Every year there are more than 11000 hospitalizations related to heart failure in children resulting in significant morbidity and mortality.Over the last two decades,our understanding,diagnosis and management of pedia...Every year there are more than 11000 hospitalizations related to heart failure in children resulting in significant morbidity and mortality.Over the last two decades,our understanding,diagnosis and management of pediatric heart failure is evolving but our ability to prognosticate outcomes in pediatric heart acute heart failure is extremely limited due to lack of data.In adult heart failure patients,the role of cardiac biomarkers has exponentially increased over the last two decades.Current guidelines for management of heart failure emphasize the role of cardiac biomarkers in diagnosis,management and prognostication of heart failure.It is also noteworthy that these biomarkers reflect important biological processes that also open up the possibility of therapeutic targets.There is however,a significant gap present in the pediatric population with regards to biomarkers in pediatric heart failure.Here,we seek to review available data regarding cardiac biomarkers in the pediatric population and also explore some of the emerging biomarkers from adult literature that may be pertinent to pediatric heart failure.展开更多
To the Editor:Owing to the heterogeneity of congenital heart disease-associated pulmonary hypertension(CHDPH)disease and the development of the pulmonary vascular system in pediatric patients,the management of CHD-PH ...To the Editor:Owing to the heterogeneity of congenital heart disease-associated pulmonary hypertension(CHDPH)disease and the development of the pulmonary vascular system in pediatric patients,the management of CHD-PH in children is signicantly different from that in adults.[1]Early identication and intervention of CHD-PH are of great signicance for disease management in children with CHD,and non-invasive,accurate clinical tools are urgently needed to predict PH risk in children with CHD.However,the applicability of the current risk-scoring system to pediatric CHD-PH remains unclear.Furthermore,there are presently no user-friendly,non-invasive online tools available for predicting CHD-PH,and there is a scarcity of related studies in Asian populations.This study aimed to develop and validate the prediction tools for estimating the risk of pulmonary hypertension and clinical deterioration in children with congenital heart disease.展开更多
Pediatric trigger thumb is an acquired flexion deformity of the interphalangeal(IP)joint.It presents most commonly as a fixed flexion deformity,but can present as intermittent triggering or even a fixed extension defo...Pediatric trigger thumb is an acquired flexion deformity of the interphalangeal(IP)joint.It presents most commonly as a fixed flexion deformity,but can present as intermittent triggering or even a fixed extension deformity.Roughly one-third of patients will develop bilateral trigger thumbs.Studies have shown that the deformity can resolve with time on its own,but prolonged deformity is concerning for permanent IP joint contracture and/or deviation and metacarpophalangeal(MCP)joint compensatory hyperextension.Treatment is controversial,ranging from observation,splinting and stretching,to surgical release of the A1 pulley.Surgery is considered the definitive treatment with low complication rates,although the timing of surgery is highly variable among surgeons.展开更多
Background The extent of our knowledge of the epidemiology of pediatric glaucoma in China is limited. To better characterize the epidemiology of pediatric glaucoma in eastern China, we report the clinical profile, eti...Background The extent of our knowledge of the epidemiology of pediatric glaucoma in China is limited. To better characterize the epidemiology of pediatric glaucoma in eastern China, we report the clinical profile, etiologies, and treatment modalities in patients 〈18 years of age in Shanghai Eye, Ear, Nose and Throat Hospital. Methods The medical records of patients presenting glaucoma between January 2003 and December 2010 were retrospectively reviewed. The demographic characteristics, the proportion of different glaucoma subtypes and surgical precedures were collected and analyzed. Results A total of 1 142 eyes of 734 pediatric patients （500 males） were included. Congenital glaucoma was the leading subtype, accounting for 47.55% of all patients. The ratio of boys to girls was 2.5：1. Patients with congenital glaucoma affecting both eyes accounted for 72.5% of all patients examined. Patients with primary juvenile glaucoma were the second most common group （n=125, 17.03%）. Traumatic glaucoma was the third most common subtype （n=81, 11.03%）. The type of surgery was related to the subtype of glaucoma. Conclusions Congenital glaucoma, primary juvenile glaucoma, and traumatic glaucoma are the most prevalent subtypes in pediatric glaucoma patients in Shanghai Eye, Ear, Nose and Throat Hospital. The characteristics of congenital glaucoma in China are similar to those in Western countries.展开更多
