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MRgFUS Vim thalamotomy is a novel, effective, minimally invasive therapeutic option for patients with essential tremor (ET). Among the selection criteria, some parameters related to the patient's anatomy, such as the skull density ratio (SDR), are well recognized. The role of brain tissue interposed between the target and the ultrasound transducers has never been explored. Therefore, the purpose of our study was to evaluate the correlation and the possible predictive value between brain tissue volumes (grey matter - GM, white matter - WM, and cerebrospinal fluid - CSF) and several treatment-related variables (periprocedural parameters, MRI imaging findings, and the clinical outcome). We analysed data from thirty ET patients previously submitted to MRgFUS thalamotomy. Pre-treatment images were automatically segmented in sopra-tentorial (ST) WM, GM, and CSF using SPM 12. The most significant findings were a positive correlation of the ST-GM with the Accumulated Thermal Dose (ATD) (p 0,001) and a negative correlation of the ATD temperature with ST-CSF and ST-TIV (p 0,001). Ultrasound propagation speed is lower in fluids than brain tissues. Also, WM has an attenuation rate of 1.5 higher than the GM. Therefore, the difference in the ATD may be explained by the different acoustic properties of normal brain tissues interposed between the transducers and the VIM.

To analyze and compare direct and indirect targeting of the Vim for MRgFUS thalamotomy. We retrospectively evaluated 21 patients who underwent unilateral MRgFUS Vim ablation and required targeting repositioning during the procedures. For each patient, in the three spatial coordinates, we recorded: (i) indirect coordinates; (ii) the coordinates where we clinically observed tremor reduction during the verification stage sonications; (iii) direct coordinates, measured on the dentatorubrothalamic tract (DRTT) at the after postprocessing of DTI data. The agreement between direct and indirect coordinates compared to clinically effective coordinates was evaluated through the Bland–Altman test and intraclass correlation coefficient. The median absolute percentage error was also calculated. Compared to indirect targeting, direct targeting showed inferior error values on the RL and AP coordinates (0.019 vs. 0.079 and 0.207 vs. 0.221, respectively) and higher error values on the SI coordinates (0.263 vs. 0.021). The agreement between measurements was higher for tractography along the AP and SI planes and lower along the RL planes. Indirect atlas-based targeting represents a valid approach for MRgFUS thalamotomy. The direct tractography approach is a valuable aid in assessing the possible deviation of the error in cases where no immediate clinical response is achieved.

Tremor is a common and very disabling symptom in patients with essential tremor and Parkinson's disease. In the recent years, transcranial ablation of thalamic nuclei using magnetic resonance guided high-intensity focused ultrasound has emerged as a minimally invasive treatment for tremor. The aim of this review is to discuss, in the light of our single-center experience, the technique, current applications, results, and future perspectives of this novel technology.

Objective: To identify possible relevant factors contributing to tremor relapse after MRgFUS thalamotomy in patients with essential tremor (ET) and Parkinson’s disease (PD). Methods: We identified patients with tremor relapse from a series of 79 treatments in a single institution. The demographic and clinical characteristics of the study group patients were compared to those of patients who did not relapse in the same follow-up period. Imaging and procedural factors were compared using a control group matched for clinical and demographic characteristics. Results: Concerning clinical and demographic characteristics, we did not find statistically significant differences in gender and age. Seventy-three percent of patients with tremor relapse were Parkinson’s disease patients. Using MRI, we found larger thalamotomy lesions at the 1-year follow-up in the control group with stable outcomes, compared to patients with tremor relapse. In the tractography evaluation, we found a more frequent eccentric position of the DRTt in patients with tremor relapse. Conclusions: The most relevant determining factors for tremor relapse after MRgFUS thalamotomy appear to be tremor from Parkinson’s disease and inaccurate thalamic targeting. Size of the thalamotomy lesion can also influence the outcome of treatment.

