Three atypical manifestations of granulomatosis with polyangiitis: lateral medullary syndrome, anterior cheek mass and melting scleritis of eye.

Granulomatosis with polyangiitis (GPA, formerly Wegener granulomatosis) is a vasculitis with various organ involvement. There have been a few cases of CNS stroke and rare cases of lateral medullary infarction (LMI) as a manifestation of GPA. Also there have been reports of sinuses, nose and laryngeal masses mistakenly referred as carcinomas and subsequently GPA was diagnosed in their pathological reports. Another severe fulminant manifestation can be necrotizing scleritis leading to perforation of sclera. Therefore, here we present some rare and fulminant manifestations of GPA in 3 separate cases for further emphasis of the unusual manifestations of GPA that should always be kept in mind.

Clinical image: Cervicofacial rotation skin flap in PLWD patients.

This article outlines a surgical protocol designed for people living with dementia (PLWD). It proposes that simultaneous resection and reconstruction of skin cancer can minimizes the need for initial care. The method outlined involves primary closure via a cervicofacial rotation flap technique and the use of monofilament resorbable sutures.

Expansive Central Ossifying Fibroma of the Right Mandible.

This case report discusses a 28-year-old patient who presented with a large expansile lesion of the right mandible. A maxillofacial CT showed a 6.7 x 9.1 x 7.6 cm right mandibular cystic mass containing an internal matrix of ground glass bone, representing a huge odontogenic keratocyte. Upon biopsy of the lesion, the specimen consisted of non-decalcified irregular fragments of cemento-osseous material, embedded in a minimally hemorrhagic, cellular fibrous tissue stroma, suggestive of central ossifying fibroma. This case presents an ossifying fibroma that far exceeds the average size of these masses, which typically range from 1.0 to 2.5 cm at its greatest dimension. The immense size of the lesion seen in this case is rarely encountered. This case also helps to emphasize the importance of timely diagnosis and complete resection of the lesion to prevent mass recurrence and possible malignant transformation.

Intraparotid facial varix.

A 72-year-old man had presented with a 4-day history of progressive left-sided facial swelling associated with pain. The physical examination revealed left facial fullness over the parotid gland without tenderness to palpation. His vital signs and laboratory test findings were within normal limits. A computed tomography scan demonstrated a left facial varix measuring 3.4 cm × 2.8 cm within an unremarkable-appearing parotid gland. Parotidectomy vs close observation were discussed, and the patient decided to pursue nonoperative management. Ultimately, his symptoms were self-limited, and the swelling had resolved within 6 months after the diagnosis. Interval computed tomography demonstrated a thrombosed left facial varix measuring 1.3 cm × 1.1 cm.

Anaplastic Large Cell Lymphoma Presenting as Ulcerative Facial Mass: A Case Report.

Anaplastic large cell lymphoma (ALCL) is a rare form of non-Hodgkin lymphoma (NHL) that can be aggressive with rapid speed, thus mandating a timely diagnosis to optimize treatment and deter progression. NHL classically presents with lymphadenopathy and constitutional symptoms. However, ALCL can present with nonspecific cutaneous manifestations with minimal or absent constitutional symptoms. The cutaneous involvement may resemble common dermatologic conditions, delaying diagnosis. We present a case of an aggressive cutaneous ALCL lesion mimicking facial cellulitis that rapidly progressed from a small comedone to a large, exophytic mass over the course of 1 month. Prior to presentation at our institution, the patient was previously diagnosed and treated for suspected bacterial infection with no response to therapy. Core needle biopsy of the forehead lesion confirmed the diagnosis of anaplastic lymphoma kinase-negative ALCL. Chemotherapy with brentuximab vedotin, cyclophosphamide, doxorubicin, and prednisone was planned for a total of 6-8 cycles with curative intent. By cycle 5, positron emission tomography and computed tomography demonstrated response to therapy with no enlarged or metabolically active lymph nodes appreciated. Our case report highlights the importance of developing a broad differential diagnosis for ulcerative facial masses, particularly when unresponsive to antimicrobial therapies. Lymphomas should be included in the differential diagnosis of patients with rapidly growing facial lesions.

Kimura Disease: A Rare and Difficult to Diagnose Entity.

Kimura disease (KD) is a rare inflammatory disorder which involves the head and neck. Due to its rarity and various findings, definitive diagnosis can be difficult to ascertain. Kimura disease is distinguished from other conditions, including angiolymphoid hyperplasia, by histopathological features including follicular hyperplasia, reactive germinal centers, abundant eosinophilia, eosinophilic microabscesses, preserved nodal architecture, Warthin-Finkeldy polykaryocytes, and capsular fibrosis. Herein, we describe the clinical presentation, pathology, and diagnosis of a single case of a 39-year-old treated at an academic center in Texas.

Progressive disfiguring facial masses with pupillary axis obstruction from Morbihan syndrome: A case report.

BACKGROUND: Morbihan syndrome is a rare illness, which presents with recurrent pitting edema on the upper two thirds of the face causing facial contour deformities, and Morbihan syndrome obstructing the pupillary axis with ptosis was seldomly reported. CASE SUMMARY: A 59-year-old woman presented with a 15-year history of facial swelling that progressively invaded bilaterally from the inner canthus and eyelids outwards. Imaging examination indicated that the bilateral periorbital and the left temporal soft tissues had swelling without cranium invasion. Histopathological analysis showed a large amount of lymphocyte infiltration, and immunohistochemistry showed positive expression of CD68 in clear-cut granulomas and D2-40 in the lymphatic endothelium. Finally, the clinical diagnosis of Morbihan syndrome was confirmed. CONCLUSION: Morbihan syndrome is an infrequent and refractory disease, which is characteristic with recurrent woody facial edema on the upper two thirds of the face. Solid facial edema is persistent and non-pitting, causing facial contour deformities and even vision field impairment. The diagnosis of Morbihan syndrome depends on clinical features, imaging information, and pathology. Blepharoplasty is optional to improve the visual field for these patients with severe pupillary axis obstruction.