Congenital vascular anomalies affecting the liver have been described in the scientific literature for decades.Understanding these malformations begins with knowledge of hepatic vascular embryology.Surgeons have appli...Congenital vascular anomalies affecting the liver have been described in the scientific literature for decades.Understanding these malformations begins with knowledge of hepatic vascular embryology.Surgeons have applied numerous classification systems to describe both intrahepatic and extrahepatic shunts,which can confuse the reader and clinician.In our experience,focusing on one classification system for extrahepatic shunts and one for intrahepatic shunts is better.Today many patients with these shunts carry good long-term prognosis thanks to advances in imaging to better detect shunts earlier and classify them.Timely intervention by skilled radiologists and surgeons have also limited complications arising from dynamic shunts and can avoid a liver transplant.Congenital hepatic shunts are not the only vascular condition affecting the liver.Hereditary hemorrhagic telangiectasia,also known as Osler Weber Rendu syndrome,particularly type 2,may have varying severity of hepatic involvement which warrants longitudinal care from an experienced hepatologist.Lastly,congenital hemangiomas,often first identified on the skin and oral mucosa,also can affect the liver.While most will resolve in infancy and childhood,the pediatric hepatologist must understand how and when to treat persistent lesions and their complications.This article serves as a concise reference to help clinicians better care for patients with these rare conditions.展开更多
Purpose: To investigate the epidemiology of strabismus in cases of pediatric cataracts. To assess visual outcome following orthoptic treatment for amblyopia in cases of cataracts in the pediatric age group. Methods: T...Purpose: To investigate the epidemiology of strabismus in cases of pediatric cataracts. To assess visual outcome following orthoptic treatment for amblyopia in cases of cataracts in the pediatric age group. Methods: This was a retrospective cohort study. We investigated a consecutive series of pediatric patients with congenital, developing, or traumatic cataracts who underwent surgery between January 1999 and April 2012 at our center. Patient demographics, cataract type, presenting symptoms, surgical intervention, postoperative visual acuity, and follow-up refractive changes were recorded. Results: In total, 1331 eyes of 1043 children were included: unilateral cataracts were present in 785 (59%) eyes. There were 605 (45.5%) traumatic and 726 (54.5%) non-traumatic cases. Ages at surgery ranged from 1 to 215 months. All eyes were examined for ocular alignment;66 (5%) were found to manifest strabismus. Deviation was significantly associated with age at intervention (p p < 0.001), and etiology of cataracts (p 0.001). We found significant differences in visual outcome following amblyopia therapy (p 0.001). Conclusions: Surgical treatment with intraocular lens implantation in children with congenital, developmental, or traumatic cataracts is effective for visual rehabilitation. Orthoptic treatment made a significant difference in visual outcome (p < 0.001).展开更多
Introduction: Children’s heart disease is a major public health problem in developing countries and especially in Mali. The purpose of our work was to determine frequency, different types of heart disease and their s...Introduction: Children’s heart disease is a major public health problem in developing countries and especially in Mali. The purpose of our work was to determine frequency, different types of heart disease and their short term evolution in the pediatric department. Methods: We performed a retrospective study among children aged 0 to 15 years, hospitalized in the pediatric department from January to December 2015 and whose diagnosis was confirmed using trans-thoracic echocardiography. Results: We included 103 cases of heart disease out of a total of 8613 admissions in the pediatric department, giving an hospital prevalence of 1.2%. Mean age was 4.1 years (from 1 day to 15 years) and children under 5 years were the most affected with 73.80% of cases. Male predominance was noted (sex ratio = 1.2). Respiratory distress was the most common circumstance of discovery (93.20%). Cardiac murmur and tachycardia were the most common cardiac signs with respectively 88.35% and 83.50%. Congenital heart disease accounted for 70.87% and was dominated by ventricular septal defect (VSD) with 30.13%. Acquired heart disease (29.13% of the sample) was dominated by mitral regurgitation (MR) with 56.67%. Mortality rate was 31.9% for congenital heart disease and 11.1% for acquired heart disease. Conclusion: children’s heart disease is responsible for high mortality. Early detection improves the management of this pathology, which remains frequent.展开更多
Purpose: To assess treatment results in pediatric patients with cataracts, to evaluate the efficacy of various surgical interventions, and to determine the factors that affect visual outcomes. Methods: This is aProspe...Purpose: To assess treatment results in pediatric patients with cataracts, to evaluate the efficacy of various surgical interventions, and to determine the factors that affect visual outcomes. Methods: This is aProspective cohort study. We studied a consecutive series of pediatric patients with congenital, developing, or traumatic cataracts who underwent surgery between January, 1999 and April, 2012 at Drashti Netralaya, Dahod. Patient demographics, cataract type, presenting symptoms, surgical intervention, postoperative visual acuity, and follow-up refractive changes were recorded. Results: In total, 1305 eyes of 1047 children were included: unilateral cataracts were present in 786 (60.2%) eyes. There were 610 (46.7%) traumatic and 695 (53.3%) non-traumatic cases. Ages at surgery ranged from 1 to 215 months. Eyes were grouped by the surgical intervention performed: Group 1, pars plana approach including 366 (28%) eyes that underwent lensectomies, and Group 2, anterior approach, including 939 (71.9%) eyes that underwent phacoemulsification ± IOL placement or small incision cataract surgery ± IOL placement. The mean follow-up time was 117 days. Ultimately, 113 (30.9%) Group 1 and 503 (53.6%) Group 2 patients achieved a visual acuity better than 20/60 (P < 0.001). Age at intervention, laterality, sensory nystagmus, pretreatment vision, IOL insertion, and etiology were all significantly related (all P < 0.001) to visual outcome. Conclusions: Surgical treatment with intraocular lens implantation for children with congenital, developmental, or traumatic cataracts is an effective treatment for visual rehabilitation. Visual outcome was significantly better in cases of traumatic cataracts versus non-traumatic cataracts.展开更多
