Postnatal CT Research Articles

Presumed Zika virus-related congenital brain malformations: the spectrum of CT and MRI findings in fetuses and newborns.

The new epidemic of Zika virus infection raises grave concerns, especially with the increasingly-recognized link between emerging cases of microcephaly and this infectious disease. Besides small cranial dimensions, there are striking morphologic anomalies in the fetal brain. Key anomalies include cortical developmental malformations and a peculiar distribution of pathologic calcifications. These potentially indicate a new pattern of congenital central nervous system infection. Eight women underwent fetal MRI. Four infants also underwent postnatal CT. Five of the women underwent amniocentesis. All neonates were born with microcephaly. On fetal MRI, ventriculomegaly, marked reduction of white matter thickness, severe sylvian fissure simplification, abnormal sulcation, and diffuse volumetric loss of cerebellar hemispheres were consistently seen. On postnatal CT, diffuse subcortical and basal ganglia calcifications were observed. The Zika virus was detected in two amniocenteses by polymerase chain reaction assays. We hope to assist the medical community in recognizing the spectrum of encephalic changes related to congenital Zika virus infection.

P28.01: Congenital lung malformations: correlation between pre‐ and postnatal imaging

To assess the accuracy of prenatal ultrasound and MRI in congenital lung malformations lesions and to compare prenatal findings with postnatal thoracic CT scan data. This retrospective study included all cases referred to our unit for congenital lung malformation after ultrasound screening, between May 2011 and April 2016. Only patients who underwent at least one postnatal thoracic CT examination during the first year of life were analysed. The prenatal findings at Ultrasound and fetal MRI were compared to final diagnosis as established by postnatal thoracic CT scans. Twenty fetuses were included after ultrasound screening at a median gestational age of 24 weeks (range 22-26). Fetal MRI were performed at a median gestational age of 31.2 (range 29 to 33.5). Ultrasound suspected 14 microcystic Congenital Cystic Adenomatoid Malformation (CCAM), 3 broncho-pulmonary sequestrations (BPS) and 3 macrocystic CCAM. At MRI there were 3 BPS, 5 microcystic CCAM, 3 macrocystic CCAM, 8 bronchial obstructions and 1 vanishing lesion. Postnatal CT scans found 9 bronchial obstructions (8 bronchial atresia, 1 compressive bronchogenic cyst), 4 BPS (in out of 3 were associated with cystic malformations), 2 microcyctic CCAM, 3 macrocystic CCAM and 2 vanishing masses. Appropriate diagnosis was reached in 8/20 (40%) cases and 17/20 cases (85%) based on US and MRI respectively (p=0,003). Final diagnosis was correctly assessed by US and MRI as follow: for bronchial obstructions 0/9 (0%) and 8/9 (88,8%) p=0,003, for BPS 3/4 (75%) and 3/4 (75%) p=1, for microcystic CCAM 2/2 and 2/2 (100%) p=1, macrocystic CCAM 3/3 and 3/3 (100%) p=1. In fetuses with suspicion of congenital lung malformations, fetal MRI performs better than US in establishing the exact diagnosis, especially in cases of bronchial obstructions. MRI should therefore be considered as a systematic adjunct to US in the management of these pathologies.

The natural history of prenatally diagnosed congenital cystic lung lesions: long-term follow-up of 119 cases

BackgroundA paucity of evidence regarding the natural history of congenital pulmonary airway malformations (CPAMs) and pulmonary sequestration (PS) has resulted in a divergence in management strategy of asymptomatic cases.MethodsWe describe...

