Case Conference at the University of Florida College of Medicine Series Editors: A. Joseph Layon, MD, Michael E. Mahla, MD, Jerome H. Modell, MD
Intraoral Vascular Malformation and Airway Management: A Case Report and Review of the Literature Marcia J. Howton, MD,* Michael E. Mahla, MDJr Department ville,
of Anesthesiology,
University of Florida College of Medicine,
Gaines-
FL.
Comment by Brent M. Seagle, MD$ A patient with a large airway venous malformation underwent anesthesia for a tooth extraction. The procedure was uneventful until extubation, immediately after which complete airway obstruction resulted. After unsuccessful attempts to relieve the problem, the patient’s trachea was reintubated. Laryngoscopy showed that the venous malformation in the airway had enlarged and was responsible for the airway obstruction. Another attempt at extubation after corrective maneuvers was again unsuccessful. A tracheostomy was required, which was eventually removed after a complete recovery. Anesthesiologists must be concerned with any airway vascular abnormality. Most abnormalities in-
*Resident
in Anesthesiology
TAssistant
Professor
of Anesthesiology
and Neurosurgery
$Assistant Professor of Surgery, Division of Plastic and Reconstructive Surgery, Department of Surgery, University of Florida College of Medicine Address reprint requests to Editorial Office, Department of Anesthesiology, University of Florida College of Medicine, Box 100254, Gainesville, FL 32610-0254, USA. Case Conference presentations partment of Anesthesiology, Medicine. Received for publication June for publication May 8, 1992.
are selected and edited at the DeUniversity of Florida College of
6, 1991; revised
manuscript
accepted
0 1992 Butterworth-Heinemann J. Clin. Anesth. 4:498-502, 1992.
498
J. Clin.
Anesth.,
vol. 4, November/December
1992
volving the airway are either hemangiomas or venous malformations. The anesthesiologist murt diagnose the problem correctly because even minor manipulation of a venous malformation may result in exsanguination, or the malforwtation may become engorged and compromise the airway.
Keywords: Anatomy-complications, intubation,
tracheal;
airway; veins, malformation of.
Case Report A healthy 33-year-old man with dental caries required extraction of tooth #32. He had no significant past medical or anesthetic history except for what was originally described as a large intraoral hemangioma. On examination, the man had a venous abnormality that involved the right side of the tongue from base to tip, the gingiva, the right tonsillar region, and portions of the hard and soft palates. The abnormality extended to just above the vallecula but did not appear to compromise the airway lumen itself. A right common and external carotid angiogram showed a large, deep oropharyngeal venous malformation on the right and centered mostly in the tonsillar and soft palatal area. The tumor slightly displaced the lingual and external maxillary arteries upward and laterally. Twenty years earlier, sclerosing liquid had been injected into the malformation in an attempt to decrease its size. This therapy was unsuccessful, and the malformation was considered inoperable. The patient reported no overt symptoms of stridor
Airway malformation: Howton and Mahlu
or exercise intolerance, but he did have occasional shortness of breath at night, which he attributed to the malformation and which was relieved by his getting up and walking around. The only other change in lifestyle he noted was a preference for small pieces of soft food. On physical examination, the muscular 95-kg man had slight swelling of the right mandible. Examination of his airway showed approximately 6 cm of mouth opening, with a normal range of motion of the head and neck. The malformation was visualized on (1) the right side of the floor of the mouth, (2) the right side of the tongue, (3) the posterior soft palate, (4) the right retromolar, and (5) the tonsillar region. The uvula was deviated to the left, but the posterior oropharynx appeared otherwise normal. The patient had numerous caries. The nasal septum was midline, and the nares were patent. The trachea was midline, and the carotid pulses were equal and without bruit. After the patient arrived in the operation room (OR), monitors, which included an electrocardiogram, noninvasive blood pressure cuff, pulse oximeter, and precordial stethoscope, were placed, and the patient was given 100% oxygen (0,) while breathing normally for 5 minutes. Arterial oxygen saturation (SpO,), which had been 97% while he was breathing room air, promptly increased to 100%. The malformation appeared to be very close to the involved tooth. As a precaution against aspiration in case of massive hemorrhage, the oral surgeon requested that the airway be secured with an endotracheal tube prior to extraction. Anesthesia was induced intravenously (IV) with thiopental sodium 500 mg in divided doses. The patient was then easily ventilated by mask, and no airway obstruction resulted. The patient was given succinylcholine 120 mg IV and robinu10.2 mg IV to facilitate intubation. Direct laryngoscopy revealed redundant vascular tissue on the right of the soft palate that extended into the posterior pharynx and stopped 2 cm above the vocal cords. No vocal cord pathology was noted. An &O-mm oral endotracheal tube was inserted atraumatically and did not contact the malformation. Anesthesia was maintained through a semiclosed circuit with O,, nitrous oxide, and isoflurane. Gas concentrations were monitored by mass spectrometry. A second 16-gauge catheter was inserted into the dorsum of the left hand to ensure adequate venous access. A full train-of-four was noted by twitch monitor 10 minutes after induction. The intraoperative course was uneventful. After the tooth was extracted, the anesthetic drugs were discontinued and the patient was allowed to wake up breathing 100% 0,. During emergence, the patient experienced some coughing and bucking. Once he was awake and following complex commands and exhibiting voluntary motion of all extremities, such as pointing to the endotracheal tube to indicate his desire to be extubated, the patient was extubated. Extubation was immediately followed by complete airway obstruction. The problem was not relieved by continuous positive airway pressure (CPAP) or by gentle placement of an oral airway. Succinylcholine 10 mg IV was administered to re-
