- Email: [email protected]
Fatal rhino-orbito-cerebral mucormycosis in an apparently normal host: case report and literature review
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unruptured giant MCA aneurysm 13 days after extracranial– intracranial bypass; it is therefore recommended that there should not be undue delay in performing proximal occlusion after bypass procedure.14 REFERENCES 1. 2. 3.
4.
5. 6. 7.
8. 9.
10.
11.
12. 13.
14.
Somach FM, Shenkin HA. Angiographic end-results of carotid ligation in the treatment of carotid aneurysm. J Neurosurg 1966; 24: 966–974. Handa H, Hashimoto N, Nagata I et al. Saccular aneurysms in rats: a newly developed animal model of the disease. Stroke 1983; 14: 857–866. Hashimoto N, Handa H, Nagata I et al. Experimentally induced cerebral aneurysms in rats, Part V: relation of hemodynamics in the circle of Willis to formation of aneurysms. Surg Neurol 1980; 13: 41–45. Hashimoto N, Handa H, Nagata I et al. Animal model of cerebral aneurysms: pathology and pathogenesis of induced cerebral aneurysms in rats. Neurol Res 1984; 6: 33–40. Drake CG. Giant intracranial aneurysms: experience with surgical treatment in 174 patients. Clin Neurosurg 1979; 26: 12–95. Drake CG, Peerless SJ, Ferguson GG. Hunterian proximal arterial occlusion for giant aneurysms of the carotid circulation. J Neurosurg 1994; 81: 656–665. Fox AL, Vinuela F, Pelz DM et al. Use of detachable balloons for proximal artery occlusion in the treatment of unclippable cerebral aneurysms. J Neurosurg 1987; 66: 40–46. Spetzler RF, Roski RA, Schuster H, Takaoka Y. The role of EC–IC bypass in the treatment of giant intracranial aneurysms. Neurol Res 1980; 2: 345–359. Spetzler RF, Schuster H, Roski RA. Elective extracranial–intracranial arterial bypass in the treatment of inoperable giant aneurysms of the internal carotid artery. J Neurosurg 1980; 53: 22–27. Awad IA, Spetzler RF. Extracranial–Intracranial bypass surgery: a critical analysis in light of the International Cooperative Study. Neurosurgery 1986; 19: 655–664. Lawton MT, Hamilton MG, Morcos JJ, Spetzler RF. Revascularisation and aneurysm surgery: current techniques, indications, and outcome. Neurosurgery 1996; 38: 83–92. Regli L, Piepgras DG, Hansen KK. Late patency of long saphenous vein bypass grafts to the anterior and posterior circulation. J Neurosurg 1995; 83: 806–811. Scott RM, Hsiu-Chin L, Yuan R et al: Rupture of a previously unruptured giant middle cerebral artery aneurysm after extracranial–intracranial bypass surgery. Neurosurgery 1982; 10: 600–603. Weill A, Cognard C, Levy D, Robert G, Moret J. Giant aneurysms of the middle cerebral artery trifurcation treated with extracranial–intracranial arterial bypass and endovascular occlusion. J Neurosurg 1998; 89: 474–478.
pathogenic nature becomes evident when the patient’s general resistance is compromised. We present a case of an invasive rhinoorbito-cerebral mucormycosis in an apparently normal adult who initially developed mild paranasal sinusitis and later developed status epilepticus and despite an aggressive management died. Interesting clinical, neuroimaging and histological findings are described, and the possibility of fatal mucormycosis in an apparently normal host is highlighted. © 2001 Harcourt Publishers Ltd Journal of Clinical Neuroscience (2001) 8(6) 583–586 © 2001 Harcourt Publishers Ltd DOI: 10.1054/jocn.2000.0818, available online at http://www.idealibrary.com on
Keywords: rhino-orbito-cerebral syndrome, mucormycosis, angiotropic fungus, immunocompromised state, cerebral infarction Received 18 May 2000 Accepted 28 August 2000 Correspondence to: Rewati Raman Sharma, Senior Specialist Neurosurgeon, PO Box 397, PC 118, Al-Harthy Complex, Muscat, Sultanate of Oman. Tel.: 00 968 567339; Fax: 00 968 567339; E-mail: [email protected]
INTRODUCTION Although still uncommon, rhino-orbito-cerebral mucormycosis is a potentially lethal fungal infection characterised by rapid progression and high mortality.1–4 This unique disease is observed worldwide, especially in the USA and often associated with diabetic ketoacidosis and intravenous drug abuse.5 Rhino-orbito-cerebral mucormycosis in diabetic ketoacidosis accounts for 80–90% of patients6–10 and the remaining cases occur in other immunocompromised states, i.e. organ transplantation,11,12 uraemia,13 liver cirrhosis,14 haematological malignancies,15,16 burns, AIDS,17 etc. Mucormycosis is rarely reported in an apparently normal host.18–20 Here we describe a case of invasive rhino-orbito-cerebral mucormycosis in a normal individual with interesting clinical, neuroimaging and histopathological findings.
