Anomaly

Embryogenic cervico-thyro-piriform tract

October 4, 2012     J. Madana, MS, DNB; Deeke Yolmo, MS; Sunil Kumar Saxena, MS; S. Gopalakrishnan, MS
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Abstract

Branchial cleft fistulae are rare congenital anomalies that arise from the abnormal persistence of branchial remnants. Branchial arch anomalies are rare. They usually present as a lateral neck mass or abscess in the form of acute suppurative thyroiditis. A complete fistula of the third arch is extremely rare. We describe such a case in a 13-year-old girl who presented with a small opening in the left lower neck, from which a mucoid discharge had been present since birth. The fistula was accompanied by recurrent neck swelling. Computed tomography with contrast injection into the external skin opening revealed a continuous tract that extended to the base of the piriform sinus. Total excision of the tract up to the piriform sinus with a left hemithyroidectomy was performed. At follow-up 28 months postoperatively, the patient exhibited no evidence of recurrence.

Middle fossa repair of bilateral large congenital tegmental defects with meningoencephaloceles

June 4, 2012     Anil Joshi, MS(ENT), MRCS; Wendy Smith, FRCS(ORL–HNS); David A. Moffat, FRCS
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Abstract

Spontaneous temporal meningoencephaloceles are unusual. When they do occur, they present with a variety of signs and symptoms, which can make diagnosis and management challenging. We report the interesting case of a 49-year-old woman with bilateral congenital temporal meningoencephaloceles. She presented with a 12-month history of bilateral fluctuating hearing loss, and she more recently developed right-sided acute otitis media with meningitis. The presentation of bilateral extensive tegmental defects and meningoencephaloceles with a fluctuating hearing loss and meningitis associated with acute otitis media affecting one ear and then subsequently the other ear is extremely rare and difficult to diagnose. It requires a very careful clinical and radiologic assessment. Methods of surgical repair differ depending on the size of the defects.

Thyroid hemiagenesis

April 30, 2012     Kuang-chun Hsieh, MD; Montu Patel, MD; Enrique Palacios, MD, FACR; Harold R. Neitzschman, MD, FACR
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In cases of incidental thyroid hemiagenesis, findings on endocrinologic and functional studies are usually normal, and there is no evidence that the incidence of thyroid pathology is any greater in these patients than it is in the general population.

Giant tracheocele with multiple congenital anomalies

April 30, 2012     J. Madana, MS, DNB; Deeke Yolmo, MS; Sunil Kumar Saxena, MS; S. Gopalakrishnan, MS
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Abstract

Tracheocele-an outpouching of tracheal mucous membrane-is an uncommon entity. It can occur as a congenital or acquired form. The congenital entity remains mostly dormant until adulthood, and then it typically presents as a herniation with multiple air-filled sacs. The acquired form develops as the result of blunt trauma, recurrent pulmonary infection, intubation, instrumentation, or surgery, and it typically presents as a single paratracheal cavity. We present an extremely rare case of a tracheocele associated with multiple congenital anomalies involving the face, limbs, and heart.

Congenital os vomer agenesis: Case report and literature review

March 31, 2012     Ediz Yorgancilar, MD, Muzeyyen Yildirim, MD, Ramazan Gun, MD, Faruk Meric, MD, and Ismail Topcu, MD
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Abstract

Defects of the nasal septum occur as a result of a variety of causes, including tuberculosis, irritation, neoplasia, trauma, infection, and chronic inflammatory diseases. Congenital os vomer agenesis as a cause is very rare. We report the case of a 28-year-old man with a defect in the posteroinferior part of the nasal septum that was discovered incidentally during a routine endoscopic examination. The patient was diagnosed with congenital os vomer agenesis, and the diagnosis was confirmed by computed tomography. We discuss the features of this case and review the literature on this rare anomaly.

Lateral ectopic thyroid: A case diagnosed preoperatively

March 31, 2012     Héctor Prado, MD, Alejandro Prado, MD, and Bertha Castillo, MD
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Abstract

Ectopic thyroid is an uncommon condition defined as the presence of thyroid tissue at a site other than the pretracheal area. When the process of embryologic migration is disturbed, aberrant thyroid tissue may appear. In most cases, ectopic thyroid is located along the embryologic descent path of migration as either a lingual thyroid or a thyroglossal duct cyst. In rare cases, aberrant migration can result in lateral ectopic thyroid tissue. Approximately 1 to 3% of all ectopic thyroids are located in the lateral neck. Ectopic tissue frequently represents the only presence of thyroid tissue; a second site of orthotopic or ectopic thyroid tissue is found in other cases. The presentation of ectopic thyroid as a lateral mass should be differentiated from metastatic thyroid cancer; other differential diagnoses include a submandibular tumor, branchial cleft cyst, carotid body tumor, and lymphadenopathy of various etiologies. In addition to the history and physical examination, the workup for a patient with a submandibular mass suspicious for ectopic thyroid should include (1) technetium-99m or iodine-131 scintigraphy, (2) ultrasonography and either computed tomography or magnetic resonance imaging, (3) fine-needle aspiration biopsy, and (4) thyroid function testing. No treatment is required for asymptomatic patients with normal thyroid function and cytology, but hypothyroid patients should be placed on thyroid hormone replacement therapy. Most cases are diagnosed postoperatively. Surgical treatment of ectopic thyroid should be considered when a malignancy is suspected or diagnosed, when the patient is symptomatic, or when thyroid suppression therapy fails.