lieve presumed laryngospasm. This maneuver did not relieve the obstruction, and the patient was given thiopental sodium 200 mg IV and succinylcholine 100 mg IV to facilitate reintubation. In addition, a cricothyroidotomy kit was readied. SpO, decreased into the 30% range for approximately 30 seconds. Laryngoscopy during reintubation showed that the vascular malformation had enlarged and extended between the vocal cords, which created a ballvalve type of obstruction. The malformation was gently and easily moved aside without causing a hemorrhage, and an &O-mm oral endotracheal tube (smaller sizes were readily available) was passed easily between the cords. Anesthesia was maintained with 0, and isoflurane. An otolaryngologist was called to the OR to perform an emergent tracheostomy if needed. The patient was placed in a semisitting position for approximately 30 minutes to facilitate venous drainage of the malformation. Anesthesia was then discontinued. When the patient had completely awakened, he was placed supine temporarily in a right lateral decubitus position to allow the ball-valve portion of the malformation to fall out and away from the trachea. Although this position change was likely to increase engorgement, we thought that the lateral head-flat position might keep the malformation from falling between the vocal cords. The trachea was extubated for a second time, and this was again followed by complete obstruction of the airway unresponsive to placement of an oral airway, CPAP, or head elevation. Atropine 1.2 mg IV, thiopental sodium 200 mg IV, and succinylcholine 120 mg IV were administered, and the patient was intubated for the third time without difficulty. Direct laryngoscopy showed a further marked increase in the size of the malformation despite the attempts to enhance venous drainage. A formal tracheostomy was performed without difficulty under O,-isoflurane anesthesia. The patient had an uneventful recovery and postoperative course without evidence of neurologic compromise. A computerized tomographic (CT) scan of the head and neck, done a month later at follow-up, showed an extensive vascular malformation of the right oropharynx, floor of the mouth, and submandibular and sublingual regions, which extended into the nasopharynx cephalad and deep into the neck and displaced the carotid artery and jugular vein medially and posteriorly. No surgical or chemotherapeutic intervention was considered possible. The tracheostomy was removed without difficulty, and the patient has since resumed normal activities.
Discussion Many inconsistent, confusing terms are used in the literature to describe vascular abnormalities in the region of the face. A system developed in 1982 describes these abnormalities according to growth rate and endothelial cell characteristics.’ A hemangioma is a vascular neoplasm occurring in infancy. It may be present at birth but usually is not noticed until early infancy. This type J. Clin. Anesth., vol. 4, November/December
1992
499
&se
Conference
of lesion grows rapidly during the first year of life and then slowly involutes. It does not involve adjacent bony structures. Microscopically, a hemangioma is characterized by increased endothelial turnover and increased numbers of mast cells.2 Hemangiomas are the most common tumors of the head and neck in infants and young children,3,4 and affect females two to three times more often than males5 The two most common histologic forms of hemangiomas are capillary and cavernous.*Jj A capillary hemangioma is comprised predominantly of vessels that have the caliber of normal capillaries. A cavernous hemangioma is made up of much larger vascular channels. A cavernous hemangioma, which is usually more extensive and extends deeper into subcutaneous tissues, is soft, elevated, poorly circumscribed, easily compressible, and purplish blue in color. Mixed capillary-cavernous hemangiomas are common. A hemangioma can occur in any tissue, but the skin and mucous membranes are the most common locations.6,7 Hemangiomas of the face and neck account for over half of all these malformations in humans,’ the most frequent sites being the mucous membranes of the tongue and lips and the less frequent sites being the larynx, palate, and trachea. Subglottic hemangiomas in infants account for 85% of all hemangiomas in this age group.’ The hemangioma characteristically enlarges during the first year of life and then undergoes some degree of spontaneous regression. Many will have virtually disappeared by the time the child reaches 5 years of age.4 For this reason, allowing the patient time to outgrow a hemangioma without medical or surgical management is the initial approach advocated.4 Active treatment is indicated only if the hemangioma interferes with the function of vital organs, compromises the airway, or ulcerates with hemorrhage or infection or if there is an accompanying thrombocytopenia. Depending on any of these conditions, several forms of treatment have been advocated (Table 1).4-10 The other major type of vascular abnormality is called a vascular malformation. This type of malformation is generally present at birth and grows not like a neoplasm but rather at the same rate as normal human growth. Malformations are commonly associated with skeletal abnormalities in surrounding bones and may expand with hormonal changes, infection, or trauma.2 Vascular malformations may be subdivided based on the type of vessels involved: capillary, venous, lymphatic, arterial, or arteriovenous. Capillary, venous, or lymphatic malforTable 1.