CASE REPORT
Fatal rhino-orbito-cerebral mucormycosis in an apparently normal host: case report and literature review Rewati Raman Sharma MB MS (NEUROSURG) DNB (NEUROSURG), Sanjay J. Pawar MB MS MCH (NEUROSURG), Adrian Delmendo MD, Santosh D. Lad MB MS FRSH, Sanjeev D. Athale MB MD The National Neurosurgery Centre, Khoula Hospital PO Box-90, PC 116, Mina-Al-Fahal, Muscat, Sultanate of Oman
Summary Fungal infections of the central nervous system (CNS) are fortunately rare but remain challenging problems occurring mostly in immunocompromised individuals, with protean manifestations, unpredictable course and unfavorable outcome in many cases despite aggressive neurosurgical intervention and recent antifungal drugs. Rhino-orbito-cerebral mucormycosis is a potentially lethal opportunistic fungal infection with rapid progression and high mortality. Its
© 2001 Harcourt Publishers Ltd
A 40 year old gentleman with no previous history of a medical illness was treated symptomatically in another hospital for a nonspecific paranasal sinusitis of one week duration with symptomatic relief. Sudden onset of repeated seizures with altered sensorium had prompted his neurosurgical admission. Following anticonvulsant treatment the seizures were brought under control. He was then fully conscious, obeying commands and had normal speech. His pupils, fundi and extraocular movements were normal though he had a slight proptosis of the right eye. Within a couple of hours this patient showed a sudden deterioration in his sensorium with pupillary inequality (RtLt) and left hemiplegia, then he lapsed into deep coma. The computed tomogram (CT) scan confirmed a mild right eye proptosis and showed opacified right middle and posterior ethmoid sinuses (Fig. 1) and a large diffuse low attenuation lesion involving right frontal lobe and basal ganglia with significant mass effect. On contrast infusion, there was some enhancement of the right sylvian fissure and anterior interhemispheric region bordering the right frontal lobe and the sylvian fissure laterally bordering the right basal ganglia. This low attenuation lesion in the frontal lobe and the basal ganglia showed no significant contrast enhancement (Fig. 2). His haematological and biochemical parameters were within normal limits. There was no evidence to suggest iron overload or haematological malignancy. In desperation, an emergency right frontal craniotomy and a wide excision of the greyish brown subcortical Journal of Clinical Neuroscience (2001) 8(6)
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Fig. 1 The axial CT scan showing an obvious right eye proptosis and opacity of the right, middle and posterior ethmoid sinuses (arrow).
Fig. 2 The axial CT scan following contrast infusion showing the low attenuation lesion as seen on the plain scan in the right frontal and basal ganglia region showing no significant contrast enhancement.
frontal portion of the lesion extending up to the base of the anterior cranial fossa was performed. Postoperatively, he showed no improvement, and died on the second postoperative day. The tissue culture on Sabouraud’s agar showed a fluffy white growth in 48 h. The histopathology of the lesion (H & E stain) showed extensive cerebral infarction due to mucormycosis, nonseptate fungal hyphae with right angle branching very well demonstrated in the cerebral blood vessels and in the brain parenchyma (Fig. 3), with inflammatory cellular infiltrate (mainly of polymorphs, few lymphocytes and plasma cells). The fungus was further demonstrated on the Gomori’s silver methamine stain confirming the presence of mucor. DISCUSSION Phycomycosis also has zygomycosis in its broad group. Zygomycosis is a term that includes mucormycosis (causing CNS mycosis) and ento-rhino-mucormycosis (causing extra CNS mycosis).1–4 The mucormycosis is mainly caused by three genera – rhizopus, mucor and absidia. The rhizopus arrhizus and rhizopus oryzae are responsible for 95% cases of mucormycosis. The organisms are ubiquitous and are found in the soil, manure and decaying vegetation and are frequently airborne. Mucormycosis occurs initially in the rhino-sino-orbital region, respiratory system, gastrointestinal tract (GIT) and skin. Journal of Clinical Neuroscience (2001) 8(6)
Fig. 3 Photomicrograph (H & E400) showing features of cerebral infarction due to mucormycosis: broad nonseptate fungal hyphae with right angle branching in the cerebral parenchyma with inflammatory cellular infiltrate.
CNS mucormycosis result either from direct invasion from rhino-orbital mucormycosis through the cribriform plate or haematogenous spread from respiratory, GIT or skin.21–23 Mucorales has nonseptate irregular hyphae with right angle branches measuring 6 to 15 mm in diameter and 200 mm in length from which root-like rhizoids grow. From rhizoids, sporoangiospores bearing sporangia develop which contain brown spores (7 mm). In the classical clinical picture of rhino-orbito-cerebral syndrome,24 there are three stages. In stage 1 there is local nasal mucosal and sinus infection resulting in nasal stuffiness, discharge, local pain and headaches. Due to tissue infarction, the classic black turbinate and palatal ulcers develop. The case under discussion had only local pain and stuffiness of one week duration but no other symptoms or signs. In stage 2 there is advanced orbital involvement resulting in the orbital apex and superior orbital syndrome (II to VI cranial nerve involvement) – our patient had a mild proptosis but no other features. In stage 3 there is cerebral involvement with spread of the infection through the superior orbital fissure