Congenital overinflation: prenatal MRI and US findings and outcomes

The purpose is to describe fetal MR and US findings of congenital overinflation (CO) and to correlate with postnatal outcome. Two radiologists reviewed fetal MR and US images in 25 fetuses diagnosed with CO. Lesion size, appearance, location, and presence of hydrops were documented. Chart review was performed for pregnancy outcome, postnatal imaging, interventions, histopathology, and clinical outcome. All lesions demonstrated primarily homogeneous increased echogenicity and MR signal with absent pulmonary vascular distortion. A tubular cystic hilar structure was consistent with a dilated bronchus (68% MR, 25% US). The right lower (32%) and left lower (23%) lobes were most commonly involved. Two cases with central bronchial obstruction resulted in perinatal demise. Of 23 live births, 17 were asymptomatic, 1 symptomatic, and 5 lost to follow-up. Postnatal CT was performed in 17 of 18 patients confirming CO. Histopathology in nine patients revealed bronchial anomalies with hyperinflated (n = 7) or polyalveolar lung (n = 2). Nine patients were observed and remained asymptomatic. Fetal MR and US demonstrate a consistent pattern of imaging findings in fetuses with CO. Many cases are asymptomatic and can be managed with nonsurgical conservative therapy. CO because of central bronchial obstruction is associated with a guarded prognosis. © 2016 John Wiley & Sons, Ltd.

Surgical treatment of congenital cystic adenomatoid malformation: a retrospective study of single tertiary center experience

Objective: To summarize the experience drawn from treatment of fetus with congenital cystic adenomatoid malformation (CCAM).Methods: This retrospective report has reviewed a clinical history of 115 patients with CCAM from September 2012 to June 2014. All cases were divided into two groups according to symptomatology: group A (n = 96, asymptomatic group) and group B (n = 19, symptomatic group). Major factors were compared between two groups. Numerical data were presented as the median (range). The difference between median was performed with ANOVA.Results: The mean gestational age of prenatal diagnosis was 24.3 weeks (range, 17–36). 49 cases with CCAM volume ratio (CVR) were retrospectively analyzed, with a median max CVR of 0.95 (range, 0.19–3.2), In the 115 cases, mean operation age was (3.7 ± 0.32) months old. Symptom is significantly associated with lesion size and chest radiographs (CXR) (p < 0.05). Compared with symptomatic CCAM patients, asymptomatic patients were associated with better clinical outcome.Conclusions: The value of CVR to evaluate prognosis showed no statistical significance. Although CXR are not reliable indicators to assess the lesion, it can predict the emergency situation. Due to potential trend of symptomatic and poor postoperative symptoms group, early postnatal CT scan and early surgical treatment are suggested.

Vanishing fetal lung malformations: Prenatal sonographic characteristics and postnatal outcomes

Background/purposeThe purpose of this study was to examine the natural history and outcomes of prenatally diagnosed lung masses that appear to undergo complete regression before birth. MethodsAn IRB-approved retrospective review was performed on 100 consecutive fetuses with a congenital lung malformation at a single fetal center. Prenatal and postnatal imaging as well as outcomes of vanishing fetal masses was analyzed and compared to those with persistent fetal masses. ResultsSeventeen lesions (17%) became sonographically undetectable at 35.3±2.3weeks gestation. Vanishing fetal masses were associated with microcystic disease (100% vs. 69%, p=0.005) and a low initial congenital pulmonary airway malformation volume ratio (CVR; 0.31±0.35 vs. 0.70±0.66, p=0.002) when compared to those with persistent fetal lesions. Based on postnatal CT imaging and pathology data, 10.3% of all fetal masses completely regressed. The positive predictive value and negative predictive value of prenatal ultrasound for detecting lung malformations in late gestation were 96% and 43%, respectively. All infants with vanishing fetal lesions were asymptomatic at birth and were more likely to be managed nonoperatively (75% vs. 22%, p<0.0001) when compared to infants with persistent fetal masses. ConclusionsVanishing lung lesions late in gestation are relatively common and are associated with a low CVR and microcystic disease.

