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)

Neurofibromatosis tipos 1 y 2 / Neurofibromatosis type 1 and 2

La neurofibromatosis (NF) comprende un grupo de enfermedades genéticas de herencia autosómica dominante, que se clasifican de la siguiente manera: neurofibromatosis tipo 1 (NF1), neurofibromatosis tipo 2 (NF2) y schwannomatosis (también conocida como neurofibromatosis tipo 3). Esta última es una enfermedad muy infrecuente, con una prevalencia aproximada de 1/126 000 personas, por lo que solo profundizaremos las dos primeras. La NF1, también conocida como la enfermedad de Von Recklinghausen, es la más frecuente de las tres y afecta principalmente la piel y el sistema nervioso periférico. Se caracteriza por la presencia de máculas "café con leche", pecas axilares o inguinales, nódulos de Lisch (hamartomas en el iris) y neurofibromas (tumores de la vaina de nervios periféricos). Otras manifestaciones menos frecuentes, aunque de mayor gravedad, incluyen gliomas del nervio óptico, meningiomas, neurofibromas malignos, escoliosis y displasia de la tibia. Su diagnóstico se suele realizar al nacimiento o durante los primeros años de vida, y se estima que un 50% de quienes la padecen presenta dificultades cognitivas. No hay datos concluyentes sobre la mortalidad en los pacientes con NF1, aunque se sabe que la expectativa de vida es menor que en la población general. La NF2 tiene una prevalencia considerablemente menor que la NF1 y su inicio es más tardío, afectando principalmente a adultos jóvenes. La presentación clínica típica se caracteriza por acúfenos, hipoacusia y ataxia en contexto de la presencia de schwannomas vestibulares bilaterales. Otros hallazgos menos frecuentes incluyen schwannomas de nervios periféricos, meningiomas, ependimomas o astrocitomas. La esperanza de vida es de unos 36 años, con una supervivencia media desde el momento del diagnóstico de 15 años. (AU)

Neurofibromatosis (NF) includes a group of genetic diseases with an autosomal-dominant inheritance pattern, and they are classified as follows: Neurofibromatosis type 1 (NF1), neurofibromatosis type 2 (NF2) and Schwannomatosis (also known as neurofibromatosis type 3). This last one is a very rare disease, with an approximate prevalence of 1/126000, so we will only deepen in the first two. NF1, also known as von Recklinghausen disease, is the most frequent, and mainly affects the skin and peripheral nervous system. Its typical manifestations are the presence of café-au-lait macules, axillary or inguinal freckles, Lisch nodules (hamartomas in the iris) and neurofibromas (peripheral nerve sheath tumors). Less frequent manifestations, although more serious, include optic nerve gliomas, meningiomas, malignant neurofibromas, scoliosis and tibial dysplasia. The diagnosis is usually made at birth or during the first years of life, and approximately 50% of patients present cognitive difficulties. There is no conclusive data on mortality in patients with NF1, although it is known that life expectancy is lower than in general population. NF2 has a considerably lower prevalence than NF1, and its onset is later in life, mainly affecting young adults. Its typical clinical presentation is characterized by tinnitus, hearing loss and ataxia in the context in the presence of bilateral vestibular schwannomas. Less frequent findings include peripheral nerve schwannomas, meningiomas, ependymomas or astrocytomas. Life expectancy is about 36 years old, with a median survival from the moment of diagnosis of 15 years. (AU)

Reporte de caso de síndrome FATCO: aplasia fibular, campomelia de tibia y oligosindactilia / A case report of a patient with FATCO syndrome: fibular aplasia, tibial campomelia and oligosyndactyly

El síndrome FATCO, por las siglas en inglés de aplasia fibular (Fibular Aplasia), campomelia de tibia (Tibial Campomelia) y oligosindactilia (Oligosyndactyly), es un conjunto de malformaciones óseas, en las que la alteración se centra en los huesos de la pierna. Es una entidad rara y hay pocos casos descritos en la Literatura internacional, y, hasta ahora, no se ha descrito ningún caso en Colombia. Presentamos aquí el caso de un recién nacido de sexo masculino con signos clínicos de manera prenatal y posnatal consistentes con síndrome FATCO sin otras malformaciones asociadas. Exponemos también una breve discusión sobre las diferentes malformaciones óseas y otros casos de FATCO en el mundo.