to the cavernous sinus (thrombosis) and internal carotid artery (occlusion-thrombosis) or the cribriform plate resulting in anterior cranial fossa and frontal lobe involvement. Our patient probably had both these features, namely involvement of the frontal lobe via cribriform plate and thrombosis of the internal carotid artery. Awareness of rhino-orbito-cerebral mucormycosis is highlighted in patients with diabetic ketoacidosis (in majority) and other immunocompromised states.6–17 The close relationship between uncontrolled diabetic ketoacidossis and mucormycosis is explained by the fact that the mucor species have an active ketone reductase medium and have their peak metabolic activity at a low pH level.23 Abrahamson6 noted that the acute alloxan-induced ketotic diabetes in rats and mice permitted invasive growth of preinstalled mucor organisms while infusional hyperglycemia and nonketotic diabetes did not. He implicated acidosis in the pathogenesis of this condition. Interestingly, our case had no diabetic ketoacidosis or other metabolic problems. Mucor, being an angiotropic fungus, has a striking tendency to proliferate along and through vascular structures producing arteritis with aneurysm and pseudo-aneurysm formation as well as vascular (internal carotid artery and basilar artery) occlusion and infarction. This rapidly progresses to stroke, encephalitis and death.20,25–27 It tends to spread along perivascular and perineural channels through the cribriform plate to frontal lobes and through the orbital apex to cavernous sinuses.28,29 The CT and MRI detect early vascular invasion and intracranial extent of the fungal invasion.28,30–32 The CT features include © 2001 Harcourt Publishers Ltd
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opacification and bony destruction of the paranasal sinuses, orbital extension from the ethmoid sinuses producing proptosis, chemosis and obliteration of the naso-pharyngeal tissue planes. Intracranial extension produces low absorption abnormalities, particularly in the anterior cranial fossa in the frontal lobes with mass effect and contrast enhancement. It may cause large vessel obstruction and cerebral infarction. In our case, the diagnosis was made on cerebral tissue histology alone as in the absence of obvious discharge from the subtle rhino-orbital involvement no tissue samples were examined at another hospital. Cerebral biopsy had revealed necrosis, perivascular and parenchymal polymorph infiltration and characteristic nonseptate fungal hyphae in the small cerebral vessels and in the cerebral parenchyma. Plain X-rays usually show mucosal thickening rather than a fluid level in the paranasal sinus involvement. Mucormycotic cerebral abscesses and hydrocephalus are well outlined by CT or MR scans.33–35 There are three primary aspects of the management of the rhino-orbito-cerebral mucormycosis:1–4,15,24,36–40 1) the control of the underlying immunocompromised state which in our case was none; 2) systemic antifungal therapy in form of amphoterecin-B or more effective liposomal amphoterecin-B; and 3) the surgical debridement of dead, infarcted or infected tissue, which, if it involves deep basal ganglionic and central structures is impossible to achieve, has happened in our case. Recently, the prognosis of the mucormycosis has markedly improved with aforementioned measures and hyperbaric oxygen therapy.21,24 There is some evidence to suggest that the latter improves the oxygen tension in the ischemic tissues and alleviates the acidosis and inhibits fungal growth.21 However, the role of hyperbaric oxygen is still unclear. There are certainly enthusiastic proponents but the clear clinical evidence of its effectiveness over and above the other treatment modalities mentioned is not compelling. According to some authors, liposomal amphoterecin-B may be more effective and less toxic as this drug complex enhances intracellular delivery of the drug to phagocytes.37 The poor prognosis in our case was related to the advanced stage of this invasive cerebral mucormycosis, although the diagnosis was suspected at operation it could only be confirmed on histology. Greater awareness of the occurrence of cerebral mucormycosis, even in the absence of diabetic ketoacidosis or other metabolic and immunocompromised states, will prompt an appropriate management strategy and improve outcome in such challenging cases.
7. 8.
9. 10. 11.
12.
13.
14. 15.
16.
17.
18.
19.
20.
21.
22. 23. 24. 25. 26.
27.
28.
ACKNOWLEDGEMENT We are sincerely grateful to Dr K. S. Mann, Senior Consultant Neurosurgeon, Khoula Hospital, for his guidance and assistance; and Department of Histopathology, Royal Hospital for the photomicrographs.
29.
30. 31.
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Dhermy P. Phycomycosis (mucormycosis). In: Vinken PJ, Bruyn GW (Eds) Vol 35. Amsterdam: North Holland Publishing Company, 1978; 541–555. Kirkpatric JB. Neurologic infections due to bacteria, fungi and parasites. In: Davis RL, Robertson DM (Eds) Textbook of neuropathology. 2nd edn. Baltimore: Williams & Wilkins 1991; 719–803. Scaravilli F. Parasitic and fungal infections of the nervous system. In: HumeAdams H, Corsellis JAN, Duchen LW (Eds) Greenfield’s neuropathology. 4th edn. London: Edward Arnold 1984: 304–337. Young RF, Gade G, Grinnell W. Surgical treatment for fungal infections in the nervous system. J Neurosurg 1985; 63: 371–381. Parfrey NA. Improved diagnosis and prognosis of mucormycosis. Medicine (Baltimore) 1986; 65: 113–123. Abrahamson E. Rhinocerebral phycomycosis in association with diabetic ketoacidosis. Ann Int Med 1967; 66: 735.
