Anomaly

Management of soft palate agenesis in Nager syndrome with an elongated, superiorly based pharyngeal flap

October 17, 2014     Oneida A. Arosarena, MD; Troy Hemme, DO
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Abstract

Nager syndrome, or preaxial acrofacial dysostosis, is associated with aberrant development of the first and second branchial arch structures, as well as abnormal development of the limb buds. It is a rare disorder, and its clinical manifestations have not been completely defined. Absence of the soft palate has been reported in patients with Nager syndrome. In this report we describe the use of an elongated, superiorly based pharyngeal flap for the treatment of severe velopharyngeal insufficiency in a patient with Nager syndrome and absence of the soft palate. We also describe the dysmorphisms associated with Nager syndrome and present a differential diagnosis for the condition.

A persistent left superior vena cava, with the accessory nerve passing through a duplicate segment of the left internal jugular vein: A unique presentation

October 17, 2014     Omar Ayoub, MSc, FRCSEd(ORL-HNS); Jamie Benton, MRCS; Shaun Jackson, FRCSEd
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Abstract

The internal jugular vein and the spinal accessory nerve are important landmarks during neck dissection; unexpected variations in their positions can result in inadvertent iatrogenic damage to adjacent structures. We report the case of a 79-year-old man who was found during neck dissection to have a duplicate segment of his left internal jugular vein (IJV), a persistent left superior vena cava, and an absent right superior vena cava. The spinal accessory nerve passed through the duplicate section of the IJV. A MEDLINE search found no previously reported case of these anomalies occurring together. We also review 10 previously reported cases of IJV duplication. Finally, we discuss the embryologic and anatomic background of these malformations so that otolaryngologists may be aware that identification of such anomalies may help to prevent postoperative morbidity.

Complete second branchial cleft anomaly presenting as a fistula and a tonsillar cyst: An interesting congenital anomaly

October 17, 2014     Prasad John Thottam, DO; Samba S. Bathula, MD; Janet M. Poulik, MD; David N. Madgy, DO
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Abstract

Branchial cleft anomalies make up 30% of all pediatric neck masses, but complete second branchial cleft anomalies are extremely rare. We report an unusual case of a complete second branchial cleft anomaly that presented as a draining neck fistula and a tonsillar cyst in an otherwise healthy 3-month-old girl. At the age of 7 months, the patient had been experiencing feeding difficulties, and there was increasing concern about the risk of persistent infections. At that point, the anomaly was excised in its entirety. Our suspicion that the patient had a complete second branchial cleft anomaly was confirmed by imaging, surgical excision, and histopathologic analysis.

Congenital airway anomaly of double aortic arch in a 2-day-old infant

October 17, 2014     Seo Moon, MD; Jessica Mayor, MD; Ramzi Younis, MD
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Double aortic vascular ring is a complete vascular ring that is formed when the distal portion of the right dorsal aorta fails to regress and the ascending aorta bifurcates to surround and compress both trachea and esophagus and rejoins to form the descending aorta.

Second branchial cleft anomaly with an ectopic tooth: A case report

September 17, 2014     Jennifer C. Alyono, MD; Paul Hong, MD; Nathan C. Page, MD; Denise Malicki, MD, PhD; Marcella R. Bothwell, MD
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Abstract

Branchial cleft cysts, sinuses, and fistulas are the most common congenital lateral neck lesions in children. They arise as a result of an abnormal development of the branchial arches and their corresponding ectoderm-lined branchial clefts. Of these diverse anomalies, second branchial cleft lesions are the most common, accounting for approximately 95% of all branchial arch pathologies. We describe what is to the best of our knowledge the first reported case of an ectopic tooth in a branchial cleft anomaly. The patient was a young girl who had other congenital abnormalities and syndromic features and who was eventually diagnosed with Townes-Brocks syndrome. We describe the clinical presentation, management, pathologic analysis, and postoperative outcomes of this case, and we present a brief review of Townes-Brocks syndrome.

Petrosquamosal sinus discovered during mastoidectomy, and its radiologic appearance on temporal bone CT: Case report and brief review

July 13, 2014     Hyun Joon Shim, MD; Seong Jun Song, MD; Ki Woong Chung, MD; Sang Won Yoon, MD
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Abstract

We report the case of a 47-year-old woman who underwent a mastoidectomy. Preoperative computed tomography demonstrated an unusually distended bony canal that passed through the superolateral portion of the right petrous bone. Intraoperatively, we identified the anomaly as a petrosquamosal sinus (PSS). This unusually dilated venous channel had arisen from the adjacent sigmoid sinus. A PSS is an emissary vein of the posterior fossa that courses along the petrosquamosal junction, connecting the sigmoid or transverse sinus with the extracranial venous system. While it usually regresses during fetal life, a dilated PSS occasionally persists into adulthood. Its anatomic course may lead to problematic bleeding during mastoidectomy.

