Abnormally wide eustachian tubes involving the sphenoid bone: A collection.

OBJECTIVES: To present nine patients with an abnormal widened bony Eustachian tube running anteriorly in the skull base through the sphenoid bone. METHODS: Patients with a particular anomaly in the bony Eustachian tube incidentally found on CT examinations were registered consecutively over a period of four years. RESULTS: Nine patients had the anomaly, eight unilaterally and one bilaterally. All our patients had additional anomalies involving the outer, middle, and/or inner ear. CONCLUSION: The consequences of this anomaly remain unknown, but the presence of the widened, bony ET should increase the awareness for complex temporal bone deformities and vice versa. LEVEL OF EVIDENCE: 4.

Adults with 22q11.2 deletion syndrome have a different velopharyngeal anatomy with predisposition to velopharyngeal insufficiency.

AIM: To find out if subjects with 22q11.2 deletion syndrome (DS) have a different velopharyngeal anatomy which could cause velopharyngeal insufficiency (VPI). METHODS: A prospective study of 16 subjects >16 years of age with 22q11.2 DS, without overt cleft palate and without previous VPI surgery, and 48 healthy controls >18 years of age were included in the study. Speech was recorded and scored blindly by two independent senior speech therapists. All 64 individuals had MRI scans, which were analyzed blindly by a consultant radiologist. RESULTS: Subjects with 22q11.2 DS had a mild degree of weak pressure consonants (mean score); borderline to mild degree of hypernasality and audible nasal emission (mean score). All controls had normal speech. When comparing subjects (22q11.2 DS) to controls, we found the subjects to have the following: A shorter distance between left and right points of origin of the levator veli palatini muscle (LVP) (p < 0.0001); a more obtuse angle of origin of the LVP (bilaterally) (p < 0.009); a thinner LVP bilaterally and in the midline (p < 0.0001); a shorter LVP bilaterally (p < 0.0001); a shorter velum (p = 0.007); a larger osseous pharyngeal depth:velar length ratio (p = 0.01); a more obtuse anterior cranial base angle (nasion to sella to basion) (p < 0.0001) and posterior cranial base angle (sella to basion to foramen magnum) (p < 0.0001); a wider velopharyngeal width (p = 0.002) and a larger pharyngeal airway volume (p = 0.0007). CONCLUSION: Compared with healthy controls, adults with 22q11.2 DS showed a different velopharyngeal anatomy, which will make these individuals more prone to VPI.

An 8-year population description from a national treatment centre on lymphatic malformations.

INTRODUCTION: Lymphatic malformations are rare developmental non-malignant abnormalities that present as masses consisting of abnormal growth of fluid filled channels or spaces in the skin or deep tissue that result in abnormalities of lymphatic flow. They may cause symptoms like pain, recurrent lymphorrea, abnormal bone growth, and disturbance of bodily functions. They may also be a cosmetic problem. The study aims to describe the population with lymphatic malformations (LM) treated at a national referral centre. MATERIALS AND METHODS: This is a retrospective review of patients with the diagnosis lymphatic malformation seen at the centre between 2006-2013. The authors performed a systematic review of the hospital records and a reevaluation of the radiological findings. Eighty-five patients with 102 lymphatic malformations were included. RESULTS: The majority of the patients (67%) were diagnosed before the age of 2 years. Lesions were located mainly to the head and neck (64%) and half (52%) were macrocystic. Treatment was: sclerotherapy (69%), surgery (49%), laser therapy (19%), or a combination of these treatment modalities (33%). The best outcome was found in the patients with macrocystic lesions (p = .029), and 76% of the patients reported a good or very good outcome. CONCLUSION: Most patients presented before the age of 2 years with complex lesions which required unique, often multimodal treatment plans and in general they experienced good outcome.

Extraordinary Large Giant Congenital Melanocytic Nevus Treated with Integra Dermal Regeneration Template.

Garment-like giant congenital melanocytic nevi are very rare, and those being treated are most often offered excision and split-thickness skin transplantation. Due to the risk of restricted mobility secondary to shrinkage and hypertrophic scarring of the transplant, we treated to date the largest reported giant congenital melanocytic nevus (16% total body surface area) with Integra dermal regeneration template (Integra Life Sciences, Plainsboro, N.J.), giving a more functional skin reconstruction. In addition, the dermal regeneration template had to be covered with split-thickness skin transplant including multiple smaller nevi due to lack of larger area with normal skin.

