ObjectiveTo summarize the research progress in clinic, development, and genetics of the Klippel-Feil syndrome and its primary ear deformity. MethodsThe related 1iterature at home and abroad concerning the Klippel-Feil syndrome with ear malformation was reviewed, analyzed, and summarized. ResultsThe clinical manifestation and classification of Klippel-Feil syndrome are complicated. As one of the most important accompany malformations, ear deformity mainly leads to hearing impairment and abnormal appearance. However, it is still unclear exactly how the ear deformity forms in Klippel-Feil syndrome, and there is little deep study on the internal connection between the ear deformity and other malformations. The premise for the treatment of Klippel-Feil syndrome includes accurate diagnoses and comprehensive disease assessment, and multidisciplinary collaboration will be the important direction of clinical practice in the future. ConclusionEar malformation is one of the most important congenital dysplasias in the Klippel-Feil syndrome. Its etiology should be based on research in the development and genetic mechanism. And its diagnosis and treatment should be followed by multidisciplinary collaboration. It is important to pay attention to identifying with ear malformation in other syndromes as well.
ObjectiveTo summarize the current progress of clinical therapy for hemifacial microsomia (HFM). MethodsThe domestic and overseas articles concerning the treatment of HFM were reviewed and analyzed. ResultsThe unified therapeutic schedule of HFM has not yet been determined due to its variable clinical manifestation. Therapies mainly include: correction of bone deformity, which attain high effectiveness by adopting distraction osteogenesis or the improvement approach based on it; repair of the hypoplasia of facial soft tissue using graft of free tissue or autologous fat, augmentation of prosthesis materials. Autologous fat is becoming a hot research area and is widely used in recent years. For the aspect of treatment of microtia, different methods are adopted according to the severity of the malformation. ConclusionThe uniform clinical diagnosis and therapy of HFM are not determined for its complicated classification and unknown etiology. The research of etiology and tissue engineering may provide the therapy of HFM.
Objective To investigate the methods and effectiveness of ear reconstruction for the microtia patients with craniofacial deformities. Methods Between July 2000 and July 2010, ear reconstruction was performed with tissue expander and autogenous costal cartilages in 1 300 microtia patients with degree II+ hemifacial microsoma, and the clinical data were reviewed and analyzed. There were 722 males and 578 females, aged 5 years and 8 months to 33 years and 5 months (median, 12 years and 2 months). The expander was implanted into the retroauricular region in stage I; ear reconstruction was performed after 3-4 weeks of expansion in stage II; and reconstructed ear reshaping was carried out at 6 months to 1 year after stage II in 1 198 patients. Results Of 1 300 patients, delayed healing occurred in 28 cases after stage II, healing by first intention was obtained in the other 1 272 cases, whose new ears had good position and appearance at 1 month after stage II. After operation, 200 cases were followed up 1-9 years (mean, 3 years). One case had helix loss because of trauma, and 1 case had the new ear loss because of fistula infection. At last follow-up, the effectiveness were excellent in 110 cases, good in 65 cases, and fair in 23 cases with an excellent and good rate of 88.4%. Conclusion It is difficulty in ear reconstruction that the reconstructed ear is symmetrical to the contralateral one in the microtia patients with degree II+ hemifacial microsoma. The key includes the location of new ear, the fabrication of framework, and the utilization of remnant ear.
ObjectiveTo explore the effect of the expanded capsule on the growth of autogenous costal cartilage. MethodsSixteen New Zealand white rabbits at the age of 3 months (weighing, 2.2-2.5 kg; male or female) were selected and four 15 mL tissue expanders were implanted on the back symmetrically. After 1 month, the expanded capsule formed, the tissue expanders were removed; the capsule of the left side was removed (experimental group), and the capsule of the right side was reserved (control group); meanwhile, the right 7th and 8th costal cartilage without the perichondrium was divided into segments and placed into the capsule of 2 groups symmetrically. At 4 and 8 weeks after transplantation, the cartilage was harvested for the general, weighing, and histological observations. ResultsOne rabbit died during the experiment, and the other 15 rabbits survived. The differences of cartilage weight between before and after transplantation showed more obvious increase in the experimental group[(0.003 4±0.002 7) g and (0.005 8±0.001 4) g] than those in the control group[(-0.000 3±0.001 9) g and (-0.003 9±0.005 3) g] at 4 and 8 weeks, showing significant differences between 2 gouprs (t=4.331, P=0.029; t=6.688, P=0.008). The change of cartilage weight at 8 weeks was significantly higher than that at 4 weeks in the experimental group (t=-3.098, P=0.001); but the change of cartilage weight at 8 weeks was significantly lower than that at 4 weeks in the control group (t=2.491, P=0.009). The histological observation showed that the activity of the cartilage was enhanced in 2 groups at 4 and 8 weeks when compared with normal cartilage, and more obvious change was observed in the experimental group than in the control group. And the acellular area was seen in the cartilage at 8 weeks in the control group. The Masson staining results showed that the color was deeper in the experimental group than in the control group. ConclusionThe removal of the expanded capsule during operation is beneficial to the growth of autogenous costal cartilage. The results can provide corresponding experimental guidance for the clinical problems.
