Jeffrey C. Rastatter

Mammary analogue secretory carcinoma of the parotid gland in a pediatric patient

S alivary neoplasms comprise 1% to 3% of all head and neck malignancies. Less than 5% of salivary gland malignancies are diagnosed in children. A recent study in adult patients describes a new salivary gland tumor, mammary analogue secretory carcinoma of the salivary gland (MASC). This lesion has pathological characteristics similar to secretory carcinoma of the breast, salivary acinic cell carcinoma, and low-grade cystadenocarcinoma. MASC is frequently associated with a translocation, t(12;15)(p13;q25), resulting in the fusion gene ETV6-NTRK3. This translocation has been demonstrated consistently in secretory carcinoma of the breast. The tyrosine kinase encoded by this fusion gene has been directly related to transformation of epithelial cells in mouse mammary glands. Children’s Memorial Hospital institutional review board approval was obtained, and we report the first case of MASC in the parotid gland of a child.

Primary Resection of Kommerell Diverticulum and Left Subclavian Artery Transfer

BACKGROUND A Kommerell diverticulum (KD) is an aneurysmal remnant of the dorsal fourth aortic arch. This can be an independent cause of tracheoesophageal compression. We previously reported resection of the KD with left subclavian artery transfer to the left carotid artery for recurrent symptoms in patients with a right aortic arch, left ligamentum, and retroesophageal left subclavian artery after prior ligamentum division. In 2001 we began resecting the KD and transferring the left subclavian artery to the left carotid artery in selected patients as a primary operation. METHODS From 2001 to 2011, 20 patients have had primary excision of a Kommerell diverticulum. Diagnosis was with computed tomographic scan (n = 14) or magnetic resonance imaging (n = 6) and bronchoscopy. Sixteen patients had a right aortic arch and 4 had a double aortic arch (right arch dominant). All patients were approached through a left thoracotomy. Fifteen patients had simultaneous division and reimplantation of the left subclavian artery into the left carotid artery. RESULTS Mean age at operation was 9.1 ± 6.5 years (range 1.5 to 29.1 years). Symptoms included cough, wheezing, stridor, dysphagia, and dyspnea on exertion. Selection criteria included KD greater than 1.5 times the size of the left subclavian artery and posterior pulsatile compression of the trachea on bronchoscopy. There were no complications related to subclavian artery transfer. No patient required a blood transfusion. No patient had a recurrent laryngeal nerve injury or chylothorax. The mean hospital stay was 4.3 ± 2.5 days. All patients had resolution of their preoperative airway and esophageal symptoms. CONCLUSIONS In selected patients with a vascular ring we now recommend resection of the associated Kommerell diverticulum and transfer of the retroesophageal left subclavian artery to the left carotid artery as a primary procedure. This strategy requires comprehensive and precise preoperative imaging with either computed tomography or magnetic resonance imaging.

Pulmonary artery sling: Current results with cardiopulmonary bypass

OBJECTIVE We have used cardiopulmonary bypass with left pulmonary artery reimplantation for pulmonary artery sling repair since 1985. This review presents our current results with this technique, emphasizing the importance of diagnosis and treatment of frequently associated tracheal stenosis. METHODS Since 1985, 34 patients have undergone pulmonary artery sling repair using a median sternotomy and cardiopulmonary bypass. Age ranged from 9 days to 43 years (mean 2.1 ± 7.5 years, median 0.2 years). Twenty-seven patients (79%) had tracheal stenosis secondary to complete cartilage tracheal rings. All patients had preoperative airway imaging with rigid bronchoscopy, and since 2000 all patients have had computed tomography imaging of the chest with 3-dimensional reconstruction (n = 14). Tracheal repair has included pericardial patch tracheoplasty (n = 7), tracheal autograft (n = 10), tracheal resection (n = 4), and slide tracheoplasty (n = 5). All patients had an echocardiogram, and cardiac lesions repaired simultaneously included atrial septal defect (4), tetralogy of Fallot (2), and ventricular septal defect (1). One patient had a severely hypoplastic right lung, and 3 patients had an absent right lung. In these patients, the left pulmonary artery was translocated anterior to the trachea. In all other patients, the left pulmonary artery was reimplanted into the main pulmonary artery. RESULTS There have been no early deaths or complications related to the use of cardiopulmonary bypass. Median hospital stay was 24 days. There have been 4 late deaths. Two late deaths were the result of complications of tracheal surgery (1 pericardial patch [6 months postoperatively] and 1 autograft [1.7 years postoperatively]). One child died of biliary atresia (0.2 years postoperatively), and 1 child died of pneumonia (5.8 years postoperatively). All recent tracheal stenosis repairs have been with slide tracheoplasty. All left pulmonary arteries are patent with a mean percent flow by perfusion scan of 41% ± 13%. Older patients (n = 2) have noted a significant improvement in exercise tolerance. CONCLUSIONS Pulmonary artery sling is best repaired with median sternotomy, cardiopulmonary bypass, and left pulmonary artery reimplantation. This resulted in uniformly patent left pulmonary arteries in all patients. Preoperative computed tomography imaging, echocardiography, and bronchoscopy are essential for precise operative planning. The frequently associated tracheal stenosis is best repaired with slide tracheoplasty.

