Management of pediatric hemoptysis: review and a case of isolated unilateral pulmonary artery agenesis

OBJECTIVES: Management of hemoptysis in the pediatric patient requires an otolaryngologist knowledgeable and skilled in the causes and management of this potentially life-threatening condition. This paper will discuss the management of pediatric hemoptysis by the otolaryngologist through an index case of massive hemoptysis seen in a child with isolated unilateral pulmonary artery agenesis (IUPAA). STUDY DESIGN: Literature review and index case. METHODS: Through a Medline search and the experience of the author (A.J.M.), we reviewed the causes and treatment options for hemoptysis in the pediatric patient. RESULTS: Management of hemoptysis by the otolaryngologist requires prompt diagnosis and patient stabilization. This allows the patient access to multiple treatment options which may include diagnostic or therapeutic bronchoscopy, angiography with embolization, and surgical intervention such as resection or revascularization. IUPAA is an unusual, and potentially life-threatening cause of hemoptysis in the pediatric patient. CONCLUSION: Hemoptysis in the pediatric patient requires prompt and thorough evaluation and treatment. An approach for the management of pediatric hemoptysis, including massive hemoptysis, is described.     

Preoperative computed tomography may fail to detect patients at risk for perilymph gusher

Perilymph gusher (PG) is a very rare occurrence that can lead to an adverse outcome during inner ear surgery. In the absence of a family history of X-linked mixed deafness syndrome, surgeons may have difficulty determining if a patient is at risk preoperatively. Radiographic imaging is often performed in an attempt to identify such a possibility, but there are few data to support the value of negative studies. We conducted a retrospective study of 3 cases of PG in which findings on preoperative high-resolution computed tomography (CT) of the temporal bone had been interpreted as normal. We reviewed these CTs to discern if they did in fact demonstrate any abnormalities that might have indicated a risk of PG, and we found that the original radiologist had missed a dilated internal auditory canal and a deformity of the cochlear modiolus in the affected ear of 1 of these patients. No abnormality was detected on review of the CTs of the other 2 patients. Therefore, we conclude that negative CT findings do not necessarily rule out a risk of PG.     

Immune stimulation for the treatment of papilloma

OBJECTIVES: There is no curative therapy for recurrent respiratory papillomatosis. Unmethylated dinucleotides of cytosine and guanine (CpG) are potent immune stimulants that have shown efficacy against tumors as monotherapy, as vaccine adjuvants, and in combination with chemotherapies. We examined the therapeutic effect of CpG oligodeoxynucleotides in the treatment of papillomavirus in a cottontail rabbit model (CRPV). METHODS: Twenty rabbits were infected with CRPV; 10 were treated with 11 weekly CpG inoculations while treatment control rabbits received intralesional saline solution. Eight rabbits (4 treatment, 4 control) were rechallenged with CRPV 17 weeks after the initial viral challenge and monitored for new papilloma development. RESULTS: Papillomas developed in all 20 rabbits (100%) within 4 weeks of infection. The diagnosis was confirmed histologically. There was no difference in the average tumor burden between the treatment and control groups after 11 weeks of CpG treatments or after 9 additional weeks of observation. There was no difference between the groups in papilloma size at the site of the injections, nor was there eradication of papillomas at remote sites in either group. No new papillomas developed in any of the 8 animals that were rechallenged. CONCLUSIONS: We have reproduced an effective mammalian papilloma model for preclinical immunotherapeutic testing. Despite the potency of CpG in triggering host immunity, CpG oligodeoxynucleotide did not show a therapeutic effect against the large papilloma burdens tested in this study. The lack of effect suggests that either enhanced papilloma antigen presentation or targeting of immune-evasive mechanisms used by the papillomas is needed to treat bulky disease with an immunotherapeutic strategy.     

Cricothyroid approximation and subluxation in 21 male-to-female transsexuals

OBJECTIVES: To evaluate the medium-term outcome of cricothyroid approximation and subluxation (CTAS) with postoperative speech therapy for pitch elevation in male-to-female transsexuals. STUDY DESIGN: Retrospective study of male-to-female transsexuals who underwent pitch-raising surgery between November 1996 and August 2001. METHODS: Twenty-one male-to-female transsexuals opted for surgical feminization of their voices after inadequate improvements with speech therapy alone. Electrolaryngographic measurements were obtained by a single speech therapist of modal fundamental frequencies and the percentage of irregularities before, at 2 weeks, and 6 months after surgery. All 21 patients underwent CTAS, and 20 underwent simultaneous cosmetic thyroid chondroplasty by a single surgeon. RESULTS: Electrolaryngographic results 2 weeks after surgery showed an average postoperative gain in modal frequency of free speech of 71.05 Hz (95% confidence interval [CI]: 42.9-99.2, P < .001). There was a concomitant average rise in irregularities of 9.9% (95% CI 0.7-18.5, P = .03). At median follow-up of 6 months after six sessions of speech therapy (n = 15), there was a decrease in irregularities to preoperative levels. The overall gain at 6 months in modal frequency of free speech was 56.9 Hz (95% CI 38.3-75.4, P < .001). Smoking and age did not predict a worse outcome. CONCLUSIONS: Cricothyroid approximation effectively raises pitch in male-to-female transsexuals. There is a concomitant rise in voice irregularities that is effectively addressed by speech therapy.     