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Friedman AH, Bullitt E. Fungal infections. In: Wilkins RH, Rengachary SS (Eds) Neurosurgery. 2nd edn. New York: McGraw Hill 1996; 3385–3394. Larkin JG, Bucher IG, Frier BM, Brebner H. Fatal rhino-cerebral mucormycosis in newly diagnosed diabetic. Diabetic Med 1986; 3: 266–268. Nolan RL, Carter RR, Griffith JE, Chapman SW. Subacute disseminated mucormycosis in a diabetic male. Am J Med Sci 1989; 298: 252–255. Sundaram C. Rhinocerebral zygomycosis – A clinicopathological study. Neurology India 1998; 46: 126–129. Carbone KM, Pennington LR, Gimenez LF, Burrow CR, Watson AJ. Mucormycosis in renal transplant patients – a report of two cases and review of the literature. Quart J Med 1958; 224: 825–831. Nampoory MR, Khan ZU, Jojny KV, Constandi JN, Gupta RK, AL-Muzairi I, Samhan M, Mozavi M, Chugh TD. Invasive fungal infections in renal transplant recipients. J Infect 1996; 33(2): 95–101. Wens R, Devriendt J, Collart F. Rhino-cerebral mucormycosis in hemodialysis patients treated with desferrioxamine: possible role of recent surgery as an additional risk factor. Nephrol Dial Transplant (Germany) 1991; 9: 656–658. Baker RD. Mucormycosis. In: human infection with Fungi, Actinomycetes and Algae. Baker RD (Ed.) New York: Springer-Verlag 1971; 832–918. Funada H, Mochizuki Y, Machi T, Ontake S, Matsuda T. Successful medical treatment of rhino-cerebral mucormycosis complicating acute leukemia. Kansenshogaku Zasshi 1991; 65. Carpenter AF, Bernard L, Poisson M, Delatte JY. Infections du systeme nerveux central chez les patients atteints d’une pathologie maligne. Revue Neurologigue 1996; 152(10): 587–601. Cuadrado LM, Guerrero A, Asejo JALG, Martin F, Palau E, Una DG. Cerebral mucormycosis in two cases of aquired immuno deficiency syndrome. Arch Neurol 1988; 45: 109–111. Bhattacharya AK, Desphande AR, Nayak SR, Kirtne MV, Ingle MV, Vora IM. Rhinocerebral mucormycosis: an unusual case presentation. J Laryngo Otol 1992. De Biscop J, Mondie JM, Ventries de la Gullaumie B, Peri G. Mucormycosis in an apparently normal host. Case study and literature review. J Craniomaxillo Fac Surg (German) 1991; 19: 275–278. Qualtrocolo G, Pignatta P, Dimanico U, Tarenzi L, Baggiore P. Rhino-cerebral mucormycosis and internal carotid artery thrombosis in a previously healthy patient. Acta Neurol Belg 1990; 20–26. Couch L, Theilen F, Mader JT. Rhino-cerebral mucormycosis with cerebral extension successfullly treated with adjunctive hyperbaric oxygen therapy. Arch Otolaryngol Head Neck Surg 1988; 114: 791–794. Ellis CJK, Daniel SE, Kennedy PG, Oppenheimer SM, Scaravilli F. Rhinoorbital zygomycosis. J Neurol Neurosurg Psychiatry 1985; 48: 455–458. Kasper LH. Bilateral rhinocerebral phycomytosis. Ann Neurol 1979; 6: 131. Abunijem ZM, Al-Shohaib S, Morrow LB. Rhinocerebral mucormycosis. Annals of Saudi Medicine 1995; 15: 496–500. Carpenter DF, Brubaker LH, Powell RD. Phycomycotic thrombosis of the basilar artery. Neurology 1968; 18: 807–812. Galetta SL, Wuk AE, Goldberg HI, Nichols CW, Glaser JS. Rhino-cerebral mucormycosis. Management and survival of carotid occlusion. Ann Neurol 1990; 28: 103–107. Martin FP, Lukeman JM, Ranson RF. Mucormycosis of the central nervous system associated with thrombosis of the internal carotid artery. J Peaditr 1954; 44: 437–442. Estreem SA, Tully R, Davis WE. Rhino-cerebral mucormycosis: Computed tomographic imaging of cavernous sinus thrombosis. Ann Otol Rhino Laryngol 1990; 160–161. Onerci M, Gursel B, Horsal S, Gulekon N, Gokaz A. Rhino-cerebral mucormycosis with extension to the cavernous sinus: a case report. Rhinology (Netherlands) 1991; 29: 321–324. Mc Devitt GR, Brantley MJ, Cawthon MA. Rhino-cerebral mucormycosis: a case report with MRI findings. Clin Imaging 1989; 13: 317–320. Terk MR, Underwood DJ, Zee CS, Colletti PM. MR imaging in rhino-cerebral and intracranial mucormycosis with CT and pathologic correlation. Margn Reson Imaging (USA) 1992; 10: 81–87. Yousen DM, Galetta SL, Gusnard DA, Goldenberg HI. MRI findings in rhino-cerebral mucormycosis. J Comput Assist Tomogr 1989; 13: 878–882. Epstein NE, Hollingsworth R, Black K, Farmer P. Fungal brain abscesses (aspergillosis/mucormycosis) in immuno-compromised patients. Surg Neurol 1991; 35: 286–289. Hamill R, Oney LA, Crase LR. Successful therapy for rhino-cerebral mucormycosis with associated bilateral brain abscesses. Arch Intern Med 1983; 13: 581–583. Bichile LS, Abhyankar SC, Hase NK. Chronic mucormycosis manifesting as hydrocephalus. J Neurol Neurosurg Psychiatry 1979; 42: 1038–1045. Masucci EF, Fabara JA, Saini N, Kurtzke JF. Cerebral mucormycosis (phycomycosis) in a heroin addict. Arch Neurol 1982; 39: 304–306. Fisher EW, Toma A, Fisher PH, Cheesman AD. Rhino-cerebral mucormycosis: use of liposomal amphoterecin B J Laryngol Otol 1991; 105: 575–577.
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Cervical (myelo)meningocoele: report of 2 cases Shunji Nishio1 MD PHD, Takato Morioka1 MD PHD, Shunji Hikino2 MD PHD, Masashi Fukui2 MD PHD Departments of 1Neurosurgery and 2Pediatrics, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higahsi-ku, Fukuoka 812-8582, Japan
Fig. 1 Photograph of case 1 showing a posterior midline cervical mass lesion.
