•
•
Rhinocerebral Mucormycosis Ronald B. Addleslone,
Neuroradiology
1
M.D., 2 and George J. BayUn, M.D.
Nine cases of rhinocerebral mucormycosis are reviewed. Eight patients had diabetes and 7 had symptoms related to the orbit. Roentgen analysis of focal bone destruction and uniform mucosal thickening will frequently suggest the diagnosis. Mucormycosis should be suspected in the diabetic patient with destruction of the walls of the bony sinuses, especially when multiple sinus involvement suggests an etiology other than neoplasm. INDEX TERMS:
Diabetes. Mucormycosis • Nose, diseases • Orbit • Sinuses, paranasal
Radiology 115:113-117, April 1975
mucormycosis is a devastating and frequently fatal disease in which accurate roentgen analysis will often suggest the diagnosis. Only with early recognition and aggressive therapy have increasing numbers of survivors been reported. We wish to report 9 patients with rhinocerebral mucormycosis seen at Duke University Medical Center during the past 10 years, providing a basis for suggested radiographic findings.
demonstrated an abnormal glucose tolerance level during evaluation for mucormycosis. Seven had symptoms related to the orbit (sudden blindness or periorbital cellulitis). Of the 6 patients alive following their illness, none experienced central nervous system symptoms, Three patients died of central nervous system disease (internal carotid occlusion, cavernous sinus thrombosis, and mycotic aneurysm). Treatment involved radical surgical excision and drainage and amphotericin B.
CASE MATERIAL (TABLE I)
ROENTGEN FINDINGS
There were 5 males and 4 females ranging in age from 11 to 69 years at the time the disease was recognized. Eight of the 9 were either known diabetics or
The roentgen hallmark of mucormycosis is focal bone destruction. Although small areas of the sinus margin are usually destroyed (Figs. 1 and 2, A), several
R
HINOCEREBRAL
Table I: Case No.
Age and Sex
I. (D.P.)
63F
II. (H.H.)
52M
in, (0.0.)*
11M
IV. (M.C.)
53F
V. (G.D.)
69M
VI. (L.W.)
16M
VII. (O.E.)
41M
VIII. (G.M.)
22F
IX. (V.H.)
43F
Presenting Symptom Sudden blindness Chronic sinusitis Periorbital cellulitis Oral/antral fistula Sudden blindness Sudden blindness Sudden blindness Periorbital cellulitis Periorbital cellulitis
Summary of Case Material Area of Bony Destruction Ethmoid, maxillary Ethmoid, maxillary Ethmoid, maxillary Ethmoid, maxillary, nasal Ethmoid, nasal Ethmoid, maxillary Ethmoid, maxillary Ethmoid, maxillary Ethmoid, maxillary, pterygoid fossa
Diabetes Yes
Central Nervous System Involvement
Survival
Internal carotid occlusion No
No
No
Yes
Intracranial mycotic aneurysm No
Yes
No
No
Yes
Yes
No
Yes
Yes Yes
Yes Yes Yes
Cavernous sinus thrombosis No No
Yes
No Yes Yes
* Case III was reported previously (8). 1
2
From the Department of Radiology, Duke University Medical Center, Durham, N. C. Accepted for publication in September 1974. Present address: St. Thomas Hospital, Nashville, Tenn.
113
sjh
114
RONALD B. ADDLESTONE AND GEORGE J. BAYLiN
April 1975
aggressiveness of the disease in each case. Pure destruction of bone without apparent repair was seen in most cases which were rapidly progressive, while increased bone density adjacent to destruction was present in patients with more chronic disease. Accompanying the bony destruction was uniform or septate thickening of the mucosal lining varying from mild thickening to total sinus opacification. One patient had an air-fluid level in the maxillary sinus on the upright examination (Fig. 6, B). The disease was generally unilateral. Bilateral changes were occasionally noted in the ethmoid sinuses only (Fig. 6, A), but one side still predominated. The ethmoid sinuses were involved in every case. In 8 of the 9 patients, the maxillary sinus or the inferior orbital wall was involved. In 2 patients the nasal bones were destroyed, with external deformity. One patient had extension into the pterygoid space. Sphenoid or frontal sinus disease was not noted, nor could any correlation be made between the sinuses involved and the presenting symptoms. Fig. 1. This 52-year-old diabetic man presented with chronic sinusitis. Septate mucosal thickening is present in the left maxillary sinus, with more uniform mucosal thickening in the right maxillary antrum. There is bilateral destruction of the orbital floor as well as the left ethmoid.
