Surgical management of choanal atresia: improved outcome using mitomycin.

OBJECTIVE: To evaluate the intraoperative use of mitomycin to improve the surgical outcome and reduce the rate of soft tissue restenosis in children undergoing choanal atresia repair. DESIGN: Retrospective chart review of all patients surgically treated for congenital choanal atresia by the senior author (W.F.M). SETTING: Tertiary children's hospital. PATIENTS: Eight consecutive patients with bony choanal atresia (6 unilateral and 2 bilateral) were compared with 15 historical controls (6 unilateral and 9 bilateral). All study and historical control patients were treated with soft plastic postoperative stenting. INTERVENTION: At the completion of the surgical repair of the choanal atresia, 0.4 mg/mL of topical mitomycin was applied to the posterior choanae for 3 minutes. MAIN OUTCOME MEASURE: The success rate of the repair of the choanal atresia as determined by the postoperative need for dilation or revision surgical procedures was compared with that of the historical controls. RESULTS: All 8 children with intraoperative use of mitomycin were treated with a mean +/- SEM of 0.375 +/- 0.183 dilations per patient. The 15 children in the control group received a mean +/- SEM of 3.667 +/- 0.583 postoperative dilations for soft tissue restenosis. The difference in the number of postoperative dilations between the study and control group was statistically significant (P =.006) using a t test. CONCLUSIONS: Mitomycin is an effective and reliable treatment for improving the surgical outcome for choanal atresia repair. This may obviate the need for postoperative dilations and may potentially eliminate the need for surgical stenting.

Avoiding intubation in the injured subglottis: the role of heliox therapy.

Intubation in the child presenting with severe viral tracheobronchitis or prior subglottic injury can be detrimental to the child and the subglottis. Intubation may lead to further mucosal ischemia, scar, subglottic stenosis, or failed extubation requiring a tracheotomy. Heliox is a combination of helium and oxygen that produces less-dense gas exchange. Its use leads to a decrease in turbulent airflow, which may obviate the need for intubation. Here we report our experience using heliox as initial therapy in 14 consecutive children presenting with severe airway distress and the need for intubation. (Five had viral tracheobronchitis, 5 had inflammatory exacerbation of subglottic stenosis, and 4 had acute iatrogenic subglottic injury.) In 10 of the 14 children, intubation, which can lead to mucosal injury and scarring, was avoided by the use of heliox therapy. Of the 4 children in whom heliox therapy failed, 3 had a prior history of subglottic stenosis. Heliox is a relatively safe and reliable alternative to intubation of children with severe subglottic edema or injury. Heliox should be considered before intubation for selected children with subglottic inflammation and severe airway distress.

Laryngospasm and diaphragmatic arrest in immature dogs after laryngeal acid exposure: a possible model for sudden infant death syndrome.

Laryngopharyngeal reflux has been proposed as a possible cause of sudden infant death syndrome (SIDS). We investigated the efferent laryngeal and diaphragmatic responses to acid exposure on the laryngeal mucosa using a neonatal canine model. Electromyographic (EMG) recordings from the thyroarytenoid muscle and the diaphragm were measured with hooked-wire electrodes. Reproducible laryngospasm responses occurred in all animals after laryngeal exposure to hydrochloric acid at pH 2.0 or less. Laryngospasm occurred in combination with tachypnea and increased diaphragmatic activity in most of the animals. Laryngospasm was associated with prolonged apnea and total cessation of diaphragmatic EMG activity in 1 animal, and in another, initial tachypnea was followed by erratic diaphragmatic activity and brief apnea. Laryngeal acid exposure (below pH 2.0) causes laryngospasm and may result in paradoxical apneic events in neonatal dogs. Acid-induced, laryngospasm-associated apnea may represent a potential cause of SIDS, and the immature dog appears to be an excellent model for further investigations.

Endovascular treatment of hemorrhage after tonsillectomy in children.

SUMMARY: Endovascular therapy for hemorrhage after tonsillectomy or adenoidectomy is an important adjunct to the definitive treatment of this life-threatening occurrence. We report two cases of hemorrhage after tonsillectomy and/or adenoidectomy and describe the endovascular management of this complication in children.

Elective extracorporeal membrane oxygenation: an improved perioperative technique in the treatment of tracheal obstruction.