Summary Two cases with posterior protruding cervical dysraphic lesions are presented; neuroimaging and clinical features of this rare clinical entity are discussed. © 2001 Harcourt Publishers Ltd Journal of Clinical Neuroscience (2001) 8(6), 586–587 © 2001 Harcourt Publishers Ltd DOI: 10.1054/jocn.2000.0862, available online at http://www.idealibrary.com on
Keywords: cervical meningocoele, myelomeningocoele, magnetic resonance imaging, spinal dysraphism Received 19 July 2000 Accepted 29 August 2000 Correspondence to: Shunji Nishio, Department of Neurosurgery, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, Fukuoka 812-8582, Japan. Fax: ;81-92-642-5526; E-mail: [email protected]
INTRODUCTION The cervical dysraphic lesion is a rare clinical entity, having been reported to comprise only 3– 6% of all cases of spinal dysraphism.1–4 Since little about cervical lesions has been described in the literature, despite many reports of lumbosacral dysraphic lesions, we report herein our experience of two patients with cervical (myelo)meningoceles.
CASE REPORT Case 1 This baby girl was the second child of a 24 year old mother, and was delivered by caesarean section in the 38th week of pregnancy because of craniopelvic disproportion. During pregnancy, no hypertension or preeclampsia was observed, nor was there any infection. Family history was unremarkable. The infant weighed 2 355 g at birth. Immediately after birth, a soft mass (3.8 cm3.8 cm2 cm) at the midline of the back in the cervical region was noted. The mass had a relatively wide dome, covered at the base by a full-thickness layer of skin and at the top by thin purplish skin. No CSF leak was noted (Fig. 1). She was transferred to our department at age 37 days, weighing 3 395 g, with normal respiratory status. No neurological deficits were found. Plain spinal radiographs and 3D spiral computed tomography (CT) imaging of the cervical spine demonstrated Journal of Clinical Neuroscience (2001) 8(6)
Fig. 2 Case 1. (A) 3D spiral CT scan of the cervical spinal dysraphism at C4–C6 vertebrae. (B) Axial CT scan through the dural fistula and posterior protruding mass. (C) T2-weighted axial MR image shows the cervical lesion consisting of two cystic lesions surrounded by two layers of membrane (a,b), and basal location of the neural nodule (c). (D) T1-weighted sagittal MR image shows tenting of the dorsal surface of the cervical cord toward the level of the spina bifida. Chiari II malformation is also noted.
spinal dysraphism at the C4–C6 vertebrae (Figs 2A,B). Magnetic resonance (MR) images revealed that the cervical mass consisted of cysts containing layers of membranes and solid tissues on the cyst wall (Fig. 2C). In addition, Chiari II malformation was noted on a T1-weighted sagittal MR image (Fig. 2D), and moderate ventricular dilatation, particularly of the occipital horns of the lateral ventricle, were found on a CT scan of the head. At surgery, a longitudinal oval skin incision was made around the base of the mass, dissected circumferentially toward the fascia where the mass narrowed into a stalk. The subcutaneous mass lesion was composed of two large cysts. An outer one containing xanthochromic fluid was not continuous with the spinal subarachnoid space, and was surrounded by thick fibrous connective tissue. The deeper cystic lesion which narrowed into a stalk was continuous with the subarachnoid space. In addition, fibroneural bands arising from the soft tissue nodules on the wall of the deeper cyst (which were revealed to be neuroglial tissues on histological examinations) tethered the cervical spinal cord (Fig. 3). Electrical stimulation to these fibroneural bands resulted in no apparent muscle reaction. A thorough release of the fibroneural bands and extirpation of the whole mass was thus performed. Postoperatively, the patient remained neurologically intact. However, her ventricular dilatation had progressed, and on the 18th postoperative day a ventriculoperitoneal shunt was placed. At a follow-up 3 years postoperatively she had no signs of neurological disturbance, and her growth and development appeared normal. Case 2 This baby girl was the third child delivered to a 33 year old mother, and was the product of a normal pregnancy and labour. © 2001 Harcourt Publishers Ltd
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unruptured giant MCA aneurysm 13 days after extracranial– intracranial bypass; it is therefore recommended that there should not be undue delay in performing proximal occlusion after bypass procedure.14 REFERENCES 1. 2. 3.
4.
5. 6. 7.
8. 9.
10.
11.
12. 13.
14.
Somach FM, Shenkin HA. Angiographic end-results of carotid ligation in the treatment of carotid aneurysm. J Neurosurg 1966; 24: 966–974. Handa H, Hashimoto N, Nagata I et al. Saccular aneurysms in rats: a newly developed animal model of the disease. Stroke 1983; 14: 857–866. Hashimoto N, Handa H, Nagata I et al. Experimentally induced cerebral aneurysms in rats, Part V: relation of hemodynamics in the circle of Willis to formation of aneurysms. Surg Neurol 1980; 13: 41–45. Hashimoto N, Handa H, Nagata I et al. Animal model of cerebral aneurysms: pathology and pathogenesis of induced cerebral aneurysms in rats. Neurol Res 1984; 6: 33–40. Drake CG. Giant intracranial aneurysms: experience with surgical treatment in 174 patients. Clin Neurosurg 1979; 26: 12–95. Drake CG, Peerless SJ, Ferguson GG. Hunterian proximal arterial occlusion for giant aneurysms of the carotid circulation. J Neurosurg 1994; 81: 656–665. Fox AL, Vinuela F, Pelz DM et al. Use of detachable balloons for proximal artery occlusion in the treatment of unclippable cerebral aneurysms. J Neurosurg 1987; 66: 40–46. Spetzler RF, Roski RA, Schuster H, Takaoka Y. The role of EC–IC bypass in the treatment of giant intracranial aneurysms. Neurol Res 1980; 2: 345–359. Spetzler RF, Schuster H, Roski RA. Elective extracranial–intracranial arterial bypass in the treatment of inoperable giant aneurysms of the internal carotid artery. J Neurosurg 1980; 53: 22–27. Awad IA, Spetzler RF. Extracranial–Intracranial bypass surgery: a critical analysis in light of the International Cooperative Study. Neurosurgery 1986; 19: 655–664. Lawton MT, Hamilton MG, Morcos JJ, Spetzler RF. Revascularisation and aneurysm surgery: current techniques, indications, and outcome. Neurosurgery 1996; 38: 83–92. Regli L, Piepgras DG, Hansen KK. Late patency of long saphenous vein bypass grafts to the anterior and posterior circulation. J Neurosurg 1995; 83: 806–811. Scott RM, Hsiu-Chin L, Yuan R et al: Rupture of a previously unruptured giant middle cerebral artery aneurysm after extracranial–intracranial bypass surgery. Neurosurgery 1982; 10: 600–603. Weill A, Cognard C, Levy D, Robert G, Moret J. Giant aneurysms of the middle cerebral artery trifurcation treated with extracranial–intracranial arterial bypass and endovascular occlusion. J Neurosurg 1998; 89: 474–478.
