Anomaly

Bilateral transversely clefted middle turbinates

March 31, 2009     Laura M. Dooley, MD and C.W. David Chang, MD
article

The value of CT venography in the diagnosis of jugular bulb diverticulum: A series of 3 cases

March 31, 2009     Matthew L. Bush, MD, Raleigh O. Jones, MD, and Curtis Given, MD
article

Abstract

Jugular bulb diverticulum is a rare diagnosis, as fewer than 50 cases have been reported in the literature. It has been reported that unilateral auditory symptoms may accompany this entity, although some patients are asymptomatic. We present a case series of 3 patients who were referred to our tertiary care neurotology center with a unilateral jugular bulb diverticulum along with unilateral sensorineural hearing loss and tinnitus. These patients were evaluated clinically and radiographically. This case series (1) adds further documentation of the presence of unilateral auditory symptoms in patients with a jugular bulb diverticulum and (2) demonstrates the value of computed tomographic venography in the diagnosis of jugular bulb diverticulum.

Unilateral submandibular gland aplasia: A rare phenomenon

March 1, 2009     Neel Gupta, MD, Enrique Palacios, MD, FACR, and Shannon Barry, MS
article

The thyroid foramen: A case report and literature review of a largely unrecognized laryngeal anomaly

March 1, 2009     Christine Gilliam, MD and Gregory Renner, MD
article

Abstract

A thyroid foramen is a congenital linear opening located in the lamina of the posterosuperior portion of the thyroid cartilage. Although its overall incidence is reported to be as high as 39%, few practitioners are aware of its existence. We report the case of a 19-year-old man who was involved in a motor vehicle accident. He experienced transient left true vocal fold paresis and what were initially thought to be “bilateral thyroid cartilage fractures” based on computed tomography of the neck performed as part of his trauma evaluation. On further scrutiny, however, the “fractures” were determined to be bilateral thyroid foramina. The presence of the paresis in the setting of these thyroid foramina prompted us to review the literature to examine the significance of thyroid foramina and the vessels and nerves that course through them. The average diameter of a thyroid foramen is approximately 2.5 mm. These openings appear in an oblique orientation, and they occur both unilaterally and bilaterally. In 70% of cases, an anastomotic branch connects the external and internal branches of the superior laryngeal nerve. Embryologically, thyroid foramina are believed to arise from a combined branchial and neurovascular origin. A detailed understanding of laryngeal anatomy, including its variations and their physiologic implications, is important to head and neck surgeons.

Report of a scalp arteriovenous malformation spontaneously hemorrhaging into a preauricular sinus

February 1, 2009     Andrew A. Winkler, MD, M.J.B. Stallmeyer, MD, and Thomas T. Le, MD
article

Abstract

Arteriovenous malformations (AVMs) of the scalp are rare congenital lesions that can be extremely disfiguring and even life-threatening. Another congenital lesion is the preauricular sinus, which forms when the auricular hillocks fail to fuse. We present a case of an AVM of the scalp that ruptured into a preauricular sinus. The occurrence of these two lesions on the same side of a patient is a notable and previously unreported event. The patient was successfully treated with intravascular embolization and surgical ligation.

Asymmetric agenesis of the mastoid antrum in a neonate

January 1, 2009     Rahul K. Shah, MD, Adarsh Vasanth, MD, and Collin S. Karmody, MD, FRCSE
article

Abstract

The authors report a case of asymmetric development of the mastoid antrum in a neonate. The lack of mastoid development most likely represents a congenital mal-development. Agenesis of the mastoid antrum has surgical implications, as failure to recognize its absence can result in disorientation during surgery, with potential to injure adjacent structures.

Lipoid proteinosis (Urbach-Wiethe disease): A case report from India

August 31, 2008     Kadambari Batra, DLO, DNB, Anil Safaya, MS, and Kiran Aggarwal, MS
article

Abstract

Lipoid proteinosis (Urbach-Wiethe disease) is a rare autosomal-recessive anomaly that primarily affects the skin and the mucosa of the upper aerodigestive tract in children. It is caused by hyaline deposits in tissues. Hoarseness secondary to laryngeal involvement is frequently the first presenting feature. It is important to consider this disease in the differential diagnosis of hoarseness because it might lead to life-threatening airway compromise. We report a case of lipoid proteinosis in a 12-year-old girl who presented with hoarseness and skin lesions.

