Newport Beach, Californica WILLIAM
REFERENCES Berliner KI, Luxford WM, House WF, Tonokawa LL: Cochlear implants in children, In Myers EN, Bluestone CD, Brackmann DE, Krause CJ (Eds): Advances in Otolaryngology: Head and Neck Surgery, Vol 4. St Louis, Mo, Mosby Year Book, 1990, pp 61-79 House WF, Berliner KI, Luxford WM: Cochlear implants in deaf children. Curr Probl Pediatr 1987; 17:347-388 National Institutes of Health: Cochlear Implants. Consensus Development Conference Statement 1988; 7:1-25
Anticytoplasmic Autoantibody Test for Wegener's Granulomatosis WEGENER'S GRANULOMATOSIS is a systemic disease characterized by necrotizing laryngeal granulomas with vasculitis of the upper and lower respiratory tract, systemic vasculitis, and focal necrotizing glomerulitis. The disease can be limited or generalized. When there are nasal, pulmonary, and renal findings, a clinical diagnosis and histologic confirmation by nasal or kidney biopsy are relatively simple. In Wegener's disease with limited and localized findings, such as isolated laryngeal subglottic involvement, diagnosis
may be difficult. Not every nasal or laryngeal biopsy specimen will demonstrate characteristic histologic findings. The finding of a nonspecific inflammatory infiltrate is more likely. The anticytoplasmic autoantibody test has been shown to have high specificity for Wegener's granulomatosis. The test was also useful to monitor reactivation and remissions of the disease. The absence of the autoantibody does not rule out Wegener's disease. The test is useful in diagnosing the disease in patients in whom histologic confirmation is not always possible-such as those with isolated subglottic stenosis. The test is done with a standard immunofluorescence technique. The cytopreparation is considered positive for anticytoplasmic autoantibody if the characteristic focal, centrally accentuated, fine granular cytoplasmic staining pattern is present in most neutrophils. Experience with pattern recognition and strict adherence to the methodologic protocol are essential for obtaining reproducible and reliable results. LAWRENCE W. DE SANTO, MD Scottsdale, Arizona REFERENCES DeRemee RA, McDonald TJ, Weiland LH: Wegener's granulomatosis: Observations on treatment with antimicrobial agents. Mayo Clin Proc 1985; 60:27-32 Specks U, DeRemee RA: Significance of antibodies to cytoplasmic components of neutrophils. Thorax 1989; 44:369-370 Van der Woude FJ, Rasmussen N, Lobatto S, et al: Autoantibodies against neutrophils and monocytes: Tool for diagnosis and marker of disease activity in Wegener's granulomatosis. Lancet 1985; 1:425-429
Gadolinium Magnetic Resonance Imaging in Bell's Palsy IT HAS BEEN DISCOVERED that the facial nerve in Bell's palsy has a characteristic appearance on gadolinium-enhanced magnetic resonance imaging (Gd-MRI) scans. Despite this important advance in imaging technology, the clinical presentation remains the mainstay of diagnosis. The diagnosis of Bell's palsy in the past has been one of exclusion, arrived at when a rapid onset of facial weakness is unaccompanied by signs or symptoms suggestive of disease involving the central nervous system, temporal bone, or parotid gland. More than 80 causes have been identified that result in facial paralysis.
Many patients with serious underlying diseases affecting the facial nerve, such as tumors and infections, have been inappropriately given the diagnosis of Bell's palsy. Such a misdiagnosis may delay needed therapy and diminish the probability of eventual nerve recovery. When a facial palsy has atypical features, the diagnosis of Bell's palsy should be questioned, and a Gd-MRI scan of the nerve obtained. The most common reason for doing a Gd-MRI scan is weakness that persists for a longer period than would be expected in an uncomplicated Bell's palsy. When paralysis persists beyond two months, the diagnosis of idiopathic paralysis should be questioned. Other atypical features include slowly progressive palsy, facial palsy accompanied by spasm, recurrent palsy, unusual degrees of pain, and the presence of multiple cranial neuropathies or other neurologic symptoms. On Gd-MRI scans, the facial nerve in Bell's palsy appears bright because of gadolinium uptake in the inflamed segment. This is usually most pronounced in the region of the geniculate ganglion at the lateral end of the internal auditory canal, but more diffuse enhancement of the intratemporal portion may be seen. This enhancement typically persists for several months and may not resolve entirely, even after paral-