cases of more marked bony loss were noted (Figs. 2, B and 3, A). Lesser degrees of bony destruction were evaluated best by conventional examination of the ethmoid sinuses. Tomography was helpful in evaluation of the maxillary sinuses (Fig. 3, B) as well as areas not readily seen on conventional radiographs (Fig. 4, B). The turbinates were commonly involved, and the nasal bones were sometimes partially destroyed as well (Figs. 3, A and 5, B). The appearance of the bony destruction reflected the
DISCUSSION
Human mucormycosis is caused by fungi of the species Mucor and Rhizopus (class Phycomycetes). These fungi are commonly called bread molds or sugar fungi and may be found in decaying animal or vegetable matter or in the soil. In tissue, the fungus can be recognized by its broad, branching, nonseptate hyphae (1, 7, 17, 24). Pathologically, these fungi show an affinity for the walls of vessels (9, 24). Necrosis, perivascular inflammatory changes, and involvement of neural elements and lymphatics are seen (21). Regardless of which of the four major clinical types of mucormycosis (rhinocerebral, pulmonary, alimentary, or disseminated)
Fig. 2. A. Focal ethmoid bone destruction (arrow) with mild mucosal thickening in an 11-year-old diabetic boy with orbital cellulitis. B. The Waters view demonstrates almost total opacification of the left maxillary sinus and destruction of the orbital floor.
Vol. 115
RHINOCEREBRAL MUCORMYCOSIS
115
Neuroradioloq;
Fig. 3. 53-year-old woman with an oral-antral fistula. A. Extensive ethmoid bone destruction is present, along with similar changes extending to the nasal bones. B. Tomogram showing left maxillary bone destruction. The uniform mucosal reaction is clearly seen, in contrast to the nodular mucosal thickening reported previously.
Fig. 4. Symptoms of sinusitis and periorbital swelling were present in this 43-year-old diabetic woman. A. The left infraorbital foramen is destroyed (arrow), along with adjacent bone loss and uniform mucosal thickening. B. Tomogram shows destruction of the pterygoid canal (arrowhead) and bony 1055 due to extension into the pterygopalatine fossa.
is present, thrombosis of vessels is a common feature (16, 24). Early vascular invasion may account for the degree of bony destruction seen (12, 24). Mucor and Rhizopus are normally saprophytic and may be found in nasal, throat, and stool cultures. Under favorable conditions they become opportunistic. Certain diseases predispose to this situation. Poorly controlled diabetes, especially when associated with acidosis, is most commonly seen with rhinocerebral mucormycosis (10). Patients debilitated by conditions such as lymphoma, leukemia, sepsis, tuberculosis, severe burns, or the use of antibiotics, steroids, or antimetabolites are prone to pulmonary, alimentary, or more widespread involvement (1,3, 10, 17).
The high incidence of diabetes with rhinocerebral mucormycosis has provided a starting point for investigation of pathogenesis. Studies suggest that either hyperglycemia or subsequent acidosis may derange the metabolism of the host, allowing the fungus to proliferate (4, 17, 24). The spores probably originate in the upper respiratory tract, proliferate in the sinuses, and extend to the brain via blood vessels or directly through the sinus walls (1, 24, 25). Clinically, the patient presents with sinus, eye, or progressive neurological changes. Proptosis, diplopia, ophthalmoplegia, blindness, and cranial nerve involvement (especially the fifth and seventh cranial nerves) are common (1-3, 5-16, 18-26). Progressive neurolog-
116
RONALD B. ADDLESTONE AND GEORGE
J.
BAYLIN
April 1975
Fig. 5. A. Focal bone destruct ion in the right ethmoid sinus (arrow) and uniform mucosal thicken ing can be seen on the initial examination in this 69-year-old man who presented with sudden blindness. B. Nasal bone destruct ion is also present.
