Novel Insights from Clinical Practice Pediatr Neurosurg 2014–15;50:165–167 DOI: 10.1159/000381750

Received: October 9, 2014 Accepted after revision: March 18, 2015 Published online: May 7, 2015

Successful Management of a Unique Condition of Isolated Intracranial Mucormycosis in an Immunocompetent Child Mohammed M. Al Barbarawi a Mohammed Z. Allouh b a

Division of Neurosurgery, Department of Neuroscience and b Department of Anatomy, Faculty of Medicine, Jordan University of Science and Technology, Irbid, Jordan

Established Facts • Isolated intracranial mucormycosis is a life-threatening infection that usually affects immunosuppressed patients. Until now, there have been 5 reports in the literature about isolated brain mucormycosis in immunocompetent patients, with only one case being in a child.

Novel Insights • This is the second case of isolated intracranial mucormycosis to be reported in a healthy immunocompetent child. The nature, location and host of the infection are considered unique variations in what is typically seen in cases of mucormycosis.

Abstract This report describes a unique case of isolated intracranial mucormycosis of a slowly progressive nature in a healthy immunocompetent child. A 4-year-old girl with a clear medical and surgical history presented with complaints of right side facial asymmetry and unsteady gait for a period of 10 months. Clinical and radiographic investigations revealed right-sided lower motor neuron facial palsy caused by an infiltrative lesion on the right cerebellopontine an-

© 2015 S. Karger AG, Basel 1016–2291/15/0503–0165$39.50/0 E-Mail [email protected] www.karger.com/pne

gle. Initial surgical debulking was performed, a biopsy was sent for histopathological examination, and a course of prophylactic antibiotic and antifungal drugs was prescribed. The pathological report confirmed the mucormycosis fungal infection, and intravenous amphotericin B was administered for 3 weeks. One month after admission, the patient left the hospital with complete recovery. Followups after 4, 8 and 12 weeks revealed no sensory or motor neurological deficits. In conclusion, this is a unique case of mucormycosis with regard to the nature and location of the infection, along with the host being a healthy child. Initial surgical exploration is a very critical step in the early diagnosis and treatment of such rare conditions. © 2015 S. Karger AG, Basel

Mohammed M. Al Barbarawi Department of Neuroscience Faculty of Medicine JUST, PO Box 3030, Irbid 22110 (Jordan) E-Mail mmalbarbarawi @ just.edu.jo

Downloaded by: UCONN Storrs 198.143.38.1 - 1/26/2016 6:11:33 PM

Key Words Amphotericin B · Fungal infection · Immunocompetence · Indolence · Mucormycosis

a

Introduction

Mucormycosis is an opportunistic fungal infection that is caused by a fungus from the order Mucorales [1]. Despite being uncommon, mucormycosis is considered a very serious condition that causes a high rate of mortality [2, 3]. Mucormycosis has been classified into 6 different types based on the site of infection, namely rhino-orbitalcerebral (ROC), pulmonary, gastrointestinal, cutaneous, disseminated and uncommon presentations, with ROC being the most common type of mucormycosis [4]. The most common associated risk factors with this infection are diabetes mellitus, hematological disorders, patients who are immunosuppressed and patients who have undergone trauma [4]. Here, we report a novel condition of indolent intracranial mucormycosis in an immunocompetent child. Both clinical and radiological findings along with the treatment regimen are described below. Case Presentation A 4-year-old girl with a clear medical and surgical history was admitted to King Abdullah University Hospital. The girl’s mother complained that her child had been suffering from right side facial asymmetry and unsteady gait for 10 months. She also noticed that her daughter was unable to close her right eye. There was no maternal history of previous medical illnesses or medications before and during the pregnancy period. Furthermore, the child had no history of previous head traumas, headaches, infections, seizures or loss of consciousness. In fact, the only history that could be found on the patient was the presence of untreated nasal polyps.

166

Pediatr Neurosurg 2014–15;50:165–167 DOI: 10.1159/000381750

b

On examination, the girl showed normal stable vital signs with a Glasgow Coma Scale of 15/15. Her pupils were equal and reactive. However, the patient had an unsteady gait and right-sided lower motor neuron facial palsy; the remaining cranial nerves were all intact. All clinical investigations, including a complete blood count, basic metabolic panel and coagulation profile, were within normal values. Radiographic examination involved magnetic resonance imaging of the brain with both T1- and T2-weighted spinning sequences. T1-weighted images revealed an infiltrative hyperintense lesion on the right cerebellopontine angle, which caused compression and deviation of the fourth ventricle (fig. 1a). T2-weighted images showed the same lesion but with an internal area devoid of signal indicating central necrosis (fig. 1b). The lesion dimensions were 3.1 × 2.5 cm. On the second day of admission, the patient underwent a surgical gross debulking of the lesion, and a biopsy was sent to the department of pathology for histopathological investigation. The patient commenced treatment with intravenous antibiotics (vancomycin and ceftriaxone) and the antifungal drug fluconazole. Three days later, the final histopathological report confirmed a mycotic brain infection of mucormycosis. The patient’s medication was then modified to an intravenous drip of amphotericin B for 3 weeks. Approximately 1 month after admission, the patient left the hospital with no signs of focal neurological deficits. Follow-ups after 4, 8 and 12 weeks revealed that the patient had complete recovery with no sensory or motor neurological deficits.