The surgical management of children with tracheal stenosis and obstruction is complicated by the perioperative needs of pressure ventilation and indwelling endotracheal tubes. These factors predispose to surgical failure and anastomotic breakdown, restenosis. and pneumomediastinum. The use of extracorporeal membrane oxygenation (ECMO) to manage ventilation during tracheal repair allows better visualization at the surgical site and obviates the need for indwelling endotracheal tubes and high-pressure ventilation. Six children were treated with elective ECMO at a tertiary care hospital. All 6 underwent successful surgical repair, and 4 of the 6 were ultimately extubated. There were no significant complications at the surgical site. There was 1 death from postoperative complications, and 2 patients required tracheotomy. One tracheotomy was performed for upper airway obstruction secondary to retrognathia, and this patient was subsequently decannulated. Medical complications were confined to 2 patients and included sepsis, hyperbilirubinemia, seizure disorder, renal failure, intracranial hemorrhage, and hydrocephalus. Elective ECMO provides a reliable perioperative technique for airway management of children with tracheal stenosis or obstruction. This technique offers the advantage of improved visibility at the operative site and eliminates the need for high-pressure ventilation, thereby likely reducing the risk of perioperative morbidity.

Serial positron emission tomography scans following radiation therapy of patients with head and neck cancer.

BACKGROUND: A single institution study was undertaken to evaluate the role of positron emission tomography (PET) scans with fluorodeoxyglucose (FDG) prior to radiation and following radiation. METHODS: Forty-five patients with head and neck cancers were evaluated with FDG-PET scans as well as either CT or MRI prior to treatment with definitive radiation (RT). These same scans were obtained following completion of RT at 1 month (36 patients), 4 months (28 patients), 12 months (19 patients), and 24 months (15 patients). Standard uptake values (SUV) normalized for blood glucose and lean body mass were calculated on the initial and 1-month post-treatment PET scans. RESULTS: Fifteen patients are alive without evidence of disease at 24 to 52 months following RT. Initial SUVs were calculated on the primary tumor site and ranged from 2.5 to 28.5. These values did not have any correlation with local control when examined for the entire group, primary site, or T stage. One-month post-RT SUV ranged from 1.8 to 6.24. Of the 36 1-month post-RT PET scans, six were interpreted as positive for residual disease and were confirmed by biopsy. Four of the five scans, which were interpreted as equivocal, were positive on biopsy. Seven of the 25 scans, which were interpreted as negative for tumor, were positive on biopsy. Four-month scans were more accurate for disease with disease noted in 0 of 18 negative scans, 6 of 7 positive scans, and 2 of 3 equivocal scans. CONCLUSIONS: PET is useful for initial imaging of head and neck cancers. SUV does not appear to be useful for predicting outcome following treatment with RT. One-month post-RT scans were inaccurate for predicting the presence of cancer. Four-month post-RT scans were a better predictor for the presence of cancer.

Velocardiofacial syndrome: incidence of immune cytopenias.

BACKGROUND: Velocardiofacial syndrome (VCFS) is associated with a broad clinical spectrum that frequently overlaps the DiGeorge syndrome. Both have been linked to chromosomal microdeletions of chromosome 22 (22q11.2). DiGeorge syndrome is associated with T-cell dysfunction. What is the incidence of immune cytopenias in children with VCFS? OBJECTIVES: To (1) identify, (2) characterize, (3) quantify, and (4) follow up the immunologic deficits in children initially seen in our institution with VCFS. DESIGN: Prospective clinical evaluation of patients with the features of VCFS. PATIENTS: Twenty consecutive children with the clinical diagnoses of VCFS. SETTING: Tertiary care children's hospital. MAIN OUTCOME MEASURES: All 20 children had genetics evaluation with chromosomal analysis. Immunologic evaluations included serum immunoglobulin concentrations, lymphocyte studies, and mitogen and antigen stimulation studies. RESULTS: Five (25%) of 20 children were noted to have T-cell dysfunction with a clinical presentation marked by recurrent upper respiratory tract infections. Three of these 5 children had resolution of the T-cell dysfunction over a 2-year period. The 2 children with persistent cytopenias combined with immunoglobulin dysfunction required intravenous IgG infusions to control their infections. CONCLUSIONS: Velocardiofacial syndrome is associated with an increased incidence of immune cytopenias and, thus, warrants evaluation in any child with the clinical diagnosis of VCFS. This immune deficit may be transient and depends on the age of the evaluation of the child.

FDG PET evaluation of head and neck cancer: value of imaging the thorax.