Fig. 6. This 16-year-old diabetic boy presented with sudden blindness. A. Bilateral focal bone destruction and mucosal thickening are present, more marked in the left ethmoid. B. An air-fluid level is present in the right maxillary sinus on the upright Waters view.
ical deterioration rapidly occurs as central nervous system infection spreads, followed by vascular thrombosis (1, 2, 8, 12, 16, 19, 21, 25, 26). The craniofacial findings on physical examination relate to vascular thrombosis (11). Mucosal necrosis or even necrosis of the hard palate and sinus walls can be seen (3). With blindness, the eye is often avascular secondary to occlusion of the ophthalmic artery (6). The diagnosis requires a high degree of suspicion to permit early therapy. Urgency in diagnosis is extremely important, as the disease is rapidly progressive. Ultimately a biopsy or smear cern-
onstrating nonseptate hyphae is needed (21), but radiological examination can demonstrate rather suggestive changes . The roentgen findings in mucormycosis have been described by Green et al. in the only previous report in the radiology literature (12). Spotty bony destruction is the hallmark of craniofacial mucormycosis. However, the present series demonstrates that more extensive bone destruction of the sinus walls or facial structures can also occur, as well as sclerotic changes in more chronic cases. Mucosal thickening was uniform along
Vol. 115
RHINOCEREBRAL MUCORMYCOSIS
the sinus wall, with a septate appearance in several cases. The inner margins were smooth and the previously described nodularity was not seen. In one case, an air-fluid level was present on the upright examination, a finding not reported previously. Although only ethmoid and maxillary sinus disease was observed in this series, frontal sinus (14, 15) and sphenoid (9) mucormycosis have also been reported. Patients with acute suppurative sinusitis also demonstrate uniform mucosal thickening coupled with either bony resorption or no bone changes; an air-fluid level is often present. However, in mucormycosis bone destruction is focal, in contrast to the more uniform demineralization seen in acute sinusitis. In chronic pyogenic sinusitis, dense mucosal thickening is seen, but there is bony sclerosis rather than destruction. Even the unusual case of mucormycosis with bony sclerosis will also involve destruction of bone. However, if pyogenic osteomyelitis is associated with chronic sinusitis, differentiation from mucormycosis may be impossible. The major differential diagnosis is between mucormycosis and neoplasm. Sinus opacification, mucosal thickening, and bony destruction are common to both conditions. Benign tumors may produce a smooth pressure atrophy or deformity of the bone, in contrast to the irregular or spotty localized changes seen in malignant tumors or mucormycosis. In addition, in malignant tumors an area of destruction is usually seen adjacent to the lesion. In the occasional case of a tumor producing more spotty bone destruction or mucormycosis with more extensive destruction, differentiation may be impossible. Involvement of multiple sinuses would favor mucormycosis. Also, the clinical presentation may vary; mucormycosis may be acute in onset, and the patient is more acutely ill than with neoplasm. The radiologist should exercise a high degree of suspicion in arriving at the diagnosis, particularly in a patient with a history of diabetes and roentgen changes suggestive of sinusitis or tumor, so that therapy can be instituted before the central nervous system is involved.
SUMMARY The presence, type, and location of bony destruction are important in the diagnosis of rhinocerebral mucormycosis. Bony destruction favors mucormycosis rather than acute or chronic sinusitis. Spotty, more diffuse bone destruction coupled with multiple sinus involvement suggests mucormycosis rather than malignant tumor. Although roentgen differentiation may be impossible, a high degree of suspicion is necessary to facilitate early diagnosis.
