Eye lens radiation exposure and repeated head CT scans: A problem to keep in mind.

OBJECTIVES: The deterministic character of radiation-induced cataract is being called into question, raising the possibility of a risk in patients, especially children, exposed to ionizing radiation in case of repeated head CT-scans. This study aims to estimate the eye lens doses of a pediatric population exposed to repeated head CTs and to assess the feasibility of an epidemiological study. METHODS: Children treated for a cholesteatoma, who had had at least one CT-scan of the middle ear before their tenth birthday, were included. Radiation exposure has been assessed from medical records and telephone interviews. RESULTS: Out of the 39 subjects contacted, 32 accepted to participate. A total of 76 CT-scans were retrieved from medical records. At the time of the interview (mean age: 16 years), the mean number of CT per child was 3. Cumulative mean effective and eye lens doses were 1.7mSv and 168mGy, respectively. CONCLUSION: A relatively high lens radiation dose was observed in children exposed to repeated CT-scans. Due to that exposure and despite the difficulties met when trying to reach patients' families, a large scale epidemiological study should be performed in order to assess the risk of radiation-induced cataracts associated with repeated head CT.

Mucocele after transnasal endoscopic repair of traumatic anterior skull base fistula in children.

OBJECTIVES: To report the long-term sinonasal complications after endoscopic repair of anterior skull base fractures in children. This study describes mucocele formation in 6 patients treated endoscopically for posttraumatic CSF fistulae. We aim to address possible etiologic factors, specific treatments and follow-up modalities. PATIENTS AND METHODS: 12 children, mean age 5.8 years (3-10), treated endoscopically at our institution between 2004 and 2010 for an anterior cranial base fracture complicated by a CSF fistula. An iatrogenic mucocele was observed in 6 cases. A retrospective review of the files of these 6 patients was carried out demonstrating demographic characteristics, presenting signs/symptoms, site of skull base defect, repair technique, timing and onset of the mucoceles, their presentation and management. A systematic CT-scan and/or MRI was carried out at 3 months, 1 year and then annually. RESULTS: 3 patients presented after cranial trauma with persistent CSF nasal leak, and 4 with meningitis. Posttraumatic defects reached the posterior wall of the frontal sinus and the junction of ethmoid and frontal bone in 3 cases, and the cribriform plate in 3 cases. Endoscopic closure was performed in all cases, with the middle turbinate as an overlay patch. No recurrence of a CSF leak was observed. After a mean period of 16 months, a single iatrogenic mucocele was observed in 4 patients (radiological detection only), and multiple mucoceles in 2 patients. Surgical treatment was advocated in cases of proptosis, quick expansion of the mucocele leading to sinus bony wall remodelling or erosion (2 cases), and meningitis related to an erosion of the cribriform plate by the mucocele. Three mucoceles were successfully treated endoscopically, and 1 required an external approach. CONCLUSION: Mucocele incidence after endoscopic repair of skull base fractures in children is not insignificant (50% in our 12 patients series). Paediatric anatomical features, cranial trauma, and the transethmoidal approach may play a role in mucocele pathogenesis. Long-term clinical and radiological follow-up is therefore recommended. These mucoceles may be managed endoscopically with good outcomes.

Autologous fat transfer in velopharyngeal insufficiency: indications and results of a 25 procedures series.

