Congenital mediastinal cysts are uncommon benign lesions generally caused by an abnormal embryological development of the foregut or coelomic cavity. They are expansive lesions, frequently asymptomatic that may manifest as a result from compression of adjacent structures. Bronchogenic, pericardial, enteric, thymic, esophageal duplication cysts and lymphangiomas are the main entities in this group of lesions. Congenital mediastinal cysts morphology is typical and imaging methods allow a correct diagnosis in a great number of cases. Surgical treatment is indicated only in cases of symptomatic lesions, considering that these lesions present no potential for malignant degeneration. The present study was aimed at commenting and illustrating the most relevant imaging findings of these lesions based on a retrospective review of ten cases collected in the records of the involved institutions.

Keywords: Congenital mediastinal cysts, Cystic neoplasms, Cysts

Thoracic asymmetries characterized by increased radiolucency of the lungs are important causes of morbidity in infants and children. Evaluation of affected patients frequently requires multiple imaging methods to diagnose the abnormality and to plan the surgical correction. The authors review and illustrate the characteristics of both common and rare thoracic asymmetries with emphasis on the radiological findings. The usefulness of different imaging methods in the diagnosis and treatment of these conditions is evaluated. The differential diagnosis as well the pitfalls and diagnostic difficulties are also discussed.

Keywords: Thoracic diseases, Congenital cystic adenomatoid malformation, Congenital lobar emphysema, Congenital diaphragmatic hernia

Although congenital heart defect is the most frequent anomaly in newborns, its antenatal detection rate through conventional ultrasound remains low. 3D and 4D ultrasound technology was developed early in the nineties, bringing great benefits in obstetrics, especially in cases of dubious diagnosis at 2D ultrasound. The spatio-temporal image correlation (STIC) is a significant development in the field of 4D ultrasound. A software coupled with a Voluson 730 Expert equipment allows a volumetric acquisition of the fetal heart and its vascular connections. Volumetric analyses are performed in multiplanar and surface rendering modes or, alternatively in association with color Doppler. Major advantages are fast images acquisition and the possibility of a later analysis by fetal echocardiography specialists. Differently from 2D ultrasound, this technique may be applied for evaluating any congenital cardiopathy because of the increased number of frames acquired from a specific anatomical region. Main disadvantage is related to the necessity of fetal immobility. The higher diffusion of this method may improve prenatal detection of heart malformations since volumes data captured by a sonographer may be sent through the Internet for analysis by fetal echocardiography specialists.

Keywords: Congenital heart defect, Prenatal diagnosis, STIC

Persistent hyperplastic primary vitreous (PHPV) is a congenital developmental anomaly of the eye that primarily affects premature infants. PHPV results from failure of regression of the embryogenic primary vitreous and hyaloid vasculature, and proliferation of connective tissue. Three types of PHPV may be found: anterior, posterior and a combination of anterior and posterior. The clinical findings include leukocoria (white pupillary reflex) and microphthalmic eye. This anomaly is usually unilateral and unassociated with other systemic findings. The differential diagnosis between PHPV and other ocular diseases may be difficult due to similar features such as leukocoria, detached retina, retinal folds and cataract. Other diseases with similar features include retinoblastoma, isolated congenital cataract, retinopathy of prematurity and pseudogliomas or leukocorias. Direct visualization of the remnants of the fetal hyaloid vascular system offers the best evidence of PHPV. However, diagnosis using the ophthalmoscope is sometimes impossible because of opaque tissues. In this circumstance an indirect visualization by ultrasound, computed tomography or magnetic resonance imaging may be useful. These imaging methods provide valuable information for the differentiation from other diseases, particularly retinoblastoma. The authors present a review of the literature and an iconographic study of the imaging findings in patients with PHPV.

Keywords: Persistent hyperplastic primary vitreous, Leukocoria, Hyaloid artery, Cloquet's canal

Persistent left superior vena cava with absent right superior vena cava is a rare anomaly, with less than 150 cases reported in the literature. Congenitally persistent left superior vena cava is the most common variant of systemic venous return to the heart, resulting embryologically from failure of the left anterior cardinal vein to become obliterated. Its incidence varies from 0.3% in patients with otherwise normal heart to 4.3% in patients with congenital heart disease. In the majority of the patients, a right superior vena cava is present as well, but rarely the right anterior cardinal vein degenerates resulting in the absence of the normal right superior vena cava. The blood from the right side is carried by the persistent left superior vena cava to the right atrium through the coronary sinus. We report the case of a patient with a persistent left superior vena cava and absence of right superior vena cava identified by chance during a chest radiograph and computed tomography examination for investigation of chronic pulmonary obstructive disease. The patient had no congenital heart disease and the blood from the right side was drained by the persistent left superior vena cava into the right atrium through the coronary sinus.

