Perioperative Techniques and Long-term Outcomes in Hypospadias: A Systematic Review

@#Introduction: Hypospadias is a condition when the urethra distal opens on the bottom of the penis and has a ventral penile curvature. Hypospadias is the second most frequent genital birth abnormality in boys after cryptorchidism. Surgery is the standard treatment of choice and has a significant risk problem. There are many surgical procedure complications, especially in appearance and function. This article discusses the appropriate operative management and the best long-term outcome. Methods: The data was culled and selected from the ten years of full-text English publication (2012-2022) utilizing the PubMed and Google Scholar databases. Meta-analyses (PRISMA) paradigm and PICO investigation of management techniques and long-term outcomes in clinical and randomized clinical trials. Result: From 149 search articles, 7 met the inclusion criteria. The gland penis size and the meatal location did not affect the outcome of the surgical technique. However, 58/432 patients required reoperation, and 61(13%) had urethroplasty complications (UC), although not statistically significant (OR 0.8, 95% CI: 0.7-0.9). Testosterone is advised for small penises, narrow glands, thin urethral plates, and proximal hypospadias (67 vs. 87%). Preoperative antibiotics reduce the incidence of infection (17/150 repairs), tubularized incised plate (TIP), and the stent is recommended. Conclusion: The recommended strategies are good perioperative treatment, including antibiotics, testosterone treatment, surgery ages (6-18 months), tubularized incised plate (TIP) technique, and stent postoperative.

Protocolo Brasileiro para Infecções Sexualmente Transmissíveis 2020: infecção pelo vírus Zika / Brazilian Protocol for Sexually Transmitted Infections 2020: Zika virus infection / Protocolo Brasileño para Infecciones de Transmisión Sexual 2020: infección por el virus Zika

Este artigo aborda as transmissões vetorial, sexual e vertical do vírus Zika, tema contemplado no Protocolo Clínico e Diretrizes Terapêuticas para Atenção Integral às Pessoas com Infecções Sexualmente Transmissíveis, publicado pelo Ministério da Saúde do Brasil em 2020. Embora no Brasil o vírus Zika seja predominantemente veiculado pelo Aedes aegypti, as vias vertical e sexual de transmissão apresentam expressiva importância para a saúde reprodutiva. A transmissão sexual demanda o uso de intervenções profiláticas específicas, incluindo o uso do preservativo masculino ou feminino, principalmente entre casais que planejam gravidez. A transmissão vertical é ligada a graves anormalidades estruturais do sistema nervoso central e ainda não há vacina e nem recursos farmacológicos conhecidos que possam preveni-la. Como a doença é predominantemente assintomática, o não cumprimento dos princípios básicos de cuidados e orientações relacionadas à dispersão da infecção transcende a gravidade dos sintomas da doença.

This article addresses vector, sexual and vertical transmission of Zika virus, a topic covered in the Clinical Protocol and Therapeutic Guidelines for Comprehensive Care for People with Sexually Transmitted Infections, published by the Brazilian Ministry of Health in 2020. Although in Brazil Zika virus is transmitted most predominantly by Aedes aegypti, the vertical and sexual transmission routes are of significant importance for reproductive health. Sexual transmission demands the use of specific prophylactic interventions, including the use of male or female condoms, especially among couples planning pregnancy. Vertical transmission is linked to severe structural abnormalities of the central nervous system and there is still no vaccine or known pharmacological resources that can prevent it. As the disease is predominantly asymptomatic, failure to comply with basic principles of care and guidelines related to the spread of infection transcends the severity of the symptoms of the disease.

Este artículo aborda la transmisión vectorial, sexual y vertical del virus Zika, tema tratado en el Protocolo Clínico y Directrices Terapéuticas para la Atención Integral a Personas con Infecciones de Transmisión Sexual, publicado por el Ministerio de Salud de Brasil en 2020. Aunque en Brasil el virus Zika sea principalmente transmitido por Aedes aegypti, las vías vertical y sexual de transmisión son de gran importancia para la salud reproductiva. La transmisión sexual exige el uso de intervenciones profilácticas específicas, incluido el uso de preservativos masculinos o femeninos, especialmente entre las parejas que planean un embarazo. La transmisión vertical está ligada a graves anomalías estructurales del sistema nervioso central y todavía no existe una vacuna o recursos farmacológicos conocidos que puedan prevenirla. Como la enfermedad es predominantemente asintomática, el incumplimiento de los principios básicos de atención y las pautas relacionadas con la propagación de la infección trasciende la gravedad de los síntomas de la enfermedad.