BACKGROUND: Patients with primary tumors of the head and neck have been reported to have a high rate of synchronous primary tumors of the upper aerodigestive tract. This study was performed to determine whether inclusion of the thorax in the scan volume would be diagnostically useful for positron emission tomography (PET) with [F-18] fluorodeoxy-D-glucose (FDG) in patients with primary tumors of the head and neck. METHODS: FDG PET scans from the midcranium to the diaphragm were obtained on 56 patients with a variety of head and neck tumors on initial examination before definitive therapy. PET findings in the chest were correlated with results of all other imaging studies, biopsy results, and clinical follow-up. RESULTS: In nine studies (16%), areas of increased FDG uptake in the chest were seen and were judged to be tumors. Six of these probably were false-positive results, although one of these six may have been unconfirmed true positives. Of the three confirmed true-positive studies, two were obvious from other routine studies. In only one case did the PET study reveal a significant lesion not found by means of routine evaluation, resulting in a case-finding yield of 2%. If the unconfirmed possible true-positive results are included, the case-finding yield increases to 4%. CONCLUSIONS: No compelling indication was seen for including the chest in PET studies of patients with head and neck cancer.

Evaluation of airway obstruction using virtual endoscopy.

OBJECTIVES: This study examines the use of virtual endoscopy (VE) in the evaluation of patients with upper airway obstruction. The utility of VE compared with actual endoscopy was investigated with respect to accuracy of diagnosis and reproduction of endoscopic images. STUDY DESIGN: A random cohort of 30 patients with various causes of airway obstruction was examined. METHODS: The computed tomography (CT) data were reconstructed using a proprietary VE software program, FreeFlight, blind to the actual endoscopic findings. The cause of obstruction was identified and compared with actual endoscopic findings. This included 21 patients with airway stenoses, 8 patients with laryngotracheomalacia, 3 tracheal tumors, 2 glottic webs, 5 patients with innominate artery compression, 2 tracheal granulomas, and 7 patients with impaired true vocal cord mobility. RESULTS: Virtual endoscopic evaluation was accurate in assessing stenosis width and length of fixed airway lesions. Correlation of stenosis shape and contour between actual endoscopy and VE was excellent. The stenosis-to-lumen ratios were compared between VE and actual endoscopy and were found to be within 10% (SD = 8). However, virtual endoscopic evaluation could not illustrate one of the glottic webs, half of the cases of tracheomalacia, or any of the cases of impaired true vocal cord mobility. CONCLUSIONS: Virtual endoscopy was not as sensitive as actual endoscopy in detecting the cause of airway obstruction that was based on dynamic movement. However, VE was excellent for the measurement and definition of fixed airway lesions.

Second laryngeal cancers in previously treated larynges.

OBJECTIVES: To review the management and outcome of patients with new second primary cases of laryngeal cancer occurring more than 3 years after the initial cancer was treated. STUDY DESIGN: Retrospective review of 855 patients with newly diagnosed and treated laryngeal cancers. METHODS: Charts were reviewed and tabulated for occurrence of second primary laryngeal tumors, method of treatment for the index and second primary laryngeal tumors, and effect of smoking status or cessation of smoking. RESULTS: Of the 855 patients, 532 patients retained their larynx; of these 377 lived more than 3 years and thus were at risk for a second new primary cancer. Of these 377 patients at risk, 19 or 5.1% developed second primary tumors. The rate of second primary disease was lower in patients whose index laryngeal cancer was treated by irradiation (4.3%) compared with those treated surgically (9.2%). However, surgical treatment of second primary laryngeal tumors resulted in far higher rates of laryngeal voicing (82%) compared with irradiation (33%). CONCLUSION: When possible, management of the index primary tumor by endoscopic resection has resulted in the highest future retention of laryngeal speech, leaving all treatment options available should a second laryngeal cancer occur. Lifelong follow-up of patients with laryngeal cancer for secondary primary tumors is important. Second primary tumors were equally distributed between patients who continued to smoke and ceased to smoke after their index primary lesion was diagnosed.

Occult primary tumors of the head and neck: lack of benefit from positron emission tomography imaging with 2-[F-18]fluoro-2-deoxy-D-glucose.

BACKGROUND: Patients who present with squamous cell carcinoma metastatic to cervical lymph nodes and no clinically apparent primary site present a therapeutic dilemma. Positron emission tomography imaging with 2-[F-18]fluoro-2-deoxy-D-glucose (FDG-PET) has been shown to be useful for the examination of known primary tumors. This study was undertaken to determine whether FDG-PET imaging improves detection of occult primary tumors in patients with metastatic squamous cell carcinoma in the lymph nodes of the head and neck. METHODS: Thirteen patients with pathology proven cervical lymph node metastases from clinically occult primary squamous cell carcinomas were evaluated prospectively with FDG-PET, in addition to standard clinical and radiographic techniques, as part of their pretreatment diagnostic evaluation. Direct panendoscopy and biopsy were performed on all patients in an attempt to detect primary tumor sites and to characterize them histologically. RESULTS: A primary squamous cell carcinoma was confirmed after panendoscopy and biopsy in 3 of the 13 patients. The site of the primary tumor was correctly identified with FDG-PET in only one of these three patients. The FDG-PET study suggested a primary tumor location where no tumor was found in 6 of 13 patients; for 5 other of the 13 patients, the FDG-PET results were negative and no primary was found. No primary tumor locations were identified by computed tomography, magnetic resonance imaging, or direct panendoscopy. FDG-PET imaging correctly detected the location of the primary tumor in 1 patient (8%) and provided apparent false-positive results for 6 (46%) of the 13 patients. CONCLUSIONS: FDG-PET imaging did not significantly improve detection of unknown primary squamous cell carcinomas in patients with metastases to lymph nodes of the neck. A high percentage of results were apparent false-positive.

