Interacción de Tratamiento Ortodóncico, Fonoaudiológico y Kinesiológico. Reporte de caso

Se describe el caso clínico de una paciente de 8 años de edad, sexo femenino que presenta respiración oral, anomalía dentomaxilar, interposición lingual en deglución, fonación, lengua descendida en reposo y adaptaciones musculoesqueléticas por lo que requiere tratamiento de Ortodoncia, Fonoaudiología y Kinesiología. El presente reporte de caso describe la interacción de las especialidades requeridas para el manejo adecuado de la paciente y expone un flujograma sugerido para abordar casos similares.

The following is a clinical case of an eight-year-old female patient who presents oral respiration, dentomaxilar anomaly, tongue interposition during swallowing and phonation, lowered tongue at rest and musculoskeletal adaptations, hence requires orthodontics, speech and physical therapy. This case report describes the interaction of the specialties necessary for the proper management of the patient and presents a suggested flow chart to address similar cases.

Displasia dentinaria tipo I: Manejo clínico y estudio histológico a propósito de dos casos / Dentin dysplasia type I: clinical management and histological study based on two cases

La displasia dentinaria tipo I (DD-I) corresponde a una alteración dentinaria de heterogeneidad genética y penetrancia completa, en donde se presenta un defecto en el desarrollo de las raíces de los dientes tanto temporales como definitivos. Clínicamente se observan dientes con extrema movilidad junto con antecedentes de exfoliación prematura o espontánea. Los defectos estructurales de los tejidos dentarios, tales como DD-I; implican un desafío ya que son pocos los casos documentados en la literatura que hablan de esta condición. Además implican un tratamiento multidisciplinario y altamente invasivo. El objetivo de este artículo es presentar dos casos de DD-I, enfatizando en su tratamiento y características histopatológicas.

Dentin Dysplasia Type I (DD-I) consists of a pathological dentinary alteration with genetic heterogeneity that results in a defectuous development of dental roots both in primary and secondary dentition. Clinically we can appreciate teeth with extreme pathological mobility and premature or spontaneous exfoliation. Alterations within normal dental structure, such as DD-I imply a challenge for the common practitioner, because of the scarce number of case reports with in the scientific literature regarding this condition and also, because of the need for a highly invasive and multidisciplinary approach they require. The aim of this article is to present two DD-I cases, emphasizing on their treatment and histopathological features.

Anomalía de Ebstein: a propósito de un caso / Ebstein anomaly: about a case

La anomalía de Ebstein es una cardiopatía congénita rara y poco frecuente caracterizada por el adosamiento de los velos valvulares tricuspídeos; en la etapa prenatal se estima que su incidencia corresponde a un 3% de todas las cardiopatías diagnosticadas. Se presenta el caso de un feto con diagnóstico de anomalía de Ebstein a quien se le realizó un diagnóstico prenatal adecuado, lo que permitió planificar el nacimiento neonatal con un equipo multidisciplinario integral. Debido a la rareza del diagnóstico prenatal de esta entidad, se describe el caso clínico y los hallazgos imagenológicos representativos.

Ebsteins anomaly is a rare and infrequent congenital heart disease characterized by the attachment of the tricuspid valve leaflets; in the prenatal stage it is estimated that its incidence corresponds to 3% of all diagnosed heart diseases. We present the case of a fetus diagnosed with Ebsteins anomaly who underwent an adequate prenatal diagnosis, which made it possible to plan the neonatal birth with a comprehensive multidisciplinary team. Due to the rarity of the prenatal diagnosis of this entity, the clinical case and the representative imaging findings are described.

Beak deformity in three bird species from the southwest of the Brazilian Amazon / Deformidad del pico en tres especies de aves del sudoeste de la Amazonia brasileña

Abstract Beak deformity have a frequency of 0.5% in wild bird populations. In addition to being rare, beak deformities are also poorly reported in the Brazilian scientific literature. Here we report beak deformities in the species: Dendrocincla merula, Amazona ochrocephala and Pheugopedius genibarbis, all of which occurred in southwestern Brazilian Amazon. Dendrocolaptids make intensive use of their beaks in the search for insects and small vertebrates, where they explore in cracks in wood with lateral blows. In the case presented here, this behaviour may have been the cause of the breakage of the tip of this individual's maxilla. In Brazilian territory, few species of parrots were recorded with deformity in the beak and in the individual in this work, everything indicates that Amazona ochrocephala was a captive animal, as it was excessively thin and its diet probably had a low content of vitamins and calcium, where their deficiency even when the animal was a puppy may have contributed to the deformity of the maxilla. Cases of beak deformities in species of the Troglodytidae family are rare, but the individual in this work presented an unusual curvature in the maxilla not observed in other individuals in museum collections. Only with more reports will we be able to better understand the occurrence and causes of these beak deformities in wild birds.

