Hemimelia tibial: Presentación de un caso / Tibial Hemimelia

Introducción: La hemimelia tibial o hemimelia paraxial longitudinal tibial, es una deficiencia congénita de la tibia. Esta deficiencia de los miembros inferiores longitudinal tibial, es muy rara y su frecuencia está en el orden de 1: 1 000 000 de niños nacidos vivos. Objetivo: Presentar un caso de hemimelia tibial diagnosticado por medio del cuadro clínico y radiografías y tratado quirúrgicamente. Presentación del caso: Paciente masculino de dos horas de nacido, atendido por presentar malformación congénita a nivel de la pierna derecha que se presentaba acortada con una prominencia dura a nivel proximal y el pie con deformidad marcada en supinación, aducción y rotación interna. Se realizó examen físico exhaustivo de la extremidad afecta y se constató el acortamiento evidente de la misma. Se indicó radiografía anteroposterior y lateral de la pierna y se observó que el segmento proximal de la tibia y el peroné estaban bien, pero con implantación alta, por lo que se diagnosticó una hemimelia tibial tipo II de Jones. Luego del alta el niño recibió seguimiento por consulta de Genética y el servicio de Ortopedia donde se decidió someterlo a un primer tiempo quirúrgico a los 6 meses de edad, mediante tibialización del peroné. A los 10 meses se realizó un segundo tiempo quirúrgico para centrar el astrágalo al peroné. Conclusiones: La hemimelia tibial se considera un diagnóstico poco frecuente en nuestro medio. No existe prevención conocida. El tratamiento es complejo y altamente especializado, y en algunos casos requiere la amputación temprana del miembro afectado para adaptar al paciente al uso de prótesis(AU)

Introduction: Tibial hemimelia or tibial longitudinal paraxial hemimelia is a congenital deficiency of the tibia. This tibial longitudinal lower limb deficiency is very rare and its frequency is in the order of 1: 1,000,000 live births. Objective: To report a case of tibial hemimelia diagnosed through the clinical condition and radiographs and treated surgically. Case report: We report the case of a two-hour-old male patient, treated for a congenital malformation at the level of the right leg that was shortened with a hard prominence at the proximal level and the foot with marked deformity in supination, adduction and internal rotation. An exhaustive physical examination of the affected limb was carried out and its evident shortening was confirmed. Anteroposterior and lateral X-rays of the leg were indicated and it revealed that the proximal segment of the tibia and fibula were fine, but with high implantation, for which a Jones type II tibial hemimelia was diagnosed. After discharge, the child was followed up by the Genetics consultation and the Orthopedics service, where it was decided to undergo a first stage surgery at 6 months of age, by means of tibialization of the fibula. At 10 months, a second surgical time was performed to center the talus to the fibula. Conclusions: Tibial hemimelia is considered a rare diagnosis in our setting. There is no known prevention. Treatment is complex and highly specialized, and in some cases requires early amputation of the affected limb to adapt the patient to the use of a prosthesis(AU)

The lateral nail fold plasty in duplicated thumbs with a common nail / 中华整形外科杂志

Objective@#In this study, a new surgical technique was developed to reconstruct the lateral nail fold in the duplicated thumbs with a common nail and postoperative outcomes were evaluated.@*Methods@#From December 2012 to October 2015, sixteen duplicated thumbs with a common nail were treated in our hospital using a flap from resected finger pulp which did not include the nail and phalanx. Unilateral inverting suture was performed between the flap and nail bed to reconstruct the lateral nail fold and nail groove. Postoperative outcomes were evaluated by Wang-Gao scoring system (total 12).@*Results@#The follow-up period ranged from 10 to 45 months. The reconstructed nails were smooth and had a natural lunula, and the nail width was about 80%-100% of their normal sides. There was a right angle between the nail epithelium and lateral nail fold in four cases, and an acute angle in two cases. The reconstructed nails were asymmetrical in these thumbs. One patient showed a hypertrophy in the lateral nail fold. The reconstructed nails were evaluated by Wang-Gao scoring system: 10 in 2 cases, 11 in 4 and 12 in 10. All the cases had excellent results.@*Conclusions@#The nail width usually is more than 80% of the normal sides in the radial polydactyly with a common nail. The surgical technique can reconstruct a natural lateral nail fold and nail groove, and avoid the nail deformity caused by the Bilhaut-Cloquet procedure.