Majewski Syndrome, Short Rib Polydactyly Syndrome (SRPS) Type II - A Rare Case Report, with Review of Literature

Short Rib Polydactyly Syndrome (SRPS) type II also known as Majewski syndrome, is the rarest of the four subtypes of SRPS which is a rare inherited skeletal dysplasia. We report a case of Majewski syndrome in a neonate with brief review of literature. A 24 years old primigravida, with history of second degree consanguineous marriage underwent a Prenatal USG which revealed anhydramnios, bilateral enlarged kidneys, extremely narrow thorax and bilateral short limbs with polydactyly. Based on these findings a probable diagnosis of lethal skeletal dysplasia was made and termination of pregnancy advised. Refusing termination the mother delivered a male foetus at 30 weeks with severe respiratory distress and gross anomalies. The foetus succumbed to respiratory failure inspite of resuscitation. Gross findings were a male Case Study Andola et al.; BJMMR, 9(12): 1-8, 2015; Article no.BJMMR.18367 2 foetus with enlarged head, hydropic face, hypertelorism, short nose, depressed nasal bridge, pseudo cleft lip, cleft palate, low set posterior rotated ears, cystic hygroma, micrognathia, short and narrow chest, all the limbs showed mesomelic limb shortening, postaxial polydactyly, syndactyly and brachydactyly. Postnatal X-ray, CT scan and USG confirmed the above mentioned features and additional findings were extremely short horizontal ribs and disproportionately shortened ovoid tibia. Autopsy revealed a bell shaped thorax, small hypoplastic lungs, bilateral enlarged kidneys, atrial septal defect, hypoplastic epiglottis and larynx and short small intestine. Microscopic findings of pulmonary hypoplasia, renal cystic dysplasia, hepatic fibrosis and markedly retarded endochondral ossification correlate with the clinical, radiological and pathological findings of Short Rib Polydactyly Syndrome Type II -Majewski Syndrome.

Fetal MRI Correlates with Postnatal CT Angiogram Assessment of Pulmonary Anatomy in Tetralogy of Fallot with Absent Pulmonary Valve

In tetralogy of Fallot with absent pulmonary valve, pulmonary stenosis and regurgitation results in significant pulmonary artery dilatation. Branch pulmonary artery dilatation often compresses the tracheobronchial tree, causing fluid trapping in fetal life and air trapping and/or atelectasis after birth. Prenatal diagnosis predicts poor prognosis, which depends on the degree of respiratory insufficiency from airway compromise and lung parenchymal disease after birth. Fetal magnetic resonance imaging (MRI) has been useful in evaluating the effects of congenital lung lesions on lung development and indicating severity of pulmonary hypoplasia. This report is the first demonstrating the utility of fetal MRI in tetralogy of Fallot/absent pulmonary valve patients, which predicted postnatal pulmonary artery size and visualized airway compression and lung parenchymal lesions. The distribution of lobar fluid trapping on fetal MRI correlated with air trapping on postnatal computed tomography angiogram.

Comparison between US and MRI in the prenatal assessment of lung malformations

The contribution of MRI in the prenatal evaluation of congenital lung abnormalities (CLA) has not been extensively investigated. (1) To compare diagnostic accuracy and assessment of prognostic factors between US and MRI in CLA and (2) to assess the diagnosis agreement between prenatal imaging and postnatal diagnosis. We included 23 consecutive fetuses who underwent concomitant US and MRI during gestation as well as postnatal CT and surgery (n = 22). US-MRI sets were performed at median gestational age of 26 (n = 16) and 34 (n = 22) weeks. Postnatal diagnoses were 11 congenital pulmonary airway malformations (CPAM), 4 bronchopulmonary sequestrations (BPS), 6 hybrid lesions and 2 cysts. US and MRI agreement was significantly better during the second trimester than during the third one (P = 0.02). Disagreements were related to missed cysts (n = 5), mediastinal shift (n = 6) and vessels (n = 5). US and MRI diagnosis agreement was present in 20 cases, including 5 cases of misdiagnosis. US and MRI were concordant with postnatal diagnosis in 17 and 16 cases, respectively. In our series, no clear superiority of MRI over US in the prenatal evaluation of CLA was demonstrated, but US better demonstrated systemic feeding vessels and MRI cysts and normal lung adjacent to the lesion.