Protocolo Brasileiro para Infecções Sexualmente Transmissíveis 2020: infecção pelo vírus Zika / Brazilian Protocol for Sexually Transmitted Infections 2020: Zika virus infection / Protocolo Brasileño para Infecciones de Transmisión Sexual 2020: infección por el virus Zika

Resumo Este artigo aborda as transmissões vetorial, sexual e vertical do vírus Zika, tema contemplado no Protocolo Clínico e Diretrizes Terapêuticas para Atenção Integral às Pessoas com Infecções Sexualmente Transmissíveis, publicado pelo Ministério da Saúde do Brasil em 2020. Embora no Brasil o vírus Zika seja predominantemente veiculado pelo Aedes aegypti, as vias vertical e sexual de transmissão apresentam expressiva importância para a saúde reprodutiva. A transmissão sexual demanda o uso de intervenções profiláticas específicas, incluindo o uso do preservativo masculino ou feminino, principalmente entre casais que planejam gravidez. A transmissão vertical é ligada a graves anormalidades estruturais do sistema nervoso central e ainda não há vacina e nem recursos farmacológicos conhecidos que possam preveni-la. Como a doença é predominantemente assintomática, o não cumprimento dos princípios básicos de cuidados e orientações relacionadas à dispersão da infecção transcende a gravidade dos sintomas da doença.

Abstract This article addresses vector, sexual and vertical transmission of Zika virus, a topic covered in the Clinical Protocol and Therapeutic Guidelines for Comprehensive Care for People with Sexually Transmitted Infections, published by the Brazilian Ministry of Health in 2020. Although in Brazil Zika virus is transmitted most predominantly by Aedes aegypti, the vertical and sexual transmission routes are of significant importance for reproductive health. Sexual transmission demands the use of specific prophylactic interventions, including the use of male or female condoms, especially among couples planning pregnancy. Vertical transmission is linked to severe structural abnormalities of the central nervous system and there is still no vaccine or known pharmacological resources that can prevent it. As the disease is predominantly asymptomatic, failure to comply with basic principles of care and guidelines related to the spread of infection transcends the severity of the symptoms of the disease.

Resumen Este artículo aborda la transmisión vectorial, sexual y vertical del virus Zika, tema tratado en el Protocolo Clínico y Directrices Terapéuticas para la Atención Integral a Personas con Infecciones de Transmisión Sexual, publicado por el Ministerio de Salud de Brasil en 2020. Aunque en Brasil el virus Zika sea principalmente transmitido por Aedes aegypti, las vías vertical y sexual de transmisión son de gran importancia para la salud reproductiva. La transmisión sexual exige el uso de intervenciones profilácticas específicas, incluido el uso de preservativos masculinos o femeninos, especialmente entre las parejas que planean un embarazo. La transmisión vertical está ligada a graves anomalías estructurales del sistema nervioso central y todavía no existe una vacuna o recursos farmacológicos conocidos que puedan prevenirla. Como la enfermedad es predominantemente asintomática, el incumplimiento de los principios básicos de atención y las pautas relacionadas con la propagación de la infección trasciende la gravedad de los síntomas de la enfermedad.

Does folic acid reduce congenital anomalies and adverse pregnancy outcomes in women received long-term low dose methotrexate?