OBJECTIVE: To assess the efficiency of autologous fat transfer (AFT/Coleman procedure) in the management of velopharyngeal insufficiency (VPI). SETTINGS: Tertiary academic center, retrospective case series over a 4 year period. Pre- and post-speech assessment by a speech pathologist using the Borel-Maisonny scale. PATIENTS AND METHOD: Twenty-five (25) procedures were performed on 22 patients during the considered period. Mean age at surgery was 12.4 ± 4.1 years-old. Main associated conditions were 22q11 deletion (n=6 including 2 with cleft palate), isolated cleft palate (n=3), and Robin sequence (n=2). Indications were VPI grade 2a (n=5), 2b (n=11) and 3 (n=6), despite prolonged speech therapy (pre-op mean duration: 4.2 years) and previous surgery (velopharyngoplasty, n=13). Four patients had a contraindication of velopharyngoplasty (aberrant internal carotid arteries). RESULTS AND CONCLUSION: Fat harvesting sites were umbilicus (n=23) and buttock (n=2). Mean injected fat volume was 7.8 ml, in the posterior wall of the pharynx (n=25) the soft palate (n=15), the peritonsillar arches (n=3), and the pre-existing flap (n=3). Mean follow-up was 17 months. Two patients relapsed once and one patient twice, requiring additional injections. Final post-operative examination 1 year after the last procedure showed an improvement of speech in 90% of cases (grade 1, n=2; 1/2a, n=5; 2a n=10; 2b, n=5). AFT is a safe technique indicated in the primary and secondary management of VPI, with stable results on speech. However, if a complete return to normal is difficult to achieve, its simplicity allows multiple procedures in the same patient.

Cartilage shield tympanoplasty in children: review of 268 consecutive cases.

OBJECTIVE: To assess the efficacy of partial ossicular chain reconstruction using autologous cartilage. DESIGN: Prospective study (April 1, 1997, through January 1, 2008). SETTING: Tertiary academic children's hospital. PATIENTS: Two hundred forty-eight children (268 ears) underwent partial ossicular chain reconstruction using a shaped block of tragal cartilage interposed between the head of the stapes and an underlay tympanic membrane reconstruction along with tragal cartilage and its perichondrium. MAIN OUTCOME MEASURES: Anatomical and audiologic results were evaluated according to the American Academy of Otolaryngology-Head and Neck Surgery guidelines. χ² Tests and multivariate analysis were used for statistical evaluation. RESULTS: Mean age at surgery was 10.9 years. Single-stage surgery was performed in 124 ears (46.3%) (62.9% for cholesteatomas and 32.3% for retraction pockets). Second-look patients (53.7%) included 93.8% of staged surgery. Audiometric results were available for 222 ears at 1 year and for 78 ears at 5 years. Closure of the average air-bone gap (ABG) to within 20 dB was achieved in 62.2% of ears at 1 year. The mean (SD) preoperative and 1-year postoperative ABGs were 25 (11.8) dB and 18.9 (10.3) dB, respectively. Anatomical results were satisfactory in 87.3%. No cases of extrusion, resorption, or displacement of the cartilage were encountered. No statistically significant difference was found between audiometric results at 1 and 5 years. Multivariate analysis showed a significant negative correlation between preoperative and postoperative ABGs and between postoperative otitis media with effusion and postoperative ABG (P < .05). CONCLUSIONS: Cartilage ossiculoplasty is a reliable technique for partial ossicular replacement. Long-term hearing outcomes remain stable and satisfactory. Preoperative ABG and postoperative otitis media are the predictive factors of the hearing outcome.

CCDC39 is required for assembly of inner dynein arms and the dynein regulatory complex and for normal ciliary motility in humans and dogs.

Primary ciliary dyskinesia (PCD) is an inherited disorder characterized by recurrent infections of the upper and lower respiratory tract, reduced fertility in males and situs inversus in about 50% of affected individuals (Kartagener syndrome). It is caused by motility defects in the respiratory cilia that are responsible for airway clearance, the flagella that propel sperm cells and the nodal monocilia that determine left-right asymmetry. Recessive mutations that cause PCD have been identified in genes encoding components of the outer dynein arms, radial spokes and cytoplasmic pre-assembly factors of axonemal dyneins, but these mutations account for only about 50% of cases of PCD. We exploited the unique properties of dog populations to positionally clone a new PCD gene, CCDC39. We found that loss-of-function mutations in the human ortholog underlie a substantial fraction of PCD cases with axonemal disorganization and abnormal ciliary beating. Functional analyses indicated that CCDC39 localizes to ciliary axonemes and is essential for assembly of inner dynein arms and the dynein regulatory complex.

Otologic features in children with primary ciliary dyskinesia.