Resumen La deformidad del pico es una característica rara y tiene una frecuencia baja, con 0.5% de casos en poblaciones de aves silvestres. Además de ser raras, las deformidades del pico también son escasamente reportadas en la literatura científica brasileña. Reportamos aquí deformidades en los picos de aves de las especies: Dendrocincla merula, Amazona ochrocephala y Pheugopedius genibarbis, en los cuales todos los registros ocurrieron en el sudoeste de la Amazonia brasileña. Los dendrocoláptidos hacen uso intensivo del pico en la búsqueda de insectos y pequeños vertebrados, explorando en grietas de la madera con golpes laterales. En el caso aquí presentado, este comportamiento de exploración pudo haber sido la causa de la rotura de la punta del maxilar de este individuo. En el territorio brasileño, pocas especies de loros fueron registradas con deformidad en el pico y en el individuo de este trabajo, todo indica que Amazona ochrocephala era un animal de cautiverio, ya que estaba excesivamente delgado y su dieta probablemente tenía un bajo contenido de vitaminas y calcio, donde su deficiencia incluso cuando el animal era un joven puede haber contribuido a la deformidad del maxilar. Los casos de deformidades del pico en especies de la familia Troglodytidae son raros, pero el individuo de este trabajo presentaba una curvatura inusual en el maxilar no observada en otros individuos de esta especie depositados en la colección científica. Sólo con más informes podremos entender mejor la ocurrencia y las causas de estas deformidades del pico en aves silvestres.

Exodontia de supranumerário paramolar e de primeiro molar com destruição coronária em região de maxila: relato de caso / Extraction of a supernumerary paramolar and first molar with coronary destruction in the maxilla area: case report

Os dentes supranumerários são descritos como uma espécie de anomalia dentária, determinada pela formação de dentes além daqueles pertencentes à dentição convencional, que geralmente promovem complicações na cavidade oral. Diversos fatores etiológicos podem estar relacionados e de acordo com sua localização, são classificados em mesiodens, distomolar e paramolar. O presente estudo tem como objetivo apresentar e descrever a exodontia de um elemento supranumerário paramolar em região de maxila, bem como a remoção cirúrgica do primeiro molar com destruição coronária em região subjacente, através de um relato de caso. Paciente E.M.J., sexo feminino, 38 anos de idade, ASA 1, procurou atendimento odontológico devido à queixa principal de dor em região do 1° molar superior esquerdo. Ao exame físico e radiográfico, foi observada grande destruição coronária do elemento 26 associado a uma fístula intraoral, além da presença de dente supranumerário paramolar na região vestibular do dente 26. O tratamento preconizado foi a remoção cirúrgica de ambos elementos e a paciente evoluiu sem intercorrências no pós-operatório. Desta maneira, pode-se concluir que a remoção cirúrgica de dente supranumerário paramolar e de unidade dentária com grande destruição coronária por lesão cariosa, mostrou-se uma alternativa segura e eficaz no presente caso.

Supernumerary teeth are described as a kind of dental anomaly, determined by the teeth´s formation other than those belonging to the conventional dentition, which generally promote complications in the oral cavity. Several etiological factors may be related and, according to their location, they are classified into mesiodens, distomolar and paramolar. This study aims to present and describe the extraction of a paramolar supernumerary element in the maxillary region, as well as the surgical removal of the first molar with coronary destruction in the underlying region, through a case report. Patient E.M.J., female, 38 years old, ASA 1, sought dental care due to the main complaint of pain in the region of the upper left 1st molar. At the clinical and radiographic examination, a great coronal destruction of element 26 associated with a intraoral fistula was observed, in addition to the presence of a supernumerary paramolar tooth on the buccal side of tooth 26. The recommended treatment was the surgical removal of both elements and the patient evolved uneventfully in the post-operative. Thus, it can be concluded that the surgical removal of a supernumerary paramolar tooth and a dental unit with great coronary destruction due to a carious lesion proved to be a safe and effective alternative in the present case.