Infradiaphragmatic extralobar pulmonary sequestration: Masquerading as suprarenal mass

Pulmonary sequestration is a rare malformation, wherein a portion of lung is non-functional and is not in normal continuity with the tracheo-bronchial tree, and may derive its blood supply from systemic vessels. Two types are described: Intralobar and extralobar types. Intralobar sequestration is more common type, which shares visceral pleura of the involved lobe and is localized within the normal pulmonary parenchyma. Whereas extralobar forms are uncommon and are totally separate from the lung and usually have own covering. Infra-diaphragmatic pulmonary sequestration is of extralobar type and is extremely rare, and usually is associated with other congenital malformations. We present an extremely rare case of isolated infra-diaphragmatic pulmonary sequestration which was antenatally detected and followed up with postnatal CT scan, where it masqueraded as suprarenal mass, and was surgically treated. This case emphasises to add a differential diagnosis of malformation in congenital supra-renal masses, which remain stable in size and appearance, and hence avoid immediate surgery.

Antenatal sonographic appearance of a large orbital encephalocele: A case report and differential diagnosis of orbital cystic mass

Orbital meningoceles and encephaloceles are rare extracranial extensions of the brain and meninges with or without direct communication between the central nervous system and the abnormal mass. We reported a rare case of large fetal orbital encephalocele; the diagnosis was suspected initially by prenatal ultrasound and confirmed by postnatal MRI and CT scans. The differential diagnosis of an intrauterine fetal cystic orbital mass includes orbital teratoma, epidermoid inclusion cysts, hemangioma or lymphangioma, congenital cystic eye, dacryocystocele, and orbital cephalocele.

Anterior mediastinal lymphangioma: Pre‐ and postnatal sonographic findings

Anterior mediastinal lymphangiomas are very rare in utero with only a few cases reported in literature. We present a case of anterior mediastinal lymphangioma that was diagnosed on prenatal sonography (US) at 22 weeks' gestation. It appeared as a well-defined, multi-septated anechoic mass, in the anterior mediastinum between the heart base and right chest wall. There was no solid component and no internal flow on color Doppler US. The pre- and postnatal US findings are correlated with prenatal MRI and postnatal CT findings.

Crouzon syndrome associated with acanthosis nigricans: Prenatal 2D and 3D ultrasound findings and postnatal 3D CT findings

Crouzon syndrome with acanthosis nigricans (CAN) is a very rare condition with an approximate prevalence of 1 per 1 million newborns. We add the first report on prenatal 2D and 3D ultrasound findings in CAN. In addition we present the postnatal 3D CT findings. The diagnosis was confirmed by molecular testing.

1171 Relationship Between Cardiac Rhabdomyomas and Tuberous Sclerosis Complex

TSC is an autosomal dominant disorder characterized by widespread harmatomas, usually occurring in the brain, eyes, skin, kidney, liver, heart and lungs, resulting in a variable clinical manifestation. Rhabdomyomas is most common cardiac tumor to be detected on fetal US. The detection of a rhabdomyoma is maybe useful for a prenatal diagnosis of TSC. This study is aimed to examine whether prenatally diagnosed cardiac rhabdomyomas is associated with TSC. We conducted a 4-year (2005 to2009) retrospective review of all children diagnosed with cardiac rhabdomyoma. Medical records were systemically reviewed for demographic date, age of diagnosis, initial presenting features, major criteria identified by time of diagnosis. Reports of postnatal CT and MRI scans were reviewed to determine the presence and extent of brain pathology. Diagnosis was based on the clinical diagnostic criteria, which was revised at TSC consensus conference in 1998. Nine presented with a cardiac rhabdomyoma. Five(55%) received a definitive diagnosis and had radiographic evidence of cortical tubers. Four(80%) had multiple lesion and one(20%) had single cardiac lesion. One(20%) presented with seizure at the time of diagnosis and presented with facial angioma. Two(40%) presented with retinal nodule. Three was performed TSC2 gene study and two(40%) was confirmed the diagnosis of TSC. This study suggests that cardiac rhabdomyoma and cortical tuber in the fetus are the earliest and the most frequent reported signs of TSC. Although cardiac rhabdomyoma was the only symptom of TSC in the patient, we performed further diagnostic evaluation and genetic counseling.