Objective In the recent years the increase of methamphetamines (MTA) abusers women has become an emerging problem. Very little data has been published regarding the effects of prenatal MTA exposure. We describe a case of MTA related toxicity in a term newborn which have early onset of neonatal encephalopathy and liver failure. Case report A term infant born to a MTA abuser mother developed seizures and severe neurological symptoms shortly after birth. Methamphetamine was detected both in maternal and in neonatal urine. The laboratoristic tests revealed severe hepatic insufficiency, coagulopathy and thrombocytopenia. Due to neonatal encephalopathy the newborn underwent hypothermia. Phenobarbital, fresh frozen plasma, platelet transfusions and vitamin K were administered. Cranial ultrasonography and magnetic resonance imaging (MRI) showed diffuse white matter damage and two ischemic-hemorrhagic cerebral lesions. Gradually the clinical conditions improved, at 1 month MRI showed a stabilization of cerebral lesions with residual diffuse leukomalacia. Physiotherapy and neurological follow up is ongoing to evaluate the long term effects. Conclusions: although infrequent, MTA-related toxicity should be suspected in infants with neurologic and hepatic symptoms. Further studies are warranted to confirm our findings in order to identify newborns at high risk of acute MTA toxicity in time to provide them the appropriate support.

OBJECTIVE To examine the relationship between electrographic seizures and developmental outcome at 18 and 24 months in neonates with moderate and severe hypoxic-ischemic encephalopathy [HIE] treated with therapeutic hypothermia [TH]. STUDY DESIGN 30 term infants with moderate-severe HIE treated with TH were enrolled prospectively from June 2012 to May 2018. All had continuous single channel amplitude integrated EEG (aEEG) monitoring for a minimum of 72 h and brain MR within 4 weeks. The aEEG was classified by severity of background and seizure burden. MR images were graded by the severity of injury. Outcome (defined abnormal in case of death, dyskinetic or spastic quadriplegic cerebral palsy, epilepsy, or Bayley III score < 85 in all three subscales or < 70 in any individual subscale) was assessed at 18 and 24 months. RESULTS Seizures were recorded in 24 out of 30 [80 %] neonates and an abnormal outcome was observed in 7 [23 %] of infants. Patients with poor outcome had a statistically significant correlation with: high seizure burden (p = 0.0004), need for more than one antiepileptic drugs (p = 0.006), a persistent abnormal aEEG trace at 48 h (p = 0.0001) and moderate-severe brain injury at MRI (p = 0.0001). Moreover, infants with status epilepticus or frequent seizures reported a significantly association with abnormal MR imaging and poor outcome than patients with sporadic seizures (p = 0.0009). CONCLUSION The role of seizures in the pathogenesis of brain injury remains controversial. In our cohort the presence of seizures, per se, was not associated with abnormal outcome; however a high seizure burden as well as a persistent abnormal aEEG background pattern and MR lesions resulted significantly associated with poor prognosis.

Objectives The objective of this study was to evaluate whether proton magnetic resonance spectroscopy and perfusion magnetic resonance imaging (MRI) are able to increase diagnostic accuracy in the follow-up of brain gliomas, identifying the progression of disease before it becomes evident in the standard MRI; also to evaluate which of the two techniques has the best diagnostic accuracy. Methods Eighty-three patients with cerebral glioma (50 high-grade gliomas (HGGs), 33 low-grade gliomas (LGGs)) were retrospectively enrolled. All patients underwent standard MRI, H spectroscopic and perfusion echo-planar imaging MRI. For spectroscopy variations of choline/creatine, choline/N-acetyl-aspartate ratio, and lipids and lactates peak were considered. For perfusion 2.0 was considered the cerebral blood volume cut-off for progression. The combination of functional parameters gave a multiparametric score (0–2) to predict outcome. Diagnostic performance was determined by the receiver operating characteristic curve, with sensitivity, specificity, positive predictive and negative predictive values. Results In patients with LGGs a combined score of at least 1 was the best predictor for progression (odds ratio (OR) 3.91) with 8.4 months median anticipation of diagnosis compared to standard MRI. The individual advanced magnetic resonance technique did not show a diagnostic accuracy comparable to the combination of the two. Overall diagnostic accuracy area under the curve (AUC) was 0.881. In patients with HGGs the multiparametric score did not improve diagnostic accuracy significantly. Perfusion MRI was the best predictor of progression (OR 3.65), with 6.7 months median anticipation of diagnosis. Overall diagnostic accuracy AUC was 0.897. Then spectroscopy and perfusion MRI are able to identify tumour progression during follow-up earlier than standard MRI. Conclusion In patients with LGGs the combination of the functional parameters seems to be the best method for diagnosis of progression. In patients with HGGs perfusion is the best diagnostic method.