Early non-surgical treatment of congenital auricular deformity / 中华整形外科杂志

Objective@#To investigate the effect of auricle deformity corrector in non-surgical treatment of congenital auricular deformity.@*Methods@#The auricular deformity correctors were applied for non-surgical treatment of congenital auricular deformities. The patients were divided into three groups according to the treatment starting age (<1 month old, 1-3 months old, 3-6 months old), followed up for every month respectively after treatment. According to the improvement of auricle morphology, the treatment results were divided into four levels (e, g, f, p) and the effective rate (e+ g)% was calculated.@*Results@#From January 2014 to December 2016, there were 140 ears of congenital malformations in children aged less than 6 months who were treated and followed up. Among them, 33 ears had helical rim abnormalities, 29 with cup-shaped ears, 12 with prominent ears, 4 with Stahl′s ears, and 62 with cryptotia ears. The therapeutic efficacies (<1 month old, 1-3 months old, 3-6 months) of these ear malformations were: cryptotia ear (100%, 100%, 87.5%), helical rim abnormality (100%, 90.47%, 66.67 %), prominent ear (-, 100%, 50%), cup ear (100%, 78.57%, 53.33%), Stahl′s ear (-, 100%, 33.33%). Follow-up more than 6 months after treatment, up to a year and a half, no recurrence was found.@*Conclusions@#The auricular deformity corrector can be used as an effective approach for achieving natural outcomes and correcting cosmetic abnormalities. Rate of satisfaction is dependent on types of deformity, the neonatal age in which treatment started and also parents′ compliance to treatment methods and principals. The method is noninvasive, easy to wear and works well. Early proper treatment can avoid future operations and save social medical resources.

Clinical efficacy of different operative techniques for reconstruction of vermilion tubercle after primary repair of cleft lip / 中华医学美学美容杂志

Objective To evaluate the clinical repair effects of the reconstruction of different vermilion tubercle after primary cleft lip repair.Methods According to the tight lip deformity and dif ferent degrees of defect of the vermilion tubercle and exposing the gums and the crown of the anterior incisors,178 patients after primary repair of the cleft lip from Department of Oral and Maxillofacial Surgery of Xiangya Hospital,Central South University were classified into the tight lip deformity and four grades of whistling deformities.According to different deformities and defects,the different operative techniques were used as follows:classic Abbé flap and modified Abbé flap were used for tight lip deformity;the bilateral advanced myomucosal flaps of lip were used for the grade Ⅰ;modified Abbé flap was used for repairing the grade Ⅱ and the grade Ⅲ whistling deformities;for grade Ⅳ whistling deformity,we mainly used classic Abbé flap.The follow-up after operation was performed,all patient's Cupid's bow,Cupid's bow peak,the vermilion tubercle and the height and width of the upper lip were observed.The scarring of the upper lip was also observed.Results The follow-up after operation was performed from six months to ninteen years,all the 187 patient's anatomical structures of the upper lip were nearly normal after recovery.The all patients showed an obvious Cupid's bow,Cupid's bow peak,the vermilion tubercle and the height and width of the upper lip.The relation of the upper lip and lower lip was harmonious.The scarring of the upper lip was not obvious 2 years after operation.Conclusions The different repair methods are chosen to repair different deformity and defect that has an important clinical value.

Abordagem cirúrgica em paciente portador da disostose cleidocraniana / Surgical approach in patient with dysostosis cleidocranial

A disostose cleidocraniana (DCC) foi descrita e conceituada, pela primeira vez, em 1897, por Marie e Sainton. O padrão de herança é autossômico dominante, porém, em alguns casos, interferências externas no período fetal poderiam causar mutações genéticas, impedindo a transformação de células-tronco mesenquimais em osteoblastos. Essa patologia é caracterizada por malformações cranianas, anormalidades nas clavículas, dentes supranumerários e retenções dentárias. Estudos radiológicos e genéticos são extremamente importantes para o seu diagnóstico preciso. O presente trabalho objetiva apresentar um tratamento cirúrgico dentário em paciente portador dessa patologia.

The Cleidocranial dysostosis (CCD) was described and conceptualized for the first time in 1897 by Marie and Sainton. The pattern of in heritance is autosomal dominant but, in some cases, externalinterference in the fetal period could cause genetic mutation preventing the transformation of mesenchymal stem cells into osteoblasts. This pathologyis characterized by cranial malformations, abnormalities of the clavicles, supernumerary teeth and dental retentions. Radiologicalstudies are extremelyimportanttoa precise diagnosis. Thisstudy aims to present a dental surgicaltreatment in patientswiththispathology.