Non-bifurcating cervical carotid artery:a case report and literature review / 中国脑血管病杂志

Non-bifurcating cervical carotid artery(NBCCA)is a very rare anatomical variation of the cervical carotid artery,which may be related to the abnormal development of internal carotid artery(ICA)and external carotid artery in embryonic period.Neither carotid bulb nor a true carotid bifurcation can be observed on the ultrasound of carotid artery while a"stump-like"change was showed at the expected bifurcation level of carotid artery on DSA.Few cases has been reported in China and abroad so far.This article reported a middle-aged male with a history of hypertension and type 2 diabetes and was admitted to the hospital due to dizziness for one month.The left NBCCA accompanied with severe stenosis of the right ICA was confirmed by cerebral angiography.The patient received the right ICA stent implantation surgery as well as antihypertensive and glucose-control treatment and was discharged as his symptoms improved.Clinical data of this case and related literatures were reviewed,in order to improve clinicians'especially imaging diagnostic physicians'understanding on NBCCA to avoid misdiagnosis and related complications.

Right heart function parameters in patients with Ebstein anomaly:Correlations of evaluation of echocardiography and MRI / 中国医学影像技术

Objective To explore the correlations of evaluations of right heart function parameters in patients with Ebstein anomaly(EA)using echocardiography and cardiac MRI.Methods Data of transthoracic echocardiography and cardiac MRI in 32 patients with EA confirmed by operation were retrospectively analyzed.The correlations of cardiac cavity size,right ventricular function and strain parameters obtained using echocardiography and the functional right ventricular(fRV)ejection fraction(EF)measured using MRI were explored.Results MRI fRV-EF in 32 cases of EA was(23.20± 7.61)％.Among echocardiographic parameters in 32 cases of EA,fractional area change(FAC)of fRV(r=0.347,P=0.015)was slightly,while global longitudinal strain(GLS)of fRV(r=0.801,P＜0.001)was highly positively correlated with MRI fRV-EF,respectively,whereas atrialized right ventricle(aRV)area/fRV area(r=-0.730,P=0.007)was highly negatively,aRV area/left ventricular area(r=-0.450,P=0.042)and right ventricular anterior-posterior diameter(r=-0.650,P=0.022)were both moderately negatively correlated with MRI fRV-EF.Both the left ventricular eccentricity index(r=-0.347,P=0.049)and Glasgow outcome scale extended(r=-0.336,P=0.024)obtained with echocardiography were slightly negatively correlated MRI fRV-EF.Conclusion Right heart function parameters in EA patients obtained with echocardiography were correlated with those of MRI fRV-GLS,among which aRV area/fRV area were highly positively correlated with MRI fRV-EF,hence having great value for evaluating right heart function in EA patients.

Mid-to-long term fate of neo-aortic root after arterial switch operation for Taussig-Bing anomaly: A retrospective study in a single center / 中国胸心血管外科临床杂志

@#Objective To explore growth pattern of neo-aortic root as well as development of neo-aortic regurgitation after arterial switch operation (ASO) for Taussig-Bing anomaly. Methods From 2002 to 2017, the patients who received ASO, and were discharged alive from Shanghai Children’s Medical Center and followed up for more than 3 years were retrospectively involved in this study. Results A total of 127 patients were enrolled. There were 98 (77.2%) males, the median age at ASO was 73.0 d and the average weight was 4.7 kg. Forty-five (35.4%) children were complicated with mild or mild-to-moderate pulmonary insufficiency (PI) before ASO. The average follow-up time was 7.0 years. During the follow-up, 14 (11.0%) children presented moderate or greater neo-aortic regurgitation (neo-AR). The diameter of neo-aortic annulus and sinus of Valsalva was beyond normal range during the entire follow-up. The average diameter of neo-aortic annulus was 18.0 mm at 5 years and 20.5 mm at 10 years. The average diameter of sinus of Valsalva was 25.9 mm at 5 years and 31.1 mm at 10 years. Neo-AR continued to develop over time. The diameter of children who developed moderate or greater neo-AR was constantly larger than that of children who did not (χ2=18.3, P<0.001). Preoperative mild or mild-to-moderate PI was an independent risk factor for the development of moderate or greater neo-AR during mid-to-long term follow-up (c-HR=3.46, P=0.03). Conclusion The diameters of neo-aortic annulus and sinus of Valsalva of Taussig-Bing children who receive ASO repair continue to expand without normalization. The dilation of annulus correlates with the development of neo-AR. PI before ASO repair increases the risk of neo-AR development.

