Hrayr K. Shahinian

Craniofacial and skull base trauma

BACKGROUND Traumatic craniofacial and skull base injuries require a multidisciplinary team approach. Trauma physicians must evaluate carefully, triage properly, and maintain a high index of suspicion to improve survival and enhance functional recovery. Frequently, craniofacial and skull base injuries are overlooked while treating more life-threatening injuries. Unnoticed complex craniofacial and skull base fractures, cerebrospinal fluid fistulae, and cranial nerve injuries can result in blindness, diplopia, deafness, facial paralysis, or meningitis. Early recognition of specific craniofacial and skull base injury patterns can lead to identification of associated injuries and allow for more rapid and appropriate management. CONCLUSION Early detection and treatment of craniofacial and skull base traumatic injuries should lead to decreased morbidity and mortality. This review discusses the most common of these injuries, their possible complications, and treatment.

Endoscope-assisted microvascular decompression of the trigeminal nerve.

Twenty-one patients with classic symptoms of trigeminal neuralgia underwent microvascular decompression of the trigeminal nerve through a retrosigmoid approach to the cerebellopontine angle. Endoscopy was used as an adjunctive imaging modality to microscopy. Specifically, endoscopes were used to confirm nerve-vessel conflicts identified by the microscope and to reveal others that escaped microscopic survey. Endoscopes were also used to assess the adequacy of the decompression performed microscopically. A total of 51 nerve-vessel conflicts were identified and treated, 14 of which were discovered only after endoscopy. Additionally, in 5 patients endoscopic examination of the surgical intervention demonstrated that further maneuvers were required to completely decompress the nerve. These results highlight the value of endoscopy in the diagnosis and therapy of cranial nerve pathology in the posterior fossa.

Cerebellopontine angle metastasis from papillary carcinoma of the thyroid : case report and literature review

BACKGROUND Papillary thyroid carcinoma is the most common type of well-differentiated thyroid malignancy and typically has an excellent prognosis and a low incidence of distant metastasis. However, once metastasis has developed in a distant site, prognosis is markedly diminished. Brain metastases from papillary thyroid carcinoma are extremely rare. Currently, there are no established therapeutic guidelines for treating brain metastases from thyroid carcinoma. CASE DESCRIPTION We report on the case of a patient who presented with worsening neurological symptoms 3 years after resection of a thyroid papillary carcinoma. Magnetic resonance imaging identified a lesion of the cerebellopontine angle that encased the lower cranial nerves. The patient underwent a left retrosigmoid craniotomy with a 98% resection of the mass and received postoperative adjuvant radioiodine therapy, external beam radiation, and Gamma Knife radiosurgery. The patient tolerated the procedure well and demonstrated significant progressive improvement in her neurological symptoms postoperatively. After the multimodal approach to therapy, she remains symptom-free at 3-year follow-up. Radiographic monitoring of the small tumor remnant reveals a decrease in size from the postoperative period. CONCLUSION This article is the first formal case report of cerebellopontine angle metastasis from papillary thyroid carcinoma. Therapeutic protocols for brain metastases of papillary thyroid carcinoma are not firmly established. This case illustrates the unique event of a cerebellopontine angle metastasis from papillary thyroid carcinoma, which was successfully treated with a combination of surgical, chemical, and radiological modalities. This aggressive course of therapy has resulted in an excellent outcome in this instance.

Pituitary Lymphoma: A Case Report and Literature Review

We report the case of a B-cell type pituitary lymphoma in a 65 year-old male immunocompetent patient who presented with hypogonadotropic hypogonadism and central hypothyroidism and subsequently developed pulmonary lymphoma. Only three cases of pituitary lymphoma have been previously reported, one in a patient with acquired immunodeficiency syndrome, one case of T-cell lymphoma reported in the Japanese literature, and one case of B-cell lymphoma. The previously reported immunocompetent patients presented with signs and symptoms of optic chiasm compression as contrasted to our patients endocrinologic presentation. B-cell lymphoma of the pituitary gland is a exceedingly rare though distinct clinical entity.

Application of the supraorbital endoscopic approach to tumors of the anterior cranial base.

Access to tumors of the anterior cranial fossa traditionally has required wide exposure of the surgical field, along with prolonged retraction of the frontal lobes or potentially disfiguring transfacial approaches. These approaches subject patients to undesirable neurologic and cosmetic morbidity. With the introduction of progressively less-invasive approaches, intracranial tumors with craniofacial involvement have become amenable to en bloc resection with a minimum of deleterious consequences. The authors report their experience with a supraorbital endoscopic approach. This technique is suitable for lesions situated in the region of the anterior cranial fossa, the suprasellar, and parasellar regions. The technique was applied to 24 patients. Pathologies treated were meningiomas, craniopharyngiomas, pituitary adenomas with extrasellarextensions, and other variable supratentorial pathologies. The use of endoscopy allowed thorough visualization of all critical structures at the paramedian skull base without the need for a bicoronal scalp flap, bifrontal osteotomies, or brain retraction. Most lesions were resected in their entirety with no perioperative complications and with excellent cosmetic results. These cases demonstrate how the application of endoscopy to surgery of the anterior skull base and craniofacial skeleton can eliminate the need for traditional open techniques without compromising surgical success.