Treatment
Options for Airway Hemangioma Option
Treatment Strategy
Noninvasive
Invasive
500
Sclerosing drugs Corticosteroids Systemic Intralesional Tracheostomy (if airway not involved) Cryosurgery Carbon dioxide laser Surgical excision
J. Clin. Anesth.,
vol. 4, November/December
1992
Table 2.
Management
Area of Concern
Anesthesia
Monitoring Positioning General
of an Airway Venous
Malformation
Strategy
Regional technique Mask ventilation if possible Hypotensive anesthesia for resection Prevention of coughing or straining as associated with extubation Adequate venous access Position associated with good venous drainage Minimal manipulation or injury to malformation
mation is a low-flow condition, and arterial or arteriovenous malformation is a high-flow condition. Most vascular malformations of the maxillofacial region are venous.2 The distinction between the types of vascular abnormalities is very important to anesthesiologists. A hemangioma generally can be resected and separated from surrounding tissue without danger of exsanguination. A vascular malformation, however, involves all the blood vessels in a given area of the body. Complete resection is generally not possible without completely removing the part of the body involved. Bleeding during surgical resection or after trauma is commonly uncontrollable. Anesthesiologists must be concerned with any type of vascular abnormality involving the airway. Patients with such a problem may present for any of the treatment options that require anesthesia, or, as in our case, a malformation may be a compounding factor during an unrelated surgery. The anesthetic-related problems involve obstruction of, or hemorrhage into, the airway. Airway obstruction may be present preoperatively or may develop later during anesthesia.s,7 Such obstruction is probably more likely with a venous malformation, which may engorge rapidly as venous pressure increases, as with the head-down position, coughing, or straining. Minor injury to a malformation may cause massive bleeding into the respiratory tract. In patients with a large malformation involving the tongue and pharynx, visualizing glottic structures and orotracheal intubation may be difficult. With the subglottic variety of hemangioma common in infants, blood may be aspirated into the tracheobronchial tree during or after tracheal intubation. Postoperative swelling of an intraoral hemangioma following a hysterectomy has been reported.5 Anesthesia for a patient with an airway vascular abnormality includes, when possible, a regional technique (Table Z), which obviates manipulation of the airway and possible vascular-related complications from airway management. Adequate intravenous access must be established before a vascular malformation is manipulated in any way. There are several case reports of exsanguination after minimal manipulation of large venous malformations, as may occur during tracheal intubation.7,‘0,11 When a patient with an airway vascular abnormality re-
Airway malformafzon: Howton and Mahla
quires general anesthesia, mask ventilation may be a viable alternative if the abnormality does not obstruct the airway and no other factors mandate endotracheal intubation, as occurred in the current case. The use of induced hypotensive anesthesia to reduce blood loss during resection of a vascular malformation is well described and may be useful during elective resection.12 Prevention of perioperative coughing and straining may reduce the likelihood of enlargement of the malformation, but enlargement has been reported even when coughing or straining does not occur.5 Our patient experienced coughing during emergence. After the first extubation failed, it became apparent during the successful reintubation that the venous malformation had increased 25% in size and was compromising his airway by extending through the cords as a ball-valve. After reestablishing airway patency, we discussed with our surgical colleagues the risks of tracheostomy to secure the postoperative airway compared with the risks of a second trial of extubation. The latter option was chosen. If a position change did not prevent the malformation from falling between the vocal cords, the risks of a second trial of extubation included airway obstruction, subsequent inability to reintubate, and emergency tracheostomy. The risks of tracheostomy included infection, inadvertent violation of a hemangioma with possible exsanguinating blood loss, and inadvertent postoperative decannulation with airway obstruction and possible inability to reestablish the airway in time to prevent neurologic morbidity. We considered the risks of the latter great enough to first attempt extubation with the patient placed in the right lateral decubitus head-neutral position after a period in the semisitting position to facilitate venous drainage. The supine lateral position was chosen to help keep the malformation from acting as a ball-valve obstruction again. To minimize trauma to the malformation, we did not perform direct laryngoscopy before the final extu-
bation. After the second extubation, the ensuing cotiplete airway obstruction was not relieved by the lateral or head-up position or by CPAP. Visualization of the airway during reintubation showed that further swelling of the malformation caused an approximate 95% compromise of the patient’s airway. The malformation was easily and atraumatically moved aside for insertion of the endotracheal tube. The tracheostomy and postoperative course were uneventful. Airway vascular abnormalities are not commonly encountered in anesthetic practice. When a patient with an intraoral or airway vascular abnormality requires anesthesia and surgery, the anesthesiologist must know the extent of the abnormality and the airway structures involved. Although no clear preference for laryngoscope blades can be recommended, if the lesion involves the epiglottis, a Macintosh blade may be preferable because it can avoid direct contact with the epiglottis. If the base of the tongue is involved, a straight blade may reduce the amount of direct contact with the lesion. If the lesion is located more on one side or the other, a right- or leftsided approach with the appropriate blade may be useful. A preoperative CT scan would have better elucidated the extent of airway involvement in our patient but, in retrospect, would not change our anesthetic management, because the malformation primarily involved the nasopharynx, oropharynx, and deep neck, ending 2 cm above the vocal cords. We would not necessarily have predicted the postextubation swelling based on the CT scan. In spite of this, however, we strongly recommend a preoperative CT scan to elucidate the extent of real or potential airway compromise. Because swelling of a venous malformation will likely occur during events such as coughing and straining, which are commonly associated with extubation, if the malformation involves structures in the vicinity of the glottis, prolonged postoperative intubation in the sitting position to facilitate drainage may help prevent the problems we encountered.
