Case Reports Facial Reconstruction of a Mucormycosis Survivor by Free Rectus Abdominis Muscle Flap, Tissue Expansion, and Ocular Prosthesis Eldad Silberstein, M.D.*, Yuval Krieger, M.D.*, Nir Rosenberg, M.D.*, Alexander Bogdanov-Berezovsky, M.D., Ph.D.*, Yaron Shoham, M.D.*, Oshra Saphier, Ph.D.†, Vasileios A. Pagkalos, M.D., M.Sc., Ph.D.*, and Ben-Zion Joshua, M.D.‡ Abstract: Invasive sinonasal mucormycosis is a rare fungal infection that usually occurs in immunocompromised or diabetic patients, and it is often fatal. The authors present a case of a woman patient suffering from systemic lupus erythematosus and diabetes mellitus treated with prednisone, presenting with a rapidly progressive rhinoorbital-cerebral mucormycosis. She was successfully treated with combined intravenous antifungal therapy and radical debridement followed by complex defect reconstruction with a free vertical rectus abdominis myocutaneous flap, tissue expander, and ophthalmic prosthesis.

on their diameter and length. Due to thick abdominal wall, the skin island of the flap was removed from the flap; the flap thinned and the skin was used as a full-thickness graft (Fig. C). Postoperative recovery was uneventful, and the patient was discharged 12 days later with continuous antifungal therapy. During regular outpatient follow up, there was no clinical or radiological evidence of recurrent fungal infection. Four months later, she underwent closure of a large oronasal fistula and creation of an ophthalmic socket in the muscle flap using split thickness skin graft to enable insertion of an eye-prosthesis. An acrylic, nonintegrated oval eye-prosthesis was used for the reconstruction of the eye. Using a tissue expander under the remaining cheek skin and moving the expanded skin upward and medially, a lower eyelid medial cantus and nose covering skin were reconstructed. All remaining exposed surfaces including parts of palate, nasal cavity, and external surface of the orbit were skin grafted. Ipsilateral nasal cavity remained patent after reconstruction, and no airway obstruction was observed. The follow-up period after complete reconstruction and conclusion of drug therapy was 4 years. Currently, the patient is off antifungal therapy. The final outcome was evaluated based on patient’s ability to swallow and speak. Apart from a socially acceptable appearance (Fig. D), the patient regained normal, understandable speech and oral nutrition without nasal escaping when swallowing.

CASE REPORT

DISCUSSION

A 41-year-old woman with a recent diagnosis of systemic lupus erythematosus and diabetes mellitus on high-dose steroid treatment was referred from another hospital due to progressive left periorbital and facial swelling. She presented with facial nerve paralysis, dysphagia, generalized edema, left proptosis, vision loss, and lack of extraocular muscle movements in her OS. A CT scan revealed swelling in the left ethmoid and sphenoid sinus mucosa, and left periorbital fat infiltration. An endoscopic examination revealed necrosis in the nasal cavity, and ethmoid, sphenoid, and maxillary sinuses. Endoscopic debridement was performed. An histopathological examination confirmed the clinical diagnosis of invasive mucormycosis infection including fungal osteomyelitis. Intravenous amphotericin B was started with a clinical diagnosis of rhino-orbital mucormycosis. Due to further deterioration with intracranial penetration, a second debridement included OS exenteration, left subtotal maxillectomy, bilateral ethmoidectomy and sphenoidectomy, partial rhinectomy, and right maxillary sinus antrostomy. As a result of debridement, a large complex cavity was created including left orbit, maxillary sinus nose and oral cavity with exposed meningeal tissue (Fig. A,B). She also underwent tracheostomy and gastrostomy insertion later on. The patient gradually improved leaving the intensive care unit. Two months later, orbital and maxillary cavities were filled using free rectus abdominis myocutaneous flap. Branches of facial artery and vein were used for anastomosis. Selection of recipient vessels was made intraoperatively based