OBJECTIVES: To analyze otologic features in patients with primary ciliary dyskinesia (PCD) aged 0 to 18 years and to evaluate the correlation between ultrastructural defects and severity of otologic features. DESIGN: Retrospective study. SETTING: Pediatric referral center. PATIENTS: Fifty-eight patients with PCD were evaluated in the following 4 age intervals: group 1, preschool (≤ 5 years [n = 47]); group 2, school (6-11 years [n = 50]); group 3, teenagers (12-17 years [n = 34]); and group 4, young adults (≥ 18 years; 27 years for the oldest [n = 10]). Follow-up was 2 to 6 years in each age group; 26 patients had total follow-up of more than 12 years. Ultrastructural defects occurred in the outer dynein arm (n = 33), the inner dynein arm (n = 13), and the central complex (n = 11). One patient had typical Kartagener syndrome with typical PCD features but normal ciliary ultrastructure. MAIN OUTCOME MEASURES: Frequency of acute otitis media, otitis media with effusion, otorrhea, chronic otitis media, hearing loss, and middle ear surgery and type of antibiotic regimen according to age and type of defect. RESULTS: Recurrent acute otitis media decreased from group 1 (32 of 47 [68%]) to group 4 (0 of 10 [0%]) (P < .001). Otitis media with effusion was more severe in groups 1 through 3 than in group 4 (P = .02). Otorrhea decreased in group 4: 30% vs 80% (3 of 10 vs 36 of 41) in the other groups (P < .001). Half of the patients with tympanostomy tubes eventually had tympanic perforation. Hearing loss was moderate in groups 1 through 3 and mild in group 4. Continuous antibiotic therapy could be slightly reduced only in group 4. Central complex defect was a significant marker of severity for all these criteria. CONCLUSIONS: Despite continuous antibiotic therapy, the middle ear condition in PCD remained severe throughout childhood, with improvement only after age 18 years. Armstrong grommet placement did not improve the middle ear condition. Central complex defect is a marker of severity.

Treatment of anterior skull base defects by a transnasal endoscopic approach in children.

OBJECT: The object of this study was to assess the efficacy and complications of endoscopic management of anterior skull base defects. METHOD: The authors reviewed the medical records of 28 children (20 boys and 8 girls) undergoing endoscopic repair of anterior skull base defects in their tertiary referral center between 2001 and 2008; 18 cases were congenital and 10 cases posttraumatic. During the endoscopic procedure, rigid telescopes--2.7 or 4 mm in diameter, with 0° or 30° lenses--were used. In 23 patients the anterior skull base defect was sealed with fragments of middle turbinate (bone and mucosa). In the remaining 5 patients it was sealed with cartilage harvested from the nasal septum (3 cases) or from the auricle (2 cases), fibrin glue, and oxidized cellulose. A combined external and endoscopic approach was required in 3 cases because of the size and extensions of the encephalocele. Outcome was primarily assessed by means of clinical examination, nasal fibroscopy, and imaging. RESULTS: The mean duration of follow-up was 26.7 months (range 9-57 months). One patient treated by a combined approach died of meningitis 2 years after surgery. In the remaining 27 patients, there was no recurrence of CSF leak, meningitis, or encephalocele. An iatrogenic frontal or ethmoidal mucocele was observed in 4 cases. CONCLUSIONS: The endoscopic approach is a minimally invasive, safe, and efficient technique for removing nasal encephaloceles in children.

Physiologic and clinical benefits of noninvasive ventilation in infants with Pierre Robin sequence.