The FATCO syndrome, (Fibular Aplasia, Tibial Campomelia and Oligosyndactyly) are bone malformations with main alteration in lower limbs. It is a rare entity and there are few cases reported in international literature, and so far there are not published cases in Colombia. Here we present a case of a male newborn with prenatal and postnatal signs consistent with FATCO syndrome without other organs malformations, and there is a brief discussion about this syndrome and other different malformations associated with it.

Tratamento da tíbia vara de blount com órteses moldadas: uma nova perspectiva / Management of infantile blount’s disease with molded orthoses: a new perspective

Objetivo: Este estudo retrospectivo visa avaliar o tratamento com órteses moldadas individualmente em estágios precoces da doença.Métodos: Foram avaliados os prontuários de pacientes submetidos a tratamento da tíbia vara de Blount com órteses moldadas para descompressão medial entre 2010 e 2014. O ângulo de deformidade(ângulo meta-diafisário de Drennan) e a classificação Langenskiõldforam aferidos antes e após o tratamento por médico ortopedista especialista com mais de 5 anos de prática, no modo cego em relação a o estudo e aos pacientes. Resultados: A média de idade dos pacientes foi de 2,57 anos. Quatro eram do sexo feminino e seis do sexo masculino. Metade da amostra apresentava doença bilateral.O ângulo de deformidade dos pacientes apresentou redução média estatisticamente significativa após o tratamento (p < 0,001). Sexo e lateralidade não influenciaram estatisticamente na alteração do ângulo de deformidade após o tratamento (p > 0,05). Conclusão:O uso noturno de órteses moldadas para descompressão medial foi efetivo para redução do ângulo meta-diafisário na Doença de Blountem pacientes menores de 3 anos de idade, independente do sexo e bi lateralidade da doença. Pacientes com mais de 3 anos não se beneficiaram do uso da órtese. Nível de EvidênciaIV, Serie de Casos

Objective: This retrospective study evaluated treatment with individuallycontoured molded bracing at early stages of the disease.Methods: We evaluated the medical records of patients undergoingtreatment of Blount’s disease with molded orthoses formedial decompression between 2010 and 2014. The deformityangle (Drennan’s metaphyseal-diaphyseal angle) and Langenskiõldclassification were measured before and after treatment by a pediatricorthopedic surgeon with over 5 years of practice, blinded forthe study and patients. Results: The mean age was 2.57 years old.Four patients were female and six male. Half of the total sample hadbilateral disease. The average deformity angle showed a statisticallysignificant reduction after treatment (p <0.001). Gender and lateralitydid not statistically influence the change of the deformity angleafter treatment (p> 0.05). Conclusion: The nightly use of moldedorthoses for medial decompression was effective in reducing themetaphyseal-diaphyseal angle in Blount’s disease in children under3 years of age, regardless of gender and bilateral disease. Patientsover 3 years old did not benefit from bracing. Level of EvidenceIV, Case Series.

Dysplasia epiphysealis hemimelica with bony and soft-tissue abnormalities: a case report and review of the literature

We report a case of a 3-year-old boy with dysplasia epiphysealis hemimelica [DEH] who presented with a hard painless swelling on the medial side of his left ankle joint. Plain X-rays, CT and MRI showed a bony tumor and associated soft-tissue abnormalities. At age one year, he had been diagnosed with an osteochondroma and underwent incomplete surgical excision at a local hospital. Due to joint impingement and restricted movement, we chose to completely excise the tumor. Pathologically, the specimen showed evidence of an osteochondroma. From its clinical, imaging, and pathological features and reports in the medical literature, a diagnosis of DEH was established. DEH is a rare variant of osteochondroma, resulting in many patients being incorrectly diagnosed or diagnosed with osteochondroma alone. It is important to low-grade malignant tumor or osteochondroma-like parosteal osteosarcoma from DEH of the talus, especially when accompanied by soft tissue abnormalities. Surgical treatment is mandatory in patients with symptoms such as pain, joint impingement and deformation. Incomplete excision of an articular lesion may lead to recurrence and additional surgery, making it less than complete excision

Diagnóstico ecográfico prenatal de sirenomelia: a propósito de un caso / Prenatal ultrasound diagnosis of Sirenomelia: about a case

RESUMEN La sirenomelia constituye una anomalía congénita rara. Su incidencia es de 1:60.000 nacidos vivos. Es causada por un defecto vascular disruptivo y caracterizado por la fusión de las extremidades inferiores, asociados a anomalías cardiacas, renales, de pared abdominal y torácica, vértebras inferiores, tubo digestivo inferiores, genitales y de las arterias umbilicales. Se presenta la experiencia de un caso de sirenomelia que se ha podido diagnosticar con la ecografia prenatal.

