Cases reported • Skull Neoplasms

1/11. Endoscopically guided midfacial degloving in infants for removal of congenital and acquired midfacial masses.

Midface degloving allows excellent exposure for a variety of congenital and acquired pediatric facial masses. The petite facial skeleton of the infant and child, however, can limit the utility of this dissection, thereby necessitating external approaches and altered cosmesis. Endoscopic assistance can aid in safe and complete removal of these masses without the need for external surgical approaches. In this series, five infants underwent midfacial degloving for midface lesions. Those masses that could not be adequately visualized underwent midfacial degloving with endoscopic assistance. Successful surgical removal was accomplished without complications, with follow-up ranging from 1 to 5 years. No surgical nasal deformity, vestibular stenosis, or decrease in midfacial growth was noted. Midfacial degloving with endoscopic guidance in selected cases is a cosmetically appealing option for lesions not otherwise resectable by standard midface degloving.

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2/11. Sellar chondroma--case report.

A 12-year-old boy presented with right visual disturbance. Skull radiography and computed tomography (CT) showed an irregular deformity of the sella turcica, hypertrophic change of the dorsum sellae, and an inhomogeneously calcified mass in the sella turcica. Magnetic resonance (MR) imaging demonstrated the mass lesion filled the hypophyseal fossa, and extended to the dorsum sellae, right cavernous sinus, and right suprasellar region. The Dolenc pterional combined epidural and subdural approach was carried out. The histological diagnosis was chondroma. Sellar chondroma requires relief of the compression to the chiasm or optic nerve as soon as possible, so partial resection can still be beneficial. However, follow-up MR imaging or CT, visual examination, and control of pituitary dysfunction are required after the operation.

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3/11. mastoid osteomas: report of two cases.

OBJECTIVE: To discuss the differential diagnosis of two cases with mastoid mass. STUDY DESIGN: Case report and review of the literature. SETTING: The study was carried out in Gulhane Military Medical Academy, Haydarpasa Training Hospital, Istanbul, turkey. PATIENT, INTERVENTION, AND RESULTS: Both cases were 23-year-old males. They admitted to our clinic with masses behind their left ears that had been progressively enlarging for 5 years. In both cases, coronal and axial computerized tomography scans revealed a wide-based lesion involving temporal bone cortex. The cases were operated on for diagnosis and for correction of the cosmetic deformity. Histopathological examinations were consistent with mixed and spongiotic osteoma. CONCLUSIONS: mastoid osteoma is a rare, benign tumor of bone. It may cause cosmetic deformity such as external mass or an auricular protrusion. Other neoplasms of the mastoid region, such as osteosarcoma and osteoblastic metastasis, should be considered for the differential diagnosis.

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4/11. Primary angiosarcoma of the cranial vault: a case report and review of the literature.

BACKGROUND: Angiosarcoma is a rare neoplasm. It is most likely to affect the scalp in elderly people and involvement of the cranium is uncommon. We report a case of primary malignant angiosarcoma of the cranial vault. CASE DESCRIPTION: An 82-year-old man presented with right hemiparesis, deformity of the cranium and mildly swollen scalp. Further studies disclosed bilateral parietal lesions, with destruction of the dura matter and infiltration of the brain parenchyma. thrombocytopenia was found. Faint enhancement of the lesions was seen on magnetic resonance imaging (MRI) after i.v. administration of contrast medium with gadolinium. The tumor was excised including the affected portion of the skull and dura matter. Histologic examination revealed typical pictures of angiosarcoma with immunohistochemical evidence of factor viii-related antigen. Postoperative radiotherapy was employed. The patient remained well at 10 months postoperative follow up. CONCLUSION: Primary angiosarcoma of the cranium is sometimes difficult to differentiate from trauma on clinical examination. We review the literature pertaining to the pathogenesis, clinical course and treatment of this condition. Determination of platelet count may be helpful in monitoring the progression of the disease. The tumor may show faint enhancement on MRI.

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5/11. Ossifying fibroma of the skull: interactive image-guided minimally invasive localization and resection.

Ossifying fibroma is a benign fibro-osseous tumor commonly affecting the craniofacial bones. It is considered to be a locally aggressive and quickly expansible bone lesion. Because of its aggressive nature and high recurrence rate, early detection and complete surgical removal are essential. Usually, these lesions are excised extensively by craniectomy, and bone loss is reconstructed by cranioplasty using acrylic resin or titanium implants. Alternatively, in the management of skull-ossifying fibroma, an image-guided technique using surgical navigation may provide precise information about localization, enabling complete removal, thereby operating with minimal exposure and within narrow resection borders and avoiding significant bone deformity. A 39-year-old male patient with a history of renal cell carcinoma was admitted to our hospital because a radionuclide scintigraphic bone scan revealed increased uptake in a small area located at the left lateral skull bone. The high-resolution computed tomography scan showed that the lesion was located inside the diploe, destroying the inner table of the calvarium. The patient underwent minimally invasive bone lesion removal using an interactive image-guided approach. Complete resection of the neoplastic lesion was achieved. The histopathological examination revealed an ossifying fibroma. The postoperative course was uneventful, and the patient was discharged 3 days after intervention. To date, there has been no evidence of local recurrence. Interactive multimodal planning and intraoperative image guidance offer an interesting approach for biopsy and minimally invasive removal of small ossifying fibroma lesions of the skull, especially in less accessible locations.