Background: Methotrexate (MTX) which is folic acid antagonist is used to treat many diseases, including rheumatic, inflammatory, autoimmune disorders or malignancies; it is also used for the medical treatment of ectopic pregnancy and termination of pregnancy. The objective of this study was to assess the impact of folic acid in preventing congenital anomalies (CAs) in the fetus of pregnant that take long-term low dose methotrexate.Methods: The study included 1569 with history of low dose methotrexate therapy <25 mg/week that stopped one month before pregnancy, they were divided into 3 groups; Group 1 comprised 272 pregnant women, Group 2 comprised 367 pregnant women both groups take folic acid 4-5 weeks preconception and for the first 12-13 weeks gestation, the dose in the 1st group was 0.4 mg/day while in 2nd Group 4 mg/day, Group 3 comprised of 930 pregnant disease-matched with no folic acid. Group 4 comprised of 1015 pregnant no underlying autoimmune and no folic acid intake.Results: The overall rate of CAs and cardiovascular CAs were higher in fetuses of Group 3 (OR: 1.7; 95% CI: 1.1-2.7) and (OR: 2.7; 95% CI: 1.4-5.2) respectively compared to the overall rate of CAs and cardiovascular CAs in other groups. Other adverse outcomes as abortion, preterm delivery, preeclampsia, placenta abruption, PROM, low birth weight, admission to NICU, IUGR and IUFD were higher in Group 3 and 4 than in Group 1 and 2. There were no significant differences in umbilical arterial PH. The Apgar scores <7 at 1, 5 min and gestational age at delivery were higher in Group 1 and 2 than Group 3 and 4.Conclusions: There was a certain reduction in methotrexate teratogenic effect on the fetuses with folic acid intake during pregnancy, these pregnancies might benefit from taking of folic acid in high doses before conception and during the first trimester of pregnancy and improvement in fetal and maternal outcomes but the explanation of this effect requires further study.

Soft tissue reconstruction in wide Tessier number 3 cleft using the straight-line advanced release technique

Craniofacial cleft is a rare disease, and has multiple variations with a wide spectrum of severity. Among several classification systems of craniofacial clefts, the Tessier classification is the most widely used because of its simplicity and treatment-oriented approach. We report the case of a Tessier number 3 cleft with wide soft tissue and skeletal defect that resulted in direct communication among the orbital, maxillary sinus, nasal, and oral cavities. We performed soft tissue reconstruction using the straight-line advanced release technique that was devised for unilateral cleft lip repair. The extension of the lateral mucosal and medial mucosal flaps, the turn over flap from the outward turning lower eyelid, and wide dissection around the orbicularis oris muscle enabled successful soft tissue reconstruction without complications. Through this case, we have proved that the straight-line advanced release technique can be applied to severe craniofacial cleft repair as well as unilateral cleft lip repair.

Dorsal midline cutaneous stigmata associated with occult spinal dysraphism in pediatric patients / 소아과

PURPOSE: To investigate the prevalence of occult spinal dysraphism (OSD) and subsequent neurosurgery in pediatric patients with isolated or combined dorsal midline cutaneous stigmata with or without other congenital malformations. METHODS: We carried out a retrospective review of patients who underwent sonography or magnetic resonance imaging (MRI) for OSD because of suspicion of dorsal midline cutaneous stigmata (presumed to be a marker for OSD) between January 2012 and June 2017. Information about patient characteristics, physical examination findings, spinal ultrasound and MRI results, neurosurgical notes, and accompanying congenital anomalies was collected. RESULTS: Totally 250 patients (249 ultrasound and one MRI screening) were enrolled for analysis. Eleven patients underwent secondary MRI examinations. The prevalence of OSD confirmed by an MRI was 2.4% (6 patients including one MRI screening). Five patients (2%) had tethered cord and underwent prophylactic neurosurgery, 3 of whom had a sacrococcygeal dimple and a fibrofatty mass. Prevalence of tethered cord increased as markers associated with a sacrococcygeal dimple increased (0.5% of the isolated marker group, 8.1% of the 2-marker group, and 50% of the 3-marker group). Incidence of OSD with surgical detethering in 17 other congenital anomaly patients was 11.8%, which was higher than the 1.3% in 233 patients without other congenital anomalies. CONCLUSION: Our results suggest that the presence of dorsal midline cutaneous stigmata, particularly fibrofatty masses, along with a sacrococcygeal dimple is associated with OSD or cord tethering requiring surgery. OSD should be suspected in patients with concurrent occurrence of other congenital anomalies.