ABSTRACT Sirenomelia is a very rare congenic anomaly. Being its incidence of approximately 1:60.000 live born, caused by a vascular disruptive defect, characterized by the fusion of lower limbs, associated to cardiac, renal, abdominal wall, and thoracic anomalies; also characterized by inferior vertebrae, inferior digestive tract, genital and umbilical artery anomalies A case of sirenomelia has been diagnosed thanks to prenatal echography.

Análisis de las deformaciones de la tibia humana a consecuencia del genu varum / Analysis of the deformations of the human tibia as a result of genu varum

El Genu Varum (piernas arqueadas), corresponde a una alteración en el eje axial que presentan todos los niños durante su desarrollo. Desde el punto de vista de la Ingeniería de los materiales, el genu varum se lo considera como un desplazamiento de la articulación en sentido externo al eje de presión Esta investigación, mediante un riguroso análisis matemático de la geometría estructural de la tibia y la función física de sus elementos, plantea la determinación de los puntos críticos de flexionamiento. Un diagrama de esfuerzos con sus respectivas coordenadas, el tipo de esfuerzo que sufre y las fuerzas que deberían aplicarse para un alineamiento sin proceder con un corte de la misma. Este estudio también plantea una metodología de análisis mediante un diagrama vectorial de los vectores fuerza que actúan en los tendones externos (peroneo) e interno como así el vector de reacción de la tibia deformada a consecuencia del genu varum. Con este análisis se plantea una explicación del por qué el genu varum se detiene a causa de la reacción compensativa de la tibia ante el desplazamiento de la articulación. Para todo lo anteriormente planteado se considera la técnica de Ilizarov para la aplicación de este método de alineamiento.

A case of bilateral tibial hemimelia type VIIa.

Congenital absence of tibia is a rare anomaly, and may be total or partial, unilateral or bilateral. Total absence is more frequent than partial, unilateral absence occurs more often than bilateral, with right limb more commonly affected than the left. In partial defect, almost always the distal end of the bone is affected, and of the bilateral cases, there may be total absence on both sides, or total on one side and partial on the other. Males are slightly more commonly affected than the females. Though, the family history is usually negative for congenital abnormalities and other diseases, there is a considerable chance of occurrence of congenital defect of the tibia or of other abnormalities, in near or remote relatives. We report a case of newborn having bilateral tibial hemimelia type VIIa.

Bilateral tibial hemimelia type 1 (1a and 1b) with T9 and T10 hemivertebrae: a novel association / Bilateral tibial hemimelia tipo 1 (1a e 1b) com hemivertebras T9 e T10: uma nova associacao

CONTEXT Congenital absence of the tibia is a rare anomaly with an incidence of one per 1,000,000 live births. It is mostly sporadic and can be identified as an isolated disorder or as part of malformation syndromes. CASE REPORT A male child, born to unaffected and non-consanguineous parents, presented with shortening of the legs and adduction of both feet. Physical examination at six months of age showed head circumference of 44.5 cm (75th percentile), length 60 cm (< 3rd percentile), weight 7,700 g (50th percentile), shortening of the left thigh and both legs with varus foot. There were no craniofacial dysmorphisms or chest, abdominal, genital or upper-extremity anomalies. Psychomotor development was normal. His workup, including renal and cranial ultrasonography, brainstem auditory evoked potential, and ophthalmological and cardiological examinations, was normal. X-rays showed bilateral absence of the tibia with intact fibulae, distally hypoplastic left femur, and normal right femur. In addition, spinal radiographs showed hemivertebrae at T9 and T10. CONCLUSION This novel association expands the spectrum of tibial hemimelia. Moreover, this observation highlights the usefulness of this inexpensive diagnostic method (X-rays) for characterizing the great clinical and radiological variability of tibial hemimelia. .

