Asociación de malformación esternal y displasia vascular en un lactante / Association of sternal malformation and vascular dysplasia in an infant

Se describe el caso clínico de un lactante primogénito de 3 meses de edad, que había sido evaluado por un especialista de Genética Clínica en el Hospital Infantil Norte "Dr. Juan de la Cruz Martínez Maceira" de Santiago de Cuba, por presentar hendidura del tercio inferior del esternón. Posteriormente aparecieron hemangiomas en el labio inferior, el lóbulo de la oreja derecha y la parte lateral derecha del cuello, así como un rafe abdominal medio desde la hendidura esternal hasta el ombligo. Se practicaron los exámenes complementarios requeridos y se interconsultó con varias especialidades, con lo cual se concluyó que el paciente presentaba una asociación de malformación esternal y displasia vascular. Se trata del primer caso notificado en Cuba.

The clinical case of a 3 months first-born infant is described who had been evaluated by a specialist of Clinical Genetics in "Dr. Juan de la Cruz Martínez Maceira" Northern Pediatric Hospital in Santiago de Cuba, for presenting a fissure in the inferior third of the sternum. Later on hemangiomas emerged in the inferior lip, the lobe of the right ear and the lateral right part of the neck, as well as a mid abdominal raphe from the sternal fissure to the navel. The required complementary tests were carried out and she was scheduled for several specialties, with which it was concluded that the patient presented an association of sternal malformation and vascular dysplasia. It is the first case notified in Cuba.

High prevalence of sternal foramen: quantitative, anatomical analysis and its clinical implications in acupuncture practice / Alta prevalencia del foramen esternal: análisis cuantitativo y anatómico y sus implicancias clínicas en la práctica de la acupuntura

The aim of our study was to verify the prevalence of the sternal foramen in adult cadavers and dry bones, as well as to determine its exact localization and relation to acupuncture points. One hundred eighty sternums fixed and contained in 10 percent formaldehyde solution were examined, of which 100 were dry bones and 80 belonged to adult cadavers. The morphometric measures were expressed in millimeters when measuring the length: 1) from jugular incision to the foramen (JI-F); 2) from sternal angle to the foramen (EA-F); 3) from xiphoid process to the foramen (XP-F). Statistical analysis was performed to compare expected vs obtained foramina frequency, using Fisher's test (p0.05 was considered significant). The results showed 16.6 percent sternal foramina, significant higher than expected (p0.0248). Of the 30 foramina, 53.3 percent were found in cadavers, of which one was a woman, and 46.7 percent sternums were found in dry bones. Of the cadavers with sternal foramen, 10 foramina were at the 5th sternochondral articulation level and 6 foramina at the 4th sternochondral level. Of the 14 analyzed dry bones, 9 foramina were at the space between the 4th and the 5th costal pit level and 5 foramina were at the 5th costal pit level. The mean±SD (mm) total length of the sternums was 151.8+/-34 and the measures JI-F, EA-F, and XP-F were, respectively, 103.8+/-22.1mm, 65.6+/-15mm and 46.9 +/-15mm. Knowledge of this occurrence is important to avoid serious heart injury by needle insertion, especially as this area holds a commonly used acupuncture point and sternal puncture...

El objetivo del estudio fue verificar la prevalencia del foramen esternal en cadáveres adultos y huesos secos, así como, determinar su localización y su relación con los puntos de acupuntura. Fueron examinados 180 esternones fijados en solución de formol al 10 por ciento, 100 eran huesos secos y 80 pertenecían a cadáveres de individuos adultos. Las medidas morfométricas se expresaron en milímetros, efectuándose las siguientes mediciones: 1) desde la incisura yugular al foramen; 2) desde el ángulo esternal al foramen y 3) desde el proceso xifoides hasta al foramen. El análisis estadístico permitió comparar la frecuencia de los forámenes, usando el test de Fisher (p 0,05 ). Los resultados mostraron la presencia del foramen esternal en 16,6 por ciento de los casos, siendo significativamente mayor a lo esperado (p 0,0248). De los 30 forámenes, el 53,3 por ciento se encontró en cadáveres y en el 46,7 por ciento en los huesos secos. De los cadáveres con foramen esternal, 10 forámenes se encontraban a nivel de la 5 articulación esternocondral y 6 a nivel de la 4 articulación esternocondral. De los 14 forámenes de los huesos secos, 9 forámenes se encontraban entre entre el 4 y el 5 espacio intercostal y 5 se localizaban a nivel del 5 articulación. La longitud total del esternón fue 151,8 mm +/- 34 y las medidas desde la incisura yugular al foramen, desde el ángulo esternal al foramen y desde el proceso xifoides hasta al foramen fueron, 103,8 +/- 22,1 mm, 65,6 +/- 15 mm y 46,9 +/- 15 mm, respectivamente. El conocimiento de este hecho es importante para evitar la lesión cardiaca grave por la inserción de agujas, ya que esta zona tiene un punto de acupuntura de uso común en la punción esternal...