Progress of PBX1 gene abnormalities causing congenital renal anomaly / 国际儿科学杂志

Congenital anomalies of the kidneys and urinary tracts（CAKUT）include a wide range of structural malformations resulting from defects in the morphogenesis of the kidney and the urinary tract. Congenital renal anomaly is common in CAKUT. The pathogenesis of congenital renal anomaly is considered to be multi-factor，involving maternal or external environment，and heredity. With the continuous progress of molecular diagnosis technology，genetic factors have attracted more and more attention. The PBX1 gene was initially discovered by the formation of an E2A-PBX1 fusion gene from a t（1；19）（q23；p13.3）chromosome translocation，which results in pre-B-cell lymphoblastic leukemia.PBX1 gene mutation can cause congenital renal and urogenital malformation syndromes with or without hearing loss，ear abnormalities，and developmental delay. This review deepens the understanding of the role of genes in regulating kidney development by describing the embryonic basis of kidney development，the structure and function of the PBX1 gene，and the pathogenesis of renal anomalies caused by mutations. Further，it summarizes the phenotype and genotype of the PBX1 gene，in order to promote the diagnosis，treatment，and determination progression of congenital renal anomaly.

Analysis of Surgical Treatment of 20 Cases of Ebstein's Anomaly Combined with Atrial Septal Defect / 昆明医科大学学报

Objective To analyze the treatment strategy of the atrial septal defect in the surgical treatment of Ebstein's anomaly combined with the atrial septal defect and the short-term follow-up results of the treatment of Ebstein's anomaly.Methods A retrospective analysis of the clinical data and follow-up results of 20 patients with Ebstein's anomaly and atrial septal defect was conducted from September 2017 to February 2021.And the statistical analysis on the preoperative and postoperative echocardiography results of this group of patients was performed.Results Sixteen patients underwent the biventricular correction surgery,among whom two cases underwent the horizontal atrial tricuspid valvuloplasty(Danielsons procedure),four cases underwent the vertical atrial tricuspid valvuloplasty(Carpentier procedure),and ten cases underwent the conical reconstruction.Two patients were given a half ventricular correction surgery(tricuspid valve reconstruction combined with bidirectional Glenn surgery)and two patients underwent the bidirectional Glenn surgery.The combined atrial septal defects were closed in one stage during extracorporeal circulation for correction of deformitie in 20 patients.At 1,3,6,and 12 months after the surgery,the patient's right ventricular size significantly decreased compared to preoperative(P<0.05),and cardiac function(left ventricular ejection fraction)significantly improved(P<0.05).Conclusion The surgical treatment of Ebstein's anomaly combined with the atrial septal defect should follow the principle of individualized treatment.Biventricular correction is still the first choice for the treatment of Ebstein's anomaly,and the atrial septal defect should be closed at one stage,so as to obtain a good therapeutic effect.

Penjujukan Eksom Bagi Penyakit Jarang Jumpa, Mullerian Agenesis dan Agenesis Anotectal anomaly: Kajian Kes (Whole Exome Sequencing of a Rare Disease, Mullerian Agenesis and Anorectal Anomaly: A Case Report) / Jurnal Sains Kesihatan Malaysia

@#Mullerian agenesis or Mayer-Rokitansky-Kuster-Hauser Syndrome (MRKH) Type-II is a congenital defect in the Mullerian duct that results in the absence of a uterus in women. The aetiology of this syndrome is unknown and has been considered a sporadic genetic disease. MRKH, together with anorectal anomaly, is an extremely rare condition and has only been reported in a few cases without any information on genetic analysis. This study investigated the mutational profile of a girl diagnosed with MRKH and anorectal anomalies with rectovaginal fistula. The whole exome sequencing (WES) trio-genetic analysis of a 5-year-old Malaysian girl diagnosed with MRKH (having anorectal anomaly with rectovaginal fistula) was performed together with her normal parents, using the Ion AmpliSeq Exome RDY kit (ThermoFisher Scientific, USA). Data were analysed using Torrent Suite v.5.0.4 and annotated using ANNOVAR. Single nucleotide polymorphisms (SNPs) with an allele frequency >0.01 were excluded, and the remaining variants were filtered based on de novo mutations, autosomal recessive, and autosomal recessive genetic traits. Related genes were analysed by biological pathway analysis (g:Profiler) and protein-protein interaction (HIPPIE v.2.3, STRING v.11.5, dan GeneMANIA). A total of 36 mutations were identified, and two of them, the LHX5 (p.P358Q), inherited from the father, and CFTR (p.R1158X), inherited from the mother. There were 28 de-novo mutations from 28 genes. All genes were involved in 27 biological processes that connected with 23 interactions, and are likely to cause MRKH syndrome in this patient.