Intratracheal ectopic thyroid tissue presenting with protracted airway obstruction: A case report

July 31, 2008     Mo Khan, MD, Peter G. Michaelson, MD, Major, USAF, MC, FS, and Michael L. Hinni, MD
article

Abstract

Intratracheal ectopic thyroid tissue is a rare finding, with few cases reported in the literature. Ectopic thyroid tissue results when the thyroid gland fails to descend to its final position during early development. These lesions present in various locations, and the clinician should be aware of their existence and presenting signs and symptoms.

An unusual case of adult airway obstruction from a lymphovenous malformation

June 30, 2008     Maj. Wayne J. Harsha, MD, Maj. James V. Crawford, MD, Lt. Col., and Douglas M. Sorensen, MD, FACS
article

Abstract

Lymphatic, venous, and mixed lymphovenous malformations are low-flow lesions that are present at birth and grow proportionately with the patient. We describe an unusual presentation of a lymphovenous malformation in an adult. A 19-year-old man presented to the emergency department with complaints of recent upper respiratory tract symptoms, increasing left-sided sore throat, voice change, odynophagia, dysphagia, and occasional subjective fevers and blood-tinged sputum. Examination revealed the presence of a left peritonsillar bulge consistent with a peritonsillar abscess; however, findings on needle aspiration were negative. The patient was admitted for intravenous steroid and antibiotic therapy. Within 24 hours, his airway became compromised, and he underwent an awake tracheotomy and biopsy, which showed a lymphovenous malformation. Magnetic resonance imaging the following day revealed a large, poorly circumscribed, heterogeneous left parapharyngeal mass consistent with a vascu-lar malformation. With continuation of the steroids and antibiotics, the lesion regressed, and the patient was subsequently decannulated. At the 1-year follow-up, he exhibited no clinical symptoms, and he was in good health off steroids.

Transcervical resection of the styloid process in Eagle syndrome

June 30, 2008     Timothy J. Martin, MD, David R. Friedland, MD, PhD, and Albert L. Merati, MD
article

Abstract

We conducted a retrospective study to determine the treatment outcomes of 6 patients who had either unilateral or bilateral elongated styloid processes and symptoms consistent with Eagle syndrome. Five of these patients had undergone transcervical resection of the styloid process, with relief of symptoms over the follow-up period (mean: 8 mo). Two of the 5 patients experienced a transient weakness in the marginal mandibular nerve, and both recovered completely. The transcervical surgical approach to resection in patients with elongated styloid processes and Eagle syndrome appears to be safe and effective, although the risk for transient marginal mandibular nerve weakness is notable.

Near-complete tracheal ring deformity: A case report

June 30, 2008     Melissa L. Somers, MD and Dana L. Suskind, MD
article

Abstract

Long-segment near-complete tracheal ring deformity is a rare condition with few documented cases. We present the case of a 7-week-old male with total anomalous pulmonary venous return and long-segment near-complete tracheal rings. We discuss the presentation, evaluation, and management of near-complete and complete tracheal rings.

Transmastoid labyrinthotomy approach for cochlear implantation in a common cavity malformation: A case report

May 31, 2008     Masoud Motasaddi Zarandy, MD
article

Abstract

The standard transmastoid facial recess approach has become the popular technique for cochlear implantation. Although this approach has been used for implantation in patients with common cavity malformations, in patients with this anomaly and severe abnormalities of the middle ear, this approach may be technically difficult and makes the facial nerve vulnerable to injury. Using a direct approach to the common cavity through the mastoid cavity, we have successfully performed cochlear implantation in 1 patient with a common cavity malformation. This transmastoid labyrinthotomy approach to the common cavity is an effective and simple technique for placing the electrode array. It minimizes the risk to the facial nerve and may decrease postoperative cerebrospinal fluid leaks.

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