Introduction: Popliteal pterygium syndrome is a rare birth defect, combining craniofacial, genitourinary and musculoskeletal abnormalities. It is an autosomal dominant disease caused by a mutation in the IRF6 gene. We...Introduction: Popliteal pterygium syndrome is a rare birth defect, combining craniofacial, genitourinary and musculoskeletal abnormalities. It is an autosomal dominant disease caused by a mutation in the IRF6 gene. We report in this observation the 1<sup>st</sup> Guinean case corrected by the surgical method as well as a review of the literature for a diagnostic and therapeutic approach. Patient and observation: We present the case of a 7-day old male newborn weighing 2700 g who was received for bilateral cleft lip and palate, lower lip fossa or sinuses, bilateral popliteal pterygium, and triangular skin fold above the hallux. The patient underwent several surgical procedures aimed at correcting these abnormalities. The correction of the pterygium of the lower limbs was ensured by excision of the fibrous band, the tenoplasty in z of the calcaneal tendon on the right side and the skin plasty in z in series then immobilized by plaster splints. The immediate postoperative follow-up was straightforward. Conclusion: Popliteal pterygium syndrome is a rare congenital malformation, the diagnosis is primarily clinical. Early soft tissue lengthening surgery and serial z-skin plasty provide better correction of the knee pterygium. Correct correction of facial abnormalities gives the child a better appearance. The management of this syndrome is multidisciplinary.展开更多
Purpose: To evaluate post-surgical complications in patients who were operated for nontraumatic pediatric cataracts in our clinic. Methods: Medical records of 62 patients (101 eyes) that underwent cataract surgery you...Purpose: To evaluate post-surgical complications in patients who were operated for nontraumatic pediatric cataracts in our clinic. Methods: Medical records of 62 patients (101 eyes) that underwent cataract surgery younger than 15 years of age, without history of ocular trauma and with a minimum follow-up period of 6 months, were reviewed retrospectively. Results: The most common initial complaint was leukocoria. Thirty-nine patients (62.9%) had bilateral cataracts and 23 patients (37.1%) had unilateral cataracts. The most common type was posterior polar cataract (22%) in patients with unilateral cataracts, and total cataract (33%) in patients with bilateral cataracts. The overall prevalence of postoperative complications was 58%, visual axis opacification (VAO) being the most common one (39%). The incidence of VAO was significantly higher in eyes with intact posterior capsules. Secondary glaucoma occurred in 12 (12%) eyes. Thirty-one (50%) patients were orthophoria, 17 (27%) patients had esotropia, and 14 (23%) patients had exotropia. Additional surgery for all of these complications was performed in 53 (53%) eyes. Conclusions: Despite appropriate surgical treatment of nontraumatic pediatric cataracts, post-surgical complications including VAO, glaucoma, or strabismus remain an important cause of morbidity in these patients. Posterior capsulotomy and anterior vitrectomy must be performed in all children below 6 years to reduce the need for additional surgery for VAO.展开更多
<strong>Background:</strong><span style="font-family:""><span style="font-family:Verdana;"> Pediatric surgery in developing countries faces serious problems of inadequa...<strong>Background:</strong><span style="font-family:""><span style="font-family:Verdana;"> Pediatric surgery in developing countries faces serious problems of inadequate infrastructures and equipment. Since July 2019, a new department was created in the north of Togo. It is in this context that this study is initiated, with the aim of taking stock of the epidemiological distribution of pediatric surgical diseases. </span><b><span style="font-family:Verdana;">Material and methods:</span></b><span style="font-family:Verdana;"> This was a retrospective and descriptive study over a period of eighteen months, from October 30, 2019 to April 30, 2021. It took place in the pediatric surgery department of the Kara teaching hospital. It concerns pathologies seen at the consultation in the department collected in consultation register. Epidemiological parameters were studied. </span><b><span style="font-family:Verdana;">Results:</span></b><span style="font-family:Verdana;"> In eighteen (18) months, two hundred and fifty-nine children, were seen in pediatric surgery consultation at the Kara Teaching Hospital. The annual frequency of consultations was 172.66. The monthly frequency was 14.39. The average age of the children was 4.56 years with extremes of 2 days and 15 years. There were 194 boys (74.90%) and 65 girls (25.10%). At the consultation, whatever the reason, the examination was normal in 17 children and 242 children had abnormalities. Two hundred and twelve children (81.85%) had each one abnormality and 30 children (11.58%) had each at least two. A total of 298 diseases were reported in the 242 children. These were 218 congenital diseases (73.15%) dominated by urological abnormalities with 37.16% of cases and 80 acquired diseases (26.85%). Acquired diseases were for 26.85% and dominated by limb trauma and infectious pathologies including chronic osteomyelitis. </span><b><span style="font-family:Verdana;">Conclusion:</span></b><span style="font-family:Verdana;"> Pathologies seen in pediatric surgery consultation were展开更多
