RESUMO
INTRODUCTION AND IMPORTANCE: Neurofibromatosis type I (NF1, OMIM: 162200) is a benign, autosomal dominant, tumorigenic predisposing syndrome with variable manifestations. Both neurofibromatosis and soft tissue sarcomas are associated with the formation of hematomas. Moreover, skeletal manifestations of NF1 include focal or generalized forms and expansive or infiltrative growth types. CASE PRESENTATION: A 19-year-old NF1 female patient presented with an expanding post-traumatic facial hematoma that resembled a soft-tissue tumor at initial presentation. A congenital neck mass was noted ipsilateral to her craniofacial skeletal deformities. Multiple imaging modalities were used to aid diagnosis, and urgent surgical intervention of the expanding facial lesion was performed. Her neck lesion and skeletal deformities were monitored, and her recovery was uneventful at 1-year follow-up, with no progression. CLINICAL DISCUSSION: A palpable, non-pulsatile soft tissue mass is a common clinical presentation with a diverse differential diagnosis. Despite the low incidence of post-traumatic vascular injuries and lesions in the maxillofacial region, neurofibromatosis-associated vasculopathy remains an underestimated and serious manifestation of NF1. The reported zygomatic arch deformity is believed to be unique. However, the NF1 tumor-associated skeletal malformations are not linearly related. CONCLUSION: NF1 is a multisystem disorder necessitating an early multidisciplinary team approach. Minimal intervention can help convert an emergent operation into an urgent one and preclude the need for major surgery. The case illustrated a rare example of simultaneous affection of soft tissue and jaw bones in NF1 patients.
RESUMO
INTRODUCTION AND IMPORTANCE: The impact of Covid-19 pandemic on the incidence and pattern of Mucormycosis (the black fungus), has increased sharply and is featured as an epidemic within a pandemic. The majority of cases were detected at late stages, which decreases the chances of survival. CASE PRESENTATION: The authors present a case of an immunocompetent male patient diagnosed with left orbital apex syndrome post covid-19 infection, which necessitated orbital exenteration. He was later hospitalized in a quarantine facility and an area of skin breakdown on his left cheek was noted and surgical debridement performed. Later, He presented to our institution with left hemifacial skin loss, exposing the underlying diseased bone. A multidisciplinary team examined the patient clinically and radiographically, reaching a primitive diagnosis of secondary cutaneous Mucormycosis due to rhino-orbital Mucormycosis, with no cavernous sinus thrombosis nor cranial extension. Radical surgical and medical treatments were given and he had an uneventful recovery. Unfortunately, he died 5 days after the reconstructive surgery with Anterolateral Thigh (ALT) flap. CLINICAL DISCUSSION: The dual effect of both covid-19 and its' associated Mucormycosis, predispose patients to increased risk of pressure injuries including Medical device related pressure injuries. Survivors of Mucormycosis are high-risk patients, and planning their reconstruction by free flaps is challenging. However, delayed reconstruction is recommended. CONCLUSION: Early diagnosis and management of covid-19 associated Mucormycosis should be prioritized. Moreover, surgical debridement of necrotic tissues should not be delayed due to an unavailable or negative histopathology.