Progressive multifocal leukoencephalopathy (PML) is a rare rapidly progressive demyelinating disease of the central nervous system caused by reactivation of latent John Cunningham (JC) polyomavirus (JCV) infection. We describe an unusual case of PML in a 54-year-old patient with follicular non-Hodgkin lymphoma who received rituximab plus cyclophosphamide, hydroxydaunorubicin, oncovicin and prednisolone (R-CHOP) therapy. She started to notice gradual progressive neurological symptoms about two months after completion of rituximab treatment and was therefore admitted to hospital. On admission, brain CT and MRI showed widespread lesions consistent with a demyelinating process involving the subcortical and deep white matter of the cerebral and cerebellar hemispheres. CT and MRI findings were suggestive of PML, and JC virus DNA was detected by polymerase chain reaction assay of the cerebrospinal fluid and serum. The patient was treated supportively but reported a progressive worsening of the clinical and radiological findings. Our report emphasizes the role of CT and MRI findings in the diagnosis of PML and suggests that PML should be considered in patients with progressive neurological disorders involving the entire nervous system and mainly the white matter, especially in the presence of previous immunomodulatory treatment or immunosuppression.

Central nervous system (CNS) vasculitis can affect both adults and children, but some of these occur almost exclusively in childhood. In children may develop as a primary condition or secondary to an underlying systemic disease. Cerebral vasculitis can be classified on the basis of the diameter of the involved vessels, although there is no univocal consensus. The diagnosis of CNS vasculitis is particularly difficult because the available investigative modalities have limited sensitivities and specificities. The most helpful diagnostic tests include cerebrospinal fluid analysis, MRI (MR angiography/venography (MRA/MRV) of the brain, and angiography. However, brain biopsy may be required to diagnose small vessel vasculitis in order to make differential diagnosis with a wide range of conditions, such as degenerative vasculopathies, embolic diseases, or coagulation disorders. This paper discusses on current understanding of most frequent primary and secondary central nervous system vasculitis in children in which are involved small vessel.

Background Reduction of apparent diffusion coefficient (ADC) values in white matter is not always ischaemic in nature. Methods We retrospectively analysed our MRI records featuring reduced ADC values in the centrum semiovale without grey matter involvement or significant vasogenic oedema. Results Several conditions showed the aforementioned MR findings: moose-horn lesions on coronal images in X-linked Charcot-Marie-Tooth disease; small fronto-parietal lesions in Menkes disease; marked signal abnormalities in the myelinised regions in the acute neonatal form of maple syrup urine disease; strip-like involvement of the corpus callosum in glutaric aciduria type 1; persistent periventricular parieto-occipital abnormalities in phenylketonuria; diffuse signal abnormalities with necrotic evolution in global cerebral anoxia or after heroin vapour inhalation; almost completely reversible symmetric fronto-parietal lesions in methotrexate neurotoxicity; chain-like lesions in watershed ischaemia; splenium involvement that normalises in reversible splenial lesions or leads to gliosis in diffuse axonal injury. Conclusion Neuroradiologists must be familiar with these features, thereby preventing misdiagnosis and inappropriate management.

The most important symptoms associated with schizophrenia are affective flattening, decreased expression of emotions, anhedonia and social isolation. The purpose of the present study was to investigate the neural response to disgusting and pleasant visual stimuli in healthy subjects and in patients with first-episode schizophrenia. Twelve subjects in the first episode of schizophrenia (DSM-IV-R, APA, 2000) with a normal IQ and 12 healthy volunteers selected for age and education underwent functional magnetic resonance imaging (fMRI) during observation of pleasant and disgusting visual stimuli. Analysis showed that in healthy subjects, the prefrontal cortex and limbic areas are activated in response to pleasant and disgusting visual stimuli, whereas this does not occur in subjects with schizophrenia since the first episode of illness.