Técnica de Puckett en el tratamiento de mamas tuberosas con lipotransferencia complementaria / The Puckett Technique for Treating Tuberous Breasts with Complementary Lipotransfer

Introducción: La mama tuberosa es una anomalía congénita de la mama que se presenta en la pubertad y es relativamente frecuente. Objetivo: El objetivo de este artículo es dar a conocer el resultado estético del tratamiento quirúrgico de esta afección con la técnica de Pukett sin incluir el implante mamario, con lipotransferencia complementaria. Presentación del caso: Se presenta una paciente femenina, de 25 años de edad, con mama tuberosa grado 4. Se le realiza corrección con técnica de Pukett sin implantes mamarios y en su lugar se usa grasa autóloga. Luego de corregir las anomalías que conforman esta malformación se obtuvo una mama armoniosa sin complicaciones. Conclusión: La técnica de Pukett combinada con lipotransferencia ofrece resultados estéticos y estables en el tratamiento de la mama tuberosa(AU)

Introduction: Tuberous breast is a congenital breast anomaly that presents at puberty and is relatively frequent. Objective: The aim of this article is to report the aesthetic outcome after treating this condition surgically using the Puckett technique without breast implant, with complementary lipotransfer instead. Case presentation: The is presented of a 25-year-old female patient with grade-4 tuberous breast. She is performed a correction using the Puckett technique without breast implants; autologous fat is used instead. After correcting the anomalies that make up this malformation, a harmonious breast was obtained without complications. Conclusion: The Puckett technique combined with lipotransfer offers aesthetic and stable outcomes in the treatment of tuberous breast(AU)

Prevalence of Congenital Anomalies of the Upper Limbs in Brazil: a descriptive cross-sectional study

ABSTRACT BACKGROUND: Congenital Anomalies of the Upper Limb (CAUL) are a group of structural or functional abnormalities that develop during intrauterine life and can lead to limb dysfunction. OBJECTIVES: To analyze the prevalence of congenital anomalies of the upper limbs in Brazil and assess maternal and neonatal variables. DESIGN AND SETTING: A cross-sectional, descriptive study was conducted on congenital upper limb malformations among live births across Brazil. METHODS: The study spanned from 2010 to 2019. Data were sourced from the Department of Informatics of the Unified Health System (DATASUS) and the Live Birth Information System (SINASC) portal. Analyses focused on the information reported in field 41 of the Live Birth Declaration Form entered into the computerized system. RESULTS: The most common anomaly in Brazil was supernumerary fingers, classified as ICD-Q69.0, affecting 11,708 children, with a prevalence of 4.02 per 10,000 live births. Mothers aged over 40 years had a 36% higher prevalence of having children with CAUL than mothers under 40 years old (OR = 1.36; 95% CI 1.19-1.56). Newborns weighing ≥ 2,499 g were 2.64 times more likely to have CAUL compared to those weighing ≥ 2,500 g (OR = 2.64; 95% CI 2.55-2.73). CONCLUSION: There was an observed increase in the reporting of CAUL cases over the decade studied. This trend serves as an alert for health agencies, as understanding the prevalence of CAUL and its associated factors is crucial for preventive medicine.