OBJECTIVE: The objective of the study was to determine the clinical and physiologic benefits of noninvasive respiratory support (NRS) (continuous positive airway pressure or noninvasive positive pressure ventilation) for infants with a Pierre Robin sequence (PRS). METHODS: Breathing patterns, respiratory efforts, and gas exchange were analyzed for 7 infants with a PRS during spontaneous breathing and during NRS. Clinical outcomes with duration of NRS and need for a tracheotomy and/or nutritional support was evaluated. RESULTS: Compared with spontaneous breathing, breathing patterns, respiratory efforts, and transcutaneous carbon dioxide pressures improved during NRS; the mean respiratory rate decreased from 55 ± 9 to 37 ± 7 breaths per minute (P = .063), the mean inspiratory time/total duty cycle decreased from 59 ± 9% to 40 ± 7% (P = .018), the mean esophageal pressure swing decreased from 29 ± 13 to 9 ± 4 cm H(2)O (P = .017), the diaphragmatic pressure-time product decreased from 844 ± 308 to 245 ± 126 cm H(2)O-second per minute (P = .018), and the mean transcutaneous carbon dioxide pressure during sleep decreased from 57 ± 7 to 31 ± 7 mm Hg (P = .043). All of the patients could be discharged successfully from the hospital with NRS. The mean duration of NRS was 16.7 ± 12.2 months. Six patients could be weaned from nutritional support, and none required a tracheotomy. CONCLUSIONS: NRS is able to improve breathing patterns and respiratory outcomes for infants with severe upper airway obstruction attributable to a PRS, which supports its use as a first-line treatment.

Stapedectomy in children: causes and surgical results in 35 cases.

OBJECTIVES: To study children who had undergone stapedectomy at an age younger than 16 years to determine the causes (particularly frequency of congenital anomalies vs otosclerosis) and to analyze the functional results over the short-term, 1-year, and long-term postsurgery time course. DESIGN: Ten-year retrospective study covering 1998 to 2008. SETTING: Pediatric tertiary care centers. PATIENTS: A total of 33 patients (35 ears) underwent stapes surgery from October 1998 to October 2008. MAIN OUTCOME MEASURE: Sex, age, preoperative and postoperative audiometric test results, associated anomalies, type of surgery (stapedotomy or partial stapedectomy), method of stapes surgery, and complications. RESULTS: The median age of patients at surgery was 13.4 years, ranging from 3.3 to 15.9 years. The major cause, which was found in 25 of 35 ears (71%), was nonprogressive conductive hearing loss due to congenital stapes fixation. The second most common cause, which was found in 6 of 35 ears (17%), was otosclerosis with progressive conductive or mixed hearing loss. Three ears presented posttraumatic stapes luxation (1 child aged 3.3 years at surgery). In 1 ear, the cause was osteogenesis imperfecta. Twenty-two ears were treated via the drill or laser-assisted small fenestra technique, and 13 ears were treated by a partial removal of the footplate covered by fascia. Early functional results were good, with a median postoperative air-bone gap of 9.8 dB, and 94% of the results were considered good or very good. There was no significant difference between early, 1-year, and longer-term audiometric results. CONCLUSIONS: Congenital fixation is the major indication for stapedectomy in children younger than 16 years. Functional results are good and remain stable over time.

Neonatal vs delayed-onset fourth branchial pouch anomalies: therapeutic implications.

OBJECTIVES: To determine the presentation of third or fourth branchial pouch anomalies in various age groups of children and evaluate endoscopic cauterization as a treatment technique. DESIGN: Retrospective study of patients treated from 2000 to 2009. SETTING: Tertiary care children's hospital. PATIENTS: Pediatric patients aged 0 to 18 years (mean age, 5.5 years), including 5 neonates. INTERVENTIONS: Endoscopic and/or open surgical management of third and fourth branchial pouch anomalies; clinical and endoscopic follow-up. MAIN OUTCOME MEASURES: Absence of clinical recurrence; closure of the sinus tract. RESULTS: Two forms of presentation were identified: a neonatal form, characterized by a voluminous and compressive cervical mass (5 of 20 [25%]) and a childhood form, presenting as a cervical abscess (15 of 20 [75%]). The vast majority of our patients regardless of presentation were treated endoscopically (n = 19), with a success rate of 68% (13 of 19) after 1 procedure, 79% (15 of 19) after 2 procedures, and 89% (17 of 19) after 3 procedures. Neonatal and adult presentations require slightly different therapeutic approaches. CONCLUSIONS: Third and fourth branchial pouch anomalies can present in 2 distinct forms: a neonatal form and a childhood form. The endoscopic technique should be the favored approach for both forms: whenever possible, in view of its simplicity, rapidity, and the lack of serious postoperative complications. Recurrences can be treated by repeated cauterization using the same technique, with good long-term outcomes. An age-based management algorithm has been developed.