AIM:To evaluate the graft rejection and visual outcomes after penetrating keratoplasty(PK)in the presence of various congenital corneal opacities in children.METHODS:In this retrospective cohort study,children who und...AIM:To evaluate the graft rejection and visual outcomes after penetrating keratoplasty(PK)in the presence of various congenital corneal opacities in children.METHODS:In this retrospective cohort study,children who underwent PK were then followed for 5 y.The patient’s medical records were collected from June 2014 until June 2019 and analyzed in December 2019.All patients were children under three years old with congenital corneal opacities with or without microcornea who came to a pediatric ophthalmologist and underwent PK in Jakarta Eye Center(JEC).Beforehand,all children have participated in a thorough evaluation for PK.In the case of severe microcornea was not advised to undergo surgery.The visual outcomes and graft survival rate were described in percentages.The graft survival plot was presented with Kaplan-Meier,while the visual acuity was analyzed using the Wilcoxon signed ranks test.RESULTS:Sixteen eyes from eleven patients(seven girls and four boys)underwent PK.The graft survival rate of the first 6,12,and 18 mo later of keratoplasty was 100%,83.3%,and 66.7%,respectively.The overall mean survival time is 22 mo(standard error 2.419),and no significant difference between the patients underwent PK before and after 36 mo of their age(P=0.52).The graft failure was 50%,and postsurgery complications included cataract 43.7%,band keratopathy 12.5%,and scleromalasia 6.25%.Wilcoxon test analysis of visual acuity post keratoplasty was not statistically significant(P=0.34),while overall showed 44%improvements of visual outcome for 5 y of follow-up.With a good survival at one year up to 22 mo(83.3%),the visual acuity could be achieved(63%),and showed improvements(44%)during follow-up.CONCLUSION:The complications are frequent for pediatric PK.Thus,corneal surgery on infants requires careful case selection,adequate pre-operative evaluation,skilled surgery(optical correction),very close cooperation family–physician,intensive post-operation care,and amblyopia management in the future.展开更多
BACKGROUND Pediatric temporal fistulae are rarely reported in the literature.Dissemination of these cases can help inform future diagnosis and effective treatment.CASE SUMMARY Three pediatric patients came to the clin...BACKGROUND Pediatric temporal fistulae are rarely reported in the literature.Dissemination of these cases can help inform future diagnosis and effective treatment.CASE SUMMARY Three pediatric patients came to the clinic due to repeated infections of the skin and soft tissue of the temporal area.One patient presented with a temporal fistula that penetrated the temporal bone and reached the dura mater.Another patient presented with a temporal fistula that penetrated into the temporal muscle fascia.The third patient presented with a fistula that penetrated the lateral wall of the orbit and entered the orbit.All patients underwent surgical fistula resection informed by preoperative computed tomography(CT)evaluation.Histopathological evaluation was also performed.All three patients were surgically treated successfully.Histopathological evaluations confirmed the fistula diagnoses in all three cases.CONCLUSION For patients who have temporal fistulae with repeated infections,surgical treatment should be performed as soon as possible to prevent serious complications.CT can be very useful for preoperative evaluation.B-mode ultrasound examination and evaluation also have a certain auxiliary role.展开更多
Arteriovenous malformation AVM is a vascular congenital defect affecting microvasculatu re of both brain and peripheral o rga ns.Arteriovenous malformation of the brain(bAVM,OMIM#108010),in particular,affects up to 15...Arteriovenous malformation AVM is a vascular congenital defect affecting microvasculatu re of both brain and peripheral o rga ns.Arteriovenous malformation of the brain(bAVM,OMIM#108010),in particular,affects up to 15 per100,000 persons with no sex predominance.Almost 50%of the patients manifest intracerebral hemorrhage and epileptic seizures,as main clinical symptoms.展开更多
Objectives:Factors influencing results of balloon valvuloplasty(BVP)of pulmonary valve stenosis(PS)in children are investigated.Background:BVP has become the standard of care for PS,medium-term results are not uniform...Objectives:Factors influencing results of balloon valvuloplasty(BVP)of pulmonary valve stenosis(PS)in children are investigated.Background:BVP has become the standard of care for PS,medium-term results are not uniform and depend on various preconditions.Methods:We analysed the medium-term results of BVP of PS in children in an observational,single centre study.Need for additional procedure was defined as outcome after initial BVP.Results:We included 143 children(83 female)at a median(IQR)age of 2.6(0.26–9.24)months and body weight of 5(3.4–8)kg at BVP with a follow–up of 5.04(1.6–10.2)years.We used balloon size of 10(9–14)mm and maximal balloon pressure of 4(3.5–10)atm,resulting in balloon–to–pulmonary annulus ratio of 1.28(1.2–1.4).Systolic pressure gradient of PS was reduced with BVP(43.5 mmHg vs.14.0 mmHg,p<0.001)and confirmed by echocardiography(68.0 mmHg vs.25.0 mmHg,p<0.001)day 1 post procedure.Pulmonary BVP with associated supravalvular PS resulted in a relevant reduction of systolic pressure gradient in 23 of 31 patients(74.2%).Early additional procedure was necessary in 14 patients(9.8%)after 0.2(0.1–0.7)years due to residual PS(n=13)and infective endocarditis(n=1).Factors for additional procedures were associated supravalvular PS with a higher residual pressure gradient,but not genetic syndrome.During further follow–up of 5.04(1.6–10.2)years no further additional procedures were needed.Conclusions:Pulmonary BVP of native pulmonary valve stenosis leads to excellent medium-term results,even in 3 of 4 infants with associated supravalvular obstruction sufficient pressure relief can be obtained.展开更多