La imagenologia médica en el laboratorio de morfología: iconografía / Medical imaging laboratory Morphology: iconography of bicéfalo fetus / A imagiologia médica no laboratório de morfologia: iconografia do feto bicéfalo

El nacimiento de gemelos siameses es un fenómeno extraño en las salas de parto de todo el mundo, son producto de un mismo óvulo que por alguna extraña razón no llegó a dividirse; se presenta 1/50.000 o 1/120.000 nacimientos; se estima que el 50% nace muerto y el 35% muere el primer día de vida; y el 70% son de génerofemenino. Un feto bicéfalo es un producto desarrollado con dos cabezas que se unen entre sí por medio de sus porciones temporo-parietales mediales, estando conectados vascularmente y compartiendo o no órganos entre sí; son de tipo monocigóticos y producidos por la separación anormal o incompleta de la porción axial del disco germinativo bilaminar en etapas avanzadas del desarrollo embrionario. En el presente trabajo se expone un caso de siameses bicéfalo de sexo femenino, cuyo espécimen hacía parte de las colecciones de embriología del Laboratorio de Morfología de la Universidad Tecnológica de Pereira, el cual por registro de Historia Clínica y Consentimiento Informado de la madre, quien lo entregó a la Universidad, fue sometido a descripción anatomopatológica con el fin de exponer aspectos relacionados con las posibles causas que los originaron, así como el diagnóstico prenatal de estos defectos congénitos y su diagnóstico Imagineológico postmortem.

The birth of conjoined twins is a strange phenomenon in the delivery rooms around the world, are the product of a single egg that for some reason did not divide; is presenta1 / 50,000 or 1 / 120,000 births; is estimated that 50% stillborn and 35% die on the first day of life; and 70% are female. A two-headed fetus is a product developed with two heads that are joined together through their temporoparietal medial portions being connected vascularized organs and sharing with each other or not; are monozygotic type and produced by abnormal or incomplete separation of the axial portion of the bilaminar germ disc in advanced stages of embryonic development. In this paper a case of two-headed Siamese female, whose specimen was part of the collections of Morphology Embryology Laboratory of the Technological University of Pereira, who for record medical history and informed consent of the mother who is exposed delivered to the University underwent pathological description to explain aspects of the possible causes of these accidents and the prenatal diagnosis of these congenital defects and postmortem imaging diagnosis.

O nascimento de gêmeos siameses é um fenômeno estranho nas salas de parto de todo o mundo. São produto de um mesmo óvulo, que por alguma estranha razão não chegou a dividir-se; se apresenta em 1/50.000 ou 1/120.000 nascimentos. Estima-se que 50% nascem mortos e 35% morre ao primeiro dia de vida; e 70% são do gênero feminino.Um feto bicéfalo é um produto desenvolvido com duas cabeças que se unem entre si por meio de suas porções temporão-parietais mediais, estando conectados vascular-mente e compartilham ou não órgãos entre si; são de tipo monozigóticos e produzi-dos pela separação anormal ou incompleta da porção axial do disco germinativo bi laminar em etapas avançadas do desenvolvimento embrionário. Neste trabalho se expõe o caso de siamesas bicéfalas do sexo feminino, cujo espéci-men fazem parte das coleções de embriologia do Laboratório de Morfologia da Uni-versidade Tecnológica de Pereira, que por registro de Historia Clínica e Consenti-mento Informado da mãe, que o entregou à Universidade, foi submetido a descrição anatomopatológica com o fim de expor aspectos relacionados às possíveis causas que os originaram, assim como o diagnóstico pré-natal destes defeitos congênitos e seu diagnóstico Imagiológico post-mortem.

Pie en espejo / Mirror foot

Paciente masculino de 79 años de edad, el cual presenta en el miembro inferior izquierdo a nivel del pie una malformación congénita que corresponde a un pie en espejo, representado por 9 artejos, articulados en 8 metatarsianos, pie zambo, seudoartrosis alta de la tibia y luxación congénita alta del peroné. Es inusual encontrar esta deformidad en el momento actual.

Here is a 79 years-old patient who had in his left foot a congenital malformation called mirror foot, which is represented by 9 knuckles articulated in 8 metatarsals, knock-kneed foot, upper pseudoarthrosis of the shinbone and upper congenital luxation of the fibula. The presentation of this type of deformity is unusual today.