Propranolol in the therapeutic strategy of infantile laryngotracheal hemangioma: A preliminary retrospective study of French experience.

OBJECTIVE: Preliminary assessment of the efficacy of propranolol on subglottic hemangioma in children on a nation-wide scale. METHODS: Multicentric, retrospective study of clinical files of 14 children; pre- and post-treatment endoscopies. RESULTS: Mean age at diagnosis was 2.3 (0.7-4) months. Mean percentage of airway obstruction was 68% (15-90) before propranolol introduction. Propranolol was started at 5.2 (0.7-16) months of age. This treatment was effective in all cases with a mean regression of the stenosis to 22% after 2 weeks and 12% after 4 weeks. Other medical treatments (steroids) could be stopped. In one patient, a side effect of propranolol motivated the switch to another ß-blocker. In four patients, treatment was stopped after 5.2 (1-10) months with a relapse in 2 (50%) cases. One of these two patients developed a resistance to propranolol and required a surgical procedure by external approach. CONCLUSION: This preliminary nation-wide survey confirms propranolol high effectiveness against airways' localization of infantile hemangiomas. Propranolol also allows alleviation or cut-off of previous medical treatments. However, recurrences are possible after early treatment interruption.

Medical and surgical complications in pediatric cochlear implantation.

OBJECTIVES: To report complications of cochlear implantation (CI) in children and to analyze risk factors. DESIGN: Retrospective study from January 1, 1990, through April 30, 2008, with a mean follow-up of 5.5 years (range, 1 month to 17 years). SETTING: Tertiary academic center. PATIENTS: Four hundred thirty-four patients younger than 16 years. Mean age at CI was 4.7 (range, 0.6-16.0) years. Forty-one children (9.4%) underwent CI when younger than 24 months. Forty-three (9.9%) had inner ear malformations. MAIN OUTCOME MEASURES: Complications after CI, classified into early (0-8 days) or delayed (>8 days) and major or minor. Spontaneous failures of internal devices were excluded. Correlation to age at CI, local trauma, and inner ear malformations were analyzed using the chi(2) test. RESULTS: Forty-three patients (9.9%) experienced complications. Delayed complications occurred in 28 patients (65.1%), with a mean delay of 2.2 (range, 0.1-8.4) years. Twenty-four patients (5.5%) had major complications, consisting of severe cutaneous infections (15 patients), magnet displacement (3), meningitis (2), cholesteatoma (2), cerebrospinal fluid leak (1), and electrode misplacement (1). Nineteen (4.4%) had minor complications, consisting of vertigo (9 patients), soft-tissue infection (5), persistent otitis media (4), and facial palsy (1). Complications led to reimplantation in 13 of the 43 patients (30.2%). Trauma to the mastoid area (14 patients) and inner ear malformations (51) were highly correlated with major delayed complications (P < .001) and early minor complications (P < .001), respectively. Young age at CI was not correlated with any type of complication. CONCLUSIONS: Complications of CI in children are common, with trauma as a major factor. Inner ear malformations should prompt specific preventive management. Cochlear implantation in young children did not appear to be a risk factor in this study.

Endoscopic surgical treatment of laryngotracheal clefts: indications and limitations.

