Jose M. Manaligod

Complications of intranasal prescription narcotic abuse.

The abuse of drugs via an intranasal route is an increasingly prevalent pattern of behavior. In the past year, a number of patients received care at our institution for complications resulting from the previously unreported phenomenon of intranasal prescription narcotic abuse. This report describes the clinical manifestations of this form of drug abuse in 5 patients. Their symptoms consisted of nasal and/or facial pain, nasal obstruction, and chronic foul-smelling drainage. Common physical findings were nasal septal perforation; erosion of the lateral nasal walls, nasopharynx, and soft palate; and mucopurulent exudate on affected nasal surfaces. In addition, 2 of the 5 patients had invasive fungal rhinosinusitis, which appears to be a complication unique to intranasal narcotic abuse.

H-type congenital tracheoesophageal fistula: University Of Iowa experience 1985 to 2005.

Objectives: We review the diagnostic workup, associated disorders, surgical technique, and postoperative course of patients who underwent repair of H-type tracheoesophageal fistulas. Methods: We performed a retrospective chart review of patients who received a diagnosis of tracheoesophageal fistula at the University of Iowa. Results: Seven patients with an H-type tracheoesophageal fistula and a single patient with a missed proximal fistula associated with esophageal atresia were identified. Their symptoms included coughing with feeding, recurrent pneumonia, and episodic cyanosis. A delay in diagnosis was seen in 4 patients and ranged from 2.5 months to 5.9 years. In all patients, the diagnosis was made with an esophagogram. The level of the fistulas was between C5 and T3, and all were successfully repaired via a right cervical approach. Conclusions: A high index of suspicion for an H-type tracheoesophageal fistula should be maintained in the presence of neonatal respiratory symptoms, as the condition can be associated with a delay in diagnosis. Repeat esophagograms and bronchoscopy may be required for diagnosis. In the postoperative period, airway obstruction is a potential risk; however, long-term difficulty with swallowing, respiration, and phonation was not observed.

EYA1 mutations associated with the branchio-oto-renal syndrome result in defective otic development in Xenopus laevis

Background information. The BOR (branchio‐oto‐renal) syndrome is a dominant disorder most commonly caused by mutations in the EYA1 (Eyes Absent 1) gene. Symptoms commonly include deafness and renal anomalies.

Lingual Dermoid Cysts

CASE REPORT A 13-year-old white girl presented with a 2-year history of recurrent swelling in the floor of mouth. Episodes of swelling usually progressed over 2 to 4 days and were accompanied by dysphagia, dysarthria, and occasional fevers. Previous treatment consisted of oral antibiotics, resulting only in temporary relief of her symptoms. One episode was complicated by the formation of a submental abscess that required surgical drainage and intravenous antibiotics at another institution. On physical examination, a small pit was seen 2 cm from the tip of the dorsal tongue. An iodine-123 thyroid scan revealed no evidence of ectopic thyroid tissue in the floor of mouth. Magnetic resonance imaging (MRI) showed a 1to 2-cm area between the geniohyoid muscles that enhanced with gadolinium (Fig 1). Exploratory surgery was undertaken through a midline tongue incision. A firm encapsulated mass was discovered that extended from the lingual pit down through the tongue into the floor of the mouth (Fig 2). The sinus tract itself only extended 5 mm past the lingual pit. The deepest portion of the mass extended into the submental space between the geniohyoid muscles. The mass was excised completely, and the tongue was closed in layers. Histopathologic evaluation showed squamous epithelium with pilosebaceous structures and apocrine and eccrine skin appendages consistent with a dermoid cyst. The patient did well after the procedure and recovered without any complications. At 2-year follow-up, she has no signs of recurrence.

EYA1 expression in the developing inner ear.

Objectives: We sought to determine the developmental anatomy and EYA1 protein distribution in the inner ear of Xenopus laevis. Methods: Xenopus laevis embryos were stained with monoclonal antibodies and imaged with confocal microscopy. Results: At stage 27, the otocyst fully forms, with strong tubulin staining of early sensory cells at its ventromedial aspect. Neuronal ingrowth follows at stage 33/34. At stage 50, the semicircular canals are complete. EYA1 localizes to the anterior aspect of the otocyst from stages 37 to 44. By stage 50, EYA1 distribution is localized primarily to the sensory maculae and the endolymphatic duct of the developing inner ear. Conclusions: Whole mount confocal imaging of the developing Xenopus inner ear delineates the exact timing of otic development, sensory cell differentiation, and innervation. EYA1 protein expression has a distinct distribution pattern at the anterior aspect of the developing otocyst in stages 41 and 44. Later stages have a more localized pattern, in which EYA1 is detected only in the sensory epithelium and endolymphatic duct. This specific pattern of expression indicates a possible role in the determination of the anterior-posterior orientation of the inner ear, as well as a later role in sensory cell differentiation.