The prevalence and effects of pectus excavatum and pectus carinatum on the respiratory function in children between 7–14 years old.

The study involved 1342 primary school students aged 7–14 years who applied to Ankara, a primary care center for general health check-up between 2006 and 2007. Forty-three students, 35 of whom had PE and 8 of whom had PC, were subjected to thorax measurement. All 43 students underwent pulmonary function tests (PFT).The prevalence rate of PC was 0.6%, and of PE, 2.6%. The thorax widths of the groups were similar (P=0.273). The thorax circumference and depth of PE group were lower than those of the controls (P<0.05). The probability rate of abnormality in PFT scores of PE group was statistically significantly higher than that of the controls (P=0.022) whereas absence of normal PFT scores the difference between PC group and the controls was not statistically significant (p=0.095). The results indicate that more than half of the individuals with pectus deformity do not have any physical complaints and do not have statistically significant differences in their PFT parameters.

Successful Vaginal Delivery of a Pregnant Woman with Cantrell's Pentalogy

Cantrell's Pentalogy is a rare condition that consists of defects involving the abdominal wall, lower sternum, anterior diaphragm, pericardium, and heart. In the literature to date, pregnant women with Cantrell's Pentalogy have not been discussed. We performed successful vaginal delivery of a 23-yr-old nulliparous, primigravid woman who had been diagnosed with this condition. Diagnosis was based on cardiac catheterization, angiography, and echocardiogram, and abdominopelvic CT. Vaginal delivery may be an option for women with Cantrell's Pentalogy and may be attempted with caution.

Pentalogía de Cantrell en un recién nacido expuesto en útero a misoprostol / Pentalogy of Cantrell in a newborn associated with prenatal exposure to misoprostol

El misoprostol es un análogo sintético de la prostaglandina E1 y es usada por sus efectos utero-tónicos como abortivo. En los países donde se utiliza como abortivo sin éxito durante el primer trimestre de gestación, se han informado un espectro de malformaciones congenitas asociadas con la exposición prenatal, que incluyen el síndrome de Moebius, defectos de la pared abdominal, artrogriposis y anomalías por reducción de los miembros, entre otros. La pentalogía de Cantrell (PC), con una prevalencia de 1 en 65.000 nacidos vivos, es un síndrome polimalformativo que consta de 5 anomalías: un defecto de la pared abdominal, defecto esternal inferior, defecto del diafragma anterior, defecto pericárdico diafragmático y anormalidades congenitas del corazón. Se reporta un caso de pentalogía de Cantrell asociado al uso de misoprostol en el primer trimestre de la gestación, siendo este reporte el primer registro de esta asociación.

Misoprostol is a synthetic analogue of prostaglandin E1 and is used for effects such as uterotonic aborti-facient. In countries where abortion is used as unsuccessful in the first trimester of gestation, reported a spectrum of congenital malformations associated with prenatal exposure, which include Moebius syndrome, abdominal wall defects, and anomalies as artrogriposis reduction members, among others. The pentalogy of Cantrell with a prevalence of 1 in 65,000 live births, is a syndrome consisting of 5 abnormalities: a defect in the abdominal wall, lower sternal defect, defect above the diaphragm, diaphragmatic pericardial defect, and congenital abnormalities of the heart. We report a case of Pentalogy of Cantrell associated with the use of misoprostol in the first trimester of pregnancy, this report is the first record of this association.

Pectus carinatum / Pectus carinatum

Dentre as deformidades da parede torácica, o pectus carinatum não tem recebido o mesmo grau de interesse que o pectus excavatum, sendo sua abordagem desconhecida por grande parcela dos pneumologistas, pediatras e cirurgiões torácicos. Isto faz com que estes pacientes não sejam encaminhados para tratamento. Trata-se de deformidade com incidência de 1:1000 adolescentes, oligosintomática, mas que leva a consultas médicas por implicações de ordem estética e emocional, sendo seus portadores introvertidos, não praticantes de exercícios físicos e não freqüentadores de praias ou piscinas para não expor o tórax. O diagnóstico é clínico e visual, e detalhes são obtidos com a radiografia do tórax e a tomografia computadorizada. O tratamento é baseado em organograma bem conhecido, que resume as condutas ortopédicas e cirúrgicas. O compressor dinâmico do tórax, associado a exercícios físicos, é indicado nos adolescentes com tórax flexível no pectus carinatum inferior e pectus carinatum lateral, tendo indicação limitada no pectus carinatum superior. A indicação cirúrgica é feita pela presença da deformidade e com motivação estética, em adolescentes com tórax não flexível, em jovens, e em adultos. Dentre as técnicas relatadas, destaca-se a esternocondroplastia modificada, pelos excelentes resultados estéticos alcançados.