pathogenic nature becomes evident when the patient’s general resistance is compromised. We present a case of an invasive rhinoorbito-cerebral mucormycosis in an apparently normal adult who initially developed mild paranasal sinusitis and later developed status epilepticus and despite an aggressive management died. Interesting clinical, neuroimaging and histological findings are described, and the possibility of fatal mucormycosis in an apparently normal host is highlighted. © 2001 Harcourt Publishers Ltd Journal of Clinical Neuroscience (2001) 8(6) 583–586 © 2001 Harcourt Publishers Ltd DOI: 10.1054/jocn.2000.0818, available online at http://www.idealibrary.com on
Keywords: rhino-orbito-cerebral syndrome, mucormycosis, angiotropic fungus, immunocompromised state, cerebral infarction Received 18 May 2000 Accepted 28 August 2000 Correspondence to: Rewati Raman Sharma, Senior Specialist Neurosurgeon, PO Box 397, PC 118, Al-Harthy Complex, Muscat, Sultanate of Oman. Tel.: 00 968 567339; Fax: 00 968 567339; E-mail: [email protected]
INTRODUCTION Although still uncommon, rhino-orbito-cerebral mucormycosis is a potentially lethal fungal infection characterised by rapid progression and high mortality.1–4 This unique disease is observed worldwide, especially in the USA and often associated with diabetic ketoacidosis and intravenous drug abuse.5 Rhino-orbito-cerebral mucormycosis in diabetic ketoacidosis accounts for 80–90% of patients6–10 and the remaining cases occur in other immunocompromised states, i.e. organ transplantation,11,12 uraemia,13 liver cirrhosis,14 haematological malignancies,15,16 burns, AIDS,17 etc. Mucormycosis is rarely reported in an apparently normal host.18–20 Here we describe a case of invasive rhino-orbito-cerebral mucormycosis in a normal individual with interesting clinical, neuroimaging and histopathological findings.
CASE REPORT
Fatal rhino-orbito-cerebral mucormycosis in an apparently normal host: case report and literature review Rewati Raman Sharma MB MS (NEUROSURG) DNB (NEUROSURG), Sanjay J. Pawar MB MS MCH (NEUROSURG), Adrian Delmendo MD, Santosh D. Lad MB MS FRSH, Sanjeev D. Athale MB MD The National Neurosurgery Centre, Khoula Hospital PO Box-90, PC 116, Mina-Al-Fahal, Muscat, Sultanate of Oman
Summary Fungal infections of the central nervous system (CNS) are fortunately rare but remain challenging problems occurring mostly in immunocompromised individuals, with protean manifestations, unpredictable course and unfavorable outcome in many cases despite aggressive neurosurgical intervention and recent antifungal drugs. Rhino-orbito-cerebral mucormycosis is a potentially lethal opportunistic fungal infection with rapid progression and high mortality. Its
© 2001 Harcourt Publishers Ltd
A 40 year old gentleman with no previous history of a medical illness was treated symptomatically in another hospital for a nonspecific paranasal sinusitis of one week duration with symptomatic relief. Sudden onset of repeated seizures with altered sensorium had prompted his neurosurgical admission. Following anticonvulsant treatment the seizures were brought under control. He was then fully conscious, obeying commands and had normal speech. His pupils, fundi and extraocular movements were normal though he had a slight proptosis of the right eye. Within a couple of hours this patient showed a sudden deterioration in his sensorium with pupillary inequality (RtLt) and left hemiplegia, then he lapsed into deep coma. The computed tomogram (CT) scan confirmed a mild right eye proptosis and showed opacified right middle and posterior ethmoid sinuses (Fig. 1) and a large diffuse low attenuation lesion involving right frontal lobe and basal ganglia with significant mass effect. On contrast infusion, there was some enhancement of the right sylvian fissure and anterior interhemispheric region bordering the right frontal lobe and the sylvian fissure laterally bordering the right basal ganglia. This low attenuation lesion in the frontal lobe and the basal ganglia showed no significant contrast enhancement (Fig. 2). His haematological and biochemical parameters were within normal limits. There was no evidence to suggest iron overload or haematological malignancy. In desperation, an emergency right frontal craniotomy and a wide excision of the greyish brown subcortical Journal of Clinical Neuroscience (2001) 8(6)
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Fig. 1 The axial CT scan showing an obvious right eye proptosis and opacity of the right, middle and posterior ethmoid sinuses (arrow).