REFERENCES 1. Abramson E, Wilson B, Arky RA: Rhinocerebral phycomycosis in association with diabetic ketoacidosis. Report of two cases
117
Neuroradiology
and a review of clinical and experimental experience with amphotericin B therapy. Ann Intern Med 66:735-742, Apr 1967 2. Baker RD: Mucormycosis-a new disease? JAMA 163: 805-808,9 Mar 1957 3. Battock DJ, Grausz H, Bobrowsky M, et al: Alternate-day amphotericin B therapy in the treatment of rhinocerebral phycomycosis (mucormycosis). Ann Intern Med 68: 122-137, Jan 1968 4. Bauer H, Flanagan JF, Sheldon WH: The effects of metabolic alterations on experimental Rhizopus oryzae (mucormycosis) infection. Yale J Bioi Med 29:23-32, Sep 1956 5. Baum JL: Rhino-orbital mucormycosis. Occurring in an otherwise apparently healthy individual. Am J Ophthalmol 63:335-339, Feb 1967 6. Bentwich Z, Rosen Z, Ganor S, et al: Chronic rhinocerebral mucormycosis (phycomycosis) with occlusion of left internal carotid artery. lsr J Med Sci 4:977-981, Sep-Oct 1968 7. Bergstrom L, Hemenway WG. Barnhart RA: Rhinocerebral and otologic mucormycosis. Ann Otol Rhinol LaryngoI79:70-81, Feb 1970 8. Blodi FC, Hannah FT, Wadsworth JAC: Lethal orbito-cerebral phycomycosis. In otherwise healthy children. Am J Opthalmol 67:698-705, May 1969 9. Case records of the Massachusetts General Hospital. Case 48-1968. N Engl J Med 279:1220-1229,28 Nov 1968 10. DeWeese DD, Schleuning AJ II, Robinson LB: Mucormycosis of the nose and paranasal sinuses. Laryngoscope 75: 13981407, Sep 1965 11. Ginsberg J, Spaulding AG, Laing va: Cerebral phycomycosts (mucormycosis) with ocular involvement. Am J Ophthalmol 62:900~906, Nov 1966 12. Green WH, Goldberg HI, Wohl GT: Mucormycosis infection of the craniofacial structures. Am J Roentgenol 101:802-806, Dec 1967 13. Groote CA: Rhinocerebral phycomycosis. Arch Otolaryngol 92:288-292, Sep 1970 14. Heitzman ER, Ferguson JH: Pain and swelling about eye. NY State J Med 69: 1059-1 061, 15 Apr 1969 15. Kaufman RS, Stone G: Osteomyelitis of frontal bone secondary to mucormycosis. NY State J Med 73: 1325- 1328, 1 Jun 1973 16. La Touche CJ, Sutherland TW, Telling M: Rhinocerebral mucormycosis. Lancet 2:811-813, 19 Oct 1963 17. McBride RA, Corson JM, Dammin GJ: Mucormycosis. Two cases of disseminated disease with cultural identification of Rhizopus; review of literature. Am J Med 28:832-846, May 1960 18. Prockop LD, Silva-Hutner M: Cephalic mucormycosis (phycomycosis). A case with survival. Arch Neurol 17:379-386, Oct 1967 19. Reeves DL, Dickson DR, Benjamin El: Phycomycosis (mucormycosis) of the central nervous system. J Neurosurg 23:82-84, Jul 1965 20. Sandler R, Tallman CB, Keamy DG, et at; Successfully treated rhinocerebral phycomycosis in well controlled diabetes. N EnglJ Med 285:1180-1182,18 Nov 1971 21. Smith HW, Kirchner JA: Cerebral mucormycosis. A report of three cases. Arch Otolaryngol 68:715-726, Dec 1958 22. Stephan T, Busis SN, Arena S, et al: Rhinocerebral phycomycosis (mucormycosis). Laryngoscope 83:173-178, Feb 1973 23. Stevens KM, Newell Re, Bergstrom L: Mucormycosis in a patient receiving Azathioprine. Arch Otolaryngol 96:250-251, Sep 1972 24. Straatsma BA, Zimmerman LE, Gass JDM: Phycomycosis. A clinicopathologic study of fifty-one cases. Lab Invest 11:963-985, Nov 1962 25. Taylor CG, Alexander RE, Green WH, et al: Mucormycosis (phycomycosis) involving the maxilla. Report of a case with survival. Oral Surg 27:806-822, Jun 1969 26. Weisskopf A: Mucormycosis-a rhinologic disease. Ann Otol Rhinol Laryngol 73:16-23, Mar 1964 St. Thomas Hospital 4220 Harding Rd. Nashville, Tenn. 37205
•
Rhinocerebral Mucormycosis Ronald B. Addleslone,
Neuroradiology
1
M.D., 2 and George J. BayUn, M.D.
