Cholesteatoma in Children with Sotos Syndrome.

BACKGROUND: Sotos syndrome is a rare genetic disorder characterized by neurodevelopmental delay and excessive childhood growth including macrocephaly. In this study, we present our experience of children with Sotos syndrome and cholesteatoma. METHODS: Retrospective case note review and cross-referencing with hospital picture archive and communication systems or cases identified from a prospective database of consecutive cholesteatoma surgeries. RESULTS: A total of 400 children underwent surgery for acquired cholesteatoma and 5 (1%) had Sotos syndrome (1 bilaterally). In comparison, 42(11%) had cleft palate which is around 10 times more common than Sotos syndrome, 5 (1%) had Down syndrome, and 3 (1%) had Turner syndrome. The median age at primary surgery was 8 years old (3.5-10.9 years), 124 children with Sotos syndrome were identified in picture archive and communication systems (4% with cholesteatoma) of which temporal bone imaging was available in 86 (70%) at the median age of 9 years (0-17.2), and 33/86 (38%) had normal ears bilaterally on all imaging. Changes consistent with fluid or inflammation were present in 9/30 (30%) computed tomography and 24/72 (33%) magnetic resonance imaging scans. Development of mastoid pneumatization was impaired in 20/30 (67%) computed tomography and 8/72 (11%) magnetic resonance imaging scans. At 5 years, children with Sotos syndrome (33%) had greater recidivism than those with cleft palate (15%) (Kaplan-Meier log-rank analysis, P=.001) CONCLUSION: Children with Sotos syndrome appear to be at increased risk of developing acquired cholesteatoma. Impaired temporal bone pneumatization is a common incidental finding in Sotos syndrome in keeping with this risk. Further study of this previously unreported association may improve the understanding of pathogenetic mechanisms in cholesteatoma.

Prospective Comparison of Pediatric Endoscopic Lateral Graft and Interlay Tympanoplasty.

OBJECTIVE: To compare outcomes and complications of endoscopic lateral graft (LGT) and interlay (IT) tympanoplasty. STUDY DESIGN: Prospective observational cohort study of totally endoscopic ear surgery (TEES) tympanoplasty. SETTING: Pediatric tertiary referral center. PATIENTS: One hundred fourteen surgeries for children with tympanic membrane perforation without cholesteatoma. INTERVENTION: Porcine-derived collagen graft tympanoplasty using either LGT or IT. MAIN OUTCOME MEASURES: Closure rates of perforation, hearing outcomes (four-tone average air conduction [AC] and air bone gap), and complications that required further surgery were assessed 12 months postoperatively. RESULTS: Perforation closure rates did not differ between LGT (52/59 (88%)) and IT (45/51 (88%)), (Fisher's exact p = 1.00) (four ears lost to follow-up). AC thresholds (p = 0.32) and air bone gap (p = 0.88) improved similarly after surgery with LGT (median 8.8 dB) and IT (median 7.5 dB). The proportion of ears with serviceably normal hearing (AC ≤ 30 dB HL) postoperatively was similar (LGT 40/49 (82%), IT 36/46 (78%), Fisher's exact p = 0.80). Following IT, three (6%) ears developed inclusion cholesteatoma requiring revision surgery. One (2%) LGT ear developed blunting after myringitis. CONCLUSIONS: IT and LGT provide similar perforation closure rates and hearing outcomes. When choosing between these two techniques to repair pediatric tympanic membrane perforations, LGT would seem to be preferable than IT due to the risk of inclusion cholesteatoma after IT. There is a small risk of anterior blunting after LGT.

Fetal therapy using rapamycin for a rapidly enlarging, obstructive, cervical lymphatic malformation: a case report.

WHAT'S ALREADY KNOWN ABOUT THIS TOPIC?: Fetal lymphatic malformations (LMs) can be detected on prenatal ultrasound and until recently, therapeutic options were limited. Recently the mammalian target of rapamycin inhibitor rapamycin has emerged as a safe, effective therapy for children with LMs and multiple studies have demonstrated improved efficacy if started early. WHAT DOES THIS STUDY ADD?: We report the first in-utero therapy with rapamycin for a rapidly enlarging, obstructive, fetal cervical LM. Fetal therapy with rapamycin was safe and effective in managing this severe malformation, despite rapamycin being started only in the last 6.5 weeks of pregnancy. We speculate that had rapamycin been commenced earlier, the reduction in mass size might have been even greater.

Hybrid Solutions for the Surgical Treatment of Multilevel Degenerative Cervical Disk Disease.