First report of ocular heterochromia in the treefrog Boana albomarginata (Spix, 1824) (Anura: Hylidae), Atlantic Forest, northeastern Brazil / Primeiro registro de heterocromia ocular na perereca Boana albomarginata (Spix, 1824) (Anura: Hylidae), Mata Atlântica, nordeste do Brasil

Abstract The scientific literature on amphibian anomalies is large, but registered cases of amphibians' ocular heterochromia are almost absent, even considering anecdotal records. In the Brazilian territory, only two cases of ocular heterochromia were formerly registered for amphibians, both for anurans, the first from the Atlantic Forest of northeastern Brazil and the second from the Amazonian region of northern Brazil. In the present account, we report two cases of ocular heterochromia in the hylid treefrog Boana albomarginata, both from the Atlantic Forest biome, Bahia state, northeastern Brazil, representing the first record of ocular heterochromia for this species and the third case registered for an amphibian's species in Brazil. One eye of each observed specimen had an unusually flashy and homogeneous blue-colored iris, filling its entire surface or only its lower half, representing cases of complete and sectoral heterochromia, respectively. Furthermore, the unusual flashy blue iris was only detectable during daylight, when the iris background color was white. At night, the standard copper to golden iris background color remains unchanged, and the blue color cannot be detected. No other detectable anomalies or unusual behaviors were observed for both specimens. This is the first report of amphibian ocular heterochromia represented by a flashy blue color iris and limited to a daylight iris color pattern. Further studies are necessary to determine the nature of this anomaly and its potential consequences for the specimen and the species.

Resumo A literatura científica sobre anomalias em anfíbios é vasta, mas casos registrados de heterocromia ocular em anfíbios estão quase ausentes, mesmo considerando registros anedóticos. No território brasileiro, apenas dois casos de heterocromia ocular foram registrados anteriormente para anfíbios, ambos para anuros, o primeiro para a Mata Atlântica do nordeste do Brasil e o segundo para a região amazônica do norte do Brasil. No presente relato, descrevemos dois casos de heterocromia ocular no hilídeo Boana albomarginata, ambos no bioma Mata Atlântica, estado da Bahia, nordeste do Brasil, representando o primeiro registro de heterocromia ocular para esta espécie e o terceiro caso registrado para uma espécie de anfíbio no Brasil. Um olho de cada exemplar observado apresentava atipicamente íris de cor azul chamativa e homogênea, preenchendo toda sua superfície ou apenas a metade inferior, representando casos de heterocromia completa e setorial, respectivamente. Além disso, a não-usual íris azul só esteve detectável sob a luz do dia, quando a cor de fundo da íris era branca. À noite, a cor de fundo padrão da íris cobreada ou dourada permaneceu inalterada e a cor azul não pôde ser detectada. Nenhuma outra anomalia detectável ou comportamento incomum foi observada para ambos os exemplares. Este é o primeiro relato de heterocromia ocular em anfíbios representado por uma cor azul bem-marcada e limitada a um padrão diurno de cor da íris. Mais estudos são necessários para determinar a natureza desta anomalia e suas potenciais consequências para o indivíduo e para a espécie.

Relato de caso: aorta dorsal dupla persistente / Case report: persistent double dorsal aorta

Resumo A aorta dorsal dupla persistente é uma anomalia congênita extremamente rara, com apenas 13 casos publicados até o presente momento. Este estudo tem como objetivo apresentar a variação embriológica observada em aorta abdominal do paciente. A descrição anatômica foi realizada a partir da revisão de prontuário eletrônico e exames de imagem. Desta forma, o presente caso refere-se a um paciente idoso de 79 anos admitido em atendimento hospitalar por queixa de dor em membro inferior esquerdo ao repouso. Foi solicitada a internação do mesmo, assim como exames laboratoriais e de imagem. Obtida como um achado de imagem a partir de angiotomografia, a variação consiste na completa separação em duas porções: uma ventral e outra dorsal, de calibres diferentes, da aorta abdominal, ao nível da terceira vértebra lombar. Isso ocorre em conjunto com a origem anômala da artéria mesentérica inferior.

Abstract Persistent double dorsal aorta is an extremely rare congenital anomaly, with only 13 cases published to date. The objective of this study is to present this embryological variant as observed in the abdominal aorta of a patient. The anatomical description was written up on the basis of a review of electronic medical records and imaging exams. The patient in this case was an elderly 79-year-old man who presented at emergency with pain at rest in the left lower limb. He was admitted and laboratory tests and imaging exams were ordered. The variation was an imaging finding observed on angiotomography, consisting of complete separation of the abdominal aorta into two portions - a ventral and a dorsal, with different calibers - at the level of the third lumbar vertebra. There was also an anomalous origin of the inferior mesenteric artery.