An abnormal sigmoid sinus with a dire clinical implication

June 8, 2014     Perianan Puraviappan, MS(ORL); Naryanan Prepageran, FRCS Ed; Cheng Ai Ong, MS(ORL); Raffique Abd Karim, MS(ORL); Omkara Rubini Lingham, MS(ORL); Rajagopalan Raman, MS(ORL)
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In reviewing CT scans before surgery, the location of the sigmoid sinus should always be noted.

Pseudo third cranial nerve palsy secondary to orbital ectopic lacrimal gland cyst: Management with functional endoscopic sinus surgery

February 12, 2014     Puneet S. Braich, BSc; Jonathan E. Silbert, MD; Andrew J. Levada, MD; and Neil R. Schiff, MD
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Abstract

An otherwise healthy 13-month-old girl was noted by her pediatrician to have developed a left head turn. The patient was referred to a pediatric ophthalmologist, who noticed signs of incomplete third cranial nerve palsy. Magnetic resonance imaging revealed the presence of an abnormal lesion in the inferonasal orbit that was abutting the ethmoid sinus. After consultation with an ENT specialist, the decision was made to remove the lesion via functional endoscopic sinus surgery because this approach was deemed to provide adequate access while limiting morbidity. Histology of the excised lesion identified it as true ectopic lacrimal gland tissue with cysts. We recognize and comment on the fact that in many reported cases of ectopic lacrimal gland cyst, the tissue was not ectopic at all but instead represented an extension of normal lacrimal gland tissue.

The significance of magnetic resonance imaging and computed tomography findings in sphenoid sinus agenesis

February 12, 2014     Lokman Uzun, MD; Omer Faik Sagun, MD; Bulent Seferoglu, MD; Omer Etlik, MD; and Kamran Mahmutyazicioglu, MD
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Abstract

Agenesis of the sphenoid sinuses is a very rarely encountered anatomic variation. Findings on magnetic resonance imaging can mislead the radiologist and clinician. Therefore, the gold standard for diagnosis is computed tomography (CT) of the paranasal sinuses. We present the case of a 28-year-old man who was admitted to our hospital with a 3-month history of headache. CT of the paranasal sinuses revealed isolated bilateral sphenoid sinus agenesis.

When is a neck lump not a neck lump? Initial presentation of a solitary sternocleidomastoid muscle in an adult

January 21, 2014     Sonia Kumar, MRCS; Suzie Jervis, MRCS; Harpreet Uppal, FRCS(ORL-HNS)
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Abstract

A 70-year-old woman was referred to us for evaluation of a presumed left-sided neck mass. The referral was based on the visual appearance of subtle neck asymmetry. The patient reported no ENT symptoms. Clinical examination and ultrasonography identified no pathology, but computed tomography revealed that the asymmetry was present because the patient's right sternocleidomastoid muscle (SCM) was absent. The SCM on the left side was normal. Congenital absence of an SCM is uncommon, and to the best of our knowledge, an initial presentation in an adult has not been previously reported in the literature.

Progressive blindness caused by an unusual sphenoid sinus dehiscence

September 18, 2013     Mariana Marquez, MD; Enrique Palacios, MD, FACR; Jeremy Nguyen, MD; Harold R. Neitzschman, MD, FACR, FACNM, FAAP
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The optic nerve and other important structures in the anterior skull base can be involved in chronic sphenoidal sinus disease.

Coexisting first and bilateral second branchial fistulas in a child with nonfamilial branchio-otic syndrome

July 21, 2013     Jose Florencio F. Lapena Jr., MA, MD; Genilou Liv M. Jimena, MD
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Abstract

We describe what we believe is only the third reported case of coexisting first and bilateral second branchial fistulas associated with nonfamilial branchio-otic syndrome. The patient was a 6-year-old girl who presented with bilaterally draining anterior neck puncta, a preauricular sinus, and moderately severe bilateral hearing loss. She had no family history of branchial anomalies. Compared with branchial cysts and sinuses, branchial fistulas are rare. Even more rare are bilateral second branchial fistulas coexisting with first branchial anomalies, as only 10 cases have been previously reported in the English-language literature. Of these 10 cases, 5 were associated with either branchio-otic syndrome or branchio-oto-renal syndrome; 2 patients had familial branchio-otic syndrome, 2 had nonfamilial branchio-otic syndrome, and 1 had nonfamilial branchio-oto-renal syndrome.

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