OBJECTIVE: To present the indications, techniques, results, and limitations of endoscopic surgical treatment of laryngotracheal cleft. DESIGN: Retrospective case note study (2005-2009). SETTING: Department of Otolaryngology-Head and Neck Surgery, Armand Trousseau Children's Hospital, Paris, France. PATIENTS: Eleven patients who underwent endoscopic cleft closure as a primary (n = 8) or secondary (n = 3) procedure among 22 patients treated for laryngotracheal clefts. We report patients' demographics, symptoms leading to the diagnosis, endoscopic evaluation method, medical examination results, and surgical techniques. INTERVENTION: Endoscopic closure of the cleft under spontaneous ventilation via 2-layer interrupted sutures after excision of the mucosal edge using a carbon dioxide laser in 10 patients and a thulium laser in 1. MAIN OUTCOME MEASURES: Analysis of postoperative complications, revision surgery, need for intensive care unit admission, closure of the cleft, and long-term symptom results. RESULTS: Successful closure of the 11 clefts (with revision surgery in 3 patients) without the need for intubation or intensive care unit admission enabled the elimination of aspiration in 10 patients and significant improvement in 1 patient with bilateral vocal cord paralysis. There were no significant postoperative complications. CONCLUSIONS: Endoscopic closure of laryngotracheal clefts is a reliable technique that significantly reduces perioperative and postoperative morbidity. The results of this technique are entirely satisfactory, and we suggest that it is suitable as a primary procedure for the treatment of type I, II, and III clefts extending to the cervical trachea, including in neonates.

Noninvasive positive-pressure ventilation avoids recannulation and facilitates early weaning from tracheotomy in children.

OBJECTIVE: To show that noninvasive positive-pressure ventilation by means of a nasal mask may avoid recannulation after decannulation and facilitate early decannulation. DESIGN: Retrospective cohort study. SETTING: Ear-nose-and-throat and pulmonary department of a pediatric university hospital. PATIENTS: The data from 15 patients (age = 2-12 yrs) who needed a tracheotomy for upper airway obstruction (n = 13), congenital diaphragmatic hypoplasia (n = 1), or lung disease (n = 1) were analyzed. Four patients received also nocturnal invasive ventilatory support for associated lung disease (n = 3) or congenital diaphragmatic hypoplasia (n = 1). Decannulation was proposed in all patients because endoscopic evaluation showed sufficient upper airway patency and normal nocturnal gas exchange with a small size closed tracheal tube, but obstructive airway symptoms occurred either immediately or with delay after decannulation without noninvasive positive-pressure ventilation. INTERVENTIONS: In nine patients, noninvasive positive-pressure ventilation was started after recurrence of obstructive symptoms after a delay of 1 to 48 mos after a successful immediate decannulation. Noninvasive positive-pressure ventilation was anticipated in six patients who failed repeated decannulation trials because of poor clinical tolerance of tracheal tube removal or tube closure during sleep. MEASUREMENTS AND MAIN RESULTS: After noninvasive positive-pressure ventilation acclimatization, decannulation was performed with success in all patients. Noninvasive positive-pressure ventilation was associated with an improvement in nocturnal gas exchange and marked clinical improvement in their obstructive sleep apnea symptoms. None of the 15 patients needed tracheal recannulation. Noninvasive positive-pressure ventilation could be withdrawn in six patients after 2 yrs to 8.5 yrs. The other nine patients still receive noninvasive positive-pressure ventilation after 1 yr to 6 yrs. CONCLUSIONS: In selected patients with upper airway obstruction or lung disease, noninvasive positive-pressure ventilation may represent a valuable tool to treat the recurrence of obstructive symptoms after decannulation and may facilitate early weaning from tracheotomy in children who failed repeated decannulation trials.

Velopharyngoplasty for noncleft velopharyngeal insufficiency: results in relation to 22q11 microdeletion.

