Long-Term Outcome of Primary Rhinoplasty with Overcorrection in Patients with Unilateral Cleft Lip: Avoiding Intermediate Rhinoplasty.

BACKGROUND: No consensus exists regarding the timing or technique of rhinoplasty for correction of the unilateral cleft lip nose deformity, with few studies examining the long-term effects of a single technique. This study appraised the long-term outcomes of primary rhinoplasty using the Tajima technique for overcorrection in a cohort of patients with unilateral cleft lip nose deformity after attaining skeletal maturity. METHODS: Consecutive nonsyndromic patients with unilateral cleft lip nose deformity ( n = 103) who underwent primary rhinoplasty with overcorrection by a single surgeon between 2000 and 2005 were reviewed. Patients with unilateral cleft lip and nasal deformity who underwent primary rhinoplasty (but with no overcorrection) ( n = 30) and noncleft individuals ( n = 27) were recruited for comparison. Outcomes were assessed through FACE-Q scales evaluating satisfaction with appearance of nose and nostrils (two scales) and computer-based objective photogrammetric analysis of nasal symmetry (nostril height, nostril width, nostril area, alar height, and alar width parameters). RESULTS: Significant differences (all P < 0.001) were observed between the Tajima and non-Tajima groups for all but one photogrammetric nasal parameter (nostril area), with the Tajima group demonstrating closer mean values to the noncleft group. The Tajima and noncleft groups demonstrated no significant difference (all P > 0.05) for scores of FACE-Q nose and nostrils scales. CONCLUSION: This study indicated that the patients who underwent primary rhinoplasty with overcorrection had improved results with no necessity for intermediate rhinoplasty, emphasizing that the procedure is an effective approach to correct the unilateral cleft nose deformity. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, III.

The cytokine-inducible Scr homology domain-containing protein negatively regulates signaling by promoting apoptosis in erythroid progenitor cells.

The small cytokine-inducible SH2 domain-containing protein (CIS) has been implicated in the negative regulation of signaling through cytokine receptors. CIS reduces growth of erythropoietin receptor (EpoR)-dependent cell lines, but its role in proliferation, differentiation, and survival of erythroid progenitor cells has not been resolved. To dissect the function of CIS in cell lines and erythroid progenitor cells, we generated green fluorescent protein (GFP)-tagged versions of wild type CIS, a mutant harboring an inactivated SH2 domain (CIS R107K), and a mutant with a deletion of the SOCS Box (CISDeltaBox). Retroviral expression of the GFP fusion proteins in BaF3-EpoR cells revealed that both Tyr-401 in the EpoR and an intact SH2 domain within CIS are prerequisites for receptor recruitment. As a consequence, both are essential for the growth inhibitory effect of CIS, whereas the CIS SOCS box is dispensable. Accordingly, the retroviral expression of GFP-CIS but not GFP-CIS R107K impaired proliferation of erythroid progenitor cells in colony assays. Erythroid differentiation was unaffected by either protein. Interestingly, apoptosis of erythroid progenitor cells was increased upon GFP-CIS expression and this required the presence both of an intact SH2 domain and the SOCS box. Thus, CIS negatively regulates signaling at two levels, apoptosis and proliferation, and thereby sets a threshold for signal transduction.