CONTEXTO Ausência congênita da tíbia é uma anomalia rara, com incidência em 1 por 1.000.000 de nascidos vivos, é principalmente esporádica e pode ser identificada como um distúrbio isolado ou como parte de síndromes de malformações. RELATO DO CASO Criança do sexo masculino, nascida de pais não afetados e não consanguíneos, apresentou-se com encurtamento das pernas e adução de ambos os pés. O exame físico realizado com seis meses de idade mostrou perímetro cefálico 44,5 cm (percentil 75), comprimento de 60 cm (percentil < 3), peso 7.700 g (percentil 50), encurtamento da coxa esquerda e as duas pernas com o pé varo bilateralhavia. Não houve dismorfismos craniofaciais, nem tórax, abdômen, genitais e anomalias das extremidades superiores. O desenvolvimento psicomotor foi normal. Os exames, incluindo ultrassonografia renal e da cabeça, potenciais auditivos evocados de tronco cerebral e exames oftalmológicos e cardiológicos, estavam normais. Raios-X revelou ausência bilateral da tíbia com fíbula intacta, hipoplasia distal do fêmur esquerdo e fêmur direito normal. Além disso, as radiografias de coluna mostraram hemivértebras em T9 e T10. CONCLUSÃO Esta associação nova expande o espectro de hemimelia tibial. Além disso, esta observação destaca a utilidade de tal método diagnóstico barato (raios-X), caracterizando a grande variabilidade clínica e radiológica de hemimelia tibial. .

Tomografia computadorizada da matriz óssea mineralizada heteróloga fragmentada e metilmetacrilato na reparação de falhas ósseas segmentares produzidas em tíbia de coelhos / Computed tomography to evaluate the association of fragmented heterolog cortical bone and methylmethacrylate to repare segmental bone defect produced in tibia of rabbits

Foi realizada falha segmentar de 6mm na região metafisária medial da tíbia de 12 coelhos, seguida de preenchimento desta por matriz óssea mineralizada heteróloga fragmentada conservada em glicerina (98%) e metilmetacrilato autoclavado, bem como avaliação por meio da tomografia computadorizada de feixe cônico (cone beam) aos 30, 60 e 90 dias. Houve incorporação gradativa do implante no leito receptor em relação ao tempo em 100% dos casos, o que mostra ser este biologicamente compatível, ao promover reparação da falha óssea, sem sinais de infecção, migração e/ou rejeição, caracterizando-se, assim, como nova opção de substituto ósseo para preenchimento de falhas ósseas.

A 6mm segmental defect was performed on the metaphyseal region of the tibia of 12 rabbits and the autoclaved fragmented heterolog cortical bone conserved in glycerin (98%) and methylmethacrylate was used as a bone graft for the reconstruction. The graft was placed in the receptor bed and its integration was evaluated by computed tomography after 30, 60 and 90 days. There was gradual bone graft incorporation in the receptor bed during the time in 100% of the cases. Fragmented cortical bone heterograft and methylmethacrylate was biologically compatible and promotes bone defect reparation without signs of infection, migration and or rejection, featuring a new option of osseous substitute to fill in bone defects.

Limb Angular Deformity Correction Using Dyna-ATC: Surgical Technique, Calculation Method, and Clinical Outcome

PURPOSE: Dyna-ATC is a unilateral external fixator with angulator, lengthener, and translator, which allows for angular correction and compensation of the secondary displacement during angular correction. The purpose of this study is to introduce surgical technique and calculation methods and to evaluate the clinical outcome of angular deformity correction using Dyna-ATC. MATERIALS AND METHODS: The amounts of secondary displacement were calculated with the distances between axis of correction of angulation, Center of Rotational Angulation, and osteotomy and the amount of angular deformity. The rate of angular correction was determined to distract the corticotomy at 1 mm/day. Clinical and radiographic evaluation was performed on 13 patients who underwent deformity correction using Dyna-ATC. There were 8 proximal tibia vara, 1 tibia valga, 2 varus and 4 valgus deformities on distal femur. One patient underwent pelvic support femoral reconstruction. Concomitant lengthening was combined in all femur cases. Mean age at surgery was 17.5 years (7 to 64). RESULTS: All but one achieved bony healing and normal alignment with the index procedure. Mean mechanical axis deviation improved from 31.9 mm to 3.0 mm. The average amount of angular correction was 11.0degrees on tibiae and 10.0degrees on femora. The average length gain on femora was 6.4 cm, and the healing index averaged to 1.1 mo/cm. One patient underwent quadricepsplasty and one patient had three augmentation surgeries due to poor new bone formation. CONCLUSION: We believe that Dyna-ATC is a useful alternative to bulky ring fixators for selective patients with angular deformity less than 30 degrees in the coronal plane around the knee joint.