Anomalias congênitas e suas principais causas evitáveis: uma revisão / Congenital anomalies and its main avoidable causes: a review

As anomalias congênitas (AC) podem ser definidas como todas as alterações funcionais ou estruturais do desenvolvimento fetal, cuja origem ocorre antes do nascimento. Elas possuem causas genéticas, ambientais ou desconhecidas. As principais causas das anomalias são os transtornos congênitos e perinatais, muitas vezes associados a agentes infecciosos deletérios à organogênese fetal, tais como os vírus da rubéola, da imunodeficiência humana (HIV), o vírus Zika, o citomegalovírus; o Treponema pallidum e o Toxoplasma gondii. O uso de drogas lícitas e ilícitas, de medicações teratogênicas, endocrinopatias maternas também podem ser citados como causa de AC. Estima-se que 15 a 25% ocorram devido às alterações genéticas, 8 a 12% são causadas por fatores ambientais e 20 a 25% podem ser causadas tanto por alterações genéticas quanto por fatores ambientais. Neste artigo, serão abordadas as principais causas das AC, com foco naquelas que podem ser evitadas. (AU)

Congenital anomalies (CA) can be defined as all functional or structural changes of fetal development that originate before birth. They have genetic, environmental or unknown causes. The main causes of anomalies are congenital and perinatal disorders, often associated with infectious agents deleterious to fetal organogenesis, such as rubella virus, human immunodeficiency virus (HIV), Zika virus, cytomegalovirus; the Treponema pallidum and the Toxoplasma gondii. The use of licit and illicit drugs, teratogenic medications, and maternal endocrinopathies can also be cited as causes of CA. It is estimated that 15 to 25% occur due to genetic alterations, 8 to 12% are caused by environmental factors and 20 to 25% can be caused by both genetic and environmental changes. In this article, the main causes of CA will be addressed, focusing on those that can be avoided. (AU)

A Case of Congenital Lacrimal Ductule Fistula on Lateral Skin to the Right Upper Eyelid

PURPOSE: To present a rare case of tear drainage since birth from a lacrimal ductule fistula, which is the first report in the Republic of Korea. CASE SUMMARY: 3-month-old female who presented with discharge of clear fluid from a small skin opening lateral to the right upper eyelid since birth visited the outpatient clinic. The patient was healthy and was receiving no medication. She was born on gestation age 35 weeks and 3 days. No other specific history or other ophthalmic abnormality was found. On examination, a small skin orifice approximately 2 mm diameter and no sign of infection or discoloration was observed. To relieve this symptom, the patient underwent a fistulectomy under general anesthesia. Histopathological examination of the surgical specimen was performed. The patient's symptom subsided postoperatively without recurrence. CONCLUSIONS: This is the first report in the Republic of Korea of tears draining from a lacrimal ductule fistula since birth and the subsequent clinical treatment procedures.

Clinical value of echocardiography and multi-slice spiral CT in the diagnosis of complex congenital heart disease in children / 中国基层医药

Objective To investigate the value of echocardiography and multi-slice spiral CT in the diagnosis of complex congenital heart disease(CCHD).Methods 73 children with CCHD were selected and diagnosed by echocardiography and multi-slice spiral CT in Children''s Hospital of Shanxi Province from April 2012 to April 2016.The distribution of disease types in 73 children with CCHD was recorded,and the diagnostic accuracy of multi-slice spiral CT was compared with echocardiography,multi-slice spiral CT and echocardiography.Results 73 children with CCHD had a total of 172 cardiac malformations confirmed by operation and pathology.By segment analysis,there were 30 cases of extracardiac vascular malformations,52 cases of abnormal heart and vascular connection,90 cases of atrial and atrioventricular junction deformity.Multislice spiral CT was performed to detect 155 cases of cardiac malformations,including 28 cases of extracardiac malformation,48 cases of maligant heart and great vessels,79 cases of intracardiac and atrioventricular malformation.Echocardiographic diagnosis of 158 cases of cardiac malformations,including 28 cases of extracardiac vascular malformations,49 cases of large vascular malformations,81 cases of intracardiac and atrioventricular junction deformity.Echocardiography combined with multi-slice spiral CT in the diagnosis of 167 cases of cardiac malformations,including 29 cases of extracardiac vascular malformations,50 cases of large vascular malformations,88 cases of intracardiac and atrioventricular malformation.The diagnostic accuracy between echocardiography [91.86％(158/172)] and multislice spiral CT [90.12％(155/172)] had no significant difference (P＞0.05).The diagnostic accuracy of echocardiography combined with multi-slice spiral CT was 97.09％(167/172),which was higher than 91.86％(158/172) of echocardiography and 90.12％(155/172) of multislice spiral CT,the difference was statistically significant(P＜0.05).Conclusion The echocardiography combined with multi-slice spiral CT in the diagnosis of CCHD has high accuracy,it can provide strong imaging support for surgical options and prognosis.