Comment Dr. Seagle: This report describes an interesting and challenging case of mechanical airway obstruction secondary to dilatation of a vascular malformation in the upper airway that occurred during anesthesia for an oral surgical procedure. That the obstructing portion of the vascular malformation was located proximal to the laryngeal inlet and that the obstruction was caused by a ball-valve prolapse of tissue into the glottis is interesting and noteworthy. That the other feared complication of airway malformation-that is, uncontrollable hemorrhage-did not occur is indeed fortunate. As Drs. Howton and Mahla note, vascular malformations can be classified according to histologic characteristics.’ Hemangioma qualifies as a proliferative neoplastic malformation that undergoes at least some degree of spontaneous involution, often total. Vascular malformations, however, show mature nonproliferative
endothelium. Their growth is commensurate with that of the growing child, and they characteristically do not involute. They may enlarge as a result of “changes in pressure and flow, ectasia, collateral formation, shunting and hormonal modulation.“ll Hemangioma and vascular malformation usually can be differentiated on the basis of careful history and physical findings; arteriography may be helpful. This differentiation is helpful in developing a prognosis, in planning therapy, and in predicting the behavior of a given malformation under circumstances such as those seen in the current case. In one study,” most vascular abnormalities in the maxillofacial region were malformations (usually venous). In this light, the substantial dilatation of the malformation in this patient was understandable. Most likely, the abnormality represented a venous malformation. The precise mechanism of the airway obstrucJ. Clin. Anesth., vol. 4, November/December 1992
501
Case Conference
tion-that is, the ball-valve prolapse mto the glottisshould be anticipated with these lesions. Further, differentiating between the two varieties of vascular malformations should improve prediction of the effect of a given malformation as well as its response to treatment. In the case presented here, the anesthetic and postanesthetic care would seem to be appropriate for any similar vascular malformation in the airway.
References Mulliken JB, Glowacki J: Hemangiomas and vascular malformations in infants and children: a classification based on endothelial characteristics. Plast Reconst Surg 1982;69:412-22. Kaban LB, Mulliken JB: Vascular anomalies of the maxillofacial region. J Oral Maxillofac Surg 1986;44:203-13. Wilson RD, Putnam L, Phillips MT, Phillips CT: Anesthetic problems in surgery for varying levels of respiratory obstruction in infants and children. Anesth Analg 1974;53:878-85. Hawkins DB, Crockett DM, Kahlstrom EJ, MacLaughlin EF: Corticosteroid management of airway malformations: long-term follow-up. Laryngoscope 1984;94:633-7.
laryngeal hemangioma. Ann 5. Benjamin B, Carter P: Congenital ($01 Rhino1 Laryngol 1983;92:448-55. of congenital heman6. Brown DA, Smith JD: Late complication gioma of the tongue. Head Neck Surg 1987;9:299-304. 7. Divekar VM, Kavadia IP, Raksha H: A massive hemangioma of the upper airway. Anaesthesia 1982;37:93-4. MT: Surgical 8. Morgan RF, Horowitz JH, Wanebo HJ, Edgerton management of vascular malformations of the head and neck. Am J Surg 1986;152:424-9. embolization by the so9. Wozniak M, Sadowski A: Intravascular called balloonization method in the treatment of cerebral arteriovenous malformations. Neural Neurochir Pol 198 1; 15: 18 l9. of pharyngocervical angiomatosis 10. Rethi A: On the therapy (pharyngeal hemangioma), of other hemangiomas of the upper airways and of the nevus flammeus. HNO 1965; 13:2958. in dental surgery. Schweirerische Mon11. Maeglin B: Emergencies atsschrift Zahnheilkunde 1974;84:964-76. for hypotensive anaesthesia. In: Enderby 12. Beare R: Indications GEH, ed. Hypotensive Anaesthesiu. Edinburgh: Churchill Livingstone, 1985;99-108.
Vascular Anomalies of the Maxillofacial Region Leonard B. Kaban, D.M.D., M.D. and John B. M&liken, M.D. University of California, San Francisco CA Summary A biologic classification based on clinical behavior and endothelial cell characteristics is used to divide vascular birthmarks into two groups: hemangiomas and vascular malformations. This system is applied to vascular anomalies of the maxillofacial region and is demonstrated with illustrative cases. The distinction between hemangioma and vascular malformation is important for maxillofacial surgeons because hemangiomas rarely affect bone, whereas malformations affect bone in 35% of cases. Reproduced with 1986;44:203-13.