Nine cases of rhinocerebral mucormycosis are reviewed. Eight patients had diabetes and 7 had symptoms related to the orbit. Roentgen analysis of focal bone destruction and uniform mucosal thickening will frequently suggest the diagnosis. Mucormycosis should be suspected in the diabetic patient with destruction of the walls of the bony sinuses, especially when multiple sinus involvement suggests an etiology other than neoplasm. INDEX TERMS:
Diabetes. Mucormycosis • Nose, diseases • Orbit • Sinuses, paranasal
Radiology 115:113-117, April 1975
mucormycosis is a devastating and frequently fatal disease in which accurate roentgen analysis will often suggest the diagnosis. Only with early recognition and aggressive therapy have increasing numbers of survivors been reported. We wish to report 9 patients with rhinocerebral mucormycosis seen at Duke University Medical Center during the past 10 years, providing a basis for suggested radiographic findings.
demonstrated an abnormal glucose tolerance level during evaluation for mucormycosis. Seven had symptoms related to the orbit (sudden blindness or periorbital cellulitis). Of the 6 patients alive following their illness, none experienced central nervous system symptoms, Three patients died of central nervous system disease (internal carotid occlusion, cavernous sinus thrombosis, and mycotic aneurysm). Treatment involved radical surgical excision and drainage and amphotericin B.
CASE MATERIAL (TABLE I)
ROENTGEN FINDINGS
There were 5 males and 4 females ranging in age from 11 to 69 years at the time the disease was recognized. Eight of the 9 were either known diabetics or
The roentgen hallmark of mucormycosis is focal bone destruction. Although small areas of the sinus margin are usually destroyed (Figs. 1 and 2, A), several
R
HINOCEREBRAL
Table I: Case No.
Age and Sex
I. (D.P.)
63F
II. (H.H.)
52M
in, (0.0.)*
11M
IV. (M.C.)
53F
V. (G.D.)
69M
VI. (L.W.)
16M
VII. (O.E.)
41M
VIII. (G.M.)
22F
IX. (V.H.)
43F
Presenting Symptom Sudden blindness Chronic sinusitis Periorbital cellulitis Oral/antral fistula Sudden blindness Sudden blindness Sudden blindness Periorbital cellulitis Periorbital cellulitis
Summary of Case Material Area of Bony Destruction Ethmoid, maxillary Ethmoid, maxillary Ethmoid, maxillary Ethmoid, maxillary, nasal Ethmoid, nasal Ethmoid, maxillary Ethmoid, maxillary Ethmoid, maxillary Ethmoid, maxillary, pterygoid fossa
Diabetes Yes
Central Nervous System Involvement
Survival
Internal carotid occlusion No
No
No
Yes
Intracranial mycotic aneurysm No
Yes
No
No
Yes
Yes
No
Yes
Yes Yes
Yes Yes Yes
Cavernous sinus thrombosis No No
Yes
No Yes Yes
* Case III was reported previously (8). 1
2
From the Department of Radiology, Duke University Medical Center, Durham, N. C. Accepted for publication in September 1974. Present address: St. Thomas Hospital, Nashville, Tenn.
113
sjh
114
RONALD B. ADDLESTONE AND GEORGE J. BAYLiN
April 1975
aggressiveness of the disease in each case. Pure destruction of bone without apparent repair was seen in most cases which were rapidly progressive, while increased bone density adjacent to destruction was present in patients with more chronic disease. Accompanying the bony destruction was uniform or septate thickening of the mucosal lining varying from mild thickening to total sinus opacification. One patient had an air-fluid level in the maxillary sinus on the upright examination (Fig. 6, B). The disease was generally unilateral. Bilateral changes were occasionally noted in the ethmoid sinuses only (Fig. 6, A), but one side still predominated. The ethmoid sinuses were involved in every case. In 8 of the 9 patients, the maxillary sinus or the inferior orbital wall was involved. In 2 patients the nasal bones were destroyed, with external deformity. One patient had extension into the pterygoid space. Sphenoid or frontal sinus disease was not noted, nor could any correlation be made between the sinuses involved and the presenting symptoms. Fig. 1. This 52-year-old diabetic man presented with chronic sinusitis. Septate mucosal thickening is present in the left maxillary sinus, with more uniform mucosal thickening in the right maxillary antrum. There is bilateral destruction of the orbital floor as well as the left ethmoid.