Multidisciplinary Aspects of 104 Patients With Pierre Robin Sequence.

OBJECTIVE: To describe Pierre Robin sequence patients with a cleft palate from a multidisciplinary perspective. PATIENTS: A total of 104 individuals with Pierre Robin sequence and cleft palate, born between 1980 and 2010. METHOD: Data were collected retrospectively and compared with large control groups. RESULTS: Of 104 patients, 19 (18.3%) were treated with a nasopharyngeal or oropharyngeal tube, continuous positive airway pressure, and/or a tracheotomy. The mean weight percentile for newborns with Pierre Robin sequence was 30.9. It decreased to 29.9 at the time of cleft palate repair (mean age, 13.7 months) (P = .78). Of 87 patients, 30 (34.5%) developed normal speech after cleft palate repair. Of 93 nonsyndromic Pierre Robin sequence patients, 31 (33.3%) had or are having surgery for velopharyngeal insufficiency, a rate that is significantly higher when compared with a control group of cleft palate-only patients (19.4%; P = .004). Of 31 patients, 25 (80.6%) developed normal resonance after surgery for velopharyngeal insufficiency. There was no significant difference in the rate of syndromes between the Pierre Robin sequence patients and a control group of cleft palate patients without Pierre Robin sequence (P = .25). Seven of 39 boys (17.9%) with Pierre Robin sequence had a diagnosis of autism spectrum disorder. CONCLUSION: Even though the mean weight percentile for newborns with Pierre Robin sequence was low, the patients did not show a growth spurt during the first year of life. The high rate of velopharyngeal insufficiency after cleft palate repair in patients with Pierre Robin sequence needs further investigation. Also, the high rate of autism spectrum disorder among boys with Pierre Robin sequence prompts further investigation.

Superiorly based pharyngeal flap for treatment of velopharyngeal insufficiency in patients with 22q11.2 deletion syndrome.

BACKGROUND: There are no previous blinded studies for comparison of preoperative versus postoperative perceptual speech assessments when using a pharyngeal flap for treating velopharyngeal insufficiency (VPI) in patients diagnosed with 22q11.2 deletion syndrome. The aim of the study was to evaluate the effect of superiorly based pharyngeal flap surgery on speech in these patients using blinded judgments of experienced speech therapists. METHODS: A retrospective study of 12 consecutive patients who had undergone pharyngeal flap surgery for treatment of VPI between 2002 and 2009 was conducted. Seven girls and 5 boys between 4 and 15 (median, 6) years old at the time of surgery were included in the study. Six patients were born with a submucous cleft palate (including 2 occult), and 1 patient, with an overt cleft palate. The remaining 5 patients had no signs of a palatal pathology. All palatal clefts had been repaired before pharyngeal flap surgery except in 2 patients with occult submucous cleft palate. Preoperative and postoperative audio recordings were blinded for scoring independently by 3 senior speech therapists. RESULTS: There was a significant improvement in hypernasality (P = 0.002), audible nasal emission (P = 0.033), weak pressure consonants (P = 0.008), and speech intelligibility (P = 0.021) after pharyngeal flap surgery. Hyponasality did not develop significantly with surgery. One patient was diagnosed with obstructive sleep apnea. CONCLUSIONS: Superiorly based pharyngeal flap resulted in a significant speech improvement in 12 consecutive patients with 22q11.2 deletion syndrome having VPI.

Autologous fat transplantation to the velopharynx for treating persistent velopharyngeal insufficiency of mild degree secondary to overt or submucous cleft palate.