Assessment of construct validity of the endoscopic sinus surgery simulator

OBJECTIVE: To study the relationship between performance on an endoscopic sinus surgery simulator (ES3) and fundamental perceptual, visuospatial, and psychomotor abilities. DESIGN: Validation study. SETTING: Tertiary care medical center. PARTICIPANTS: Thirty-four medical students and 4 otolaryngology residents voluntarily enrolled. INTERVENTIONS: Subjects performed tasks on the ES3, minimally invasive surgical trainer virtual reality (MIST-VR), pictorial surface orientation (PicSOr), and 3 visuospatial tests (cube comparison, card rotation, and map planning). MAIN OUTCOME MEASURES: The MIST-VR was scored for time, task error, economy of hand movement, economy of diathermy, and total score. Scores were generated for the PicSOr task and visuospatial tests. Scores were correlated with time, accuracy, and total subscore on navigation, injection, and dissection tasks, as well as hazard score and total trial score on the ES3. RESULTS: The PicSOr score was statistically significantly correlated with the hazard score on the ES3 (r = 0.50, P < .001). Cube comparison (r = 0.43, P < .01) and card rotation (r = 0.45, P < .01) scores correlated significantly with the ES3 trial score, as did the MIST-VR total score and the ES3 trial score (r = 0.57, P < .001). In a multiple regression model, the PicSOr, cube comparison, and MIST-VR total scores were statistically significant predictors of ES3 performance (r = 0.63, P < .01). CONCLUSIONS: Scores on the ES3 correlate strongly with scores on previously validated measures of perceptual, visuospatial, and psychomotor performance. The ES3 provides a reliable assessment of factors that are important to the acquisition of minimally invasive surgical skills, demonstrating construct validity.     

Long-term performance of indwelling tracheoesophageal speaking valves in Chinese patients undergoing laryngectomy

OBJECTIVES: To assess the use of indwelling tracheoesophageal speaking valves in Chinese patients undergoing laryngectomy, to identify the clinicopathologic factors for favorable outcome, and to evaluate the factors that determine prosthesis lifetime. DESIGN: Retrospective review. SETTING: Tertiary care institution. PATIENTS: Sixty consecutive patients with total laryngectomy were included. All had indwelling tracheoesophageal speaking valves for voice restoration. INTERVENTION: Anterograde replacement of voice prosthesis in case of failure of functional speech production or leakage of saliva. MAIN OUTCOME MEASURES: Incidence of successful tracheoesophageal speech rehabilitation for daily communication, influence of different clinicopathologic factors on the outcome, and prosthesis lifetime and its relationship to different clinicopathologic factors. RESULTS: The success rate of speech rehabilitation was 78%. Age younger than 60 years was the only clinicopathologic factor associated with successful speech rehabilitation (P = .04, Fisher exact test). The median device lifetime was 8.2 months. Both age of 60 years or older and the use of subsequent prosthetic valves were identified as risk factors for valve failure on univariate analysis (n = 192; log-rank test; P = .02 and P = .03, respectively), with age of 60 years or older as the only risk factor that reduced the device lifetime in the Cox proportional hazards model (P = .03; relative hazard ratio, 1.5; 95% confidence interval, 1.1-2.4). CONCLUSIONS: Our success rate in using indwelling tracheoesophageal speaking valves was comparable to that reported in the Western literature. The median device lifetime of 8.2 months was satisfactory. Patient age was found to be a significant predictor of successful tracheoesophageal speech rehabilitation, with age of 60 years or more adversely affecting device lifetime.     