Aspiration before and after Supraglottoplasty regardless of Technique

Objective. To determine the incidence of preoperative and postoperative aspiration in infants who undergo supraglottoplasty. To determine the effect of cold steel and CO2 laser supraglottoplasty on aspiration in infants with severe laryngomalacia. Design. Retrospective study. Setting. Tertiary pediatric hospital. Patients. Thirty-nine patients who underwent CO2 laser-assisted supraglottoplasty (CLS) or cold steel supraglottoplasty (CSS) for severe laryngomalacia. Main Outcome Measures. Aspiration and upper-airway obstruction. Results. Thirty-nine patients met inclusion criteria (18 males, 21 females). Eighteen patients underwent CSS and 21 patients underwent CLS. 10/39 (25.6%) of the patients had preoperative aspiration, and 2/10 (20%) resolved after supraglottoplasty. New onset aspiration was found in 4/13 (30.8%) in the CSS group and 9/16 (56.3%) in the CLS group. Conclusions. There is no significant difference in the rate of postoperative new-onset aspiration or relief of upper-airway obstruction in the CLS or CSS, is temporary and can be managed with thickened diet or temporary tube feedings. The rate of persistent postoperative aspiration was statistically similar regardless of the method of surgery.

The second branchial cleft fistula.

OBJECTIVE To review the surgical anatomy and histopathology of second branchial cleft fistulae. STUDY DESIGN Retrospective study of patients treated for second branchial cleft fistulae at a tertiary care pediatric hospital. The senior author noted anatomic and histologic features of second branchial cleft fistulae, not previously described. SETTING Tertiary care childrens hospital. PATIENTS AND METHODS Retrospective examination of 28 patients was conducted who were operated upon for second branchial cleft fistula. Data collected included age at surgery, initial presentation, imaging characteristics prior to surgery, laterality of the fistula tract, pathology results and follow-up data. RESULTS Twenty-eight patients met the criteria for inclusion. Three patients (11%) had bilateral fistulae. 11 (39%) were male and 17 (61%) were female. 23 (74.2%) tracts were lined with ciliated columnar epithelium, 3 (9.7%) had cuboidal epithelium, and 5 (16.7%) had squamous epithelium. Nineteen (61.3%) tracts contained salivary tissue. Of the unilateral fistula tracts, 25 (100%) were on the right side. Of the 3 patients with bilateral lesions, 2 (66%) had associated branchio-oto-renal syndrome (BORS). CONCLUSIONS Second branchial cleft fistulae are rare. They are usually right-sided. If bilateral fistulae are present, one should consider an underlying genetic disorder. The histology of the fistulae mostly demonstrates ciliated columnar epithelium with the majority of specimens showing salivary tissue. There is a clear association with the internal jugular vein (IJV). Dissection should continue until superior to the hyoid bone, ensuring near complete surgical dissection and less risk of recurrence.