Mucormycosis is a rare, invasive, and often fulminant fungal infection. It is caused by ubiquitous fungi in the class Zygomycetes and of the order Mucorales. Most cases affect immunocompromised patients1 and so was the case with this patient who suffered from Lupus disease and diabetes with highdose steroid therapy. The mortality rate in such cases is in the range of 36% to 44%.2 Therefore, high index of suspicion, early diagnosis, and immediate combined medical and surgical treatment is required. Amphotericin B is the drug of choice; however, serious drug toxicity may limit its use in some patients. In these instances, posaconazole can be used as an alternative antifungal agent in the treatment of this severe fungal infection.3 These organisms can be cultured from the stool, nasal passages, and oral cavities of healthy individuals, and for this reason swab wound cultures are not reliable for diagnosis of invasive infection. Tissue biopsies, including staining techniques of hematoxylin-eosin, periodic acid-Schiff, and mehenamine-silver nitrate to demonstrate the fungal hyphae, are essential to make the correct and early diagnosis. Surgical debridement enables fungal culturing and reduces the load of organisms as well as changes the anaerobic and microaerophilic conditions favoring fungal thriving. Often repeated debridement sessions are required to gain disease control. After surviving the acute phase of the disease, long-standing antifungal therapy should be continued to prevent recurrence. At this stage, the patient is left with a large bony and soft tissue defect as in this case where there was practically one large cavity that included the left maxillary sinus, orbit, nasal cavity, and oral cavity. When planning her reconstruction, the authors were trying to restore as much function and get a socially acceptable appearance for the patient. Functional goals included restoring oral nutrition and understandable speech along with nasal breathing. Aesthetic goals were restoring missing cheek and medial cantus skin, and trying to enable insertion of an eye prosthesis that will look natural. These goals were drawn nearby filling up the large cavity using free tissue transfer of a rectus abdominis flap and tissue expansion of cheek skin. Filling up orbital and maxillary cavity with local or free flaps has been well described before and so is the use of tissue

Accepted for publication July 30, 2014. *Division of Plastic and Reconstructive Surgery, Soroka University Medical Center, Ben Gurion University, Beer Sheva, Israel; †Department of Chemical Engineering, Sami Shamoon College of Engineering, Beer-Sheva, Israel; and ‡Otolaringology and Head and Neck Surgery Division, Soroka University Medical Center, Ben-Gurion University of the Negev, Beer-Sheva, Israel The authors have no financial or conflicts of interest to disclose. Address correspondence and reprint requests to Eldad Silberstein, M.D., Division of Plastic and Reconstructive Surgery, Soroka University Medical Center, Ben-Gurion University, P.O.B. 151, Beer-Sheva 84101, Israel. E-mail: [email protected] DOI: 10.1097/IOP.0000000000000314

Ophthal Plast Reconstr Surg, Vol. XX, No. XX, 2014

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Copyright © 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc. Unauthorized reproduction of this article is prohibited.

Ophthal Plast Reconstr Surg, Vol. XX, No. XX, 2014

Case Reports

Mucormycosis survivor: patient facial defect (A), axial CT scan showing nasal defect and temporal lobe infarct (B), after filling the defect with free rectus abdominis flap (C), and after skin expansion and eye prosthesis insertion (D).

expanders.4 Nevertheless, those survivors of mucormycosis are high-risk patients. They carry the risk for disease recurrence and predisposition for more conventional bacterial infection and sepsis with insertion of a foreign body, and create intentional controlled ischemic tissue conditions. Finally, an ophthalmic socket reconstruction has been performed in this case. Unlike ordinary reconstruction, after ophthalmic enucleation the presented patient has no eyelids as natural support for an ocular prosthesis. This demands creation of a precise pocket and additional tissue reinforcement using an expanded skin flap. The authors conclude that mucormycosis infection survivors with large composite facial defects can be successfully reconstructed, achieving functional and socially acceptable appearance, using modern reconstructive options. A close follow up to ensure no disease relapse occurs along the way is required.

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ACKNOWLEDGMENT The authors thank Mrs. Rudnitsky Valentina ocularist at Soroka Occular Prosthesis Laboratory for her help in treating this patient.

REFERENCES 1. Mantadakis E, Samonis G. Clinical presentation of zygomycosis. Clin Microbiol Infect 2009;15(suppl 5):15–20. 2. Roden MM, Zaoutis TE, Buchanan WL, et al. Epidemiology and outcome of zygomycosis: a review of 929 reported cases. Clin Infect Dis 2005;41:634–53. 3. Rutar T, Cockerham KP. Periorbital zygomycosis (mucormycosis) treated with posaconazole. Am J Ophthalmol 2006;142:187–8. 4. Odessey E, Cohn A, Beaman K, et al. Invasive mucormycosis of the maxillary sinus: extensive destruction with an indolent presentation. Surg Infect (Larchmt) 2008;9:91–8.

© 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.

Copyright © 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc. Unauthorized reproduction of this article is prohibited.

Facial Reconstruction of a Mucormycosis Survivor by Free Rectus Abdominis Muscle Flap, Tissue Expansion, and Ocular Prosthesis.

Invasive sinonasal mucormycosis is a rare fungal infection that usually occurs in immunocompromised or diabetic patients, and it is often fatal. The a...
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