Two-flap palatoplasty: Description of the surgical technique and reporting of results at a single center

March 1, 2012     Emmanouel Koudoumnakis, MD, Ioannis M. Vlastos, MD, PhD, Kostas Parpounas, MD, PhD, and Michael Houlakis, MD, PhD
article

Abstract

Two-flap palatoplasty is commonly used to treat cleft palate defects, but only a few reports on outcomes have been published in the literature. We conducted a retrospective analysis of 257 cases of cleft palate treated with two-flap palatoplasty at a single center in Greece over a 13-year period. Our outcomes data included surgical complication rates, the results of speech assessments, midface development, and other parameters. We found a low rate of short- and long-term complications that required revision surgery, such as symptomatic fistula (5.4%) and velopharyngeal insufficiency (5.3%). Speech outcomes in relation to intelligibility, hypernasality, and nasal emissions were satisfactory in 70 to 86% of patients. Dental arch relationships, as estimated by the 5-Year-Olds Index, were judged to be either good or excellent in 62% of those evaluated. A considerable proportion of patients (45%) who had otitis media with effusion experienced a spontaneous resolution without the use of tympanostomy tubes 2 to 8 months after their operation. We conclude that two-flap palatoplasty is an effective procedure that warrants further attention. We describe the surgical technique in detail. Our technique includes a modified intravelar veloplasty that incorporates near-total muscle retropositioning.

Frontal infundibular cells: Pathway to the frontal sinus

March 1, 2012     Ozum Tuncyurek, MD, Murat Songu, MD, Zehra Hilal Adibelli, MD, and Kazim Onal, MD
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Abstract

A frontal infundibular cell (FIC) is an uncommon anatomic variant of an anterior ethmoid pneumatization that originates from the frontal recess and typically extends into the lumen of the frontal sinus ostium. FICs may show several anatomic variants that impact the anatomy of the frontal recess. Familiarity with these variants is required for safe endoscopic sinus surgical procedures. We conducted a retrospective chart review, examining 1,040 frontal recesses in 520 adults with no frontal sinus disease, to explore the anatomy of the healthy frontal recess and to describe the prevalence of FICs in patients without a history of conditions that cause frontal pneumatization. Review of axial and coronal computed tomography data, which were reformatted for sagittal reconstruction, was performed at a computer workstation. Bent and Kuhn's classification was used to define types of FICs. We identified 167 patients (32.1%) as having unilateral or bilateral FICs. Type I infundibular cells were the most common type (found in 15.7% of the entire study population). Type II and type III FICs were found in 3.8% and 12.1%, respectively; type IV FICs were present in 0.4%. The FIC has not been emphasized as an important potential cause of frontal sinus obstruction in the radiology literature, but clinicians should specifically watch for it on all routine computed tomography of the frontal sinus. Fine-cut computed tomography scans aid in the identification of each individual cell and allow the surgeon to formulate a clear and precise surgical plan. A surgical plan with a thorough understanding of the anatomy enables confident dissection of this complex and difficult area.

Paradoxical giant inferior concha

July 13, 2011     Barış Naiboğlu, MD, Atılay Yaylacı, MD, and Çağatay Oysu, MD
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Abstract

Anatomic variations of the middle and superior conchae are common, but inferior concha anomalies are rare. These anomalies have included pneumatization, agenesis, and duplication. We describe what we believe is the first reported case of a paradoxical curvature of the bony part of the inferior concha. The patient was a 13-year-old girl. The development of a huge inferior concha blocked the left nasal cavity and deviated the nasal septum to the contralateral side, resulting in bilateral obstruction. The patient was successfully treated with surgery.

Type 1 first branchial cleft anomaly: Duplication of the external auditory canal

July 13, 2011     Matthew L. Carlson, MD, David J. Archibald, MD, and Shelagh A. Cofer, MD
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Stahl ear deformity associated with Finlay-Marks syndrome

May 31, 2010     Young S. Paik, MD and C.W. David Chang, MD
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Adult presentation of a fourth branchial pouch sinus

September 30, 2009     Jon Robitschek, MD, Nici Bothwell, MD, Guy Takahashi, MD, Daniel Hall, MD, FACS, and Joseph Sniezek, MD, FACS
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Abstract

A fourth branchial pouch sinus is a rare clinical entity. The handful of previously reported cases have all involved children. We report a rare case of an intrathyroidal fourth branchial pouch sinus in an adult. A 36-year-old man presented with a recurrent left-sided deep neck abscess. A diagnostic evaluation by barium pharyngoesophagography in conjunction with computed tomography revealed the presence of a sinus tract that extended inferiorly from the left piriform sinus to the left thyroid lobe. The synchronous use of both of these imaging modalities with shared contrast enabled us both to establish the diagnosis and formulate a successful surgical approach. The patient underwent elective neck exploration with resection of the left thyroid lobe and superior sinus tract. Postoperative pathology confirmed the presence of an epithelium-lined sinus within the left lobe of the thyroid consistent with a branchial pouch derivative. Follow-up barium pharyngoesophagography demonstrated successful closure of the sinus tract. This unique case represents a delayed clinical presentation, and it broadens the differential diagnosis of recurrent deep neck abscess and thyroid cyst in the adult.

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