BACKGROUND: Autologous fat transplantation to the velopharynx has been described in a few smaller studies including heterogeneous groups of patients for the treatment of velopharyngeal insufficiency (VPI). The aim of this study was to evaluate speech and to measure velopharyngeal closure with magnetic resonance imaging (MRI) in patients who underwent autologous fat transplantation for the treatment of persistent VPI of mild degree secondary to overt or submucous cleft palate. METHODS: A prospective study of 16 patients with persistent VPI of mild degree secondary to overt or submucous cleft palate who underwent autologous fat transplantation to the velopharynx. The patients were injected with a median of 5.6 (3.8-7.6) ml autologous fat to the velopharynx. Pre- and 1-year postoperative audio recordings were blinded for scoring independently by three senior speech therapists. Hypernasality, hyponasality, nasal turbulence and audible nasal emission were scored on a five-point scale. Pre- and 1-year postoperative MRIs were obtained during vocal rest and during phonation in 12 patients. Data measured were the velopharyngeal distance in the sagittal plane and the velopharyngeal gap area in the axial plane. RESULTS: Hypernasality improved significantly (p=0.030), but not audible nasal emission (p=0.072) or nasal turbulence (p=0.12). The velopharyngeal distance during phonation decreased significantly (p=0.013), but not the velopharyngeal gap area (p=0.16). There was no significant correlation between speech and MRI results. CONCLUSION: Autologous fat transplantation to the velopharynx improved hypernasality significantly, but not audible nasal emission or nasal turbulence in patients with persistent VPI of mild degree secondary to overt or submucous cleft palate. Given the low number of patients and the lack of a control group, the value of fat transplantation for the treatment of mild VPI is not proven for sure.

Speech and magnetic resonance imaging results following autologous fat transplantation to the velopharynx in patients with velopharyngeal insufficiency.

OBJECTIVE: To measure velopharyngeal closure with magnetic resonance imaging (MRI) and to evaluate speech when treating velopharyngeal insufficiency (VPI) with autologous fat transplantation to the velopharynx. PATIENTS: Nine patients were recruited. Six patients had undergone cleft palate repair and subsequently developed VPI. Three were noncleft patients of which one had developed VPI after nasopharyngeal cancer treatment; another patient had developed VPI after combined adenotonsillectomy, and a third patient had VPI of unknown etiology. MAIN OUTCOME MEASURE: Preoperative and 1-year postoperative MRIs were obtained during vocal rest and during phonation. Data measured were the velopharyngeal distance in the sagittal plane and the velopharyngeal gap area in the axial plane. Preoperative and 1-year postoperative audio recordings were blinded for scoring independently by three senior speech therapists. RESULTS: When comparing preoperative and 1-year postoperative MRI during phonation we found a significant reduction of the median velopharyngeal distance from 4 to 0 mm (p = .011), and a significant reduction of the median velopharyngeal gap area from 42 to 34 mm(2) (p = .038). Nasal turbulence improved significantly (p = .011). Hypernasality/hyponasality and audible nasal emission did not change significantly. CONCLUSIONS: Autologous fat transplantation to the velopharynx resulted in a significant reduction of the velopharyngeal distance and the velopharyngeal gap area during phonation, as measured by MRI. This was in accordance with a significant improvement in nasal turbulence. However, hypernasality and audible nasal emission did not change significantly and could not be correlated to the MRI findings.

Comparison of perioperative morbidity after LeFort III and monobloc distraction osteogenesis.

We have compared distraction by monobloc and LeFort III osteotomy in the treatment of midfacial retrusion. We treated 14 patients with midface distraction (Crouzon syndrome (n = 9), Apert disease (n = 3), and other (n = 2)), 7 of whom had monobloc distraction and 7 who had LeFort III osteotomy. We compared duration of operation, peroperative blood loss, and complications. The two groups were comparable with respect to diagnosis, type of distraction (internal or external device), and duration of distraction. The operating time was longer in the monobloc than in the LeFort III group, but not significantly so (p = 0.09). The weight-adjusted blood losses were significantly different (66 ml/kg and 34 ml/kg, respectively (p = 0.05). The two groups had similar numbers of complications (p = 0.3), and similar duration of hospital stay. Both techniques seem safe. The choice of operation, therefore, should be tailored to the individual patient and the monobloc procedure should be used if indicated.

A 3-year patient-centred follow-up of 516 consecutively treated orthognathic surgery patients.

The purpose of this study was to evaluate the outcome of a scheme for provision of orthognathic surgery by examining whether patients' expectations were fulfilled as well as their opinions about the result of treatment and side-effects. Data were obtained during a pre-planned 3-year follow-up clinical examination which included the administration of patient questionnaires. Of 583 subjects (281 females, 235 males; mean age 27.2 years) who underwent surgery during the period 1994-2002, 516 attended (89 per cent). Differences between subgroups and associations between variables were analysed by chi-square or Fisher's exact test. The most frequent motives for treatment were improvement of dental appearance and chewing ability, indicated by 83 and 81 per cent of the patients, respectively. The expectations were fulfilled for most patients, and satisfaction with the treatment result was reported by 92 per cent. Dissatisfaction was significantly related to gender, skeletal malocclusion, and surgical procedure (P = 0.001). Of all patients, 36.8 per cent reported impaired sensory function, the most frequent side effect. Sensory impairment and concern due to disturbed sensation were significantly related to age at surgery (P = 0.001 and P = 0.041, respectively). The treatment outcome as perceived by the patients was generally favourable. Even if sensory disturbance was frequently observed, most patients, especially in the younger age groups, seemed to adapt. Certain patient characteristics and surgical procedures were associated with an increased risk of dissatisfaction. These observations are relevant in the guidance of prospective orthognathic patients.