Síndrome Klippel-Trenaunay en el embarazo. Reporte de un caso y revisión de la literatura / Klippel-Trenaunay syndrome in pregnancy. Case report and literature review

Resumen Antecedes: El síndrome de Klippel-Trenaunay (KTS) es un síndrome de malformación vascular que comprende una afectación variable de capilares cutáneos, venas y linfáticos con hipertrofia de tejidos blandos y huesos de la extremidad afectada. Durante el embarazo estas malformaciones se incrementan, con afectación pélvica e intraabdominal. Caso clínico: Paciente de 15 años, primigesta, con síndrome Klippel-Trenaunay diagnosticado al nacimiento, con embarazo a término, referida por iniciar con trabajo de parto para finalización del embarazo en un hospital de tercer nivel. No cuenta con control obstétrico, estudios prenatales ni ultrasonidos obstétricos. Se realiza una ecografía pélvica en donde se descartóla presencia de varices pélvicas y un doppler que evidenció un sistema venoso conservado. Se realiza terminación del embarazo vía abdominal obteniendo un recién nacido vivo masculino, peso de 3045 gramos, APGAR 9 y 9 al minuto y a los 5 minutos. Resultados: El embarazo en pacientes con síndrome de Klippel-Trenaunay tiene alto riesgo de tromboembolismo y complicaciones hemorrágicas. La valoración debe ser por un equipo multidisciplinario capacitado para anticiparse a las potenciales complicaciones. Limitaciones del estudio o implicaciones: La principal limitación es la baja incidencia de esta patología. Se puede concluir que el diagnostico de SKT no es indicación de interrupción del embarazo. El éxito del manejo de estas pacientes requiere la participación de un equipo multidisciplinario. Originalidad o valor: Este caso clínico es de primordial relevancia ya que en la bibliografía internacional están reportados menos de 100 casos de embarazos complicados con este síndrome.

Abstract: Background: Klippel-Trenaunay syndrome (KTS) Klippel- Trenaunay syndrome (KTS) is a vascular malformation síndrome that includes variable involvement of skin capillaries, veins and lymphatics with hypertrophy of soft tissues and bones of the affected limb. During pregnancy, these malformations increase, with pelvic and intra-abdominal involvement. Clinical case: 15-year-old patient, gravida 0, with Klippel- Trenaunay syndrome diagnosed at birth, with full-term pregnancy, referred for initiating labor for resolution of pregnancy in a third level hospital. Without obstetric control, without prenatal studies or obstetric ultrasounds. A pelvic ultrasound was performed, which ruled out the presence of pelvic varices and a Doppler that showed a preserved venous system. The pregnancy was terminated by abdominal route, obtaining a male newborn, weighting 3045 grams, APGAR 9 and 9 after 1 and 5 minutes. Results: Pregnancy in patients with Klippel-Trenaunay síndrome has a high risk of thromboembolism and bleeding complications. They should be evaluated by a trained multidisciplinary team to anticipate possible complications. Study limitations or implications: The main limitation is the low incidence of this pathology. It can be concluded thatthe diagnosis of SKT is not an indication for termination of pregnancy. Successful management of these patients requires the participation of a multidisciplinary team. Originality or value: This clinical case is of primary relevance since fewer than 100 cases of complicated pregnancies with this syndrome are reported in the international literature.

Radix em molares decíduos inferiores: revisão sistemática com metanálise / Radix in lower primary molars: systematic review with meta-analysis

Objective: To estimate the prevalence of three roots in deciduous mandibular molars. Methodology: Electronic searches were carried out in PubMed and Scopus to identify cross-sectional studies published up to September 2023. The Joanna Briggs Institute tool was used to critically appraise the studies. STATA 16.0 was used to generate risk of bias figures and perform the meta-analysis. Results: Eighteen studies evaluating 9,067 patients (8,969 first molars and 10,765 second molars) were included in this review. The overall prevalence of radix in mandibular deciduous molars was 9.61% (3.67% for first molars and 18.72% for second molars). The prevalence rate of teeth diagnosed using Cone Beam Computed Tomography (CBCT) was similar to the diagnoses made using conventional radiographic techniques together (periapical, interproximal and panoramic). Final considerations: Lower deciduous molars with three roots have a prevalence of almost 10%, with a higher prevalence in second molars. The diagnosis of this morphological alteration can be made using conventional radiographic techniques, but the use of CBCT is recommended.(AU)

