Objective To determine the accuracy and utility of a scoring system designed to allow an objective appraisal of the outcome of hypospadias repair, based on evaluating meatal location, meatal shape, urinary stream, straightness of erection, and the presence and complexity of any complicating urethral ®stula. Patients and methods Twenty patients (median age 23 months) were randomly selected and reviewed at a median of 8.9 months after their hypospadias repair. Two paediatric surgeons, a nurse and one of the child's parents independently assessed each patient using the`hypospadias objective scoring evaluation' (HOSE) system. The results were collated and the level of interobserver variation assessed using the weighted kappa test. Results The mean weighted kappa was 0.66, indicating good agreement among observers. The level of agreement was highest between surgeon and nurse at 0.70, but remained good between surgeon and parent, at 0.65. Conclusions Interobserver variation using the HOSE system was minimal, supporting its use as an objective outcome measure after hypospadias surgery, and facilitating an impartial evaluation of operations used in correcting hypospadias.

Mutations or deletions involving ZFHX1B (previously SIP1) have recently been found to cause one form of syndromic Hirschsprung disease (HSCR), associated with microcephaly, mental retardation, and distinctive facial features. Patients with the characteristic facial phenotype and severe mental retardation, but without HSCR, have now also been shown to have mutations in this gene. Mutations of ZFHX1B are frequently associated with other congenital anomalies, including congenital heart disease, hypospadias, renal tract anomalies, and agenesis of the corpus callosum (ACC). We present the clinical data and mutation analysis results from a series of 23 patients with this clinical syndrome, of whom 21 have proven ZFHX1B mutations or deletions (15 previously unpublished). Two patients with the typical features (one with and one without HSCR) did not have detectable abnormalities of ZFHX1B. We emphasize that this syndrome can be recognized by the facial phenotype in the absence of either HSCR or other congenital anomalies, and needs to be considered in the differential diagnosis of dysmorphism with severe mental retardation +/- epilepsy.

Facial fractures were uncommon overall but occurred more frequently in children with major trauma. Plain facial radiographs provided limited additional diagnostic information to careful clinical examination and often fail to detect or clearly define a facial fracture in children. In the correct clinical setting, a facial CT scan allows accurate diagnosis of the injury and can reveal previously unsuspected additional fractures.

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