Prenatal ultrasound diagnosis of fetal ear auricle abnormalities / 中华医学超声杂志（电子版）

Objective The purpose of this study was to assess the value of prenatal ultrasound diagnosis for fetal ear auricle malformations.Methods The coronal and sagittal planes of fetuses ears were obtained prospectively in 6239 singleton fetuses in the First Affiliated Hospital of Shantou University Medical College for the period from 2012 February to 2015 December,the ultrasound images and pregnancy outcomes were analyzed in 11 cases of fetuses ear auricle malformations diagnosed prenatally.Results Eleven Cases of fetuses ear auricle malformations include with 7 cases of microtia,3 cases of low-set ears and 1 case of anotia.Eleven cases were combined with other structural malformations were as followings,3cases with craniocerebral congenital malformation,5 cases with dentofacial deformity,5 cases with malformation of heart,3 cases with limb deformity.Cordocentesis was performed in 7 cases among which 6 with abnormal karyotype,including 2 cases of trisomy 21,2 cases of trisomy 13,2 cases of trisomy 18,1 case of 22ql 1 abnormalities.Compared with the postpartum facial examination,prenatal ultrasound correctly diagnosed 10 cases of fetal ear auricle malformations,missed diagnosis 1 case of microtia.Conlusions Fetus with ear auricle abnormalities have characteristic prenatal ultrasound imaging;prenatal ultrasonography can provide reliable information in the diagnosis of this disease.This study suggests that antenatal ear auricle length measurements might be a promising sonographic screening method for the detection of abnormal karyotype in pregnancy.

An adult asymptomatic pulmonary artery sling / 영남의대학술지

A pulmonary artery sling is a very rare congenital abnormality in which the left pulmonary artery rises from the posterior surface of the right pulmonary artery and then passes between the trachea and the esophagus, causing tracheal compression. It is associated with tracheo-bronchial abnormalities (50%) and cardiovascular abnormalities (30%). It may produce respiratory symptoms through the airway compression of the abnormal left pulmonary artery and congenital abnormalities associated with it. Because most (90%) pulmonary artery sling patients present symptoms during infancy, their condition is often diagnosed in the first year of life. However, a pulmonary artery sling is occasionally found in adults. It is usually asymptomatic and found incidentally. This is a very rare case of an asymptomatic pulmonary artery sling in an adult. A 38-year-old man presented symptoms of mild exertional dyspnea. His spiral computed tomography showed a pulmonary artery sling. He was discharged without specific treatment because his symptoms improved without specific treatment and might not have been associated with a pulmonary artery sling. We report an adult case of an asymptomatic pulmonary artery sling diagnosed via spiral computed tomography, accompanied by a literature review.

The Comparative Morphometric Study of the Posterior Cranial Fossa : What Is Effective Approaches to the Treatment of Chiari Malformation Type 1?

OBJECTIVE: The objective of this study was to investigate changes in the posterior cranial fossa in patients with symptomatic Chiari malformation type I (CMI) compared to a control group. METHODS: We retrospectively reviewed clinical and radiological data from 12 symptomatic patients with CMI and 24 healthy control subjects. The structures of the brain and skull base were investigated using magnetic resonance imaging. RESULTS: The length of the clivus had significantly decreased in the CMI group than in the control group (p=0.000). The angle between the clivus and the McRae line (p<0.024), as the angle between the supraocciput and the McRae line (p<0.021), and the angle between the tentorium and a line connecting the internal occipital protuberance to the opisthion (p<0.009) were significantly larger in the CMI group than in the control group. The mean vertical length of the cerebellar hemisphere (p<0.003) and the mean length of the coronal and sagittal superoinferior aspects of the cerebellum (p<0.05) were longer in the CMI group than in the control group, while the mean length of the axial anteroposterior aspect of the cerebellum (p<0.001) was significantly shorter in the CMI group relative to control subjects. CONCLUSION: We elucidate the transformation of the posterior cranial fossa into the narrow funnel shape. The sufficient cephalocaudal extension of the craniectomy of the posterior cranial fossa has more decompression effect than other type extension of the craniectomy in CMI patients.