Difficult pediatric airway management using the intubating laryngeal airway

OBJECTIVE To evaluate the intubating laryngeal airway (ILA) in providing safe endotracheal intubation in pediatric patients with difficult airway; to describe a method for using flexible fiberoptic bronchoscopy with the ILA for evaluating the pediatric airway. METHODS Case series with chart review of the medical records of patients who had the ILA and fiberoptic intubation used to secure the airway at a tertiary pediatric hospital from January 2009 to January 2011. We documented the circumstances necessitating airway management, ILA success, airway evaluation findings, and medical conditions contributing to difficult airway. RESULTS Fifty patients met criteria for review. The median age was 59.8 months (0.3-244.1), and the median weight was 19.0 kg (2.6-86). Four cases (8%) were unanticipated difficult airways and 46 (92%) were anticipated difficult airways. Nine (18%) of the 50 procedures were performed emergently. Comorbid conditions included craniofacial syndromes (n=36), cervical spine instability/immobility (n=9), and airway hemorrhage (n=3). 48 (96%) patients were fiberoptically intubated on first attempt through the ILA. In 2 patients, fiberoptic intubation required a second attempt. The overall success rate using the ILA and fiberoptic intubation to secure the airway was 100%. CONCLUSION ILA and fiberoptic-guided tracheal intubation is a safe and effective method for securing the airway in pediatric patients with difficult airway and can be a useful alternative to direct laryngoscopy when laryngeal exposure is suboptimal.

Head and Neck Vascular Lesions

Vascular lesions of the head and neck are complex and diverse. These include infantile hemangioma, venous malformations, lymphatic malformations, and arteriovenous malformations, among others. Vascular malformations and tumors display different growth patterns and require different approaches to treatment. Therefore, accurate diagnosis is of utmost importance. This article is a guide for the diagnosis and management of vascular lesions of the head and neck.

Synchronous airway lesions in children younger than age 3 years undergoing adenotonsillectomy.

Objective. Determine the prevalence of synchronous airway lesions (SALs) in children younger than age 3 years undergoing adenoidectomy or adenotonsillectomy for sleep-disordered breathing (SDB) at Children’s Memorial Hospital. Design. Case series with chart review. Setting. Tertiary care pediatric hospital. Children. One hundred ten children 3 years of age or younger who underwent adenoidectomy or adenotonsillectomy along with a full-airway evaluation that included flexible fiber-optic laryngoscopy, direct laryngoscopy, and rigid bronchoscopy for SDB from January 2003 to January 2009. Outcome Measures. Prevalence of SALs and rate of SALs that required intervention. Results. Sixty-seven percent of children were found to have at least 1 SAL. Four children required surgical intervention for a SAL. There was no significant difference in preoperative respiratory distress index (RDI) between children with normal airway examinations compared with children with a SAL. There was no significant difference in the rate of SALs between children younger than 18 months old and those 18 to 36 months old. Conclusions. There is a high incidence of SALs in children younger than 3 years old with SDB. There was no significant difference in the rate of SALs in children younger than 18 months old compared with children 18 to 36 months old. The RDI determined by a polysomnography was not predictive of the presence of a SAL. Tracheal cobblestoning was the most common SAL discovered.

Reoperation after Vascular Ring Repair

The majority of patients having surgical intervention for a vascular ring have resolution of their symptoms. However, 5% to 10% of these patients develop recurrent symptoms related either to airway or esophageal compression and may require reoperation. In our series of 300 patients with vascular rings, we performed a reoperation on 26 patients, not all of whom were originally operated on at our institution. The four primary indications for reoperation were Kommerell diverticulum (n = 18), circumflex aorta (n = 2), residual scarring (n = 2), and tracheobronchomalacia requiring aortopexy (n = 4). All patients undergoing reoperation have had preoperative evaluation with bronchoscopy and computed tomographic scanning (CT) with 3-dimensional reconstruction. Patients with dysphagia have had a barium esophagram and esophagoscopy. Patients with a Kommerell diverticulum have undergone resection of the diverticulum and transfer of the left subclavian artery to the left carotid artery. The aortic uncrossing procedure has been used in patients with a circumflex aorta. Aortopexy has been used to treat anterior compression of the trachea by the aorta. Results of these reinterventions have been successful in nearly all cases. Lessons learned from these reoperations can be applied to prevent the need for reoperation by properly selecting the correct initial operation. A dedicated team caring for these children consisting of medical imaging, otolaryngology, cardiovascular-thoracic surgery, and critical care is imperative.

Endoscopic endonasal surgery for sinonasal and skull base lesions in the pediatric population.

Endoscopic endonasal skull base surgical techniques, initially developed in adult patients, are being utilized with increasing frequency in pediatric patients to treat sinonasal and skull base lesions. This article reviews the current state of endoscopic endonasal approaches to the skull base to both treat disease and reconstruct the skull base in pediatric patients. Sinonasal and skull base embryology and anatomy are reviewed as a foundation for understanding the disease processes and surgical techniques. Selected skull base pathologies and conditions that involve the pediatric skull base are also reviewed.