Pathologic lower extremity fractures in children with Alagille syndrome.

OBJECTIVES: : In this retrospective study, we aimed to determine the incidence and distribution of fractures in patients with Alagille syndrome, 1 of the leading inherited causes of pediatric cholestatic liver disease. MATERIALS AND METHODS: : Surveys regarding growth, nutrition, and organ involvement were distributed to patient families in the Alagille Syndrome Alliance of the Children's Hospital of Philadelphia research database. Patients with a history of fracture were identified by their response to 1 question, and details characterizing each patient's medical, growth, and fracture history were obtained through chart review and telephone contact. RESULTS: : Twelve of 42 patients (28%) reported a total of 27 fractures. Patients experienced fractures at a mean age of 5 years, which contrasts with healthy children, in whom fracture incidence peaks in adolescence. Fractures occurred primarily in the lower extremity long bones (70%) and with little or no trauma (84%). Estimated incidence rate calculations yielded 399.6 total fractures per 10,000 person-years (95% confidence interval 206.5, 698.0) and 127.6 femur fractures per 10,000 person-years (95% confidence interval 42.4, 297.7). There were no differences in sex, age distribution, or organ system involvement between the fracture and no-fracture groups. CONCLUSIONS: : Children with Alagille syndrome may be at risk for pathologic fractures, which manifest at an early age and in a unique distribution favoring the lower extremity long bones. Although this preliminary study is limited by small sample size and potential ascertainment bias, the data suggest that larger studies are warranted to further characterize fracture risk and explore factors contributing to bone fragility in these children.

Transseptal suture to secure middle meatal spacers.

Middle meatal spacers are used by many sinus surgeons to aid postoperative care. Aspiration of a spacer is a concern. We demonstrate a novel method of securing spacers with a transseptal suture to prevent aspiration. We fashion each spacer from a powder-free, nonlatex glove finger that is packed with a Merocel sponge. The open end of the finger is closed with 2-0 Prolene sutures. For a bilateral procedure, the needle is left attached to one of the spacers. A spacer is placed in each ethmoid cavity, the attached needle is passed through the anterior cartilaginous septum, and the suture is tied to the suture on the opposite spacer. For unilateral procedures, the suture on the single spacer is passed to the contralateral side and tied on itself. One week later, the transseptal suture is cut and the spacers are removed. We performed this procedure on 78 patients who had undergone total ethmoidectomy. None of the spacers migrated during the 1 week they were in place. One patient complained of pain during removal of a spacer. During follow-up of 2 to 10 months, we found no evidence of injury to the septum at the site of the transseptal suture. We conclude that the transseptal suture is a safe and cosmetically superior method of securing middle meatal spacers.

Lateral sinus thrombosis as a complication of otitis media: 10-year experience at the children's hospital of Philadelphia.

OBJECTIVES: Lateral sinus thrombosis is a rare intracranial complication of otitis media that is traditionally described in countries with poor access to medical care. Our goal was to describe the clinical presentation, management, and outcome of patients diagnosed with lateral sinus thrombosis in a US tertiary care center and to highlight the clinically relevant differences in presentation between these patients and those described in previous reports. PATIENTS AND METHODS: The medical charts of 13 patients diagnosed with otogenic lateral sinus thrombosis were reviewed. These patients were identified from a manual search of 156 subjects with International Classification of Diseases, Ninth Revision codes corresponding with a diagnosis of mastoiditis or thromboembolism over a 10-year period (1997-2007) at the Children's Hospital of Philadelphia. RESULTS: In contrast to previous reports in the literature, the majority of patients in this series exhibited cranial neuropathies and signs of raised intracranial pressure. Nearly all of the patients had a history of acute otitis media treated with antibiotics in the weeks preceding admission. However, many patients denied high fevers or active otomastoid symptoms, which are classically associated with lateral sinus thrombosis. The diagnosis was made in all of the children by using computed tomography and MRI/venography. Treatment strategies included myringotomy tube placement, simple mastoidectomy, intravenous antibiotics, and anticoagulation. Posthospitalization follow-up data revealed no significant long-term complications. CONCLUSION: Despite appropriate antibiotic therapy, lateral sinus thrombosis and other intracranial complications of otitis media are still a threat to children in the modern era. Neurologic, rather than otologic, symptoms may dominate the presentation of otogenic lateral sinus thrombosis. Thus, a high index of suspicion may be critical for ensuring timely diagnosis of this rare condition.