Objetivo: estimar a prevalência de três raízes em molares inferiores decíduos. Metodologia: foram realizadas buscas eletrônicas na PubMed e Scopus para identificar estudos transversais publicados até setembro/2023. Para a avaliação crítica dos estudos foi utilizada a ferramenta do Instituto Joanna Briggs. STATA 16.0 foi usado para gerar figura do risco de viés e realizar a metanálise. Resultados: dezoito estudos que avaliaram 9.067 pacientes (8.969 primeiros molares e 10.765 segundos molares) foram incluídos nesta revisão. A prevalência global de radix em molares decíduos inferiores foi de 9,61% (3,67% para primeiros molares e 18,72% para segundo molares). A taxa de prevalência de dentes com diagnóstico através de Tomografia Computadorizada Cone Beam (TCCB) foi semelhante aos diagnósticos realizados pelas técnicas radiográficas convencionais em conjunto (periapical, interproximal e panorâmica). Considerações finais: os molares decíduos inferiores com três raízes têm uma prevalência de quase 10%, com maior prevalência em segundo molares. O diagnóstico desta alteração morfológica pode ser feito através das técnicas radiográficas convencionais, porém recomenda-se a utilização de TCCB.(AU)

Un caso de leucismo en el Juil de Jamapa, Rhamdia laticauda (Siluriformes, Heptapteridae) en el noreste del estado de Oaxaca, México

Se reporta la primera observación de un espécimen leucístico en el Juil de Jamapa, Rhamdia laticauda en el noreste del estado de Oaxaca. En un estudio ictiológico de la Reserva de la Biosfera Tehuacán-Cuicatlán en el río Xiquila de la cuenca alta del río Papaloapan, el pez se capturó con pesca eléctrica y se fotografió. El bagre vivo mostró una reducción de pigmentos en la piel, manchas blancas y ojos negros normales. Esto contribuye al conocimiento de la variabilidad intraespecífica y la historia natural de R. laticauda. Se discuten las causas potenciales de esta anomalía de color.

Here, we report the first observation of a leucistic specimen in the Rock catfish, Rhamdia laticauda in the Northeast of Oaxaca, Mexico. We caught the specimen by electric fishing and then photographed during an ichthyological survey carried out in the Tehuacán-Cuicatlán Biosphere Reserve in the Xiquila River of the upper Papaloapan River basin. The live catfish showed a lack of pigment in the skin, white spots, and normal eye color. This information contributes to the knowledge about intraspecific variability and natural history of R. laticauda. We discuss the potential causes of this color anomaly.

An atypical presentation of talon cusp: Double talon cusps

Talon cusp is an uncommon developmental anomaly resulting in an extra cusp or cuspal projection on an anterior tooth consisting of normal enamel, dentin, and varying degrees of pulp tissue. Talon cusp shows varied presentations of which the palatal talon cusp has been the most common of all. The management requires an extensive understanding of this clinical entity and the complications associated with its occurrence. In this case series, we are reporting double talon cusps which is a rarity itself, involving the palatal aspect of maxillary central incisors

Mullerian Anomaly with Large Ovarian Mass — Where Two Worlds Collide

When young women diagnosed with Large Ovarian Masses present with associated mullerian abnormality it shakes the world of Gynaecology and requires the joint help of General Gynaecologists and gynae-oncologists as reconstruction of Mullerian anomaly and fertility preservation is as important as management of Malignant Ovarian Masses. We report a case of 21 year, unmarried woman with complaints of primary amenorrhea and large abdominopelvic mass and short, blind vagina. Magnetic Resonance Imaging showed Uterus Didelphis with normal endometrium and upper vaginal agenesis. Computed Tomography images revealed a large ovarian mass. Intraoperatively there was a 30cm large Ovarian Tumour, Didelphyic and hypoplastic (2cm) uterus and 2 cm blind vagina. Management of this case involved extensive discussion among the Gynecologists, Gynae-oncologist and radiologists. In non-oncology setup where the rate of surgeries are high, option between frozen section and Mullerian reconstruction are required especially when the Ovarian mass looks benign as both surgeries require time and expertise. Hence, involvement of patient and family members in decision making form an integral part of management.Intraoperative findings also influence surgical decisions in Mullerian anomaly