Reflux in infants with laryngomalacia: results of 24-hour double-probe pH monitoring.

Laryngomalacia is the most common cause of stridor in children. Previous studies using barium esophagrams or single-probe esophageal pH testing have indicated that 68% to 80% of infants with laryngomalacia have reflux. A recent study in a large series of pediatric patients has shown that these 2 testing modalities are relatively insensitive in detecting reflux when compared with 24-hour double-probe pH testing. This study was undertaken to determine the incidence and frequency of reflux in children with laryngomalacia by use of 24-hour double-probe pH monitoring. Twenty-four children with endoscopically diagnosed laryngomalacia underwent 24-hour double-probe pH testing. The distal probe was placed in the lower esophagus, and the proximal probe was placed just above the cricopharyngeus immediately posterior to the larynx. All 24 (100%) children had pharyngeal acid exposure as judged by the proximal pH probe. These children had a mean of 15.21 episodes of reflux to the level of the pharynx during the 24-hour study period. In contrast, only 16 (66%) children had abnormal acid exposure as measured by the distal esophageal probe. These results indicate that essentially all children with laryngomalacia have reflux of gastric acid to the pharyngeal level. Multiple authors have documented the detrimental effects of acid and the accompanying pepsin in the larynx and tracheobronchial tree. Persistent laryngeal edema is an almost universal finding in patients with reflux to the pharyngeal level and is a common finding in children with laryngomalacia. In some patients with laryngomalacia, reflux may be the primary cause of their airway compromise, whereas in others it may be a significant cofactor exacerbating a preexisting neurologic or anatomic abnormality.

Free jejunal grafts of the pharynx: surgical methods, complications, and radiographic evaluation.

Free jejunal grafts have been used in the surgical treatment of patients with carcinoma of the pharynx and upper esophagus. Post-operative complications, including swallowing difficulty, are frequent and radiographic assessment may be required. In this pictorial paper, we describe the surgical technique of free jejunal grafting of the pharyngoesophagus, and the radiographic appearances and clinical importance of early and delayed complications following the procedure. Dysphagia after placement of a jejunal graft is a common occurrence which is often multifactorial, and may be related to functional, anatomic, or a combination of factors.

Surgical management and follow-up of a complex tracheobronchial injury.

Tracheobronchial trauma is an uncommon condition with potentially devastating consequences. Appropriate pre-, intra-, and postoperative management is mandatory for a satisfactory functional outcome. We report a case of extensive tracheobronchial injury secondary to blunt trauma, which was managed successfully with emergent surgical repair and careful endoscopic follow-up. We review the important management decisions made in this case.

The neck mass.

Many head and neck disease processes are manifest as neck masses. These conditions are treated by surgical excision except for some inflammatory masses, and often those too must be excised before a diagnosis can be made. The real question is when to excise the lesion to expedite treatment in the most cost-effective manner. In general, when signs of inflammation are associated with the mass, antibiotic treatment with short-term observation is acceptable. Persistence of the mass or an increase in mass size during observation mandates for their evaluation. Biopsy of a mass is considered for progressive growth, isolated nature or asymmetry of the mass, location (supraclavicular), development of symptoms associated with lymphoma (fever and hypertrophy of the spleen, liver, or Waldeyer's ring), or static size (if > 3 cm). In the adult patient, a complete head and neck physical examination is mandatory before biopsy. Needle biopsy of the neck mass is the current standard of care if no cause of the mass is found on examination. Identified benign cystic lesions or lymphomas indicate a need for excision, either as definitive treatment or for diagnostic reasons. If results of the needle biopsy are positive, equivocal, or even negative in the presence of a high index of suspicion for metastatic squamous cell carcinoma, an endoscopic examination is mandatory before open excision. If no discrete lesion is seen, guided biopsy of the upper aerodigestive tract is performed. Open biopsy of the mass should be accompanied by a frozen-section examination of the mass. A concomitant definitive neck dissection should be performed if the mass proves to be metastatic carcinoma.