Anomalia de Ebstein: relato de caso / Ebstein Anomaly: a case report

Justificativa e objetivos: a anomalia de Ebstein é a doença congênita mais comum da valva tricúspide. É caracterizada por aderência de porção variável das cúspides posterior e septal de uma valva tricúspide redundante para o interior da parede ventricular direita e implantada mais caudalmente em direção ao ápice. Suas manifestações clínicas bem como seu tratamento são variáveis conforme o grau de anomalia valvar. Relato do caso: paciente de 11 anos com diagnóstico de anomalia de Ebstein desde o período pós-natal imediato. Apresentava percentil 10 de peso e estatura; desenvolvimento normal. Foi indicada intervenção cirúrgica devido a quadro de dispneia aos médios esforços, piora da cianose e diaforese. Submetido à plicatura do VD atrializado, plastia da valva tricúspide com diminuição do anel tricúspide, correção da CIA e CIV sob anestesia geral balanceada associada à peridural torácica. Antes de entrar em CEC, apresentou TSV após manipulação do AD. A CEC durou 89 min e o CAo, 60 min. Saiu de CEC com adrenalina a 0,3 mcg/kg/min e milrinona a 0,75 mcg/kg/min. Aproximadamente 15 minutos após receber uma bolsa de concentrado de hemácias, duas unidades de plaquetas e 10 mL de gluconato de cálcio 10 mL a 10%, cursou com TV sem pulso revertida a ritmo juncional após a segunda desfibrilação. Foi transferido para o CTI em ritmo juncional, FC: 130 BPM, PIA: 56/40 mmHg. Conclusões: portadores de anomalia de Ebstein com mais de 10 anos frequentemente apresentam arritmias cardíacas. Há forte associação com vias de condução acessórias ­ fenômenos de pré-excitação ­ WPW (ressalta-se que no caso em questão o estudo eletrofisiológico pré-CEC não evidenciou qualquer via anômala ou fenômeno de pré-excitação). É comum a coexistência de defeitos septais. O tratamento clínico-cirúrgico deve ser individualizado, sendo mandatório conhecer alterações cardiovasculares e hemodinâ- micas associadas para otimizar o ato anestésico-cirúrgico

Justification and objectives: Ebstein anomaly is the most common congenital disorder in the tricuspid valve. It is characterized by a variable portion of the posterior and septal leaflets of a redundant tricuspid valve adhering the right ventricular wall and caudally towards the apex. Its clinical manifestations and treatments vary according to the degree of valve anomaly. Case report: An eleven-year-old patient diagnosed with Ebstein anomaly since the immediate postnatal period. The patient was in the 10th percentile of height and weight, and had normal development. Surgery was indicated due to effort dyspnea, worsening cyanosis, and diaphoresis. The patient underwent plicature of the atrialized right ventricle; tricuspid valve plastic surgery to reduce the tricuspid ring, correction of interatrial and interventricular communication with the aid of balanced, general anesthesia associated with thoracic epidural. Before initial- izing extracorporeal circulation (ECC), the patient had supraventricular tachycardia upon manipulation of the right atrium. ECC and aortic clamping lasted 89 and 60 minutes respectively. CEC stopped with 0.3 mcg/ kg/min adrenalin and 0.75 mcg/kg/min milrinone. Approximately 15 minutes after receiving a red blood cell concentrate bag, two plaque units and 10 mL of 10 % calcium gluconate, he had ventricular tachycardia without pulse that was reverted to junctional rhythm upon the second defibrillation.The patient was transferred to ICU in junctional rhythm, heart rate: 130 BPM; intra-abdominal pressure: 56/40 mmHg. Conclusions: Elbstein anomaly patients aging over 10 years old frequently have cardiac arrhythmias. There is strong association with accessory conduction pathways ­ pre-excitation phenomena ­ WPW (in the case under scrutiny, a pre-CEC electrophysiological study did not point to any anomalous pathway or pre-excitation phenomena). Coexistence of septal defects is common. Clinical and surgical treatment should be customized, and the practitioners must be aware of the associated cardiovascular and hemodynamic alterations in order to optimize both anesthetic and surgical procedures.