Long-term skeletal and soft-tissue responses after advancement genioplasty.

INTRODUCTION: The objectives of this cephalometric study were to assess the skeletal stability of advancement genioplasty 3 years after surgery and to evaluate the predictability of soft-tissue changes. METHODS: The subjects comprised 21 consecutive patients who had no additional orthognathic surgical procedures. Lateral cephalograms were taken at 5 times: immediately preoperative, immediately postoperative, 6 months postoperative, and 1 and 3 years postoperative. RESULTS: Mean surgical advancement at pogonion was 8.4 mm. Three years after surgery, mean relapse at pogonion was 8% of the surgical advancement. Part of this change was most likely due to bone remodeling. No patient demonstrated a clinically significant postoperative change at pogonion. The soft tissue of the chin was found to follow bony movement in a ratio of 0.9:1. Great individual variability was observed. The mentolabial fold depth increased as a result of the treatment. Effects of advancement genioplasty on the lips were small. CONCLUSIONS: A prediction ratio based on long-term skeletal changes is likely to generate an estimate that is more appropriate to present to the patient.

LeFort I maxillary advancement: 3-year stability and risk factors for relapse.

INTRODUCTION: The objectives of this retrospective cephalometric study were to assess the amount, direction, and timing of postoperative changes after LeFort I maxillary advancement, and to identify risk factors for skeletal relapse. METHODS: The material was selected from the files at the Department of Orthodontics, University of Oslo, and comprised 43 patients who underwent 1-piece LeFort I advancement as the only surgical procedure from 1990 to 1998. All patients were followed for 3 years by using a strict data collection protocol. Lateral cephalograms were obtained before surgery and at 5 times after surgery. RESULTS: A mean relapse of 18% of the surgical advancement occurred. In 14% of the patients, clinically significant skeletal relapse (> or = 2 mm) was observed. Most (89%) postoperative change occurred during the first 6 months after surgery. Skeletal relapse increased significantly with degree of surgical advancement (P = .001) and degree of inferior repositioning of the anterior maxilla (P = .004) (linear regression analysis). At the end of follow-up, overjet and overbite were within clinically acceptable ranges for all patients. CONCLUSIONS: Maxillary advancement with a 1-piece LeFort I osteotomy is a relatively stable procedure. Identified risk factors for horizontal relapse were degree of surgical advancement and degree of inferior repositioning of anterior maxilla.

Monobloc distraction osteogenesis in pediatric patients with severe syndromal craniosynostosis.

The management of the hypoplastic midface in syndromic craniosynostosis remains a great challenge. Frequently, patients have to be operated on numerous times to achieve a satisfactory end result, partially because of the limited skeletal advancement possible when using traditional surgical techniques. During the last decade, however, methods for gradual midfacial distraction have been presented, whereby greater advancements can be obtained. We present four children aged 17 months to 15 years with severe syndromal craniosynostosis in need of midface advancements because of severe respiratory obstruction or severe exophthalmos. These patients were complex cases with several previous craniofacial surgeries (mean of three times, range of two to six times) that yielded insufficient skeletal advancements. They were operated on with gradual monobloc advancements using the Modular Internal Distraction System. The mean length of operations was 370 minutes (range: 240-455 minutes), and the mean amount of perioperative blood transfusion needed was 1,300 ml (range: 280-2,700 ml) or 66.9 ml/kg (range: 31.1-94.9 ml/kg). The patient with the greatest number of previous operations also had the longest operation time as well as the most blood loss. The average midface advancement obtained was 25 mm (range: 20-30 mm), resulting in cessation or a significant decrease of preoperative respiratory problems, reduced exophthalmos, and improved facial profile. Apart from a local infection in one patient with a connective tissue disorder and several previous wound infections, no major postoperative complications were recorded. Distraction osteogenesis has become a versatile and safe technique that allows for large advancements of the midface.