502
J. Clin.
Anesth.,
permission
vol. 4, November/December
from
1992
the Journal
of Oral and Muxillofacial
Surge9
Intraoral Vascular Malformation and Airway Management: A Case Report and Review of the Literature Marcia J. Howton, MD,* Michael E. Mahla, MDJr Department ville,
of Anesthesiology,
University of Florida College of Medicine,
Gaines-
FL.
Comment by Brent M. Seagle, MD$ A patient with a large airway venous malformation underwent anesthesia for a tooth extraction. The procedure was uneventful until extubation, immediately after which complete airway obstruction resulted. After unsuccessful attempts to relieve the problem, the patient’s trachea was reintubated. Laryngoscopy showed that the venous malformation in the airway had enlarged and was responsible for the airway obstruction. Another attempt at extubation after corrective maneuvers was again unsuccessful. A tracheostomy was required, which was eventually removed after a complete recovery. Anesthesiologists must be concerned with any airway vascular abnormality. Most abnormalities in-
*Resident
in Anesthesiology
TAssistant
Professor
of Anesthesiology
and Neurosurgery
$Assistant Professor of Surgery, Division of Plastic and Reconstructive Surgery, Department of Surgery, University of Florida College of Medicine Address reprint requests to Editorial Office, Department of Anesthesiology, University of Florida College of Medicine, Box 100254, Gainesville, FL 32610-0254, USA. Case Conference presentations partment of Anesthesiology, Medicine. Received for publication June for publication May 8, 1992.
are selected and edited at the DeUniversity of Florida College of
6, 1991; revised
manuscript
accepted
0 1992 Butterworth-Heinemann J. Clin. Anesth. 4:498-502, 1992.
498
J. Clin.
Anesth.,
vol. 4, November/December
1992
volving the airway are either hemangiomas or venous malformations. The anesthesiologist murt diagnose the problem correctly because even minor manipulation of a venous malformation may result in exsanguination, or the malforwtation may become engorged and compromise the airway.
Keywords: Anatomy-complications, intubation,
tracheal;
airway; veins, malformation of.
Case Report A healthy 33-year-old man with dental caries required extraction of tooth #32. He had no significant past medical or anesthetic history except for what was originally described as a large intraoral hemangioma. On examination, the man had a venous abnormality that involved the right side of the tongue from base to tip, the gingiva, the right tonsillar region, and portions of the hard and soft palates. The abnormality extended to just above the vallecula but did not appear to compromise the airway lumen itself. A right common and external carotid angiogram showed a large, deep oropharyngeal venous malformation on the right and centered mostly in the tonsillar and soft palatal area. The tumor slightly displaced the lingual and external maxillary arteries upward and laterally. Twenty years earlier, sclerosing liquid had been injected into the malformation in an attempt to decrease its size. This therapy was unsuccessful, and the malformation was considered inoperable. The patient reported no overt symptoms of stridor
Airway malformation: Howton and Mahlu
or exercise intolerance, but he did have occasional shortness of breath at night, which he attributed to the malformation and which was relieved by his getting up and walking around. The only other change in lifestyle he noted was a preference for small pieces of soft food. On physical examination, the muscular 95-kg man had slight swelling of the right mandible. Examination of his airway showed approximately 6 cm of mouth opening, with a normal range of motion of the head and neck. The malformation was visualized on (1) the right side of the floor of the mouth, (2) the right side of the tongue, (3) the posterior soft palate, (4) the right retromolar, and (5) the tonsillar region. The uvula was deviated to the left, but the posterior oropharynx appeared otherwise normal. The patient had numerous caries. The nasal septum was midline, and the nares were patent. The trachea was midline, and the carotid pulses were equal and without bruit. After the patient arrived in the operation room (OR), monitors, which included an electrocardiogram, noninvasive blood pressure cuff, pulse oximeter, and precordial stethoscope, were placed, and the patient was given 100% oxygen (0,) while breathing normally for 5 minutes. Arterial oxygen saturation (SpO,), which had been 97% while he was breathing room air, promptly increased to 100%. The malformation appeared to be very close to the involved tooth. As a precaution against aspiration in case of massive hemorrhage, the oral surgeon requested that the airway be secured with an endotracheal tube prior to extraction. Anesthesia was induced intravenously (IV) with thiopental sodium 500 mg in divided doses. The patient was then easily ventilated by mask, and no airway obstruction resulted. The patient was given succinylcholine 120 mg IV and robinu10.2 mg IV to facilitate intubation. Direct laryngoscopy revealed redundant vascular tissue on the right of the soft palate that extended into the posterior pharynx and stopped 2 cm above the vocal cords. No vocal cord pathology was noted. An &O-mm oral endotracheal tube was inserted atraumatically and did not contact the malformation. Anesthesia was maintained through a semiclosed circuit with O,, nitrous oxide, and isoflurane. Gas concentrations were monitored by mass spectrometry. A second 16-gauge catheter was inserted into the dorsum of the left hand to ensure adequate venous access. A full train-of-four was noted by twitch monitor 10 minutes after induction. The intraoperative course was uneventful. After the tooth was extracted, the anesthetic drugs were discontinued and the patient was allowed to wake up breathing 100% 0,. During emergence, the patient experienced some coughing and bucking. Once he was awake and following complex commands and exhibiting voluntary motion of all extremities, such as pointing to the endotracheal tube to indicate his desire to be extubated, the patient was extubated. Extubation was immediately followed by complete airway obstruction. The problem was not relieved by continuous positive airway pressure (CPAP) or by gentle placement of an oral airway. Succinylcholine 10 mg IV was administered to re-