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6/11. Extensive neglected psammomatoid ossifying fibroma with craniofacial deformity.

The psammomatoid ossifying fibroma is a rare and aggressive lesion that usually arises from the sinonasal region. The lesion is well circumscribed with the pathognomonic feature of concentric or laminated spherical ossicles that appear similar to psammoma bodies. The authors report a case of an extensive and neglected psammomatoid ossifying fibroma in a 10-year-old West African girl. She had a 5-year history of a slowly growing tumor, which at presentation was 15 cm in diameter and had resulted in left craniofacial deformity as well as left visual impairment. Due to the large tumor, the surgical resection plan necessitated a cranio-facial neurosurgical approach in combination with autogenous bone grafts for cranofacial reconstruction, and allogenous demineralized bone grafts for donor site coverage. In addition, preoperative embolization was utilized to reduce tumor vascularity. However, this embolization resulted in reduction of pericranial flap vascularity, leading to flap ischemia, infection and subsequent removal of necrotic autogenous bone graft. Ultimately, the objectives of large tumor resection and acceptable aesthetic outcome were met after controlling an episode of postoperative infection.

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7/11. surgery of massive fibrous dysplasia and osteoma of the midface.

Since most bony lesions of the midface will stop growing or regress, it is generally not appreciated when fibrous dysplasia and osteoma occasionally continue to grow, causing functional and cosmetic problems. To demonstrate the potential of the disease processes and management options available to the surgeon, a series of patients with massive fibrous dysplasia and osteoma of the midface is reviewed. Most of the patients show a facial deformity alone or in combination with telecanthus, epiphora, exophthalmos, or nasal obstruction. Several types of radical and conservative surgical procedures are described to remove or control the bony lesions, to restore facial contour, and to reconstruct the sinuses, nasal passageways, lacrimal collecting system, and medial wall of the orbit.

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8/11. Hemangiomatous anomaly of bone in Crouzon's syndrome: case report.

A striking and diffuse hemangiomatous architecture was identified by microscopic examination of bone specimens obtained at cranial synostectomy and from the craniofacial area of a young boy with Kleeblattschadel deformity and Crouzon's syndrome. A retrospective review of the histopathological appearance of bone specimens from other patients with Crouzon's syndrome treated on our service and a review of the literature on craniosynostosis suggest that the hemangiomatous pattern seen in this patient is rare if not unique. The unusual pathological findings provide evidence that the underlying pathophysiology of this disorder may be heterogeneous.

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9/11. Surface reconstructions from computerized tomographic scans for evaluation of malignant skull destruction.

The head and neck surgeon is dependent on radiographic technology to diagnose deformity, plan surgery, and evaluate operative results. The development of computerized tomographic scanning has facilitated osseous definition as well as allowed study of intracranial and extracranial soft tissue. Nonetheless, the computerized tomographic image slice format requires an educated interpreter and a fertile imagination for correlation with physical findings. We have created computer software that presents computerized tomographic data as surface images that resemble photographs of the patient or a specimen skull. The program runs on standard computerized tomographic scanners, requires no operator intervention, and is efficient in computation time and space. The surgeon can directly visualize the osseous deformity and mathematically relate it to the overlying soft tissue. The surface images can be viewed from a variety of perspectives, with or without selected structures, such as removing the body of the mandible in the pseudo-water's projection to demonstrate the palate. The image data can be manipulated to obtain a variety of useful results beyond the images themselves. The addition of this technology to the preoperative evaluation and longitudinal follow-up of patients with head and neck cancer has increased our understanding of the extent of their malignancy.

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10/11. Total en bloc resection of the temporal bone and carotid artery for malignant tumors of the ear and temporal bone.

A technique for single stage total en bloc resection of the temporal bone and intratemporal carotid artery with immediate reconstruction has been described. This formidable procedure requires the collaborative efforts of neurotologic skull base surgeons, neurosurgeons, and head and neck surgeons. Two patients have undergone this procedure; one with squamous cell carcinoma and one with extensive basal cell carcinoma. The quality of life following this procedure is adequate and the cosmetic deformity can be minimized. Longer follow-up is necessary to determine the value and overall impact of this approach to cancer of the temporal bone on disease free interval and patient survival. Further experience with the technical aspects of this procedure should reduce the operating time, blood loss, and resultant morbidity.

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