cases of more marked bony loss were noted (Figs. 2, B and 3, A). Lesser degrees of bony destruction were evaluated best by conventional examination of the ethmoid sinuses. Tomography was helpful in evaluation of the maxillary sinuses (Fig. 3, B) as well as areas not readily seen on conventional radiographs (Fig. 4, B). The turbinates were commonly involved, and the nasal bones were sometimes partially destroyed as well (Figs. 3, A and 5, B). The appearance of the bony destruction reflected the
DISCUSSION
Human mucormycosis is caused by fungi of the species Mucor and Rhizopus (class Phycomycetes). These fungi are commonly called bread molds or sugar fungi and may be found in decaying animal or vegetable matter or in the soil. In tissue, the fungus can be recognized by its broad, branching, nonseptate hyphae (1, 7, 17, 24). Pathologically, these fungi show an affinity for the walls of vessels (9, 24). Necrosis, perivascular inflammatory changes, and involvement of neural elements and lymphatics are seen (21). Regardless of which of the four major clinical types of mucormycosis (rhinocerebral, pulmonary, alimentary, or disseminated)
Fig. 2. A. Focal ethmoid bone destruction (arrow) with mild mucosal thickening in an 11-year-old diabetic boy with orbital cellulitis. B. The Waters view demonstrates almost total opacification of the left maxillary sinus and destruction of the orbital floor.
Vol. 115
RHINOCEREBRAL MUCORMYCOSIS
115
Neuroradioloq;
Fig. 3. 53-year-old woman with an oral-antral fistula. A. Extensive ethmoid bone destruction is present, along with similar changes extending to the nasal bones. B. Tomogram showing left maxillary bone destruction. The uniform mucosal reaction is clearly seen, in contrast to the nodular mucosal thickening reported previously.
Fig. 4. Symptoms of sinusitis and periorbital swelling were present in this 43-year-old diabetic woman. A. The left infraorbital foramen is destroyed (arrow), along with adjacent bone loss and uniform mucosal thickening. B. Tomogram shows destruction of the pterygoid canal (arrowhead) and bony 1055 due to extension into the pterygopalatine fossa.
is present, thrombosis of vessels is a common feature (16, 24). Early vascular invasion may account for the degree of bony destruction seen (12, 24). Mucor and Rhizopus are normally saprophytic and may be found in nasal, throat, and stool cultures. Under favorable conditions they become opportunistic. Certain diseases predispose to this situation. Poorly controlled diabetes, especially when associated with acidosis, is most commonly seen with rhinocerebral mucormycosis (10). Patients debilitated by conditions such as lymphoma, leukemia, sepsis, tuberculosis, severe burns, or the use of antibiotics, steroids, or antimetabolites are prone to pulmonary, alimentary, or more widespread involvement (1,3, 10, 17).
The high incidence of diabetes with rhinocerebral mucormycosis has provided a starting point for investigation of pathogenesis. Studies suggest that either hyperglycemia or subsequent acidosis may derange the metabolism of the host, allowing the fungus to proliferate (4, 17, 24). The spores probably originate in the upper respiratory tract, proliferate in the sinuses, and extend to the brain via blood vessels or directly through the sinus walls (1, 24, 25). Clinically, the patient presents with sinus, eye, or progressive neurological changes. Proptosis, diplopia, ophthalmoplegia, blindness, and cranial nerve involvement (especially the fifth and seventh cranial nerves) are common (1-3, 5-16, 18-26). Progressive neurolog-
116
RONALD B. ADDLESTONE AND GEORGE
J.
BAYLIN
April 1975
Fig. 5. A. Focal bone destruct ion in the right ethmoid sinus (arrow) and uniform mucosal thicken ing can be seen on the initial examination in this 69-year-old man who presented with sudden blindness. B. Nasal bone destruct ion is also present.