lieve presumed laryngospasm. This maneuver did not relieve the obstruction, and the patient was given thiopental sodium 200 mg IV and succinylcholine 100 mg IV to facilitate reintubation. In addition, a cricothyroidotomy kit was readied. SpO, decreased into the 30% range for approximately 30 seconds. Laryngoscopy during reintubation showed that the vascular malformation had enlarged and extended between the vocal cords, which created a ballvalve type of obstruction. The malformation was gently and easily moved aside without causing a hemorrhage, and an &O-mm oral endotracheal tube (smaller sizes were readily available) was passed easily between the cords. Anesthesia was maintained with 0, and isoflurane. An otolaryngologist was called to the OR to perform an emergent tracheostomy if needed. The patient was placed in a semisitting position for approximately 30 minutes to facilitate venous drainage of the malformation. Anesthesia was then discontinued. When the patient had completely awakened, he was placed supine temporarily in a right lateral decubitus position to allow the ball-valve portion of the malformation to fall out and away from the trachea. Although this position change was likely to increase engorgement, we thought that the lateral head-flat position might keep the malformation from falling between the vocal cords. The trachea was extubated for a second time, and this was again followed by complete obstruction of the airway unresponsive to placement of an oral airway, CPAP, or head elevation. Atropine 1.2 mg IV, thiopental sodium 200 mg IV, and succinylcholine 120 mg IV were administered, and the patient was intubated for the third time without difficulty. Direct laryngoscopy showed a further marked increase in the size of the malformation despite the attempts to enhance venous drainage. A formal tracheostomy was performed without difficulty under O,-isoflurane anesthesia. The patient had an uneventful recovery and postoperative course without evidence of neurologic compromise. A computerized tomographic (CT) scan of the head and neck, done a month later at follow-up, showed an extensive vascular malformation of the right oropharynx, floor of the mouth, and submandibular and sublingual regions, which extended into the nasopharynx cephalad and deep into the neck and displaced the carotid artery and jugular vein medially and posteriorly. No surgical or chemotherapeutic intervention was considered possible. The tracheostomy was removed without difficulty, and the patient has since resumed normal activities.
Discussion Many inconsistent, confusing terms are used in the literature to describe vascular abnormalities in the region of the face. A system developed in 1982 describes these abnormalities according to growth rate and endothelial cell characteristics.’ A hemangioma is a vascular neoplasm occurring in infancy. It may be present at birth but usually is not noticed until early infancy. This type J. Clin. Anesth., vol. 4, November/December
1992
499
&se
Conference
of lesion grows rapidly during the first year of life and then slowly involutes. It does not involve adjacent bony structures. Microscopically, a hemangioma is characterized by increased endothelial turnover and increased numbers of mast cells.2 Hemangiomas are the most common tumors of the head and neck in infants and young children,3,4 and affect females two to three times more often than males5 The two most common histologic forms of hemangiomas are capillary and cavernous.*Jj A capillary hemangioma is comprised predominantly of vessels that have the caliber of normal capillaries. A cavernous hemangioma is made up of much larger vascular channels. A cavernous hemangioma, which is usually more extensive and extends deeper into subcutaneous tissues, is soft, elevated, poorly circumscribed, easily compressible, and purplish blue in color. Mixed capillary-cavernous hemangiomas are common. A hemangioma can occur in any tissue, but the skin and mucous membranes are the most common locations.6,7 Hemangiomas of the face and neck account for over half of all these malformations in humans,’ the most frequent sites being the mucous membranes of the tongue and lips and the less frequent sites being the larynx, palate, and trachea. Subglottic hemangiomas in infants account for 85% of all hemangiomas in this age group.’ The hemangioma characteristically enlarges during the first year of life and then undergoes some degree of spontaneous regression. Many will have virtually disappeared by the time the child reaches 5 years of age.4 For this reason, allowing the patient time to outgrow a hemangioma without medical or surgical management is the initial approach advocated.4 Active treatment is indicated only if the hemangioma interferes with the function of vital organs, compromises the airway, or ulcerates with hemorrhage or infection or if there is an accompanying thrombocytopenia. Depending on any of these conditions, several forms of treatment have been advocated (Table 1).4-10 The other major type of vascular abnormality is called a vascular malformation. This type of malformation is generally present at birth and grows not like a neoplasm but rather at the same rate as normal human growth. Malformations are commonly associated with skeletal abnormalities in surrounding bones and may expand with hormonal changes, infection, or trauma.2 Vascular malformations may be subdivided based on the type of vessels involved: capillary, venous, lymphatic, arterial, or arteriovenous. Capillary, venous, or lymphatic malforTable 1.