Congenital cholesteatoma（CC）is a rarely seen benign tumor of the temporal bone. There are five general sites of extradural occurrence: the middle ear, external auditory meatus, mastoid, squamous portion and the petr...Congenital cholesteatoma（CC）is a rarely seen benign tumor of the temporal bone. There are five general sites of extradural occurrence: the middle ear, external auditory meatus, mastoid, squamous portion and the petrous apex of the temporal bone. CC grows slowly and presents no symptoms at the early stage. Delayed and mis-diagnosis are common with this condition. Case report A 10-year-old boy presented with a 3-month history of hearing loss on right side. There was no history of otorrhea, facial palsy, previous otological procedures or trauma. Otoscopy revealed a bulging posterosuperior quadrant in the otherwise intact right tympanic membrane （Fig.1）. Pure tone audiometry showed an average threshold of 51 dB for 500, 1000, 2000 and 4000Hz, with a 40 dB air-bone gap, suggesting a moderate conductive hearing loss（Fig.4）. CT scan of the temporal bone showed an isolated soft tissue density lesion in the middle ear（Fig.2）.展开更多
文摘Every year there are more than 11000 hospitalizations related to heart failure in children resulting in significant morbidity and mortality.Over the last two decades,our understanding,diagnosis and management of pediatric heart failure is evolving but our ability to prognosticate outcomes in pediatric heart acute heart failure is extremely limited due to lack of data.In adult heart failure patients,the role of cardiac biomarkers has exponentially increased over the last two decades.Current guidelines for management of heart failure emphasize the role of cardiac biomarkers in diagnosis,management and prognostication of heart failure.It is also noteworthy that these biomarkers reflect important biological processes that also open up the possibility of therapeutic targets.There is however,a significant gap present in the pediatric population with regards to biomarkers in pediatric heart failure.Here,we seek to review available data regarding cardiac biomarkers in the pediatric population and also explore some of the emerging biomarkers from adult literature that may be pertinent to pediatric heart failure.
基金National Natural Science Foundation of China(Nos.82000034,82120108001,and 82170069)Chongqing Postdoctoral International Exchange Training Program(No.2021JLPY001)+2 种基金National Clinical Medical Research Center(No.NCRC-2022-GP-08)Guangdong Department of Science and Technology(No.2022A0505030017)Basic Science and Application of Guangzhou Science and Technology Plan(No.202201010069)
文摘To the Editor:Owing to the heterogeneity of congenital heart disease-associated pulmonary hypertension(CHDPH)disease and the development of the pulmonary vascular system in pediatric patients,the management of CHD-PH in children is signicantly different from that in adults.[1]Early identication and intervention of CHD-PH are of great signicance for disease management in children with CHD,and non-invasive,accurate clinical tools are urgently needed to predict PH risk in children with CHD.However,the applicability of the current risk-scoring system to pediatric CHD-PH remains unclear.Furthermore,there are presently no user-friendly,non-invasive online tools available for predicting CHD-PH,and there is a scarcity of related studies in Asian populations.This study aimed to develop and validate the prediction tools for estimating the risk of pulmonary hypertension and clinical deterioration in children with congenital heart disease.
文摘Pediatric trigger thumb is an acquired flexion deformity of the interphalangeal(IP)joint.It presents most commonly as a fixed flexion deformity,but can present as intermittent triggering or even a fixed extension deformity.Roughly one-third of patients will develop bilateral trigger thumbs.Studies have shown that the deformity can resolve with time on its own,but prolonged deformity is concerning for permanent IP joint contracture and/or deviation and metacarpophalangeal(MCP)joint compensatory hyperextension.Treatment is controversial,ranging from observation,splinting and stretching,to surgical release of the A1 pulley.Surgery is considered the definitive treatment with low complication rates,although the timing of surgery is highly variable among surgeons.
基金This work was supported by grants from the National Natural Science Foundation （No. NSFC81100667） and the Science and Technology Commission of Shanghai （No. 114119a7300）.Acknowledgments： We thank Prof. Chen Dongfeng and Yang Lanbo in Massachusetts Eye and Ear, Harvard Medical School, Boston, USA, for their great help to edit the English in the paper.
文摘Background The extent of our knowledge of the epidemiology of pediatric glaucoma in China is limited. To better characterize the epidemiology of pediatric glaucoma in eastern China, we report the clinical profile, etiologies, and treatment modalities in patients 〈18 years of age in Shanghai Eye, Ear, Nose and Throat Hospital. Methods The medical records of patients presenting glaucoma between January 2003 and December 2010 were retrospectively reviewed. The demographic characteristics, the proportion of different glaucoma subtypes and surgical precedures were collected and analyzed. Results A total of 1 142 eyes of 734 pediatric patients （500 males） were included. Congenital glaucoma was the leading subtype, accounting for 47.55% of all patients. The ratio of boys to girls was 2.5：1. Patients with congenital glaucoma affecting both eyes accounted for 72.5% of all patients examined. Patients with primary juvenile glaucoma were the second most common group （n=125, 17.03%）. Traumatic glaucoma was the third most common subtype （n=81, 11.03%）. The type of surgery was related to the subtype of glaucoma. Conclusions Congenital glaucoma, primary juvenile glaucoma, and traumatic glaucoma are the most prevalent subtypes in pediatric glaucoma patients in Shanghai Eye, Ear, Nose and Throat Hospital. The characteristics of congenital glaucoma in China are similar to those in Western countries.