Keywords: Persistent left superior vena cava, Absent right superior vena cava, Chest radiography, Computed tomography

We report the radiographic findings in the long bones of a newborn with confirmed diagnosis of congenital syphilis and discuss the differential diagnosis with battered child syndrome. The radiographic findings in the long bones from birth to the fourth month of life are presented. Clinical and laboratory data, the serial x-ray films and the characteristic radiological signs were essential to establish the correct diagnosis.

Keywords: Congenital syphilis, Battered child syndrome, Bone fractures

Acalvaria is a rare congenital malformation of unknown pathogenesis characterized by the absence of the flat bones of the cranial vault, dura mater and associated muscles, while the central nervous system is usually preserved. The most accepted physiopathogenic theory suggests the presence of a postneurulation defect with normal placement the embryonic ectoderm. The present report describes neonatal imaging findings of primary acalvaria.

Keywords: Primary acalvaria; Skull defect; Congenital malformation.

Objetivo: A principal utilidade da tomografia por emissão de pósitrons com 18-fluordeoxiglicose (FDG-PET) está no estadiamento do câncer de pulmão. Porém, ela também pode ser utilizada para diferenciar lesões pulmonares indeterminadas, mas seu impacto na ressecção cirúrgica de lesões benignas é desconhecido. O objetivo deste estudo foi comparar a prevalência de lesões benignas em toracotomias feitas por suspeição de câncer de pulmão, antes e após a introdução do FDG-PET, em um centro de referência de cirurgia torácica. Materiais e Métodos: Os autores analisaram, prospectivamente, uma base de dados cirúrgicos de todos os pacientes consecutivos submetidos a toracotomia por câncer de pulmão suspeito ou comprovado e compararam a prevalência de lesões benignas em dois grupos ao longo de dois anos consecutivos, respectivamente antes (grupo I) e depois (grupo II) da introdução da FDG-PET. Resultados: Ressecção cirúrgica foi feita em 1.233 pacientes durante o período do estudo. A prevalência de lesões benignas na cirurgia nos grupos I e II foi similar (44/626 e 41/607, ambas correspondendo a 7%), e também no grupo II, entre aqueles submetidos a FDG-PET e os restantes (21/301 e 20/306 respectivamente, ambos correspondendo a 7%). No grupo II, dos 21 pacientes com lesões benignas submetidos a FDG-PET, 19 tiveram um estudo falso-positivo (valor médio padrão de captação 5.3 [faixa 2.6–12.7]). Desses, respectivamente 13 e 4 pacientes tiveram broncoscopia não diagnóstica e biópsia transtorácica percutânea de pulmão antes da toracotomia. Não houve diferença na proporção de lesões benignas diferentes ressecadas entre o grupo I e aqueles submetidos a FDG-PET no grupo II. Conclusão: A introdução da FDG-PET não alterou a proporção de pacientes submetidos a toracotomia por lesões benignas, principalmente devido à avidez pelo isótopo de algumas lesões não malignas. Tais resultados falsos-positivos devem ser considerados nos casos em que se contempla a possibilidade de ressecção cirúrgica em pacientes com câncer de pulmão não confirmado.

Keywords: Tomografia por emissão de pósitrons; Tomografia computadorizada; Pulmão – lesões benignas ou congênitas; Câncer de pulmão – diagnóstico e estadiamento; Câncer de pulmão – cirurgia.

Association of basal sphenoethmoidal encephalocele with midline cleft lip and palate is extremely rare. The authors report the case of a nine-year-old girl presenting a midline facial cleft with meningocele that was noticeable through the palatine defect as a medial intranasal pulsatile mass. An analysis of clinical and radiological findings of the present case of cranial dysraphism is carried out.

Keywords: Encephalocele; Cleft palate; Congenital malformation.

Poland's syndrome is a rare non-inherited congenital anomaly. The authors describe the classic radiologic findings of Poland's syndrome by reporting the case of a male four-year old patient with asymmetry of hands and chest, illustrating the fundamental imaging criteria for a conclusive diagnosis.

Keywords: Poland's syndrome; Chest asymmetry; Congenital malformation.

OBJETIVO: Avaliar a completude das fissuras, resposta radiológica ao tratamento e análise quantitativa por TC (AQTC) em população de pacientes com enfisema grave submetidos ao implante de valvas endobrônquicas (VEB). MATERIAIS E MÉTODOS: Foram estudados exames de TC multidetectores de 29 pacientes, realizados com protocolo de baixo dose, cortes finos, sem contraste. Dois radiologistas revisaram, retrospectivamente, os exames em consenso. A completude das fissuras foi estimada em escala de 5% e a resposta radiológica (atelectasia/perda volumétrica do lobo alvo) foi avaliada. AQTC foi realizada nos exames pré e pós-tratamento utilizando programa completamente automático. RESULTADOS: Resposta radiológica positiva foi vista em 16/29 pacientes. Os 13 pacientes sem resposta à TC apresentavam fissuras incompletas e completude média das fissuras obliquas de 72,8%, contra 88,3% no outro grupo. Os resultados mais significativos da AQTC mostraram redução do volume pulmonar (VP) total (média de 542 ml), VP tratado (700 ml) e volume de enfisema (331,4 ml) no grupo com resposta positiva, que também apresentou melhora nos testes funcionais. CONCLUSÃO: O benefício das VEB é maior em pacientes com fissuras completas e que desenvolvem atelectasia lobar. Nos pacientes sem resposta radiológica encontramos maior prevalência de fissuras incompletas e grau de incompletude das fissuras. Nossa AQTC automática detectou as alterações pós-tratamento, principalmente considerado a análise do pulmão tratado.