Primary tracheal papillomatosis presenting as reactive airway disease

Typically patients with recurrent respiratory papillomatosis (RRP) initially manifest laryngeal disease with symptoms of hoarseness, weak cry, and stridor. Extralaryngeal spread is not uncommon, and distal progression may be facilitated by airway epithelial injury, inducing squamous metaplasia. Kashima et al1 found laryngeal papilloma in 97.9% of 417 patients with RRP, but tracheal papilloma only when there was a positive history of tracheostomy. Indeed the diagnosis of RRP may be delayed, as its clinical features often resemble those of more common respiratory disorders.2 We present an 11-year-old boy with no history or evidence of laryngeal papillomata who nevertheless was discovered to have extensive tracheal papillomas.

Pediatric Airway Reconstruction: Principles, Decision-Making, and Outcomes at the University of Iowa Hospitals and Clinics

Pediatric airway stenosis challenges the treating surgeon, from the initial evaluation and decision-making process to the final postoperative care and follow-up setting. As our approach to these cases evolves, we must critically evaluate our outcomes in reference to our stated goals. We describe our process in treating this population and critically examine our outcome data from 1990 to the present. Emphasis is placed on selection of procedure and postoperative management.

Congenital laryngeal webs: surgical course and outcomes.

Objectives We compare the success of different surgical options in the treatment of laryngeal webs. Methods We performed a retrospective study spanning the years 1980 to 2005. Results Eighteen patients were identified. The average age at diagnosis was 6 months (range, 1 day to 2.5 years). The presenting symptoms included weak cry, stridor, airway obstruction, and difficulty breathing. Associated cardiac defects consistent with the diagnosis of 22q-syndrome were present in 7 patients. Webs were classified as grade I (5 patients), grade II (2 patients), grade III (10 patients), or grade IV (1 patient) according to the Cohen classification. In 5 patients, only endoscopic lysis was required. The remaining 13 patients underwent open procedures; 9 patients in this group required tracheotomy. An average of 1.3 open airway procedures was necessary to achieve a decannulation rate of 89%. After operation, 34% of patients had residual webbing and 20% had a weak or aphonic voice. Conclusions Management of laryngeal webs is dependent on the severity of airway obstruction. Grade I and II webs can be treated endoscopically; more severe laryngeal webs usually require tracheotomy and open airway reconstruction.

Tracheal Spindle Cell Tumor in a Child

Tracheal tumors in the pediatric population are rare neoplasms that have devastating consequences if not evaluated and diagnosed in a timely fashion. As illustrated by this case, the tumors typically do not become symptomatic until marked airway obstruction occurs, and patients may present with acute respiratory distress. Radiologic advances, including 3-D CT tracheal reconstruction, are invaluable in the diagnosis of and surgical planning for tracheal neoplasms.

Unilateral laryngeal dysgenesis

A 5-year-old girl had a lifelong history of hoarseness at presentation. The hoarseness was not progressive, and there were no symptoms of dysphagia, dyspnea, or stridor. She was the product of an uneventful full-term pregnancy in a mother with diabetes. She had no history of endotracheal intubation or chronic medical problems. Review of symptoms revealed gait abnormalities and urinary incontinence; further evaluation documented bilateral bifid eleventh ribs, right thoracolumbar scoliosis, cervical and thoracic spinal syrinxes, and an L4 hemivertebra with a malformed L5 vertebra, resulting in a tethered lumbar spinal cord. Flexible laryngoscopy showed both vocal cords to be mobile, but there appeared to be a polyp originating from the right ventricle. Direct laryngoscopy confirmed a normal left hemilarynx and a polypoid appearing right laryngeal mass (Fig. 1). Examination of this tissue allowed it to be identified as a vestigial false vocal cord. Removal demonstrated a hypoplastic right vestibule without a distinct ventricle or vocal cord ligament. There were no other airway anomalies, and a CT scan showed the laryngeal framework to be normal. Her voice was unchanged after surgery, and no further otolaryngologic intervention was planned. Spinal cord decompression effectively relieved her incontinence and altered gait. Normal laryngeal development features formation of an epithelial lamina by the seventh postovulatory week. This structure obliterates the future glottis and supraglottis with the exception of a tiny dorsally located pharyngoglottic duct. Arrest of development at this phase leads to type I laryngeal atresia. 1,2