Expressão da caderina na discondroplasia tibial / Cadherin expression in the tibial dyschondroplasia

By immunohistochemistry the expression of a pan-cadherin antibody that recognizes a wide variety of cadherins in chondrocytes from normal and tibial dyschondroplasia (TD) growth plates was compared. Surprisingly, an upregulated expression that was not expected in TD lesion chondrocytes was observed. The reason for this apparent upregulation is not clear. The increased expression may simply be due to the particular phenotype of lesion chondrocytes, and cadherin may be involved in apoptosis of chondrocytes of TD lesion. Another possibility, is that a low level of calcium in the lesion may be responsible for the observed upregulation. The results of the present study suggest that the formation of the dyschondroplastic lesion is not merely due to the impaired terminal differentiation of lesion chondrocytes and that other mechanisms are probably involved in TD etiology. Further studies will be necessary to provide insight into the precise nature of the condition.

Pyle metaphyseal dysplasia.

Pyle type metaphyseal dysplasia is a rare autosomal recessive disease that primarily affects metaphyses. We report a 12 year old boy with Pyles disease. He had mild facial dysmorphism, genu valgum and wasting of legs. Skeletal radiology revealed the characteristic Erlenmeyer flask sign at distal femoral and proximal tibial metaphyses along with platyspondyly.

[Campomelic dysplasia a case report]

Campomelic dysplasia is a skeletal disease, first reported by Bound. The clinical feature associates stature deficiency bowing of tibia associated with agenesis of toes. Diagnosis is based on radiological features showing bowed tibia, fibula aplasia with skeletal dysplasia, nanow chest. We report one case confirmed radiologically

Congenital osteofibrous dysplasia of the tibia, associated with pseudoarthrosis of the ipsilateral fibula

We describe an otherwise normal male neonate who presented shortly after birth with rare congenital osteofibrous dysplasia of the right tibia associated with pseudoarthrosis of the ipsilateral fibula. The lesion was curetted, and the defect was packed with a fibular bone graft from the other leg. Histopathological examination was typical for osteofibrous dysplasia. The ipsilateral fibular pseudoarthrosis was observed with no active intervention. Seven years follow-up showed good functional recovery without recurrence of the lesion. The case is a new presentation of congenital osteofibrous dysplasia, and is presented to draw attention to this rare condition that must be considered in the differential diagnosis of congenital lesions of the tibia

[ clinical use of combined cancellous bone graft and bone marrow on the induced tibial bone defect in dogs]

To evaluate the induction of osteogenesis of combined autogenous cancellous bone graft and bone marrow in experimental midshaft tibial bone defect. Experimental study. Seven adult mixed-breed dogs with an average age of 1.57 years and an average weight of 22.57 kg. the animals were randomly divided in to experimental [n=5] and control [n=2] groups. In each dog a bone defect [2.5 cm] was created on the midshaft of left tibia and fixed by plate and screws. In the experimental group, tibial bone defect was filled with autogenous cancellous bone and bone marrowo. These matter were harvested from the iliac crest and the medullary canal of the femur, respectively. In the control group, the defect was not filled. Postoperative clinical assessment including measurement of body temperature, heart rate, respiratory rate and evaluation of the degree of lameness was done. Moreover, radiographic assessment of bone based on the process of bone healing periosteal reaction and soft tissue swelling were done before surgery, immediately after surgery and then 1,2,3,5,9,13 and 17 weeks after surgery. The degree of lameness in experimental group was lesser than the control ones from the day 12 to 49 [p<0.05]. Significant difference was observed in the degree of soft tissue swelling between two groups at the week 5 [p<0.05]. Moreover, difference of the periosteal reaction was not significant between two groups. The degree of bone healing process was significantly greater in the experimental group compare to control [p<0.05]. While clinical union was seen in the experimental group up to 100% [n=4] and 75% [n=1], it was not seen in the control group. The result of this study showed that the autogenous cancellous bone and bone marrow can be a reliable method for filling of 2.5 cm segmental tibial bone defect in dog. In this regard, healing of the higher size of bone defect needs to be further investigated

Early proximal tibial valgus osteotomy as a very important prognostic factor in Thai children with infantile tibia vara.