文摘Congenital vascular anomalies affecting the liver have been described in the scientific literature for decades.Understanding these malformations begins with knowledge of hepatic vascular embryology.Surgeons have applied numerous classification systems to describe both intrahepatic and extrahepatic shunts,which can confuse the reader and clinician.In our experience,focusing on one classification system for extrahepatic shunts and one for intrahepatic shunts is better.Today many patients with these shunts carry good long-term prognosis thanks to advances in imaging to better detect shunts earlier and classify them.Timely intervention by skilled radiologists and surgeons have also limited complications arising from dynamic shunts and can avoid a liver transplant.Congenital hepatic shunts are not the only vascular condition affecting the liver.Hereditary hemorrhagic telangiectasia,also known as Osler Weber Rendu syndrome,particularly type 2,may have varying severity of hepatic involvement which warrants longitudinal care from an experienced hepatologist.Lastly,congenital hemangiomas,often first identified on the skin and oral mucosa,also can affect the liver.While most will resolve in infancy and childhood,the pediatric hepatologist must understand how and when to treat persistent lesions and their complications.This article serves as a concise reference to help clinicians better care for patients with these rare conditions.
文摘Purpose: To investigate the epidemiology of strabismus in cases of pediatric cataracts. To assess visual outcome following orthoptic treatment for amblyopia in cases of cataracts in the pediatric age group. Methods: This was a retrospective cohort study. We investigated a consecutive series of pediatric patients with congenital, developing, or traumatic cataracts who underwent surgery between January 1999 and April 2012 at our center. Patient demographics, cataract type, presenting symptoms, surgical intervention, postoperative visual acuity, and follow-up refractive changes were recorded. Results: In total, 1331 eyes of 1043 children were included: unilateral cataracts were present in 785 (59%) eyes. There were 605 (45.5%) traumatic and 726 (54.5%) non-traumatic cases. Ages at surgery ranged from 1 to 215 months. All eyes were examined for ocular alignment;66 (5%) were found to manifest strabismus. Deviation was significantly associated with age at intervention (p p < 0.001), and etiology of cataracts (p 0.001). We found significant differences in visual outcome following amblyopia therapy (p 0.001). Conclusions: Surgical treatment with intraocular lens implantation in children with congenital, developmental, or traumatic cataracts is effective for visual rehabilitation. Orthoptic treatment made a significant difference in visual outcome (p < 0.001).
文摘Introduction: Children’s heart disease is a major public health problem in developing countries and especially in Mali. The purpose of our work was to determine frequency, different types of heart disease and their short term evolution in the pediatric department. Methods: We performed a retrospective study among children aged 0 to 15 years, hospitalized in the pediatric department from January to December 2015 and whose diagnosis was confirmed using trans-thoracic echocardiography. Results: We included 103 cases of heart disease out of a total of 8613 admissions in the pediatric department, giving an hospital prevalence of 1.2%. Mean age was 4.1 years (from 1 day to 15 years) and children under 5 years were the most affected with 73.80% of cases. Male predominance was noted (sex ratio = 1.2). Respiratory distress was the most common circumstance of discovery (93.20%). Cardiac murmur and tachycardia were the most common cardiac signs with respectively 88.35% and 83.50%. Congenital heart disease accounted for 70.87% and was dominated by ventricular septal defect (VSD) with 30.13%. Acquired heart disease (29.13% of the sample) was dominated by mitral regurgitation (MR) with 56.67%. Mortality rate was 31.9% for congenital heart disease and 11.1% for acquired heart disease. Conclusion: children’s heart disease is responsible for high mortality. Early detection improves the management of this pathology, which remains frequent.
文摘Purpose: To assess treatment results in pediatric patients with cataracts, to evaluate the efficacy of various surgical interventions, and to determine the factors that affect visual outcomes. Methods: This is aProspective cohort study. We studied a consecutive series of pediatric patients with congenital, developing, or traumatic cataracts who underwent surgery between January, 1999 and April, 2012 at Drashti Netralaya, Dahod. Patient demographics, cataract type, presenting symptoms, surgical intervention, postoperative visual acuity, and follow-up refractive changes were recorded. Results: In total, 1305 eyes of 1047 children were included: unilateral cataracts were present in 786 (60.2%) eyes. There were 610 (46.7%) traumatic and 695 (53.3%) non-traumatic cases. Ages at surgery ranged from 1 to 215 months. Eyes were grouped by the surgical intervention performed: Group 1, pars plana approach including 366 (28%) eyes that underwent lensectomies, and Group 2, anterior approach, including 939 (71.9%) eyes that underwent phacoemulsification ± IOL placement or small incision cataract surgery ± IOL placement. The mean follow-up time was 117 days. Ultimately, 113 (30.9%) Group 1 and 503 (53.6%) Group 2 patients achieved a visual acuity better than 20/60 (P < 0.001). Age at intervention, laterality, sensory nystagmus, pretreatment vision, IOL insertion, and etiology were all significantly related (all P < 0.001) to visual outcome. Conclusions: Surgical treatment with intraocular lens implantation for children with congenital, developmental, or traumatic cataracts is an effective treatment for visual rehabilitation. Visual outcome was significantly better in cases of traumatic cataracts versus non-traumatic cataracts.