Prevalence of GH and other anterior pituitary hormone deficiencies in adults with nonsecreting pituitary microadenomas and normal serum IGF‐1 levels

Objective  GH is usually the first pituitary hormone to be affected following a pathological insult to the pituitary; however, data on the prevalence of GH deficiency in patients with nonsecreting pituitary microadenomas and normal serum IGF‐1 levels are scarce. This study aims to evaluate the prevalence of GH and other anterior pituitary hormone deficiencies, and to determine whether microadenomas per se could be associated with reduced GH response rates to GHRH–arginine stimulation.

Congenital teratoma of the oropharyngeal cavity with intracranial extension: case report and literature review.

Congenital teratoma of the oropharyngeal cavity is a unique clinical entity that poses immediate threats to the neonate in the postpartum period. Establishment of a secure airway is a primary goal after delivery; complete surgical resection is the subsequent guiding principle of management. Even more rare than teratomas confined to the oropharynx are tumors that extend into the intracranial cavity during development. Descriptions of these lesions in the medical literature are uncommon. In the majority of these reports, the tumors are associated with stillbirth, perinatal death, or significant morbidity after attempted resection. We present the rare case of a patient with congenital oropharyngeal teratoma with intracranial extension who has demonstrated long-term survival without any neurological or functional deficits. A unique delayed staged approach to resection of the extracranial and intracranial components of the tumor is described.

High prevalence of normal tests assessing hypercortisolism in subjects with mild and episodic cushing's syndrome suggests that the paradigm for diagnosis and exclusion of cushing's syndrome requires multiple testing

Many Endocrinologists believe that a single determination of eucortisolism or a single demonstration of appropriate suppression to dexamethasone excluded Cushings syndrome, except in what was previously thought to be the rare patient with episodic or periodic Cushings syndrome. We hypothesize that episodic Cushings syndrome is relatively common and a single test assessing hypercortisolism may not be sufficient to accurately rule out or diagnose Cushings syndrome and retrospectively examined the number of normal and abnormal tests assessing hypercortisolism performed on multiple occasions in 66 patients found to have mild and/or episodic Cushings syndrome compared to a similar group of 54 patients evaluated for, but determined not to have Cushings syndrome. We found that 65 of the 66 patients with Cushings syndrome had at least one normal test of cortisol status and most patients had several normal tests. The probability of having Cushings syndrome when one test was negative was 92% for 23:00 h salivary cortisol, 88% for 24-h UFC, 86% for 24-h 17OHS, and 54% for nighttime plasma cortisol. These results demonstrated that episodic hypercortisolism is highly prevalent in subjects with mild Cushings syndrome and no single test was effective in conclusively diagnosing or excluding the condition. Rather, the paradigm for the diagnosis should be a careful history and physical examination and in those patients in whom mild Cushings syndrome/disease is strongly suspected, multiple tests assessing hypercortisolism should be performed on subsequent occasions, especially when the patient is experiencing signs and symptoms of short-term hypercortisolism.

Fully Endoscopic Supraorbital Resection of Congenital Middle Cranial Fossa Arachnoid Cysts: Report of 2 Cases

Intracranial congenital arachnoid cysts are benign intra-arachnoidal fluid collections with a wall composed of arachnoid cells encompassing a cavity containing a fluid similar to cerebrospinal fluid. This cavity frequently communicates with the subarachnoid space. Arachnoid cysts are reported to account for about 1% of all intracranial space-occupying lesions. The most frequent localization of congenital intracranial arachnoid cysts is in the middle cranial fossa, constituting more than half of the cases reported in the literature. We report our use of a fully endoscopic supraorbital approach through the eyebrow for accessing and resecting congenital middle cranial fossa arachnoid cysts. The approach was performed on 2 patients, a 9-month-old infant and a 12-year-old female patient. In both cases, the cysts were resected in their entirety utilizing a 1-cm ‘keyhole’ craniotomy, without the need for a corticotomy and with virtually no brain retraction. The outcomes were favorable and both patients were discharged from hospital within 48 h of surgery. There were no perioperative complications and the cosmetic outcomes were excellent.

Neural transformation in a pituitary corticotroph adenoma

Abstract A pituitary mass was removed by the transsphenoidal approach from a 63-year-old man with the clinical history and laboratory findings characteristic of Cushings disease with partial hypopituitarism. Histological, immunohistochemical, ultrastructural and immunoelectron microscopic investigation demonstrated a periodic acid-Schiff-positive, adrenocorticotropic hormone (ACTH)-immunoreactive, pituitary corticotroph adenoma with the formation of neural tissue resembling neuropil within the tumor. The neural elements showed immunopositivity for neurofilament protein and ACTH, but were immunonegative for other adenohypophysial hormones and for corticotropin-releasing hormone. Although the molecular mechanism accounting for neural transformation in this corticotroph adenoma remained obscure, based on the clinical, histological and morphological findings it appears that formation of neural tissue most likely indicate a favorable prognosis.