High prevalence of obstructive sleep apnea among patients with head and neck cancer

OBJECTIVES: To determine the prevalence of obstructive sleep apnea (OSA) in patients with cancer of the oral cavity and oropharynx scheduled for primary surgical resection. To correlate the presence of OSA and the occurrence of postoperative morbidities in this patient population. METHODS: This was a prospective study involving 17 patients with malignancies of the oral cavity and oropharynx scheduled for primary surgical resection. Consecutive patients were approached to undergo overnight polysomnography to determine the apnea-hypopnea index (AHI). OSA was defined by an AHI value > or = 20 events per hour. Postoperative morbidities were evaluated in a blinded fashion for the patients completing surgery. RESULTS: OSA was present in 13 of 17 patients, yielding a striking prevalence of 76% in this patient group. The mean AHI for patients with OSA was 44.7 +/- 3.5 (standard error) events per hour, with a mean nadir oxygen saturation of 88.2 +/- 1.8%, consistent with moderate to severe sleep-disordered breathing. The OSA and non-OSA patients were similar with respect to age and body mass index. The mean size of the primary tumour was 3.3 cm in patients with an AHI < 20 and 3.5 cm in those with an AHI > or = 20 (p = not significant). Overall, postoperative complications, defined as prolonged intensive care unit stay (> 24 hours), need for mechanical ventilation, and cardiopulmonary morbidities, were observed in 67% of OSA and 25% of non-OSA patients. CONCLUSIONS: These findings point to a strong association between OSA and malignancies of the oral cavity and oropharynx. This relationship was independent of the size of the primary malignancy in this patient population with tumours ranging from 1 to 7 cm (p = not significant). When comparing the two groups (AHI < 20 and AHI > or = 20), there was a tendency for the group with OSA to have an increase in postoperative morbidities. Further research is warranted to further evaluate the postoperative morbidities and mortalities associated with OSA in this patient population and to determine the potential roles for preoperative treatment with continuous positive airway pressure and tracheotomy.     

Subannular ventilation tubes: retrospective study

OBJECTIVE: To evaluate the efficiency of a subannular tube insertion technique in a group of pediatric patients with adhesive otitis or severe atelectasis of the tympanic membrane. DESIGN: Retrospective nonrandomized case series. Setting: Tertiary referral centre. MAIN OUTCOME MEASURES: The main outcomes of this study are tube duration according to the type of tube used, the complication rate, and the audiometric gain associated with this procedure. RESULTS: The study group consisted of 190 patients (316 tubes) aged between 3 and 19 years (average 9 years old) and operated on between 1993 and 1999 by four pediatric otolaryngologists. The average follow-up was 53 months. The tubes remained in place for an average of 21.8 months, with fluoroplastic tubes lasting 17.8 months and Goode T tubes lasting 23.8 months. When used in children between 5 and 9 years of age and in cases of adhesive otitis, Goode T tubes showed statistically significantly better results than fluoroplastic tubes. The complications of this technique were otorrhea (17.7%), perforation (7.9%), a plugged tube (7.0%), and cholesteatoma (1.6%). The 5- to 9-year-old group and the reintervention group of patients showed statistically higher complication rates compared with all other groups. Sixty-four patients (128 tubes) were eligible for audiogram analysis, which showed a gain of 13.4 dB (speech reception threshold). CONCLUSIONS: The technique of subannular tube insertion is a safe and effective method for long-term middle ear ventilation in cases of adhesive otitis or severely atelectatic tympanic membrane or for patients with pathology related to dysfunction of the eustachian tube. It offers an alternative to repeated short-term tube insertions for otitis media with effusion or recurrent acute otitis media.     

Facial surface analysis by 3D laser scanning and geometric morphometrics in relation to sexual dimorphism in cerebral--craniofacial morphogenesis and cognitive function

Over early fetal life the anterior brain, neuroepithelium, neural crest and facial ectoderm constitute a unitary, three-dimensional (3D) developmental process. This intimate embryological relationship between the face and brain means that facial dysmorphogenesis can serve as an accessible and informative index of brain dysmorphogenesis in neurological and psychiatric disorders of early developmental origin. There are three principal challenges in seeking to increase understanding of disorders of early brain dysmorphogenesis through craniofacial dysmorphogenesis: (i) the first, technical, challenge has been to digitize the facial surface in its inherent three-dimensionality; (ii) the second, analytical, challenge has been to develop methodologies for extracting biologically meaningful shape covariance from digitized samples, making statistical comparisons between groups and visualizing in 3D the resultant statistical models on a 'whole face' basis; (iii) the third, biological, challenge is to demonstrate a relationship between facial morphogenesis and brain morphogenesis not only in anatomical-embryological terms but also at the level of brain function. Here we consider each of these challenges in turn and then illustrate the issues by way of our own findings. These use human sexual dimorphism as an exemplar for 3D laser surface scanning of facial shape, analysis using geometric morphometrics and exploration of cognitive correlates of variation in shape of the 'whole face', in the context of studies relating to the early developmental origins of schizophrenia.