Fig. 2 The axial CT scan following contrast infusion showing the low attenuation lesion as seen on the plain scan in the right frontal and basal ganglia region showing no significant contrast enhancement.
frontal portion of the lesion extending up to the base of the anterior cranial fossa was performed. Postoperatively, he showed no improvement, and died on the second postoperative day. The tissue culture on Sabouraud’s agar showed a fluffy white growth in 48 h. The histopathology of the lesion (H & E stain) showed extensive cerebral infarction due to mucormycosis, nonseptate fungal hyphae with right angle branching very well demonstrated in the cerebral blood vessels and in the brain parenchyma (Fig. 3), with inflammatory cellular infiltrate (mainly of polymorphs, few lymphocytes and plasma cells). The fungus was further demonstrated on the Gomori’s silver methamine stain confirming the presence of mucor. DISCUSSION Phycomycosis also has zygomycosis in its broad group. Zygomycosis is a term that includes mucormycosis (causing CNS mycosis) and ento-rhino-mucormycosis (causing extra CNS mycosis).1–4 The mucormycosis is mainly caused by three genera – rhizopus, mucor and absidia. The rhizopus arrhizus and rhizopus oryzae are responsible for 95% cases of mucormycosis. The organisms are ubiquitous and are found in the soil, manure and decaying vegetation and are frequently airborne. Mucormycosis occurs initially in the rhino-sino-orbital region, respiratory system, gastrointestinal tract (GIT) and skin. Journal of Clinical Neuroscience (2001) 8(6)
Fig. 3 Photomicrograph (H & E400) showing features of cerebral infarction due to mucormycosis: broad nonseptate fungal hyphae with right angle branching in the cerebral parenchyma with inflammatory cellular infiltrate.
CNS mucormycosis result either from direct invasion from rhino-orbital mucormycosis through the cribriform plate or haematogenous spread from respiratory, GIT or skin.21–23 Mucorales has nonseptate irregular hyphae with right angle branches measuring 6 to 15 mm in diameter and 200 mm in length from which root-like rhizoids grow. From rhizoids, sporoangiospores bearing sporangia develop which contain brown spores (7 mm). In the classical clinical picture of rhino-orbito-cerebral syndrome,24 there are three stages. In stage 1 there is local nasal mucosal and sinus infection resulting in nasal stuffiness, discharge, local pain and headaches. Due to tissue infarction, the classic black turbinate and palatal ulcers develop. The case under discussion had only local pain and stuffiness of one week duration but no other symptoms or signs. In stage 2 there is advanced orbital involvement resulting in the orbital apex and superior orbital syndrome (II to VI cranial nerve involvement) – our patient had a mild proptosis but no other features. In stage 3 there is cerebral involvement with spread of the infection through the superior orbital fissure to the cavernous sinus (thrombosis) and internal carotid artery (occlusion-thrombosis) or the cribriform plate resulting in anterior cranial fossa and frontal lobe involvement. Our patient probably had both these features, namely involvement of the frontal lobe via cribriform plate and thrombosis of the internal carotid artery. Awareness of rhino-orbito-cerebral mucormycosis is highlighted in patients with diabetic ketoacidosis (in majority) and other immunocompromised states.6–17 The close relationship between uncontrolled diabetic ketoacidossis and mucormycosis is explained by the fact that the mucor species have an active ketone reductase medium and have their peak metabolic activity at a low pH level.23 Abrahamson6 noted that the acute alloxan-induced ketotic diabetes in rats and mice permitted invasive growth of preinstalled mucor organisms while infusional hyperglycemia and nonketotic diabetes did not. He implicated acidosis in the pathogenesis of this condition. Interestingly, our case had no diabetic ketoacidosis or other metabolic problems. Mucor, being an angiotropic fungus, has a striking tendency to proliferate along and through vascular structures producing arteritis with aneurysm and pseudo-aneurysm formation as well as vascular (internal carotid artery and basilar artery) occlusion and infarction. This rapidly progresses to stroke, encephalitis and death.20,25–27 It tends to spread along perivascular and perineural channels through the cribriform plate to frontal lobes and through the orbital apex to cavernous sinuses.28,29 The CT and MRI detect early vascular invasion and intracranial extent of the fungal invasion.28,30–32 The CT features include © 2001 Harcourt Publishers Ltd
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opacification and bony destruction of the paranasal sinuses, orbital extension from the ethmoid sinuses producing proptosis, chemosis and obliteration of the naso-pharyngeal tissue planes. Intracranial extension produces low absorption abnormalities, particularly in the anterior cranial fossa in the frontal lobes with mass effect and contrast enhancement. It may cause large vessel obstruction and cerebral infarction. In our case, the diagnosis was made on cerebral tissue histology alone as in the absence of obvious discharge from the subtle rhino-orbital involvement no tissue samples were examined at another hospital. Cerebral biopsy had revealed necrosis, perivascular and parenchymal polymorph infiltration and characteristic nonseptate fungal hyphae in the small cerebral vessels and in the cerebral parenchyma. Plain X-rays usually show mucosal thickening rather than a fluid level in the paranasal sinus involvement. Mucormycotic cerebral abscesses and hydrocephalus are well outlined by CT or MR scans.33–35 There are three primary aspects of the management of the rhino-orbito-cerebral mucormycosis:1–4,15,24,36–40 1) the control of the underlying immunocompromised state which in our case was none; 2) systemic antifungal therapy in form of amphoterecin-B or more effective liposomal amphoterecin-B; and 3) the surgical debridement of dead, infarcted or infected tissue, which, if it involves deep basal ganglionic and central structures is impossible to achieve, has happened in our case. Recently, the prognosis of the mucormycosis has markedly improved with aforementioned measures and hyperbaric oxygen therapy.21,24 There is some evidence to suggest that the latter improves the oxygen tension in the ischemic tissues and alleviates the acidosis and inhibits fungal growth.21 However, the role of hyperbaric oxygen is still unclear. There are certainly enthusiastic proponents but the clear clinical evidence of its effectiveness over and above the other treatment modalities mentioned is not compelling. According to some authors, liposomal amphoterecin-B may be more effective and less toxic as this drug complex enhances intracellular delivery of the drug to phagocytes.37 The poor prognosis in our case was related to the advanced stage of this invasive cerebral mucormycosis, although the diagnosis was suspected at operation it could only be confirmed on histology. Greater awareness of the occurrence of cerebral mucormycosis, even in the absence of diabetic ketoacidosis or other metabolic and immunocompromised states, will prompt an appropriate management strategy and improve outcome in such challenging cases.