This paper focuses on radiological approach in pediatric stroke including both ischemic stroke (Arterial Ischemic Stroke and Cerebral Sinovenous Thrombosis) and hemorrhagic stroke. Etiopathology and main clinical findings are examined as well. Magnetic Resonance Imaging could be considered as the first-choice diagnostic exam, offering a complete diagnostic set of information both in the discrimination between ischemic/hemorrhagic stroke and in the identification of underlying causes. In addition, Magnetic Resonance vascular techniques supply further information about cerebral arterial and venous circulation. Computed Tomography, for its limits and radiation exposure, should be used only when Magnetic Resonance is not available and on unstable patients.

Background. Cerebral amyloid angiopathy-related inflammation (CAA-ri) results from autoimmune response to beta-amyloid deposits in cerebral vessels. Its clinical course and complications have seldom been described in literature.Case Report. In a patient presenting with delirium and left hemiparesis the diagnosis of CAA-ri was supported by the finding of elevated anti-amyloid autoantibodies in the cerebrospinal fluid (CSF). Steroid therapy produced significant improvements in clinical and investigational assessments, but after two months, it caused Acute Respiratory Distress Syndrome. After steroid therapy discontinuation the patient presented a rapidly progressive dementia, Guillain-Barré syndrome, new cerebral ischemic lesions, and thrombosis of the right cephalic and subclavian veins that were treated with subcutaneous heparin. After a week the patient died because of brain hemorrhage.Conclusion. This case suggests caution in steroid therapy discontinuation and antithrombotic therapy administration in patients with CAA-ri. The CSF search of anti-amyloid autoantibodies could be helpful to support the diagnosis.

The extreme variability of clinical and MRI findings in the suspicion of Devic's disease always requires the detection of specific antibodies (AQP4). MRI scans were performed with a high-field MRI scanner (1.5T General Electric Signa Horizon): the MRI protocol of the brain employed axial DP, T2, T1, FLAIR and DWI weighted images (wi) and coronal T2-wi. After intravenous administration of contrast medium axial and sagittal T1-weighted images of the brain were repeated. The spine protocol employed after contrast medium included sagittal T2-wi, T2-wi with fat suppression and T1-wi. In May 2004, a 64-year-old healthy Caucasian woman began to suffer loss of motor and thermal sensitivity in the left lower limb. MRI showed nonspecific areas of abnormal signal intensity on the deep left frontal and right frontoparietal white matter with no pathological enhancement after contrast medium and a small intramedullary area of altered signal at metameric level C2–C4 with a diagnosis of post-viral transverse myelitis. The patient had two similar episodes years later so the neurologist decided to search for circulating IgG AQP4 with the definitive diagnosis of neuromyelitis optica. In this case, compared to a clinical presentation of atypical deficit neurological involvement, the neuroradiological results of a progressive diffuse involvement of the white matter were atypical.

The Intracranial Complications of infection of the paranasal sinuses are uncommon and subdural empyema is rarer still. Roughly half of the cases of subdural empyema are caused by sinusitis and the most common source of infection is the frontal sinuses. MR scan is the method of choice for the diagnosis and monitoring of subdural empyema, allowing prompt institution of appropriate treatment thereby reducing mortality. We describe a 15-year-old girl with interhemispheric subdural empyema caused by fronto-ethmoidal sinusitis. Clinical onset was characterised by high fever, severe headache, nausea, vomiting and diplopia. CT scan showed evidence of a left parafalcial hypodense collection. MR scan, especially after contrast administration, showed the exact extension and location of the interhemispheric subdural empyema, also displaying the associated leptomeningitis and fronto-ethmoidal sinusitis. Prompt medical treatment led to a remission of symptoms and sinus infection with associated leptomeningitis as documented by serial MR monitoring. However, the slow progressive increase in size of the subdural collection was deemed an indication for neurosurgical management (evacuation of the collection by ventricular drainage catheters inserted under stereotaxic control) leading to a complete resolution of the subdural empyema without neurological sequelae.