Otologic management in children with the CHARGE association.

OBJECTIVES: To characterize otologic management of two patient groups, those with the CHARGE association and those not strictly labeled as CHARGE but with several features of the disorder (CHARGE-like), in order to determine: (1) the clinical validity and utility of managing CHARGE-like children in a similar manner to patients with the strictly defined CHARGE association, (2) the progression and prognosis of hearing loss and (3) the identification of factors that may predict the degree of hearing loss. DESIGN: Case series. SETTING: Tertiary care urban children's hospital. PATIENTS: 37 children, 22 in the CHARGE group and 15 in the CHARGE-like group. INTERVENTIONS: Otorhinolaryngologic and audiologic management. MAIN OUTCOME MEASURES: Otorhinolaryngologic and audiologic evaluation. RESULTS: All patients required otologic and/or audiologic care. Bilateral hearing loss was found in 32 patients (86%) and unilateral hearing loss in five patients (14%) when hearing was assessed in the absence of otitis media. Among the 32 patients with bilateral hearing loss, 31 (97%) were able to be fit with useful hearing aids. External ear anomalies were present in 25/37 (68%) patients, and middle ear and ossicular anomalies were identified in four cases (4/37, 11%), 36/37 (97%) patients required surgical management of otitis media. Three patients (3/37, 8%) exhibited radiographic evidence of inner ear deformity. Facial nerve dysfunction was noted in the records of 14/37 (38%) patients. No statistically significant difference was found when CHARGE and CHARGE-like patients were compared for degree of hearing loss (P = 0.5964), type of hearing loss (P = 0.2657), worsening of hearing level (P = 0.7908), or anomalies of the external ear (P = 0.6921), ossicles (P = 0.7908), inner ear (P = 0.7908) or facial nerve (P = 0.6409). Patients with external ear anomalies did not exhibit statistically different degrees (P = 0.3125) or types (P = 0.1515) of hearing loss from patients without auricular anomalies. The presence of facial nerve anomaly correlated significantly (P = 0.0021) with profound hearing loss. CONCLUSIONS: Children who are CHARGE-like may be may be considered equivalent in terms of otologic and audiologic management to children strictly defined as CHARGE patients. These children all require otologic care due to the high prevalence of middle ear disease and the underlying permanent hearing loss that is both stable and aidable. The degree of hearing loss cannot be predicted by external ear morphology, but may be predicted by facial nerve palsy.

PET scanning in head and neck oncology: a review.

BACKGROUND: The objective of this study was to review and describe the usage of fluorine-labeled deoxyglucose (FDG) and positron emission tomography (PET) in the diagnosis and management of head and neck cancer. METHODS: Several prospective series,-including 159 newly diagnosed and previously untreated and 23 previously irradiated head and neck squamous cell carcinoma patients initially seen at the Wake Forest University Medical Center and evaluated by clinical examination, conventional computed tomography/ magnetic resonance imaging (CT/MRI) scans, PET scans, and histopathologic studies,-were reviewed and the findings summarized for comparison of the correct differentiation of primary and metastatic cancers and for postirradiation tumor clearance in a subsegment of those cases. RESULTS: Positron emission tomography scanning using a fluorine-labeled deoxyglucose (FDG) radiotracer proved as reliable as conventional scanning for primary and metastatic tumor identification. Compared with clinical examination, PET was better for identification of nodal metastatic tumors but poorer for small primary tumors. For previously irradiated patients treated at least 4 months before the test, PET scanning was clearly superior to clinical examination and conventional imaging in differentiating tumor recurrence from soft-tissue irradiation effects. CONCLUSIONS: Fluorine-labeled deoxyglucose-PET scanning is comparable to conventional imaging of head and neck cancers in detecting primary and metastatic carcinoma. Lack of anatomic detail remains its major drawback. Currently, its greatest role is in the evaluation of the postradiotherapy patient.

Laryngeal radionecrosis versus recurrent cancer: a clinical approach.

A group of 38 patients was studied by positron emission tomography utilizing fluorine18-labeled deoxyglucose (FDG-PET) scanning to examine the ability to differentiate postirradiation laryngeal recurrent cancer from radionecrosis. The resulting 79% correct diagnosis showed the technique to be far superior to conventional computed tomography scanning (61%) and clinical examination (43%). An algorithmic approach to evaluating and treating patients with radionecrosis and/or recurrent cancer by utilizing FDG-PET scanning is presented.