Defectos congénitos mayores en un hospital de tercer nivel en Cali, Colombia 2004-2008 / Major birth defects in a third-level hospital in Cali, Colombia, 2004-2008

Objetivos Determinar la prevalencia de los defectos congénitos diagnosticados al nacimiento y describir los factores sociodemográficos y del recién nacido asociados a la ocurrencia de estas patologías en un hospital de tercer nivel de la ciudad de Cali en Colombia. Metodología Se analizó la información del sistema de vigilancia del Hospital Universitario del Valle entre marzo de 2004 y octubre de 2008. Para determinar la ocurrencia de defectos congénitos se siguió la metodología propuesta por el ECLAMC. Se realizaron tablas y gráficos para describir las características de los recién nacidos y sus padres. Resultados Durante el período de estudio, un total de 4 años y 7 meses entre 2004 y 2008, se atendieron 32 995 nacimientos, de los cuales 735 presentaron al menos un defecto congénito, para una prevalencia de 2,22 por 10 000 nacimientos. Por grupos específicos de defectos congénitos se registraron las prevalencías más altas (casos x 10 000 nacimientos) para: polidactilia (22,1), pie equinovaro (17,6), hidrocefalia (16,4), defectos del tubo neural (16,7), defectos por reducción de extremidades (8,2), labio y/o paladar hendido (10,9), cardiopatías (14,6), hidronefrosis (11,5), gastrosquisis (7,3), ciclopía (2,4) y sirenomelia (1,2). Discusión Las prevalencias de defectos congénitos relacionados con factores ambientales como hidrocefalia, defectos por reducción de extremidades, pie equino varo, hidronefrosis, gastrosquisis, ciclopía y sirenomelia presentaron prevalencias más altas en comparación con lo reportado en la literatura. Se presentan algunas hipótesis que podrían explicar el aumento registrado.

Objective Determining the prevalence of infant birth defects and describing sociodemographic factors associated with the occurrence of these diseases in a tertiary hospital in the city of Cali in Colombia. Methodology Data from the Valle Teaching Hospital's surveillance system was analysed, covering March 2004 to October 2008. The methodology proposed by the Collaborative Study of Congenital Malformations in Latin-America (ECLAMC) was followed for determining birth defect occurrence. Tables and graphs were constructed for describing the characteristics of the new-born, their parents and the resulting pregnancies. Results There were 32,995 births during the study period (4 years and 7 months); there was at least one birth defect in 735 of them, giving a 2.22 per 10,000 births prevalence. The major birth defects having the highest prevalence were polydactyly (22.1 per 10,000 births), clubfoot (17.6), hydrocephalus (16.4), neural tube defects (16.7), limb reduction defects (8.2), cleft lip or cleft palate (10.9), heart defects (14.6), hydronephrosis (11.5), gastroschisis (7.3), cyclopia (2.4) and sirenomelia (1.2), Discussion The prevalence of birth defects related to environmental factors such as hydrocephalus, limb reduction defects, clubfoot, hydronephrosis, gastroschisis, cyclopia and sirenomelia showed higher prevalence compared to those reported in the pertinent literature. Some hypotheses which could explain such increase are reported.