Congenital Segmental Emphysema: An Evolving Lesion

Congenital segmental emphysema (CSE) is a newly-recognised sub-type of congenital parenchymal lung anomaly. It is characterised by antenatal detection and post-natal evolution from an initially solid segmental appearance to a hyperlucent and hyperinflated segment. A retrospective review of a single-centre tertiary referral database between Jan 1994 and Dec 2007 was performed. 130 infants had antenatally detected lung anomalies, and of these 12 (9.2%) infants (initially labelled as congenital cystic adenomatoid malformation (CCAM)), showed features better defined as CSE. The lesions were described antenatally as non-progressive microcystic (n=6), hyperechogenic (n=2) or both (n=2). Early post-natal CT scans showed areas of solid segmental parenchyma, initial hyperlucency or microcysts. Subsequent CT imaging, however, showed evolution to segmental hyperlucency in areas previously solid and in 2 cases a central bronchocele was noted. Ten children underwent resectional surgery (segmentectomy n=4, lobectomy n=6) at a median age of 1 (range 0.4-5.2) year and the gross appearance of the resected specimen confirmed hyperinflated (not cystic) segments. Histological review showed localised abnormally dilated alveolar spaces in 7 cases. Adjacent areas consistent with type 2 CCAM were also seen (n=3). CSE lies within the spectrum of both CCAM and sequestration but there is a definite post-natal evolution and volume change which presage symptoms. This may be associated with segmental bronchial atresia and progressive air trapping via collateral airways such as the interalveolar pores of Kohn.

Elective surgery for congenital cystic adenomatoid malformation at one year of age is safe and without respiratory sequelae

Conclusion: Concordance between postnatal lung CT and postnatal histopathology was superior to concordance between prenatal lung ultrasound and postnatal histopathology. In this study the type of lesion (CPAM versus non-CPAM) was suggestive to predict postnatal outcome, however patient cohorts were too small and divers to be statistically significant. Therefore the ability of perinatal imaging to predict outcome still needs to be refined.

Congenital lung malformation: Evaluation of prenatal and postnatal radiological findings

This study evaluated the accuracy of prenatal MRI and postnatal CT imaging in the identification of congenital cystic adenomatoid malformation and bronchopulmonary sequestration by comparison with histological analysis. Over a 3-year period, 15 patients with lung malformations diagnosed prenatally by ultrasound were referred for prenatal MRI, and all were investigated postnatally by chest CT. All asymptomatic newborns with unresolved lesions underwent elective surgery by thoracoscopy. All surgical specimens were analysed histologically. Among the 15 patients with an abnormality diagnosed by ultrasound, prenatal MRI findings differed from the final histological diagnosis with respect to extent (n = 3), type of lesion (n = 1) and aberrant vessel identification (n = 4). Postnatal chest CT failed to visualize the aberrant vessel in one patient. Complete regression of the lesion was noted in two patients with bronchopulmonary sequestration, and in one patient with congenital cystic adenomatoid malformation and was confirmed by CT. Elective thoracoscopic lobectomy of the affected lobe was performed for 12 patients. Two conversions to thoracotomy were required. All operated patients had an uneventful hospital course. Prenatal MRI is less accurate than postnatal CT scan, which remains the most reliable diagnostic modality to specify the location and extent and kind of lesions.