Treatment
Options for Airway Hemangioma Option
Treatment Strategy
Noninvasive
Invasive
500
Sclerosing drugs Corticosteroids Systemic Intralesional Tracheostomy (if airway not involved) Cryosurgery Carbon dioxide laser Surgical excision
J. Clin. Anesth.,
vol. 4, November/December
1992
Table 2.
Management
Area of Concern
Anesthesia
Monitoring Positioning General
of an Airway Venous
Malformation
Strategy
Regional technique Mask ventilation if possible Hypotensive anesthesia for resection Prevention of coughing or straining as associated with extubation Adequate venous access Position associated with good venous drainage Minimal manipulation or injury to malformation
mation is a low-flow condition, and arterial or arteriovenous malformation is a high-flow condition. Most vascular malformations of the maxillofacial region are venous.2 The distinction between the types of vascular abnormalities is very important to anesthesiologists. A hemangioma generally can be resected and separated from surrounding tissue without danger of exsanguination. A vascular malformation, however, involves all the blood vessels in a given area of the body. Complete resection is generally not possible without completely removing the part of the body involved. Bleeding during surgical resection or after trauma is commonly uncontrollable. Anesthesiologists must be concerned with any type of vascular abnormality involving the airway. Patients with such a problem may present for any of the treatment options that require anesthesia, or, as in our case, a malformation may be a compounding factor during an unrelated surgery. The anesthetic-related problems involve obstruction of, or hemorrhage into, the airway. Airway obstruction may be present preoperatively or may develop later during anesthesia.s,7 Such obstruction is probably more likely with a venous malformation, which may engorge rapidly as venous pressure increases, as with the head-down position, coughing, or straining. Minor injury to a malformation may cause massive bleeding into the respiratory tract. In patients with a large malformation involving the tongue and pharynx, visualizing glottic structures and orotracheal intubation may be difficult. With the subglottic variety of hemangioma common in infants, blood may be aspirated into the tracheobronchial tree during or after tracheal intubation. Postoperative swelling of an intraoral hemangioma following a hysterectomy has been reported.5 Anesthesia for a patient with an airway vascular abnormality includes, when possible, a regional technique (Table Z), which obviates manipulation of the airway and possible vascular-related complications from airway management. Adequate intravenous access must be established before a vascular malformation is manipulated in any way. There are several case reports of exsanguination after minimal manipulation of large venous malformations, as may occur during tracheal intubation.7,‘0,11 When a patient with an airway vascular abnormality re-
Airway malformafzon: Howton and Mahla
quires general anesthesia, mask ventilation may be a viable alternative if the abnormality does not obstruct the airway and no other factors mandate endotracheal intubation, as occurred in the current case. The use of induced hypotensive anesthesia to reduce blood loss during resection of a vascular malformation is well described and may be useful during elective resection.12 Prevention of perioperative coughing and straining may reduce the likelihood of enlargement of the malformation, but enlargement has been reported even when coughing or straining does not occur.5 Our patient experienced coughing during emergence. After the first extubation failed, it became apparent during the successful reintubation that the venous malformation had increased 25% in size and was compromising his airway by extending through the cords as a ball-valve. After reestablishing airway patency, we discussed with our surgical colleagues the risks of tracheostomy to secure the postoperative airway compared with the risks of a second trial of extubation. The latter option was chosen. If a position change did not prevent the malformation from falling between the vocal cords, the risks of a second trial of extubation included airway obstruction, subsequent inability to reintubate, and emergency tracheostomy. The risks of tracheostomy included infection, inadvertent violation of a hemangioma with possible exsanguinating blood loss, and inadvertent postoperative decannulation with airway obstruction and possible inability to reestablish the airway in time to prevent neurologic morbidity. We considered the risks of the latter great enough to first attempt extubation with the patient placed in the right lateral decubitus head-neutral position after a period in the semisitting position to facilitate venous drainage. The supine lateral position was chosen to help keep the malformation from acting as a ball-valve obstruction again. To minimize trauma to the malformation, we did not perform direct laryngoscopy before the final extu-
bation. After the second extubation, the ensuing cotiplete airway obstruction was not relieved by the lateral or head-up position or by CPAP. Visualization of the airway during reintubation showed that further swelling of the malformation caused an approximate 95% compromise of the patient’s airway. The malformation was easily and atraumatically moved aside for insertion of the endotracheal tube. The tracheostomy and postoperative course were uneventful. Airway vascular abnormalities are not commonly encountered in anesthetic practice. When a patient with an intraoral or airway vascular abnormality requires anesthesia and surgery, the anesthesiologist must know the extent of the abnormality and the airway structures involved. Although no clear preference for laryngoscope blades can be recommended, if the lesion involves the epiglottis, a Macintosh blade may be preferable because it can avoid direct contact with the epiglottis. If the base of the tongue is involved, a straight blade may reduce the amount of direct contact with the lesion. If the lesion is located more on one side or the other, a right- or leftsided approach with the appropriate blade may be useful. A preoperative CT scan would have better elucidated the extent of airway involvement in our patient but, in retrospect, would not change our anesthetic management, because the malformation primarily involved the nasopharynx, oropharynx, and deep neck, ending 2 cm above the vocal cords. We would not necessarily have predicted the postextubation swelling based on the CT scan. In spite of this, however, we strongly recommend a preoperative CT scan to elucidate the extent of real or potential airway compromise. Because swelling of a venous malformation will likely occur during events such as coughing and straining, which are commonly associated with extubation, if the malformation involves structures in the vicinity of the glottis, prolonged postoperative intubation in the sitting position to facilitate drainage may help prevent the problems we encountered.
