Rhino-orbito-cerebral mucormycosis after allogeneic hematopoietic stem cell transplantation for very severe aplastic anemia in a child: a case report

Abstract

Rhino-orbito-cerebral mucormycosis (ROCM) is a rare and life-threatening fungal infection that predominantly affects immunocompromised individuals, such as those undergoing hematopoietic stem cell transplantation (HSCT). This report describes the case of a 10-year-old girl with very severe aplastic anemia (VSAA) who underwent an haploidentical HSCT from her father. She initially achieved successful engraftment with a donor chimerism rate of 98.25% on day 60. However, on day 65 post-transplant, she developed severe right-eye pain, progressive swelling, and visual impairment. Comprehensive evaluations, including contrast-enhanced magnetic resonance imaging (MRI) and cerebrospinal fluid (CSF) analysis, revealed extensive orbital and cerebral involvement consistent with ROCM. Intensive antifungal therapy with liposomal amphotericin B and posaconazole, along with endoscopic surgical debridement of the infected sinuses and orbital regions, was initiated. Post-treatment MRI scans demonstrated a significant reduction in cerebral edema and other abnormalities, while repeated CSF analyses confirmed the absence of fungal elements. This case underscores the critical need for early diagnosis and aggressive management of ROCM in immunocompromised patients, particularly following HSCT.

Keywords: antifungal therapy; hematopoietic stem cell transplantation; rhino-orbito-cerebral mucormycosis; surgical intervention; very severe aplastic anemia.

Figure 1

(A) Upon admission, the patient presented with significant redness and swelling in the right eye, accompanied by ptosis of the upper eyelid, impaired vision, and an inability to elevate the eyelid. Antifungal therapy with liposomal amphotericin B and posaconazole was initiated to address the suspected fungal infection. (B) Coronal view and (C) axial view of contrast-enhanced magnetic resonance imaging of the head showed bilateral frontal lobe parenchymal edema with abnormal signal intensities, indicative of a potential infectious process.

Figure 2

(A) Following nasal endoscopic surgery by the otolaryngology team, persistent marked edema was observed in the right conjunctiva, while the swelling in the left eye had improved. (B) Histopathological examination of the excised tissue revealed a multinucleated giant cell response, suggestive of a fungal infection (200× magnification). (C) Further histopathological analysis identified chronic inflammatory changes with fungal hyphae within necrotic tissue (200× magnification). (D) Coronal and (E) axial views of contrast-enhanced magnetic resonance imaging of the head, performed 9 weeks post-surgery, displayed radiologic findings consistent with the nasal endoscopic procedure, confirming the ongoing resolution of the infection.

Figure 3

(A) Photographic representation of the tarsorrhaphy surgery performed to address the patient's visual complications. (B) At the 24-week postoperative follow-up, both eyes exhibited a lack of light perception, indicating persistent visual impairment. (C) Coronal and (D) axial views of contrast-enhanced magnetic resonance imaging of the head, conducted 24 weeks after surgery, provided a radiologic assessment of the patient's condition, showing the extent of recovery and ongoing changes in the affected areas.