OBJECTIVES: To find the effectiveness of the early surgery (2-3 years of age)as a very important prognostic factor affecting the outcomes in Thai children with infantile tibia vara and all the prognostic factors including the usefulness of arthrographic study in correcting the deformity. MATERIAL AND METHOD: From 1994 to 2004, sixteen children aged average 3.61 years old (2.08-7.0) were treated in Siriraj Hospital and diagnosed as infantile tibia vara by Langenskiold radiographic staging were included in the present study and retrospectively reviewed with an average of 6.4 years follow up (range 6 month - 11.1 years). All cases were initially treated by surgery because of low compliance for brace or brace failure. They consisted of 3 boys and 13 girls. There were 24 legs including the bilateral involvement in 8 cases (2 boy and 6 girls). After arihrography, the midshaft fibular osteotomy was performed then the proximal tibial dome-shaped valgus osteotomy was done and fixed with 2 pins.The desired position was 12 degree knee valgus . The patients were divided in two groups, 1)group A,the successful group with the knee becoming normal without any deformity after single osteotomy, 2)group B,the recurrent group with recurrence of the varus deformity required further corrective osteotomies to make normal axis of the knee. All variables were analyzed and compared between group A and group B. The general characteristics and radiographic findings were recorded in 1)age, 2)sex, 3)side, 4)weight in kilogram and in percentage of normal or overweight(obesity) compared with the standard Thai weight chart, 5)tibiofemoral angle (TFA) pre and postoperative treatment, 6) metaphyseal diaphyseal angle (MDA), 7)the medial physeal slope angle (MPS, 8)The preoperative arthrographic articulo-diaphyseal angle (ADA), 9.arthrographic articulo-medial physeal angle (AMPA). RESULTS: There were 14 legs in group A and the remaining 10 legs were in group B (average 2.4 operations). All cases healed in good alignment of the legs without major complication. All patients who were operated on early before 3 years old were 100% cured by single osteotomy in group A(11 legs).Arthrography was useful in evaluating the knee joint and drawing the angle. Considering the prognostic factors affecting the outcomes after surgery, there were 6 prognostic factors . First, the age less than 3 years old (P<0.001). Second, the normal weight (P<0.047). Third, the Langenskiold stage 1-2 (P=0.002). Fourth, the MPS angle equal or less than 59 degree (P < 0.001). Fifth, the ADA preperative angle equal or less than 18 degrees (P<0.001). Sixth and the last factor, the TFA angle postoperative treatment, equal or more than 10 degrees valgus (mean 13 degrees valgus) (P=0.009).In multivariate analysis with stepwise logistic regression of these 6 prosnostic factors, the MPS angle had the most important significance. The proximal tibial valgus osteotomy was a very important factor(P < 0.001). CONCLUSION: The 6 prognostic factors and usefulness of arthrography were identified. The authors suggest that surgery should be performed early in Thai children who have met these criterias 1)age of the patients more than 2 years old, 2)Langenskiold roentgenographic characteristics of infantile tibia vara stage 2 or more at the time of diagnosis, 3)Low compliance for brace treatment.or brace failure but not more than 3 years old. The surgery should not be delayed more than 3 years of age by waiting for effectiveness of brace treatment in Thai children with infantile tibia vara. The early proximal valgus dome- shaped osteotomy was a very important controllable prognostic factor by surgeon decision.

Bilateral congenital split hand with tibial aplasia.

The authors report on a child with bilateral typical split-hand (ectrodactyly) and tibial aplasia, probably the first Indian report. Minor limb malformations in the maternal side suggests an autosomal dominant inheritance. The clinical spectrum and the inheritance of this malformation are discussed. Prenatal screening in families with such malformations is essential.