Comment Dr. Seagle: This report describes an interesting and challenging case of mechanical airway obstruction secondary to dilatation of a vascular malformation in the upper airway that occurred during anesthesia for an oral surgical procedure. That the obstructing portion of the vascular malformation was located proximal to the laryngeal inlet and that the obstruction was caused by a ball-valve prolapse of tissue into the glottis is interesting and noteworthy. That the other feared complication of airway malformation-that is, uncontrollable hemorrhage-did not occur is indeed fortunate. As Drs. Howton and Mahla note, vascular malformations can be classified according to histologic characteristics.’ Hemangioma qualifies as a proliferative neoplastic malformation that undergoes at least some degree of spontaneous involution, often total. Vascular malformations, however, show mature nonproliferative
endothelium. Their growth is commensurate with that of the growing child, and they characteristically do not involute. They may enlarge as a result of “changes in pressure and flow, ectasia, collateral formation, shunting and hormonal modulation.“ll Hemangioma and vascular malformation usually can be differentiated on the basis of careful history and physical findings; arteriography may be helpful. This differentiation is helpful in developing a prognosis, in planning therapy, and in predicting the behavior of a given malformation under circumstances such as those seen in the current case. In one study,” most vascular abnormalities in the maxillofacial region were malformations (usually venous). In this light, the substantial dilatation of the malformation in this patient was understandable. Most likely, the abnormality represented a venous malformation. The precise mechanism of the airway obstrucJ. Clin. Anesth., vol. 4, November/December 1992
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tion-that is, the ball-valve prolapse mto the glottisshould be anticipated with these lesions. Further, differentiating between the two varieties of vascular malformations should improve prediction of the effect of a given malformation as well as its response to treatment. In the case presented here, the anesthetic and postanesthetic care would seem to be appropriate for any similar vascular malformation in the airway.
References Mulliken JB, Glowacki J: Hemangiomas and vascular malformations in infants and children: a classification based on endothelial characteristics. Plast Reconst Surg 1982;69:412-22. Kaban LB, Mulliken JB: Vascular anomalies of the maxillofacial region. J Oral Maxillofac Surg 1986;44:203-13. Wilson RD, Putnam L, Phillips MT, Phillips CT: Anesthetic problems in surgery for varying levels of respiratory obstruction in infants and children. Anesth Analg 1974;53:878-85. Hawkins DB, Crockett DM, Kahlstrom EJ, MacLaughlin EF: Corticosteroid management of airway malformations: long-term follow-up. Laryngoscope 1984;94:633-7.
laryngeal hemangioma. Ann 5. Benjamin B, Carter P: Congenital ($01 Rhino1 Laryngol 1983;92:448-55. of congenital heman6. Brown DA, Smith JD: Late complication gioma of the tongue. Head Neck Surg 1987;9:299-304. 7. Divekar VM, Kavadia IP, Raksha H: A massive hemangioma of the upper airway. Anaesthesia 1982;37:93-4. MT: Surgical 8. Morgan RF, Horowitz JH, Wanebo HJ, Edgerton management of vascular malformations of the head and neck. Am J Surg 1986;152:424-9. embolization by the so9. Wozniak M, Sadowski A: Intravascular called balloonization method in the treatment of cerebral arteriovenous malformations. Neural Neurochir Pol 198 1; 15: 18 l9. of pharyngocervical angiomatosis 10. Rethi A: On the therapy (pharyngeal hemangioma), of other hemangiomas of the upper airways and of the nevus flammeus. HNO 1965; 13:2958. in dental surgery. Schweirerische Mon11. Maeglin B: Emergencies atsschrift Zahnheilkunde 1974;84:964-76. for hypotensive anaesthesia. In: Enderby 12. Beare R: Indications GEH, ed. Hypotensive Anaesthesiu. Edinburgh: Churchill Livingstone, 1985;99-108.
Vascular Anomalies of the Maxillofacial Region Leonard B. Kaban, D.M.D., M.D. and John B. M&liken, M.D. University of California, San Francisco CA Summary A biologic classification based on clinical behavior and endothelial cell characteristics is used to divide vascular birthmarks into two groups: hemangiomas and vascular malformations. This system is applied to vascular anomalies of the maxillofacial region and is demonstrated with illustrative cases. The distinction between hemangioma and vascular malformation is important for maxillofacial surgeons because hemangiomas rarely affect bone, whereas malformations affect bone in 35% of cases. Reproduced with 1986;44:203-13.
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