Analysis of diagnosis and treatment for the congenital superior cervical deformity / 中国矫形外科杂志

[Objective]To analysis the unstable pathogeny of the superior cervical congenital deformity for the operative method selection. [Methods]65 patients with congenital superior cervical deformity were treated from January 2003 to June 2007,in which there were 39 male patients,and 26 female patients. The X-ray,CT and MRI were examined generally before operation. In the images,there were 32 cases with formation failure deformity,18 cases with segmentation failure deformity,and 15 cases with abnormal structure deformity. 37 cases with easy reduction and no anterior compression were treated with posterior internal fixation and self cancellous bone graft for fusion. The other 28 cases with impossible reduction and anterior spinal cord compression were treated with anterior odontoid resection and decompression,and then posterior internal fixation and self cancellous bone graft for fusion.[Results]All of the operations were sucesseful without never and vascular injury and other serious complication. There were 47 cases following up 12 to 24 months in the 65 cases,and the mean follow up 15 6 months.One case's broken up titanium rod were treated with replacing new rod and bone graft,and the other cases showing bone fusion. There 28 cases following up in 34 cases with superior cervical pain and resticted movement,in which the pain was decreased or disappeared. There were 19 cases following up in 31 cases with numb limbs and superior never center injury. The preoperative Frankel score showed 5 cases B,8 cases C,and 17 cases D. The postoperative Frankel score of following up cases showd 5 cased C,6 cases D,and 8 cases E,and the score increased one or two degree.[Conclusion]The posterior,or anterior-posterior approach was selected by the condition of congenital superior cervical pathogeny,atlantoaxial reduction and spinal cord compression.

Treatment of concealed penis / 中华医学美学美容杂志

Objective To introduce the clinical manifestations and treatment methods of concealed penis, which is relative rare in plastic surgery. Methods Vertical preputiotomy was performed. The hypoplastic tissuely was excised. After that, there was no prepuce redundant, so that no prepuce excision was needed. Results 7 patients were treated. All were satisfied with the results. Conclusion Concealed penis should be distinguished from phimosis. Circumcision should be avoided in concealed penis patient.

Management of Anesthesia of Hirschsprung's Disease Radical Operation By Abdominoscope / 中国医师杂志

Objective To investigate the effect of the general anesthesia combined with peridural anesthesia on hirschsprung's radical operation through children abdominoscope. Methods 40 children patients with hirschsperung were elective eradicative operated by using endotrachal intubation complex vein general anesthesia combined with continuons peridural anesthesia.ECG,HR,MAP,SPO 2,P ET CO 2 were monitored, and blood gas were recorded and analyzed before pneumoperitoneum,15 minutes and 40 minutes after pneumoperitoneum,1 minute after deflation,before decannulation at the operation termination.Results Compared with before pneumoperitoneum date,HR inereased obviously 40 minutes after pneumoperitoneum,and before decannulation at the operation termination,peak pressure in airway increased obviously after pneumoperitoneum,the difference was notable,SPO 2 decreased after pneumoperitoneum,the difference was also significant.pH decreased gradually with the time prolongation,there was obviously difference.The trend of P ET CO 2 and PaCO 2 changes were analogous,P ET CO 2 had much notable difference after pneumoperitoneum,PaCO 2 had notable difference at the operation termination,and before decannulation,it was decreased to the basic value.Conlusions Hirschsprung's Disease eradical operation using general anesthesia combined with peridural anesthesia under abdominoscope guide,can immobilize ill children completely,relieve pain perfectly,muscular relaxation satisfactory,the physical interference to body is little and less complication.

Application of Spiral CT in Atlantoaxial Lesion / 实用放射学杂志

Objective To evaluate the diagnostic value of three-dimensional CT(3DCT)in atlantoaxial lesions.Methods The findings of 3DCT were analyzed and compared with conventional CT and radiography in 124 patients with atlantoaxial disease.Results 124 cases of atlantoaxial lesion included fracture in 42,atlantoaxial rotatory dislocation in 73,hypoplasia of the dens in 4,and congenital atlantoaxial deformity in 5.Among the 124 cases,89 cases were diagnosed by X-ray,109 cases by conventional CT,121 cases by MIP,122 cases by SSD,124 cases by MPR and VR.Conclusion 3DCT can find the slight rotatory dislocation,so it is the most effective means for the diagnosis of atlantoaxial rotatory dislocation.3D and MPR of spiral CT are useful in the diagnosis of atantoaxial fracture.Combining conventional CT and X-ray film with 3DCT can improve the acuracy of the diagnosis.

Epidemiological Survey on Congenital De-formities of Bornbabies In Pollution Area of Taiyuan Suburbs / 环境与健康杂志

In order to the further prove the rela tions between the environmental pollution and the congenital deformities. We selected two villages in the Taiyuan Suburbs for our investigations. Wu village used the industrial sewages for irrigations and Ho village used the rainfall. All of the babies born during 1985.1.1-1988.12.31 were investigation. In Wu village the frequency of deformities was 67.42%. and in Ho village the frequency of deformities was 20.07%. The X2 significance test was 30.56 and P