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ACKNOWLEDGEMENT We are sincerely grateful to Dr K. S. Mann, Senior Consultant Neurosurgeon, Khoula Hospital, for his guidance and assistance; and Department of Histopathology, Royal Hospital for the photomicrographs.
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Cervical (myelo)meningocoele: report of 2 cases Shunji Nishio1 MD PHD, Takato Morioka1 MD PHD, Shunji Hikino2 MD PHD, Masashi Fukui2 MD PHD Departments of 1Neurosurgery and 2Pediatrics, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higahsi-ku, Fukuoka 812-8582, Japan
Fig. 1 Photograph of case 1 showing a posterior midline cervical mass lesion.
Summary Two cases with posterior protruding cervical dysraphic lesions are presented; neuroimaging and clinical features of this rare clinical entity are discussed. © 2001 Harcourt Publishers Ltd Journal of Clinical Neuroscience (2001) 8(6), 586–587 © 2001 Harcourt Publishers Ltd DOI: 10.1054/jocn.2000.0862, available online at http://www.idealibrary.com on
Keywords: cervical meningocoele, myelomeningocoele, magnetic resonance imaging, spinal dysraphism Received 19 July 2000 Accepted 29 August 2000 Correspondence to: Shunji Nishio, Department of Neurosurgery, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, Fukuoka 812-8582, Japan. Fax: ;81-92-642-5526; E-mail: [email protected]
INTRODUCTION The cervical dysraphic lesion is a rare clinical entity, having been reported to comprise only 3– 6% of all cases of spinal dysraphism.1–4 Since little about cervical lesions has been described in the literature, despite many reports of lumbosacral dysraphic lesions, we report herein our experience of two patients with cervical (myelo)meningoceles.
CASE REPORT Case 1 This baby girl was the second child of a 24 year old mother, and was delivered by caesarean section in the 38th week of pregnancy because of craniopelvic disproportion. During pregnancy, no hypertension or preeclampsia was observed, nor was there any infection. Family history was unremarkable. The infant weighed 2 355 g at birth. Immediately after birth, a soft mass (3.8 cm3.8 cm2 cm) at the midline of the back in the cervical region was noted. The mass had a relatively wide dome, covered at the base by a full-thickness layer of skin and at the top by thin purplish skin. No CSF leak was noted (Fig. 1). She was transferred to our department at age 37 days, weighing 3 395 g, with normal respiratory status. No neurological deficits were found. Plain spinal radiographs and 3D spiral computed tomography (CT) imaging of the cervical spine demonstrated Journal of Clinical Neuroscience (2001) 8(6)
Fig. 2 Case 1. (A) 3D spiral CT scan of the cervical spinal dysraphism at C4–C6 vertebrae. (B) Axial CT scan through the dural fistula and posterior protruding mass. (C) T2-weighted axial MR image shows the cervical lesion consisting of two cystic lesions surrounded by two layers of membrane (a,b), and basal location of the neural nodule (c). (D) T1-weighted sagittal MR image shows tenting of the dorsal surface of the cervical cord toward the level of the spina bifida. Chiari II malformation is also noted.
spinal dysraphism at the C4–C6 vertebrae (Figs 2A,B). Magnetic resonance (MR) images revealed that the cervical mass consisted of cysts containing layers of membranes and solid tissues on the cyst wall (Fig. 2C). In addition, Chiari II malformation was noted on a T1-weighted sagittal MR image (Fig. 2D), and moderate ventricular dilatation, particularly of the occipital horns of the lateral ventricle, were found on a CT scan of the head. At surgery, a longitudinal oval skin incision was made around the base of the mass, dissected circumferentially toward the fascia where the mass narrowed into a stalk. The subcutaneous mass lesion was composed of two large cysts. An outer one containing xanthochromic fluid was not continuous with the spinal subarachnoid space, and was surrounded by thick fibrous connective tissue. The deeper cystic lesion which narrowed into a stalk was continuous with the subarachnoid space. In addition, fibroneural bands arising from the soft tissue nodules on the wall of the deeper cyst (which were revealed to be neuroglial tissues on histological examinations) tethered the cervical spinal cord (Fig. 3). Electrical stimulation to these fibroneural bands resulted in no apparent muscle reaction. A thorough release of the fibroneural bands and extirpation of the whole mass was thus performed. Postoperatively, the patient remained neurologically intact. However, her ventricular dilatation had progressed, and on the 18th postoperative day a ventriculoperitoneal shunt was placed. At a follow-up 3 years postoperatively she had no signs of neurological disturbance, and her growth and development appeared normal. Case 2 This baby girl was the third child delivered to a 33 year old mother, and was the product of a normal pregnancy and labour. © 2001 Harcourt Publishers Ltd