ObjectiveTo investigate the accuracy of multi-slice spiral CT (MSCT) scan and image reconstruction technology for measuring morphological parameters of costal cartilages and to evaluate the volume of costal cartilages. MethodsBetween March and August 2013, 75 patients with congenital microtia and scheduled for auricle reconstruction were included in the study. Of 75 patients, there were 49 males and 26 females with a mean age of 8 years and 5 months (range, 5 years and 7 months to 32 years and 7 months) and a mean weight of 29.5 kg (range, 21-82 kg). A Philips Brilliance 64 MSCT machine was used to scan 1st-12th costal cartilages with the parameters based on the age and weight of the patients. All the data were transported to the workstation for reconstructing the image of the costal cartilages with the technique of maximum intensity projection (MIP) and volume rendering technique (VRT). Then the morphologies of costal cartilages were observed through the images on VRT; the width of the costal cartilaginous ends close to ribs (W) and the length of the total cartilage (L) were measured and compared with their counterparts (W' and L') after the costal cartilages were harvested during the processes of auricle reconstructions to analyze consistency between these two sets of data. ResultsThe morphologies of ribs and costal cartilages shown on VRT image got fine sharpness, verisimilitude, and stereoscopic impressions. A total of 192 costal cartilages were examined. The results showed that the widths of the costal cartilaginous ends close to ribs (W) was (9.69±1.67) mm, and W' was (9.73±1.64) mm, showing no significant difference between W and W' (t=-1.800, P=0.073), and interclass correlation coefficient (ICC) test showed Cronbach's α=0.993. The length of the total cartilage (L) was (83.03±23.86) mm, and L' was (81.83±16.43) mm, showing no significant difference between L and L' (t=1.367, P=0.173), and ICC test showed Cronbach's α=0.904. Linear-regression analysis showed L=1.28×L'-21.93 (R2=0.780, F=673.427, P=0.000). The results suggested there was a good consistency between these two sets of data. ConclusionScanning costal cartilages with appropriate parameters and reconstructing the cartilaginous image with MIP is an effective method to measure the width and length of costal cartilage and to estimate costal cartilage volume, which can provide accurate reference for plastic surgery together with reading the morphology from the image on VRT.
【Abstract】 Objective To summarize different treatments of the residual ear in auricular reconstruction, toinvestigate the reasonable appl ications of the residual ear. Methods From September 2005 to July 2006, 128 patients（79 males, 49 females; aging 5-21 years with an average of 11 years）with unilateral microtia underwent the staged repair. In the patients, there were 44 cases of left-unilaterally microtia and 84 cases of right-unilaterally microtia. The residual ears looked l ike peanut in 56 patients, l ike sausage in 35 patients, l ike boat in 27 patients, and l ike shells in 10 patients. Among all the patients, the external acoustic meatus was normal in 5 patients, stenosis in 11 patients, and atresia in 112 patients. According to auricular developmental condition, the patients were divided into three types: 17 cases of type I, 98 cases of type II, and 13 cases of type III. In the first stage operation, a 50 mL kidney-l iked expander was implanted into post aurem subcutaneous tissue. The residualear whose superior extremity was close to the hair l ine was treated. The middle and superior part of the residual ear was cut. The redundant residual auricular cartilage was removed. In the second stage operation, the inferior part of the cartilage frame was covered by the middle and superior part of the residual ear. According to the location of the residual ear, “V-Y” plasty, “Z”-plasty and reversal of the residual ear were used to correct the location of the residual ear. In the third stage operation, the remained residual ear was used to reconstruct crus of hel ix or cover the wound surface which was resulted from repairing the reconstructed ear. Results The residual ears which were reshaped and transferred had good blood circulation. All residual ears were survival. The wounds healed by first intention. The follow-up for 8-15 months showed that the auricular lobule of the reconstructed ear was turgor vital is and natural. The locations of the reconstructed ear and normal side ear were symmetry. The auricular lobules of the reconstructed ear survived well. The reconstructed crus of hel ix, hel ix, antihel ix and triangular fossawere clear. The results were satisfactory. Conclusion Using residual ear reasonably is an important procedure of successful auricular reconstruction and the symmetry of the reconstructed ear and uninjured side ear.
ObjectiveTo explore the anthropometric changes of the auricle after auricular cartilage unfolding in moderate concha-type microtia patients, so as to provide the basis to help evaluate surgical timing and prognostic.MethodsA total of 33 children with moderate concha-type microtia, who were treated with auricular cartilage unfolding between October 2016 and September 2018 and met the inclusive criteria, were included in the study. There were 24 boys and 9 girls with an average age of 1.4 years (range, 1-3 years). Sixteen cases were left ears and 17 cases were right ears. The follow-up time was 12-23 months (mean, 17.5 months). The affected auricular detailed structures were observed and quantitatively analyzed before operation and at immediate after operation. The width, length, and perimeter of auricle before operation and at immediate after operation and at last follow-up were noted with three dimensional-scanning technology. The normal auricle was noted as control.ResultsThere were (7.5±1.0) and (11.3±0.8) structures of the affected auricle at pre- and post-operation, respectively, showing significant difference between pre- and post-operation (t=23.279, P=0.000). The length, width, and perimeter of the affected auricle constantly increased after operation, and there were significant differences between pre-operation and immediately after operation and between immediately after operation and last follow-up (P<0.05). The differences of length, width, and perimeter of the affected auricle between immediately after operation and last follow-up were (3.13±1.44), (2.44±0.92), and (8.50±3.76) mm, respectively. And the differences of length, width, and perimeter of the normal auricle between pre-operation and last follow-up were (3.16±1.54), (2.35±0.86), and (9.79±4.60) mm, respectively. There was no significant difference in the differences of length, width, and perimeter between the affected auricle and the normal auricle (P>0.05).ConclusionThe auricular cartilage unfolding in treatment of the moderate concha-type microtia can receive more ear structures and increase auricle sizes, which make it possible for free composite tissue transplantation. In addition, the affected and the contralateral normal auricles have a very similar growth rate and it offers the theoretical foundation for the early treatment for moderate concha-type microtia.