Fig. 6. This 16-year-old diabetic boy presented with sudden blindness. A. Bilateral focal bone destruction and mucosal thickening are present, more marked in the left ethmoid. B. An air-fluid level is present in the right maxillary sinus on the upright Waters view.
ical deterioration rapidly occurs as central nervous system infection spreads, followed by vascular thrombosis (1, 2, 8, 12, 16, 19, 21, 25, 26). The craniofacial findings on physical examination relate to vascular thrombosis (11). Mucosal necrosis or even necrosis of the hard palate and sinus walls can be seen (3). With blindness, the eye is often avascular secondary to occlusion of the ophthalmic artery (6). The diagnosis requires a high degree of suspicion to permit early therapy. Urgency in diagnosis is extremely important, as the disease is rapidly progressive. Ultimately a biopsy or smear cern-
onstrating nonseptate hyphae is needed (21), but radiological examination can demonstrate rather suggestive changes . The roentgen findings in mucormycosis have been described by Green et al. in the only previous report in the radiology literature (12). Spotty bony destruction is the hallmark of craniofacial mucormycosis. However, the present series demonstrates that more extensive bone destruction of the sinus walls or facial structures can also occur, as well as sclerotic changes in more chronic cases. Mucosal thickening was uniform along
Vol. 115
RHINOCEREBRAL MUCORMYCOSIS
the sinus wall, with a septate appearance in several cases. The inner margins were smooth and the previously described nodularity was not seen. In one case, an air-fluid level was present on the upright examination, a finding not reported previously. Although only ethmoid and maxillary sinus disease was observed in this series, frontal sinus (14, 15) and sphenoid (9) mucormycosis have also been reported. Patients with acute suppurative sinusitis also demonstrate uniform mucosal thickening coupled with either bony resorption or no bone changes; an air-fluid level is often present. However, in mucormycosis bone destruction is focal, in contrast to the more uniform demineralization seen in acute sinusitis. In chronic pyogenic sinusitis, dense mucosal thickening is seen, but there is bony sclerosis rather than destruction. Even the unusual case of mucormycosis with bony sclerosis will also involve destruction of bone. However, if pyogenic osteomyelitis is associated with chronic sinusitis, differentiation from mucormycosis may be impossible. The major differential diagnosis is between mucormycosis and neoplasm. Sinus opacification, mucosal thickening, and bony destruction are common to both conditions. Benign tumors may produce a smooth pressure atrophy or deformity of the bone, in contrast to the irregular or spotty localized changes seen in malignant tumors or mucormycosis. In addition, in malignant tumors an area of destruction is usually seen adjacent to the lesion. In the occasional case of a tumor producing more spotty bone destruction or mucormycosis with more extensive destruction, differentiation may be impossible. Involvement of multiple sinuses would favor mucormycosis. Also, the clinical presentation may vary; mucormycosis may be acute in onset, and the patient is more acutely ill than with neoplasm. The radiologist should exercise a high degree of suspicion in arriving at the diagnosis, particularly in a patient with a history of diabetes and roentgen changes suggestive of sinusitis or tumor, so that therapy can be instituted before the central nervous system is involved.
SUMMARY The presence, type, and location of bony destruction are important in the diagnosis of rhinocerebral mucormycosis. Bony destruction favors mucormycosis rather than acute or chronic sinusitis. Spotty, more diffuse bone destruction coupled with multiple sinus involvement suggests mucormycosis rather than malignant tumor. Although roentgen differentiation may be impossible, a high degree of suspicion is necessary to facilitate early diagnosis.