Among the deformities of the thoracic wall,pectus carinatum has not received the same attention as has pectus excavatum. Few pulmonologists, pediatricians, and thoracic surgeons are aware of the approaches to treating this condition. As a consequence, patients with pectus carinatum are not referred for treatment. This deformity, with an incidence of 1:1000 teenagers, is oligosymptomatic. However, for aesthetic and emotional reasons, it accounts for a large number of medical appointments. Such patients are introverted and do not engage in physical activities, since they are unwilling to expose their chest, which also discourages them from going to the beach or to swimming pools. The diagnosis is clinical and visual, and details are obtained through chest X-rays and computed tomography. The treatment is based on a well-known organogram that summarizes orthopedic and surgical procedures. Dynamic compression, combined with physical exercises, is indicated for teenagers with flexible thorax in inferior and lateral pectus carinatum, with limited indication for those with superior pectus carinatum. For individuals of any age with rigid thorax, surgery is indicated for aesthetic reasons. Among the techniques described, the modified sternum chondroplasty stands out due to the excellent aesthetic results achieved.

Pentalogía de Cantrell. A propósito de un caso / Pentalogy of Cantrell. A case report

Ectopia cordis is an extremely rare cardiac anomaly. The heart is localized partially or totally outside the thorax cavity. This anomaly occurs as an isolated defect or combined with others midline defects. Cantrell and colleagues described, in 1958, a syndrome including defects of the abdominal wall, sternum, diaphragm, pericardium and heart. There are few successful surgical cases with this pentalogy. We describe a case with this Cantrell's pentalogy. The cardiac malformation was a univentricular heart with pulmonary stenosis. The patient underwent successful surgical palliation with a systemic-to-pulmonary anastomosis and uneventful recovering.

Surgical reconstruction for absence of sternum and pericardium in a newborn.

We report the surgical management of a 3 day old baby with absent sternum and pericardium with impending rupture of the skin cover. The repair described is innovative and may be used in place of primary repair by bringing the ribs together. Review of embryology and various modalities of closure are provided.

[ teratogenic effect of lamotrigine on vertebral column, sternum and ribs of mice fetus]

One of the most important complications of anti-epileptic drugs [AEDs], used during pregnancy, is the increasing of congenital malformation in neonates. One of these drugs, lamotrigine [LTG], in addition to treatment of epilepsy, is used in many of the chronic neural diseases. There are a few controversial reports about teratogenic effect of this drug. Based on these reports, oral usage of this drug is not teratogenic. Therefore, the aim of this study was to investigate about the teratogenic effect of this drug on a number of mice fetuses following the interaperitoneal injections. In this study, 150 mice were divided in four groups: 1] control 1, no material administration, 2] control 2, ip ethanol solution was administrated, 3] case 1, LTG was administrated ip [25 mg/kg, tid] 4] fourth group [case 2], LTG was administrated ip 3 time/day [75 mg/kg] from the 9th to 18th day of gestation. Fetuses were collected on the GD 18 and then some of embryos were used for alizarin red and alcian blue staining. By this method of staining soft tissue would become clear, cartilages become blue, and bones turn to red. Then malformations in vertebral column, sternum and ribs were considered in four groups and were compared. In the group treated with LTG, different types of skeletal malformations were increased [p<0.001] and the incretion was drug dose-dependent. The results of this study showed that LTG is likely a risk factor in occurrence of skeletal malformation in mice fetus

Hendidura esternal total en un recién nacido y parcial en una niña de 4 años / Cleft sternum: 2 clinical cases

La hendidura esternal es una malformación congénita de la pared torácica que se origina en una falla embrionaria de la fusión de las valvas esternales en la línea media. El defecto debe ser reparado precozmente al nacer, para reestablecer la protección ósea de las estructuras del mediastino, prevenir el movimiento paradojal de las vísceras en la respiración, eliminar la deformidad visible y permitir un desarrollo normal de la caja torácica. Objetivo: Notificar 2 pacientes portadores de esta infrecuente malformación y revisar sus características clínicas, permitiendo realizar un diagnostico preciso, orientar el estudio y definir un adecuado tratamiento. Casos clínicos: Recién nacido masculino con una hendidura esternal completa que provoca un "distress" respiratorio y una preescolar de 4 años, con una hendidura parcial del tercio superior esternal, asociada a una cardiopatía congénita operada. Los pacientes son sometidos a una reparación quirúrgica que permitió un alta precoz y una evolución clínica favorable. Conclusión: La Hendidura Esternal es una malformación de baja frecuencia que debe ser corregida precozmente para evitar el uso de técnicas de mayor complejidad con resultados variables.