Congenital high airway obstruction syndrome: MR/US findings, effect on management, and outcome

Congenital high airway obstruction syndrome (CHAOS) is a rare disorder defined as any fetal abnormality that obstructs the larynx or trachea. Prompt airway intervention at delivery after accurate prenatal diagnosis may allow survival of this otherwise fatal condition. To identify prenatal MRI findings in CHAOS, to compare these findings with those of fetal US, to determine if imaging alters diagnosis and management decisions, and to correlate prenatal with postnatal imaging findings. Records and MRI scans of ten fetuses with CHAOS were reviewed, and the findings correlated with outside and same-day fetal US and postnatal imaging findings. Fetal lung volumes were measured on MRI scans. Large lung volumes were found in 90% of the fetuses. Increased lung signal intensity, inverted diaphragm, and a dilated, fluid-filled lower airway were identified in all. The obstruction level was identified in 90%. MRI changed screening US diagnosis in 70%, but was concordant with the tertiary care US imaging in 90%. Seven fetuses were terminated or died in utero, and three fetuses survived after ex utero intrapartum tracheostomy placement. Autopsy or bronchoscopy performed in 60% confirmed CHAOS. Postnatal chest radiographs and CT showed hyperinflation, while US and fluoroscopy showed diminished diaphragmatic motion. MRI demonstrates large lung volumes, increased lung signal intensity, inverted diaphragm, and dilated fluid-filled lower airway, and usually identifies the obstruction level. The degree of correlation between MRI and tertiary prenatal US is high, but CHAOS is frequently misdiagnosed on screening US. Correct diagnosis may enable planned airway management. Voluminous lungs and diaphragmatic abnormalities persist on postnatal imaging.

Neonatal pulmonary sequestration: Clinical experience with transumbilical arterial embolization

Pulmonary sequestration (PS) is a rare congenital malformation of the lower respiratory tract. The exact natural course of PS is not well understood and there are no well-established treatment guidelines for antenatally diagnosed PS. The aim of this study was to describe clinical outcomes in neonates with PS and to evaluate the efficacy of transumbilical arterial embolization (TUE). From 1998 to 2006, total 30 neonatal cases were included. Serial antenatal ultrasound in 26 cases found 6 (23%) regressed lesions, all of which were demonstrated on postnatal chest CT. Six (20%) cases were classified as mixed-type (combined cystic) lesions. Surgery was performed early (during initial hospitalization) in two cases and lately (after the neonatal period) in four cases. TUE was performed for 17 (57%) cases of intrapulmonary PS. Follow-up images obtained a median of 19 months (range, 4-51) after TUE demonstrated complete (9, 53%), partial (5, 29%), and no (3, 18%) regression. The regression rate was significantly higher in solid-type lesions (13/13, 100%) than in mixed-type (1/4, 25%) (P = 0.006). Complications included transient hypertension (two cases, 12%), post-embolization fever (two cases, 12%) and migration of a microcoil (one case, 6%), without long-term morbidities. Natural courses could be observed in 10 cases of extralobar PS and regression was observed in 2 cases (20%) during a median follow-up of 12 months (range, 6-45). A well-designed comparative study is warranted to evaluate the long-term efficacy and safety of TUE.

Diagnosis of Chiari III malformation by second trimester fetal MRI with postnatal MRI and CT correlation

We report a case of Chiari III malformation diagnosed by fetal MRI. Ultrasound (US) performed at a gestational age of 18 weeks demonstrated a posterior skull base cyst. Repeat US at 19 weeks demonstrated neural tissue in the cyst, consistent with an encephalocele. MR imaging at 23 weeks confirmed the presence of an occipital encephalocele, demonstrated additional bony defect in the upper cervical spine, and identified abnormal morphology and position of the brainstem consistent with the diagnosis of Chiari III. Postnatal MRI and CT confirmed the fetal MRI findings and demonstrate the utility of fetal MRI in the early evaluation of songraphically detected posterior fossa abnormalities.