REFERENCES 1. Abramson E, Wilson B, Arky RA: Rhinocerebral phycomycosis in association with diabetic ketoacidosis. Report of two cases
117
Neuroradiology
and a review of clinical and experimental experience with amphotericin B therapy. Ann Intern Med 66:735-742, Apr 1967 2. Baker RD: Mucormycosis-a new disease? JAMA 163: 805-808,9 Mar 1957 3. Battock DJ, Grausz H, Bobrowsky M, et al: Alternate-day amphotericin B therapy in the treatment of rhinocerebral phycomycosis (mucormycosis). Ann Intern Med 68: 122-137, Jan 1968 4. Bauer H, Flanagan JF, Sheldon WH: The effects of metabolic alterations on experimental Rhizopus oryzae (mucormycosis) infection. Yale J Bioi Med 29:23-32, Sep 1956 5. Baum JL: Rhino-orbital mucormycosis. Occurring in an otherwise apparently healthy individual. Am J Ophthalmol 63:335-339, Feb 1967 6. Bentwich Z, Rosen Z, Ganor S, et al: Chronic rhinocerebral mucormycosis (phycomycosis) with occlusion of left internal carotid artery. lsr J Med Sci 4:977-981, Sep-Oct 1968 7. Bergstrom L, Hemenway WG. Barnhart RA: Rhinocerebral and otologic mucormycosis. Ann Otol Rhinol LaryngoI79:70-81, Feb 1970 8. Blodi FC, Hannah FT, Wadsworth JAC: Lethal orbito-cerebral phycomycosis. In otherwise healthy children. Am J Opthalmol 67:698-705, May 1969 9. Case records of the Massachusetts General Hospital. Case 48-1968. N Engl J Med 279:1220-1229,28 Nov 1968 10. DeWeese DD, Schleuning AJ II, Robinson LB: Mucormycosis of the nose and paranasal sinuses. Laryngoscope 75: 13981407, Sep 1965 11. Ginsberg J, Spaulding AG, Laing va: Cerebral phycomycosts (mucormycosis) with ocular involvement. Am J Ophthalmol 62:900~906, Nov 1966 12. Green WH, Goldberg HI, Wohl GT: Mucormycosis infection of the craniofacial structures. Am J Roentgenol 101:802-806, Dec 1967 13. Groote CA: Rhinocerebral phycomycosis. Arch Otolaryngol 92:288-292, Sep 1970 14. Heitzman ER, Ferguson JH: Pain and swelling about eye. NY State J Med 69: 1059-1 061, 15 Apr 1969 15. Kaufman RS, Stone G: Osteomyelitis of frontal bone secondary to mucormycosis. NY State J Med 73: 1325- 1328, 1 Jun 1973 16. La Touche CJ, Sutherland TW, Telling M: Rhinocerebral mucormycosis. Lancet 2:811-813, 19 Oct 1963 17. McBride RA, Corson JM, Dammin GJ: Mucormycosis. Two cases of disseminated disease with cultural identification of Rhizopus; review of literature. Am J Med 28:832-846, May 1960 18. Prockop LD, Silva-Hutner M: Cephalic mucormycosis (phycomycosis). A case with survival. Arch Neurol 17:379-386, Oct 1967 19. Reeves DL, Dickson DR, Benjamin El: Phycomycosis (mucormycosis) of the central nervous system. J Neurosurg 23:82-84, Jul 1965 20. Sandler R, Tallman CB, Keamy DG, et at; Successfully treated rhinocerebral phycomycosis in well controlled diabetes. N EnglJ Med 285:1180-1182,18 Nov 1971 21. Smith HW, Kirchner JA: Cerebral mucormycosis. A report of three cases. Arch Otolaryngol 68:715-726, Dec 1958 22. Stephan T, Busis SN, Arena S, et al: Rhinocerebral phycomycosis (mucormycosis). Laryngoscope 83:173-178, Feb 1973 23. Stevens KM, Newell Re, Bergstrom L: Mucormycosis in a patient receiving Azathioprine. Arch Otolaryngol 96:250-251, Sep 1972 24. Straatsma BA, Zimmerman LE, Gass JDM: Phycomycosis. A clinicopathologic study of fifty-one cases. Lab Invest 11:963-985, Nov 1962 25. Taylor CG, Alexander RE, Green WH, et al: Mucormycosis (phycomycosis) involving the maxilla. Report of a case with survival. Oral Surg 27:806-822, Jun 1969 26. Weisskopf A: Mucormycosis-a rhinologic disease. Ann Otol Rhinol Laryngol 73:16-23, Mar 1964 St. Thomas Hospital 4220 Harding Rd. Nashville, Tenn. 37205
