Objective To report use of the Vibrant Soundbridge (VSB) in patients with congenital deformation of the middle and outer ears and investigate its utility in this patient population.Method Four patients with congenital...Objective To report use of the Vibrant Soundbridge (VSB) in patients with congenital deformation of the middle and outer ears and investigate its utility in this patient population.Method Four patients with congenital deformation of middle and outer ears underwent VSB implantation. All were male (aged 3-18 years,average 13.5 years) and operated on the left side.Malformation was bilateral in 3 patients and unilateral in 1 patient. Surgical techniques were modified to accommodate each patient’s unique conditions and needs.The implant site was approached via the facial recess in 3 patients and through a retro-facial nerve route in 1 patient. The VSB implant was connected to either the stapes (2 cases) or the round window (2 cases).Pure tone and speech audiometry results and daily communication capabilities before and after VSB activation were compared.Results The operations were successful in all patients, with no complications. The patient communication level improved significantly after VSB activation. Average air conduction pure tone threshold or conditioned reflex audiometry threshold improved by 35 dB in the 0.25-4 kHz range,from 69 dB HL before VSB activation to 34 dB HL after.The sentence recognition rate in quiet at 65 dB SPL went up to 86% from 0% without VSB for patients with bilateral deformation and remained at 100% for the patient with unilateral deformity. However, for the latter patient, the rate improved to 20% from 0% without VSB in noise (-8 dB SNR).Conclusion VSB is an excellent solution for improving hearing in patients with congenital deformation of middle and outer ears.Operation can be completed and good results can be achieved even in patients with unique conditions and needs.展开更多
BACKGROUND Congenital extrahepatic portosystemic shunt, also known as Abernethy deformation, is a rare malformation caused by dysplasia in the portal vein system. There are few reports of liver transplantation as a tr...BACKGROUND Congenital extrahepatic portosystemic shunt, also known as Abernethy deformation, is a rare malformation caused by dysplasia in the portal vein system. There are few reports of liver transplantation as a treatment for Abernethy deformation, and our report is the first case in China. This is the second reported case with congenital extrahepatic portosystemic shunt combined with focal nodular hyperplasia and hepatopulmonary treated with liver transplantation.CASE SUMMARY The patient was a 14-year-old girl, diagnosed preoperatively as type Ib Abernethy deformation, intrahepatic multiple space-occupying lesion, and hepatopulmonary syndrome. The patient recovered well after undergoing classic orthotopic liver transplantation. Liver function, pulmonary function, and portal vein computed tomography angiography imaging were reexamined 20 mo postoperatively, and no abnormality was observed.CONCLUSION Liver transplantation is an effective treatment for type I Abernethy deformation combined with focal nodular hyperplasia and hepatopulmonary syndrome.展开更多
Background:Congenital thumb deformities account for one-third or more of all cases of congenital hand deformity.However,the current classification schemes of congenital thumb hypoplasia are no longer adequate due to t...Background:Congenital thumb deformities account for one-third or more of all cases of congenital hand deformity.However,the current classification schemes of congenital thumb hypoplasia are no longer adequate due to their lack of adaptability to increasing knowledge in the field.Hence,a modified system with the potential to adapt to ongoing advances in knowledge and understanding is desperately needed.Methods:Based on the photographs collected from thousands of cases of congenital deformities of the hand and upper limb over multiple decades in our department,we subdivided thumb hypoplasia according to the variables of morphological characteristics,anatomical structures,functional status,the relationship between thumb deformity and hand deformity,the relationship between congenital hand deformity syndrome and thumb hypoplasia,and the selection of treatment methods.Results:A total of 10 types were presented,which were elucidated with nomenclatures as well as pathological feature and symptoms.Conclusion:This modified system may shed additional light on the classification of congenital thumb anomalies,which will assist in a more effective selection of treatment modalities and offers significant benefits to both patients and practice.展开更多
BACKGROUND Hypoplasia of bilateral cruciate ligaments is a rare congenital malformation.The diagnosis of such diseases and indications for the various treatment options require further analysis and discussion.CASE SUM...BACKGROUND Hypoplasia of bilateral cruciate ligaments is a rare congenital malformation.The diagnosis of such diseases and indications for the various treatment options require further analysis and discussion.CASE SUMMARY The patient is a 26-year-old Chinese woman who has been suffering from knee pain since the age of 8 years,2-3 episodes a year.Three years ago,due to the practice of advanced yoga poses,the frequency of left knee pain increased,requiring prompt medical treatment.Magnetic resonance imaging demonstrated an absence of both anterior and posterior cruciate ligaments of both knees with abnormal posterior tilting of the tibial plateau.Bilateral subluxation of the knee joint was also found,therefore tibial osteotomy was performed.The patient reported at the 24 mo follow-up that the frequency of pain and instability had been reduced and function restored.CONCLUSION Osteotomy may be an effective method to treat patients with congenital cruciate ligament deficiency with posterior tibial plateau tilting.The diagnosis of congenital cruciate ligament deficiency shall be based on the combination of patient’s medical history,clinical manifestations,and findings from imaging to avoid possible misdiagnosis.Based on the symptoms,frequency of attacks,and intent of the individual,appropriate treatment options shall be identified.展开更多