Incidental Abnormal Ocular Findings of Neonates in Ophthalmic Examinations

PURPOSE: To investigate incidental abnormal ocular findings that may cause visual impairment in neonates. METHODS: The medical records of patients requested for screening for retinopathy of prematurity (ROP) at our hospital's pediatrics department between March 2005 and July 2009 were retrospectively reviewed. Patients were classified into premature and mature neonates according to gestational age (under 37 weeks). RESULTS: A total of 2,090 eyes from 1,045 patients were comprised of 1,514 eyes of 757 premature neonates and 576 eyes of 288 mature neonates. Among 154 eyes of 78 patients (10.17%) who were diagnosed with ROP, 34 eyes of 18 patients had laser treatment. Seven eyes of 5 patients in the premature neonate group (0.46%), and 38 eyes of 23 patients in the mature neonate group (6.57%) were found to have abnormal ocular findings, excluding incidental ROP. Twenty-six eyes of 16 patients were found to have a retinal hemorrhage, and the majority had a birth history of perinatal distress, including birth asphyxia, meconium aspiration, and transient tachypnea of the newborn. Other findings included congenital cataracts, corneal opacity, macular coloboma, persistent hyperplastic primary vitreous, and abnormal fundus. CONCLUSIONS: The incidence of ocular abnormalities in neonates that cause visual impairment is low, but theses may threaten a child's life. Early detection and prompt treatment of ocular disorders in children are important in order to avoid permanent, lifelong visual impairment. Therefore, ophthalmic examinations are essential, even in normal neonates, for early detection of ocular disorders.

Unilateral Pulmonary Aplasia: A Case Report.

Chest X-Ray findings of unilateral lung or lobar collapse with a shift of mediastinal shift towards the affected side may prompt differential diagnoses of suspected foreign body aspiration, mucus plug occlusion, and bronchial mass lesions. We must also consider the rare condition of pulmonary agenesis. It is one of the rare congenital abnormalities in the development of the lungs in which there is complete absence of a lung. We report a three month old child with right sided pulmonary aplasia.

Congenital Defect of the Posterior Arch of Cervical Spine : Report of Three Cases and Review of the Current Literature

Abnormalities of the posterior arch, including congenitally absent cervical pedicle and cervical spondylolysis, are rare entities that are usually found incidentally on neck radiographs. It is important to recognize these characteristic radiological features because their radiographic appearance may cause them to be confused with more serious entities such as fractures, locked facets, and tumor-induced bony erosions. Also, it is important to distinguish these abnormalities from similar pathologies to prevent the use of inappropriate treatment. We report the relevant clinical and radiological findings seen in three cases of posterior arch defect after trauma with review of pertinent literature.

A Case of Aryepiglottoplasty with Apnea Technique in Laryngomalacia Patient / 대한이비인후과학회지

Laryngomalacia is the most common congenital laryngeal anomaly. Usually, the symptoms of laryngomalacis are mild and the disease does not interfere with the general body growth as it resolves itself spontaneously. Occasionally, however, it causes a failure to grow, apnea or cardiopulmonary diseases. We have recently experienced a case of severe laryngomalacia neonate. Aryepiglottoplasty with apnea technique was carried out under the general anesthesia using CO2 Laser.

Segmental Spinal Dysgenesis: Case report

Segmental spinal dysgenesis (SSD) is a rare congenital abnormality in which a segment of the spine and spinal cord fails to develop properly. Segmental vertebral anomalies involve the thoracolumbar, lumbar, or lumbosacral spine. We report two cases of SSD associated with other spinal dysraphism. Radiologic investigations revealed segmental dysgenesis of thoracic spinal cord without an associated vertebral bony anomaly in one case and segmental dysgenesisof the cervico-thoracic cord with diastematomyelia in the other case. The neurologic picture depends on the severity of the malformation and on its segmental level along the longitudinal embryonic axis. The pathogenesis of SSD syndrome is not clear but its cause is probably related to a segmental maldevelopment of the neural tube.

The Correction of Auricular Deformities Using Various Otoplasty Techniques / 대한이비인후과학회지

The otoplasty or auriculoplasty is characterized by the correction of morphological deformity of auricle including its accessory tissue and covering skin to normal or satisfying level to patient. The indication of this surgery is classified as congenital anomaly and secondary deformity caused by trauma and inflammation. Of these, the congenital auricular deformities are closely associated with the anatomical development of auricular muscle as well as cartilage malformation. In addition, the correction of secondary deformities should be based upon complete anatomical understanding of the auricle, so otolaryngologists have more superior accessibility to patients than plastic surgeons and can easily correct the deformity. In this paper, we investigated the types of each auricular deformity, the methods of surgical correction, and results through case reports commonly found in the field of otolaryngology with literature review.