Congenital hemangioma of the face-Value of fetal MRI with prenatal ultrasound.

We report the presentation, workup, and pre/perinatal management of a fetus with a large congenital hemangioma of the face. Hemangiomas are benign vascular neoplasms frequently encountered in neonates and infants. The less common congenital variant develops in utero and can present on prenatal ultrasound with diagnostic uncertainty as well as clinical implications for delivery. The differential diagnosis for these solid vascular masses when located in the head and neck includes myofibroma, kaposiform hemangioendothelioma, teratoma, and encephalocele. Potential clinical issues relate to size and location of the mass and include airway obstruction, disruption in development or compression of the globe, invasion into the cranial vault, bleeding and ulceration, and high output heart failure. Prenatal ultrasound and MRI play an important role in the diagnosis of congenital hemangiomas and planning for delivery and immediate postnatal cares.

Defecto oncológico maxilar reconstruido mediante colgajo de músculo temporal / Maxillary oncologic defect reconstructed with temporal muscle flap / Defeito oncológico maxilar reconstruído por aba muscular temporal

El macizo facial es una región anatómica compleja que alberga órganos sensoriales. El desarrollo de una enfermedad oncológica, así como su tratamiento, causan defectos funcionales y estéticos con un alto costo físico y psíquico para el paciente y su entorno. Es por ello que la reconstrucción representa un reto. Las diferentes posibilidades incluyen prótesis obturatríces, colgajos libres, pediculados o microvascularizados. En este artículo se describirá el colgajo de músculo temporal y se desarrolla un caso clínico de cirugía oncológica maxilar reconstruido mediante éste en un paciente que presenta una lesión exofítica en cuadrante superior derecho, que se extiende sobre el flanco vestibular, reborde alveolar y zona palatina, desde zona de premolares hasta la zona del segundo molar inclusive, impidiéndole usar la prótesis dental. Esta cirugía es una técnica económica, que requiere menor tiempo quirúrgico que otras técnicas, asociándose a poco porcentaje de fracaso y pocas complicaciones post operatorias.

The facial mass is a complex anatomical region that houses sensory organs. The development of an oncologic disease, as well as its treatment, causes functional and esthetic defects with a high physical and psychological cost for the patient and his environment. This is why reconstruction represents a challenge. The different possibilities include obturator-root prostheses, free, pedicled or microvascularized flaps. In this article the temporal muscle flap will be described and a clinical case of maxillary oncologic surgery reconstructed by means of it is developed in a patient who presents an exophytic lesion in the right upper quadrant, which extends over the vestibular flank, alveolar ridge and palatal area, from the premolar area up to and including the second molar area, preventing him from using the dental prosthesis. This surgery is an economical technique that requires less surgical time than other techniques, and is ass

A massa facial é uma região anatômica complexa que abriga órgãos sensoriais. O desenvolvimento de uma doença oncológica, assim como seu tratamento, causa defeitos funcionais e estéticos com alto custo físico e psicológico para o paciente e seu ambiente. A reconstrução é, portanto, um desafio. As diferentes possibilidades incluem as próteses de raiz obturadora, abas livres, pediculadas ou microvascularizadas. Este artigo descreve o retalho muscular temporal e descreve um caso clínico de cirurgia oncológica maxilar reconstruída utilizando-o em um paciente com lesão exofítica no quadrante superior direito, estendendo-se pelo flanco vestibular, rebordo alveolar e área palatina, desde a área do pré-molar até a área do segundo molar inclusive, impedindo-o de utilizar a prótese dentária. Esta cirurgia é uma técnica econômica, que requer menos tempo cirúrgico que outras.

An incidental finding of pleomorphic adenoma of the minor salivary glands in the skin area of the lower lip.

Salivary gland tumors are uncommonly seen and account for less than 3% of the head and neck tumors. Pleomorphic adenoma is a well-described benign tumor of the salivary glands, originating from myoepithelial and intercalated duct cells. It is most commonly found in major salivary glands. We present a rare and unusual case of pleomorphic adenoma of the minor salivary glands in the lower lip. The tumor was diagnosed upon excision of 1.5 × 1.2 cm(2) well-circumscribed nodule at the junction of the lower lip and chin in a 46-year-old man. The histopathological analysis confirmed presence of an epithelial salivary gland tumor with islands of plasmacytoid cells, and duct-like structures within a variable and mixed stroma.

Surgical Treatment of the Craniofacial Dysostoses-Crouzon's Disease

Craniofacial dysostoses(Crouzon's disease) are characterized by cranial and facial deformities but the relationship between the craniosynostosis and facial deformities has not been clearly established. However, we believe that certain facial hypoplasias are comparable in nature with craniostenosis. Our objective in treatment of the Crouzon's disease is to achieve a postero-anterior movement of the whole facial mass, with the double aim of establishing normal dental occlusion and of increasing orbital capacity. The method we describe uses Tessier's basic procedures for surgical correction of the Crouzon's diseases patient who was 16 year-old boy. It would appear that the best treatment of these requires a radical and total osteostomy of the middle third of the face, to permit complete repositioning of the involved segment. These total osteotomies frequently include the creation of a line of separation between the anterior cranial base above and the facial bone below and also between the pterygoid processes behind and the maxilla anteriorly. Thus one is detaching the face from the cranial base. The authors described the surgical management of the Crouzon's disease and discussed the warning points of intraoperative technique in correction of the craniofacial dysostosis.