Discussion

Mucormycosis of the brain is a very serious and lifethreatening condition. Common routes of brain infection are either through direct expansion from the paranasal sinuses and the orbits, as in ROC type infections, or Al Barbarawi/Allouh

Downloaded by: UCONN Storrs 198.143.38.1 - 1/26/2016 6:11:33 PM

Fig. 1. Brain magnetic resonance images of a 4-year-old girl with mucormycosis. a T1-weighted image showing a hyperintense lesion on the right cerebellopontine angle (arrow). b T2-weighted image showing the same lesion but with central necrosis.

through hematological dissemination [4]. Isolated cases of mucormycosis have been reported in patients with no rhino-orbital expansion, dissemination from other locations or head trauma [3, 5–7]. However, most isolated cases occur in immunosuppressed patients who suffer from systemic conditions or intravenous drug addiction [8, 9]. Interestingly, there have been 5 reports in the literature on isolated brain infection due to mucormycosis in immunocompetent patients [5, 8, 10]. Among these, only 1 case was reported in a child [10]. To our knowledge, this is the second case of isolated intracranial mucormycosis to be reported in a healthy immunocompetent child. The previous history of nasal polyps in the child reported in the present case study raises the possibility of a direct rhinocerebral route for fungal invasion. Nasal polyps comprise the most common nonneoplastic lesions found in the nose [11], with allergic fungal sinusitis being a common etiology [12, 13]. In addition to being the primary infectious agents, fungi may present as secondary opportunistic invaders in nasal polyposis [14]. The prevalence of allergic fungal sinusitis in patients with nasal

polyps has been estimated to be between 7 and 12% [12, 13]. However, due to the lack of histopathological examination, it will be very difficult to confirm the possibility of the rhinocerebral route of infection in this child. Treatment of the child consisted of an initial surgical debridement followed by systemic antifungal therapy. Surgical intervention was a very critical step in this case since it helped in decompressing brain structures and allowed for early histological diagnosis. Medical intervention consisted of an intravenous drip containing the liposomal form of amphotericin B, since this is the drug of choice for cerebral mucormycosis [2, 9]. Previous studies confirmed the efficacy of this form of the drug in the management of intracranial infection of mucormycosis where less nephrotoxicity and better cerebrospinal fluid penetration were reported compared to other forms of amphotericin B [2, 15]. In conclusion, this is a unique case of mucormycosis with regard to the nature, location and host of the infection. We believe that surgical intervention is a very important step in the early diagnosis and proper management of similar cases.

References

Isolated Intracranial Mucormycosis in a Child

6 Malik AN, Bi WL, McCray B, Abedalthagafi M, Vaitkevicius H, Dunn IF: Isolated cerebral mucormycosis of the basal ganglia. Clin Neurol Neurosurg 2014;124:102–105. 7 Rumboldt Z, Castillo M: Indolent intracranial mucormycosis: case report. Am J Neuroradiol 2002;23:932–934. 8 Verma A, Brozman B, Petito CK: Isolated cerebral mucormycosis: report of a case and review of the literature. J Neurol Sci 2006; 240: 65–69. 9 Uy EM, Rustagi T, Khera S: Cerebral mucormycosis in a diabetic man. Conn Med 2011; 75:273–279. 10 Tsung LL, Zhu XL, Chu WC, Sun DT, Cheung KL, Leung TF: Intraventricular amphotericin for absidiomycosis in an immunocompetent child. Hong Kong Med J 2010;16:137–140.

11 Jang TY: Pathogenesis of nasal polyps. J Rhinol 1999;6:5–11. 12 Telmesani LM: Prevalence of allergic fungal sinusitis among patients with nasal polyps. Ann Saudi Med 2009;29:212–214. 13 Braun H, Buzina W, Freudenschuss K, Beham A, Stammberger H: ‘Eosinophilic fungal rhinosinusitis’: a common disorder in Europe? Laryngoscope 2003;113:264–269. 14 Corradini C, Del Ninno M, Buonomo A, Nucera E, Paludetti G, Alonzi C, Sabato V, Schiavino D, Patriarca G: Amphotericin B and lysine acetylsalicylate in the combined treatment of nasal polyposis associated with mycotic infection. J Investig Allergol Clin Immunol 2006;16:188–193. 15 Spellberg B, Walsh TJ, Kontoyiannis DP, Edwards J Jr, Ibrahim AS: Recent advances in the management of mucormycosis: from bench to bedside. Clin Infect Dis 2009; 48: 1743– 1751.

Pediatr Neurosurg 2014–15;50:165–167 DOI: 10.1159/000381750

167

Downloaded by: UCONN Storrs 198.143.38.1 - 1/26/2016 6:11:33 PM

1 Kwon-Chung KJ: Taxonomy of fungi causing mucormycosis and entomophthoramycosis (zygomycosis) and nomenclature of the disease: molecular mycologic perspectives. Clin Infect Dis 2012;54:S8–S15. 2 Alrefai AH, Berger JR, Saadeh RS: Against all odds: surviving rhino-orbital-cerebral mucormycosis: a case report. J Neurovirol 2012; 18:144–147. 3 Han SR, Choi CY, Joo M, Whang CJ: Isolated cerebral mucormycosis. J Korean Neurosurg Soc 2007;42:400–402. 4 Petrikkos G, Skiada A, Lortholary O, Roilides E, Walsh TJ, Kontoyiannis DP: Epidemiology and clinical manifestations of mucormycosis. Clin Infect Dis 2012;54:S23–S34. 5 Air EL, Vagal AA, Kendler A, McPherson CM: Isolated cerebellar mucormycosis, slowly progressive over 1 year in an immunocompetent patient. Surg Neurol Int 2010;1:81.

Successful Management of a Unique Condition of Isolated Intracranial Mucormycosis in an Immunocompetent Child.

This report describes a unique case of isolated intracranial mucormycosis of a slowly progressive nature in a healthy immunocompetent child. A 4-year-...
294KB Sizes 0 Downloads 8 Views