Malformaciones de la pared torácica en el adolescente y el adulto: experiencia quirúrgica / Techniques and results in the thoracic wall malformations surgical treatment

Antecedentes: El tratamiento de las anomalías de la pared torácica fue encarado desde los comienzos de la cirugía torácica. Tratándose de una patología de presentación proteiforme, múltiples han sido los métodos que aquella ha propuesto para su resolución. La implicancia psicológica que esta malformación conlleva es por lo general el motivo debido al cual debe ser resuelta. Objetivo: Exposición de técnicas y resultados obtenidos en la resolución de las malformaciones de la pared torácica. Lugar: Hospital Francés de la ciudad de Buenos Aires, Argentina. Diseño: Retrospectivo y descriptivo. Población: 38 pacientes operados con variada patología de pared torácica, atendidos en un hospital de comunidad de Buenos Aires. Método: De acuerdo a los distintos tipos de patologías presentadas fueron utilizadas las osteocondrotomías con soportes metálicos retroesternales y fijación externa con materiales termoplásticos temporarios. En algunas oportunidades fue necesario la aplicación de prótesis acrílicas mamarias. Resultados: De 38 pacientes operados, la conformidad absoluta fue obtenida en el 52 por ciento de los casos, la conformidad en 15 por ciento y se encontraron disconformes en 7,9 por ciento de los casos. La mejoría de la capacidad respiratoria fue escasamente significativa en los casos tratados. Conclusiones: Las malformaciones de la pared torácica se presentan con escasa frecuencia. Deben ser resueltas por su implicancia psicológica. Es una patología proteiforme por lo que han de conocerse las diferentes técnicas propuestas. Las osteocondrotomías con sujeción transtorácica mediante clavos de acero y sujeción externa temporal con plancha termoplástica acrílica sujeta a las costillas subyacentes, dieron muy buenos resultados en la mayoría de los casos

Pectus excavatum: avances en el tratamiento / Pectus excavatum: advances in the treatment

Trabajo presentado en la Sesión Plenaria del Congreso del American College of Surgeons, Santiago, Chile, 2003. Premio al Mejor Trabajo Científico. El pectus excavatum, pecho excavado, hundido o en embudo es la malformación torácica más frecuente. Se presenta en uno de cada 1000 recién nacidos. La deformidad se caracteriza por la presencia de una profunda depresión esternal. En los casos sintomáticos, el manejo es fundamentalmente quirúrgico. El objetivo es reportar una experiencia clínica de 30 pacientes portadores de pectus excavatum tratados con una novedosa técnica quirúrgica mínimamente invasiva con apoyo de videotoracoscopía, denominada Técnica de Nuss, que consiste en el implante, temporal, de una barra metálica retroesternal.

Disorders in the sternal growth and "pectus" deformities: an experimental model and clinical correlation

O crescimento da parede torácica anterior nao tem sido bem explorado. Controvérsia sobre esse assunto inclui a descriçao de suturas no esterno por alguns autores. Os objetivos deste estudo sao demonstrar que placas de crescimento cartilaginosas estao presentes entre os segmentos ósseos do esterno em crescimento e que lesao dessas placas pode causar deformidades pectus. A parede torácica anterior normal de ratos Sprague-Dawley e a de três crianças foram comparadas macroscópica e histologicamente. Foi desenvolvido um modelo experimental no qual a epifisiodese parcial das placas de crescimento esternais resultou em deformidades pectus. Um caso clínico no qual o distúrbio do crescimento do esterno, causado iatrogenicamente por cirurgia, resultou em deformidade pectus em uma criança foi correlacionado com o modelo animal. Placas de crescimento cartilaginosas existem entre os segmentos ósseos do esterno em crescimento. Lesao dessas placas de crescimento resulta em deformidade pectus. Deformidades da parede torácica anterior podem ser causadas por distúrbios de crescimento das placas de crescimento esternais. Lesoes dessas placas de crescimento em um modelo animal experimental e lesoes causadas iatrogenicamente em um paciente demonstram a formaçao de deformidades pectus.

Diagnóstico antenatal de pentalogía de Cantrell / Prenatal diagnosis of Cantrell's pentalogy

Presentamos un caso clínico de diagnóstico antenatal de Pentalogía de Cantrell. Se discute diagnóstico y manejo perinatal

Correçäo cirúrgica de fenda esternal total: relato de caso / Surgical correction of the total clefts sternum: case report

A fenda esternal total é uma doença grave, rara e que traz repercussöes clínicas importantes quando näo tratada precocemente. Os autores procuram aqui relatar um caso de fenda esternal total que foi submetido a correçäo cirúrgica no Hospital Infantil Pequeno Príncipe, cuja evoluçäo pós-operatória imediata foi bem sucedida. A raridade da doença demonstra a pouca experiência no tratamento cirúrgico da fenda esternal total na literatura mundial