Keywords: Enfisema; Terapia de colapso pulmonar; Tomografia computadorizada.

Congenital upper urinary tract abnormalities imply a variable clinical spectrum of morphofunctional changes ranging from asymptomatic conditions to renal failure and incompatibility with life. Computed tomography, which has overcome excretory urography imaging, has been playing a key role in the diagnosis of congenital anomalies, serving as a better guidance in the therapeutic and surgical decision-making process, besides acting as an essential tool in the identification of associated complications and aiding in the performance of minimally invasive surgery techniques.

Keywords: Congenital abnormalities; Urinary tract; Multislice computed tomography.

Persistent stapedial artery is a rare congenital anomaly that occurs by a failure in the involution of such artery. Most patients with persistent stapedial artery are asymptomatic. The imaging diagnosis is made principally by means of multidetector computed tomography. In the present case, persistent stapedial artery was an incidental computed tomography finding. The authors discuss the embryogenesis, computed tomography findings and the importance of an early diagnosis of such anomaly.

Keywords: Persistent stapedial artery; Congenital; Tomography.

OBJECTIVE: To establish benchmarks and study some sonographic characteristics of the thyroid gland in a group of euthyroid children aged up to 5 years as compared with age-matched children with congenital hypothyroidism. MATERIALS AND METHODS: Thirty-six children (17 female and 19 male) aged between 2 months and 5 years were divided into two groups - 23 euthyroid children and 13 children with congenital hypothyroidism - and were called to undergo ultrasonography. RESULTS: In the group of euthyroid children (n = 23), mean total volume of the thyroid gland was 1.12 mL (minimum, 0.39 mL; maximum, 2.72 mL); a homogeneous gland was found in 17 children (73.91%) and 6 children (26.08%) had a heterogeneous gland. In the group of children with congenital hypothyroidism (n = 13), mean total volume of the thyroid gland was 2.73 mL (minimum, 0.20 mL; maximum, 11.00 mL). As regards thyroid location, 3 patients (23.07%) had ectopic thyroid, and 10 (69.23%) had topic thyroid, and out of the latter, 5 had a homogeneous gland (50%) and 5, a heterogeneous gland (50%). In the group with congenital hypothyroidism, 6 (46.15%) children had etiological diagnosis of dyshormoniogenesis, 3 (23.07%), of ectopic thyroid, and 4 (30.76%), of thyroid hypoplasia. CONCLUSION: Thyroid ultrasonography is a noninvasive imaging method, widely available, easy to perform and for these reasons could, and should, be performed at any time, including at birth, with no preparation or treatment discontinuation, to aid in the early etiological definition of congenital hypothyroidism.

Keywords: Congenital hypothyroidism; Ultrasonography; Main diagnosis.

OBJETIVO: Avaliar os padrões tomográficos relacionados aos agentes etiológicos da pneumonia em pacientes durante o primeiro ano após transplante renal. MATERIAIS E MÉTODOS: Foram analisados dados de prontuários de 956 pacientes submetidos a transplante renal, no período de 2013 a 2018, em um centro transplantador renal do nordeste do Brasil. Nos pacientes que desenvolveram pneumonia, os agentes etiológicos foram classificados em bactérias piogênicas, micobactérias, fungos, vírus e pneumonia polimicrobiana, enquanto os padrões tomográficos foram classificados em consolidação, broncopneumonia, pneumonia intersticial e nódulos e massas. Para verificar associação estatística entre micro-organismos e padrões tomográficos, foi utilizado o teste exato de Fisher, com p < 0,001. RESULTADOS: Foram encontrados 101 casos de pneumonia, dos quais 60 (59,4%) tiveram agente etiológico identificado, sendo as bactérias piogênicas as mais frequentes, detectadas em 22 (36,7%) dos casos. Entre os pacientes com agente causal identificado, o padrão tomográfico predominante foi o de nódulos e massas, identificado em 25 (41,7%) casos. Foi observada associação entre bactérias piogênicas e o padrão de consolidação, fungos com nódulos e massas, bem como entre agentes virais e padrão intersticial. CONCLUSÃO: Foi demonstrada associação estatística entre micro-organismos causadores de pneumonia e padrões tomográficos, informação que pode contribuir para o planejamento da terapia de pacientes transplantados renais.

Keywords: Pneumonia; Transplante de rim; Tomografia computadorizada.