This editorial discusses the significant findings and implications of the study conducted by Alomran et al.This retrospective study,soon to be published,provides valuable insights into the epidemiology of and risk fac...This editorial discusses the significant findings and implications of the study conducted by Alomran et al.This retrospective study,soon to be published,provides valuable insights into the epidemiology of and risk factors associated with clubfoot in a specific Saudi population.By highlighting the study’s key outcomes and discussing its broader implications for public health and clinical practices,this editorial aims to underscore the importance of continued research and targeted interventions in addressing congenital deformities such as clubfoot.展开更多
文摘Objective To report use of the Vibrant Soundbridge (VSB) in patients with congenital deformation of the middle and outer ears and investigate its utility in this patient population.Method Four patients with congenital deformation of middle and outer ears underwent VSB implantation. All were male (aged 3-18 years,average 13.5 years) and operated on the left side.Malformation was bilateral in 3 patients and unilateral in 1 patient. Surgical techniques were modified to accommodate each patient’s unique conditions and needs.The implant site was approached via the facial recess in 3 patients and through a retro-facial nerve route in 1 patient. The VSB implant was connected to either the stapes (2 cases) or the round window (2 cases).Pure tone and speech audiometry results and daily communication capabilities before and after VSB activation were compared.Results The operations were successful in all patients, with no complications. The patient communication level improved significantly after VSB activation. Average air conduction pure tone threshold or conditioned reflex audiometry threshold improved by 35 dB in the 0.25-4 kHz range,from 69 dB HL before VSB activation to 34 dB HL after.The sentence recognition rate in quiet at 65 dB SPL went up to 86% from 0% without VSB for patients with bilateral deformation and remained at 100% for the patient with unilateral deformity. However, for the latter patient, the rate improved to 20% from 0% without VSB in noise (-8 dB SNR).Conclusion VSB is an excellent solution for improving hearing in patients with congenital deformation of middle and outer ears.Operation can be completed and good results can be achieved even in patients with unique conditions and needs.
基金Supported by the Natural Science Foundation of Hunan Province,No.2016JJ3165 and No.2015JJ2169the Fundamental Research Funds for the Central Universities of Central South University,No.2017zzts902
文摘BACKGROUND Congenital extrahepatic portosystemic shunt, also known as Abernethy deformation, is a rare malformation caused by dysplasia in the portal vein system. There are few reports of liver transplantation as a treatment for Abernethy deformation, and our report is the first case in China. This is the second reported case with congenital extrahepatic portosystemic shunt combined with focal nodular hyperplasia and hepatopulmonary treated with liver transplantation.CASE SUMMARY The patient was a 14-year-old girl, diagnosed preoperatively as type Ib Abernethy deformation, intrahepatic multiple space-occupying lesion, and hepatopulmonary syndrome. The patient recovered well after undergoing classic orthotopic liver transplantation. Liver function, pulmonary function, and portal vein computed tomography angiography imaging were reexamined 20 mo postoperatively, and no abnormality was observed.CONCLUSION Liver transplantation is an effective treatment for type I Abernethy deformation combined with focal nodular hyperplasia and hepatopulmonary syndrome.
文摘Background:Congenital thumb deformities account for one-third or more of all cases of congenital hand deformity.However,the current classification schemes of congenital thumb hypoplasia are no longer adequate due to their lack of adaptability to increasing knowledge in the field.Hence,a modified system with the potential to adapt to ongoing advances in knowledge and understanding is desperately needed.Methods:Based on the photographs collected from thousands of cases of congenital deformities of the hand and upper limb over multiple decades in our department,we subdivided thumb hypoplasia according to the variables of morphological characteristics,anatomical structures,functional status,the relationship between thumb deformity and hand deformity,the relationship between congenital hand deformity syndrome and thumb hypoplasia,and the selection of treatment methods.Results:A total of 10 types were presented,which were elucidated with nomenclatures as well as pathological feature and symptoms.Conclusion:This modified system may shed additional light on the classification of congenital thumb anomalies,which will assist in a more effective selection of treatment modalities and offers significant benefits to both patients and practice.
文摘BACKGROUND Hypoplasia of bilateral cruciate ligaments is a rare congenital malformation.The diagnosis of such diseases and indications for the various treatment options require further analysis and discussion.CASE SUMMARY The patient is a 26-year-old Chinese woman who has been suffering from knee pain since the age of 8 years,2-3 episodes a year.Three years ago,due to the practice of advanced yoga poses,the frequency of left knee pain increased,requiring prompt medical treatment.Magnetic resonance imaging demonstrated an absence of both anterior and posterior cruciate ligaments of both knees with abnormal posterior tilting of the tibial plateau.Bilateral subluxation of the knee joint was also found,therefore tibial osteotomy was performed.The patient reported at the 24 mo follow-up that the frequency of pain and instability had been reduced and function restored.CONCLUSION Osteotomy may be an effective method to treat patients with congenital cruciate ligament deficiency with posterior tibial plateau tilting.The diagnosis of congenital cruciate ligament deficiency shall be based on the combination of patient’s medical history,clinical manifestations,and findings from imaging to avoid possible misdiagnosis.Based on the symptoms,frequency of attacks,and intent of the individual,appropriate treatment options shall be identified.
文摘This editorial discusses the significant findings and implications of the study conducted by Alomran et al.This retrospective study,soon to be published,provides valuable insights into the epidemiology of and risk factors associated with clubfoot in a specific Saudi population.By highlighting the study’s key outcomes and discussing its broader implications for public health and clinical practices,this editorial aims to underscore the importance of continued research and targeted interventions in addressing congenital deformities such as clubfoot.