OBJECTIVE: To evaluate the results of velopharyngoplasty for velopharyngeal insufficiency (VPI) in relation to 22q11 deletion or nonsyndromic VPI. DESIGN: Retrospective study. SETTING: Academic medical center. PATIENTS: Eleven of 45 patients with 22q11 microdeletion (group 1) and 9 patients without 22q11 microdeletion (group 2) with noncleft VPI (hypoplastic velum or hypodynamic velopharynx and deep pharynx) underwent velopharyngoplasty (midline pharyngeal flap with superior pedicle). Exclusion criteria included cleft palate, submucous cleft palate, all syndromic cases, and all associated malformations (except those related to 22q11 microdeletion in patients with DiGeorge syndrome). MAIN OUTCOME MEASURES: Speech assessment before surgery using the Borel-Maisonny scale and at 9 months and 24 months after surgery. Velopharyngeal insufficiency was classified as normal, inconsistent, mild, moderate, and severe. RESULTS: Before surgery, in group 1, 3 patients had mild and 8 had severe VPI, and in group 2, 1 had mild and 8 had severe VPI. Postoperative outcomes at 9 months showed that in group 1, 2 patients had excellent results (normal and inconsistent) and 9 had mild VPI, while in group 2, 6 patients had excellent results and 3 had mild VPI (P = .03). Postoperative outcomes at 24 months showed that in group 1, 10 patients had excellent results and 1 had mild VPI, while in group 2, 8 patients had excellent results and 1 had mild VPI. CONCLUSIONS: Surgical treatment of noncleft VPI by pharyngoplasty was efficient in 10 of the 11 patients (91%) in the 22q11 group and in 8 of the 9 patients (89%) in the nonsyndromic group. Postoperative remission took longer for patients with the 22q11 microdeletion than for the control group. However, long-term results following surgical treatment were equally good in the 2 groups.

Slide tracheoplasty in infant with congenital tracheal stenosis and tracheomalacia after esophageal atresia with tracheoesophageal fistula repair.

Slide tracheoplasty can be applied successfully to all types of long segment congenital tracheal stenosis. In case of short segment tracheal stenosis, direct resection and anastomosis is often preferred. We report a case illustrating a new side benefit of the slide tracheoplasty in a patient presenting a relatively short hypoplastic tracheal segment coexisting with tracheomalacia after EA with TEF repair.

Role of Propranolol in the therapeutic strategy of infantile laryngotracheal hemangioma.

There are recent reports of effective treatment of cutaneous hemangiomas with Propranolol. The current study aims to assess efficacy of systemic Propranolol for subglottic hemangiomas and to discuss its place among the other available therapies. We report 2 infants with subglottic hemangiomas, which were resistant to other established medical treatments. One infant presented with PHACES association, the other with widespread cutaneous congenital hemangiomas. Both were subsequently treated with systemic Propranolol. Both patients' subglottic hemangiomas responded dramatically to systemic Propranolol. No side effects of the therapy occurred, and a safety protocol previously designed for Propranolol prescribed for other indications was applied to our patients. Propranolol appears to be an effective treatment for subglottic hemangiomas and should now be used as a first-line treatment in subglottic hemangiomas when intervention is required.

Cholesteatoma secondary to temporal bone involvement by Langerhans cell histiocytosis: a complication amenable to curative surgery.

OBJECTIVES: To describe secondary acquired cholesteatoma in patients previously treated for Langerhans cell histiocytosis (LCH). To focus on misleading symptoms suggesting LCH relapse. PATIENTS: This study involved 3 patients aged 12, 20, and 58 months whose conditions were diagnosed with LCH. All 3 had involvement of the mastoid and of the skin of the external ear canal (EAC) at diagnosis. They were treated with steroids and vinblastine. INTERVENTIONS: Serial computed tomographic (CT) scans and clinical follow-up. MAIN OUTCOME MEASURE: Exploratory surgery of the mastoid. RESULTS: Otorrhea recurred in all 3 patients at 24, 17, and 26 months, respectively, with difficulties to clinically identify a hole in the posterior part of the EAC. The otorrhea was considered a sign of a new occurrence of LCH, leading to systemic chemotherapy in 2 cases. A CT scan showed a defect in the posterior wall of the EAC and suggested cholesteatomatous invasion of the mastoid (2 true cholesteatomas and 1 precholesteatomatous case).Surgery (canal wall up mastoidectomies) successfully removed the cholesteatoma (bilateral in 2 cases) and reconstructed the bony defect using cartilage. Biopsies ruled out LCH recurrence. CONCLUSION: Secondary acquired cholesteatoma (through a bony defect of the EAC) may occur in patients previously treated for LCH. Recurrence of symptoms and bone destruction on CT may wrongly suggest LCH recurrence. Surgery allows removal of the cholesteatoma as well as EAC reconstruction and control biopsy.