文摘Introduction: Popliteal pterygium syndrome is a rare birth defect, combining craniofacial, genitourinary and musculoskeletal abnormalities. It is an autosomal dominant disease caused by a mutation in the IRF6 gene. We report in this observation the 1<sup>st</sup> Guinean case corrected by the surgical method as well as a review of the literature for a diagnostic and therapeutic approach. Patient and observation: We present the case of a 7-day old male newborn weighing 2700 g who was received for bilateral cleft lip and palate, lower lip fossa or sinuses, bilateral popliteal pterygium, and triangular skin fold above the hallux. The patient underwent several surgical procedures aimed at correcting these abnormalities. The correction of the pterygium of the lower limbs was ensured by excision of the fibrous band, the tenoplasty in z of the calcaneal tendon on the right side and the skin plasty in z in series then immobilized by plaster splints. The immediate postoperative follow-up was straightforward. Conclusion: Popliteal pterygium syndrome is a rare congenital malformation, the diagnosis is primarily clinical. Early soft tissue lengthening surgery and serial z-skin plasty provide better correction of the knee pterygium. Correct correction of facial abnormalities gives the child a better appearance. The management of this syndrome is multidisciplinary.
文摘Purpose: To evaluate post-surgical complications in patients who were operated for nontraumatic pediatric cataracts in our clinic. Methods: Medical records of 62 patients (101 eyes) that underwent cataract surgery younger than 15 years of age, without history of ocular trauma and with a minimum follow-up period of 6 months, were reviewed retrospectively. Results: The most common initial complaint was leukocoria. Thirty-nine patients (62.9%) had bilateral cataracts and 23 patients (37.1%) had unilateral cataracts. The most common type was posterior polar cataract (22%) in patients with unilateral cataracts, and total cataract (33%) in patients with bilateral cataracts. The overall prevalence of postoperative complications was 58%, visual axis opacification (VAO) being the most common one (39%). The incidence of VAO was significantly higher in eyes with intact posterior capsules. Secondary glaucoma occurred in 12 (12%) eyes. Thirty-one (50%) patients were orthophoria, 17 (27%) patients had esotropia, and 14 (23%) patients had exotropia. Additional surgery for all of these complications was performed in 53 (53%) eyes. Conclusions: Despite appropriate surgical treatment of nontraumatic pediatric cataracts, post-surgical complications including VAO, glaucoma, or strabismus remain an important cause of morbidity in these patients. Posterior capsulotomy and anterior vitrectomy must be performed in all children below 6 years to reduce the need for additional surgery for VAO.
文摘<strong>Background:</strong><span style="font-family:""><span style="font-family:Verdana;"> Pediatric surgery in developing countries faces serious problems of inadequate infrastructures and equipment. Since July 2019, a new department was created in the north of Togo. It is in this context that this study is initiated, with the aim of taking stock of the epidemiological distribution of pediatric surgical diseases. </span><b><span style="font-family:Verdana;">Material and methods:</span></b><span style="font-family:Verdana;"> This was a retrospective and descriptive study over a period of eighteen months, from October 30, 2019 to April 30, 2021. It took place in the pediatric surgery department of the Kara teaching hospital. It concerns pathologies seen at the consultation in the department collected in consultation register. Epidemiological parameters were studied. </span><b><span style="font-family:Verdana;">Results:</span></b><span style="font-family:Verdana;"> In eighteen (18) months, two hundred and fifty-nine children, were seen in pediatric surgery consultation at the Kara Teaching Hospital. The annual frequency of consultations was 172.66. The monthly frequency was 14.39. The average age of the children was 4.56 years with extremes of 2 days and 15 years. There were 194 boys (74.90%) and 65 girls (25.10%). At the consultation, whatever the reason, the examination was normal in 17 children and 242 children had abnormalities. Two hundred and twelve children (81.85%) had each one abnormality and 30 children (11.58%) had each at least two. A total of 298 diseases were reported in the 242 children. These were 218 congenital diseases (73.15%) dominated by urological abnormalities with 37.16% of cases and 80 acquired diseases (26.85%). Acquired diseases were for 26.85% and dominated by limb trauma and infectious pathologies including chronic osteomyelitis. </span><b><span style="font-family:Verdana;">Conclusion:</span></b><span style="font-family:Verdana;"> Pathologies seen in pediatric surgery consultation were
文摘AIM:To evaluate the graft rejection and visual outcomes after penetrating keratoplasty(PK)in the presence of various congenital corneal opacities in children.METHODS:In this retrospective cohort study,children who underwent PK were then followed for 5 y.The patient’s medical records were collected from June 2014 until June 2019 and analyzed in December 2019.All patients were children under three years old with congenital corneal opacities with or without microcornea who came to a pediatric ophthalmologist and underwent PK in Jakarta Eye Center(JEC).Beforehand,all children have participated in a thorough evaluation for PK.In the case of severe microcornea was not advised to undergo surgery.The visual outcomes and graft survival rate were described in percentages.The graft survival plot was presented with Kaplan-Meier,while the visual acuity was analyzed using the Wilcoxon signed ranks test.RESULTS:Sixteen eyes from eleven patients(seven girls and four boys)underwent PK.The graft survival rate of the first 6,12,and 18 mo later of keratoplasty was 100%,83.3%,and 66.7%,respectively.The overall mean survival time is 22 mo(standard error 2.419),and no significant difference between the patients underwent PK before and after 36 mo of their age(P=0.52).The graft failure was 50%,and postsurgery complications included cataract 43.7%,band keratopathy 12.5%,and scleromalasia 6.25%.Wilcoxon test analysis of visual acuity post keratoplasty was not statistically significant(P=0.34),while overall showed 44%improvements of visual outcome for 5 y of follow-up.With a good survival at one year up to 22 mo(83.3%),the visual acuity could be achieved(63%),and showed improvements(44%)during follow-up.CONCLUSION:The complications are frequent for pediatric PK.Thus,corneal surgery on infants requires careful case selection,adequate pre-operative evaluation,skilled surgery(optical correction),very close cooperation family–physician,intensive post-operation care,and amblyopia management in the future.