Background In different stages of cervical degenerative disk disease, the combination of dynamic and nondynamic implants may be considered. The aim of this study was to investigate the applicability of criteria to assist decision making in these cases. Methods Thirty patients with spondylotic cervical radiculopathy and a coincidence of soft disk and hard disk herniation were surgically treated with a hybrid solution (combination of total disk replacement and cage fusion). The control group included 32 patients who underwent two-level cage fusion. Pre- and postoperative Japanese Orthopaedic Association (JOA) scores and range of motion (ROM) were compared. Results Twenty-three patients underwent two-level hybrid solution and 7 underwent three-level treatment. The most frequent solution (n = 13) was a combination of a dynamic implant at C5-C6 and a nondynamic implant at C6-C7. The mean JOA score improved from 13.9 to 15.6 points after surgery (mean deviation [MD] 1.6, 95% confidence interval [CI] 2.1 to 1.2, p < 0.001). ROM showed a slight trend to increase (MD 0.8, 95% CI -0.9 to 2.6, p = 0.193). In the control group, the mean JOA score improved from 13.3 to 15.1 points after surgery (MD 1.4, 95% CI 2.1 to 1.2, p < 0.001). The comparison of the postoperative JOA scores and recovery rates between the hybrid treatment group and the control group did not show significant differences. Conclusions In cases of coincident soft and hard degenerative cervical disk disease at adjacent levels, the combination of a disk prosthesis and a nondynamic implant is a safe and effective treatment option and an alternative to multilevel fusion.

Reconstruction of the anterior skull base after major trauma or extensive tumour resection.

BACKGROUND: The authors describe their experience with the reconstruction of complex anterior skull base defects after trauma or tumour resection using a "sandwich" technique with pericranial flap, titanium mesh and TachoSil. METHODS: Description of surgical anatomy, surgical technique, indications, limitations, complications, specific perioperative considerations and specific information to give to the patient about surgery and potential risks. A summary of ten key points is given. CONCLUSIONS: After a bifrontal craniotomy and a subfrontal approach, it is possible to achieve a reliable reconstruction of the anterior skull base in a watertight manner by fixing a pericranial flap or a fascia lata graft to the orbital roofs and planum sphenoidale with an individually tailored titanium mesh and closing the frontobasal dura leasion with TachoSil.

Management of complex cervical instability.

PURPOSE: Illustrative cases are presented to demonstrate the surgical management of complex instability of the cervical spine. METHODS: Six patients with different underlying pathologies are presented along with their clinical and radiologic findings, surgical procedures, complications, and outcomes. RESULTS: Five patients underwent anteroposterior (AP) decompression and stabilization, of which two required secondary posterior stabilization because of dislocation or subsidence of the anterior osteosynthesis. In another case, a patient with a two-level corpectomy, a stable situation was achieved with an anterior approach only. The outcomes, measured according to Odom's criteria, were excellent in one patient, good in three patients, and fair in two patients. CONCLUSIONS: In cases of complex cervical instability, combined AP decompression and stabilization minimizes the risk of anterior plate failure or dislocation of the vertebral body prosthesis. However, there may be increased risk of adjacent-level degeneration. Therefore, a combined procedure should be considered in selected patients. Not all patients with cervical instability require circumferential surgery. In two-level corpectomy cases, the decision between the less invasive anterior-only approach and the more stable combined approach can be difficult. However, in patients with proof of poor bone quality or with metabolic disorders, a more stable combined approach should be considered.

A bilateral infratentorial neurenteric cyst.

We report a patient with a large infratentorial neurenteric (NE) cyst. Intracranial NE cysts, also known as enterogenous cysts, constitute a rare, generally benign entity of unknown aetiology. The presentation, imaging characteristics and management of the case is discussed, including illustrative peri-operative images.

Perioperative epidural steroids for lumbar spine surgery in degenerative spinal disease. A review.

OBJECT: This systematic review assesses the efficacy of epidural steroids on adults undergoing lumbar spine surgery for degenerative spinal disease. METHODS: The Cochrane Central Register of Controlled Trials (CENTRAL), MEDLINE, and Embase databases were searched for relevant articles. Search terms included "laminectomy," "discectomy," and "steroid." Randomized and quasi-randomized controlled trials of adults undergoing lumbar spinal surgery for degenerative spinal disease were included. The main outcomes were pain, quality of life, total analgesic agent consumption, postoperative length of hospital stay, the ability to return to full-time work, and adverse events. RESULTS: Twelve trials (involving 1053 patients) were included. Epidural steroids reduced back pain at 12-24 hours postoperatively (standardized mean difference [SMD] -1.26, 95% CI -2.35 to -0.0.18, p = 0.02), and radicular pain at 1 week postoperatively (SMD -0.71, 95% CI -1.19 to -0.24, p = 0.003) and 1-2 months postoperatively (SMD -2.14, 95% CI -3.47 to -0.81, p = 0.002). Epidural steroids decreased postoperative consumption of analgesic agents (SMD -0.38, 95% CI -0.62 to -0.14, p = 0.002), length of stay (SMD -0.95, 95% CI -1.62 to -0.27, p = 0.006) and the risk of not returning to full-time work at 1 year (relative risk of 0.27, 95% CI 0.13-0.57, p = 0.0006). There was no significant difference in quality of life or in adverse events. CONCLUSIONS: There is evidence that epidural steroids decrease pain in the short term and shorten length of stay in adults undergoing lumbar spinal surgery for degenerative spinal disease. Most of the evidence comes from studies without validated outcomes and that selectively report positive results. More research is required before establishing perioperative epidural steroids as an effective adjunct to surgery for reducing pain in the long term.