基金Supported by the Cross Research Fund of Biomedical Engineering of Shanghai Jiaotong University in 2017,No.YG2017MS36.
文摘BACKGROUND Pediatric temporal fistulae are rarely reported in the literature.Dissemination of these cases can help inform future diagnosis and effective treatment.CASE SUMMARY Three pediatric patients came to the clinic due to repeated infections of the skin and soft tissue of the temporal area.One patient presented with a temporal fistula that penetrated the temporal bone and reached the dura mater.Another patient presented with a temporal fistula that penetrated into the temporal muscle fascia.The third patient presented with a fistula that penetrated the lateral wall of the orbit and entered the orbit.All patients underwent surgical fistula resection informed by preoperative computed tomography(CT)evaluation.Histopathological evaluation was also performed.All three patients were surgically treated successfully.Histopathological evaluations confirmed the fistula diagnoses in all three cases.CONCLUSION For patients who have temporal fistulae with repeated infections,surgical treatment should be performed as soon as possible to prevent serious complications.CT can be very useful for preoperative evaluation.B-mode ultrasound examination and evaluation also have a certain auxiliary role.
文摘Arteriovenous malformation AVM is a vascular congenital defect affecting microvasculatu re of both brain and peripheral o rga ns.Arteriovenous malformation of the brain(bAVM,OMIM#108010),in particular,affects up to 15 per100,000 persons with no sex predominance.Almost 50%of the patients manifest intracerebral hemorrhage and epileptic seizures,as main clinical symptoms.
文摘Objectives:Factors influencing results of balloon valvuloplasty(BVP)of pulmonary valve stenosis(PS)in children are investigated.Background:BVP has become the standard of care for PS,medium-term results are not uniform and depend on various preconditions.Methods:We analysed the medium-term results of BVP of PS in children in an observational,single centre study.Need for additional procedure was defined as outcome after initial BVP.Results:We included 143 children(83 female)at a median(IQR)age of 2.6(0.26–9.24)months and body weight of 5(3.4–8)kg at BVP with a follow–up of 5.04(1.6–10.2)years.We used balloon size of 10(9–14)mm and maximal balloon pressure of 4(3.5–10)atm,resulting in balloon–to–pulmonary annulus ratio of 1.28(1.2–1.4).Systolic pressure gradient of PS was reduced with BVP(43.5 mmHg vs.14.0 mmHg,p<0.001)and confirmed by echocardiography(68.0 mmHg vs.25.0 mmHg,p<0.001)day 1 post procedure.Pulmonary BVP with associated supravalvular PS resulted in a relevant reduction of systolic pressure gradient in 23 of 31 patients(74.2%).Early additional procedure was necessary in 14 patients(9.8%)after 0.2(0.1–0.7)years due to residual PS(n=13)and infective endocarditis(n=1).Factors for additional procedures were associated supravalvular PS with a higher residual pressure gradient,but not genetic syndrome.During further follow–up of 5.04(1.6–10.2)years no further additional procedures were needed.Conclusions:Pulmonary BVP of native pulmonary valve stenosis leads to excellent medium-term results,even in 3 of 4 infants with associated supravalvular obstruction sufficient pressure relief can be obtained.
文摘Congenital cholesteatoma（CC）is a rarely seen benign tumor of the temporal bone. There are five general sites of extradural occurrence: the middle ear, external auditory meatus, mastoid, squamous portion and the petrous apex of the temporal bone. CC grows slowly and presents no symptoms at the early stage. Delayed and mis-diagnosis are common with this condition. Case report A 10-year-old boy presented with a 3-month history of hearing loss on right side. There was no history of otorrhea, facial palsy, previous otological procedures or trauma. Otoscopy revealed a bulging posterosuperior quadrant in the otherwise intact right tympanic membrane （Fig.1）. Pure tone audiometry showed an average threshold of 51 dB for 500, 1000, 2000 and 4000Hz, with a 40 dB air-bone gap, suggesting a moderate conductive hearing loss（Fig.4）. CT scan of the temporal bone showed an isolated soft tissue density lesion in the middle ear（Fig.2）.