Serial magnetic resonance imaging findings are described in a patient with a sporadically occurring pilocytic astrocytoma that underwent spontaneous regression over 6 years. To the authors' knowledge, this is the first report in which spontaneous involution of a pilocytic astrocytoma not associated with neurofibromatosis type 1 has been described. A literature review regarding sporadic and syndrome-associated pilocytic astrocytoma was undertaken, with particular reference to treatment and natural history.

Multiple sclerosis (MS) is an inflammatory CNS disease characterized by multifocal areas of demyelination; usually it arises in young adults, but can also occur in children (under the age of 10) and adolescents (under the age of 18). As in adult, pediatric MS (PMS) diagnosis is based on the demonstration of multiple demyelination episodes separated in time and spaces. Diagnostic criteria realized for childhood are similar to those employed for adults. Although clinical and imaging features of PMS can be similar to those of adults, the disease is often characterized by a more aggressive course and atypical imaging findings, with giant and pseudotumoral plaques. Differential diagnosis between PMS and ADEM could be difficult: clinical findings and MRI are necessary; sometimes MRI follow-up is required for definitive diagnosis.

Rosai-Dorfman disease (RDD) was firstly described in 1969 as a benign proliferative disorder of histiocytes with systemic symptoms and lymphadenopathy. This disease is of uncertain pathogenesis and mostly occurs in children and young adults. The typical clinical features of RDD include bilateral painless cervical lymphadenopathy, but extranodal involvement may also be present. The most common extranodal sites include organs such as the respiratory tract, skin, nasal cavity, orbit and bone. Isolated central nervous system (CNS) manifestations are extremely rare. In case of CNS involvement, the commonest imaging findings are dural-based extra-axial enhancing masses. We describe a case of intracranial RDD mimicking multiple meningiomas both clinically and radiologically in a 57-year-old man presenting with a six-year history of progressive right visual and hearing loss and tinnitus. In cases of multiple extra-axial lesions it is worth bearing in mind the possible differential diagnosis for intracranial RDD and eventually propose to the patient further investigations.

Myxopapillary ependymoma is a rare variant of ependymoma, almost exclusively occurring in the region of the cauda equina and filum terminale. We describe a myxopapillary ependymoma located in the left cerebellopontine angle of a young man suffering from peripheral vertigo and left sensorineural hearing loss for years. The patient underwent surgical removal of the tumour. Microscopic examination showed histological and immunohistochemical features consistent with a diagnosis of myxopapillary ependymoma. Imaging studies of the spine yielded normal findings, confirming the lesion’s primary nature. To the best of our knowledge, this is the first case of primary intracranial myxopapillary ependymoma described in this location.

Aim of our study is to present the findings of parietal cortex alterations in patients affected by movement disorders. We observed 4 patients with movement disorders (3 parkinsonian syndromes, I diffuse dystonia) and MR evidence of cortical alterations, without additional cerebral lesions. MR was performed with a 0.5T unit, SE T1-w, TSE T2-w, and Turbo Fluid Attenuated Inversion Recovery (T-FLAIR) sequences on axial and coronal planes, contiguous slices and 5 mm slice thickness. In the parkinsonian patients, parietal atrophy was observed in 2 cases and fronto-parietal atrophy in I case; in all cases T-FLAIR sequences showed bilateral parietal cortex signal alteration. The patient with diffuse dystonia presented severe bilateral parietal atrophy with bilateral fronto-parietal signal alteration. Our findings are strongly suggestive for cortico-basal degeneration. The evidence of signal alteration within the cortex suggests a primary pathogenetic involvement of cortico-basal pathways.

The hand and wrist are common sites for trauma, resulting in various bony, capsular, ligamentous and tendinous injuries. Traumatic injuries of the wrist are very frequent and occur at every age. Some lesions happen characteristically in a specific decade of life. Bone mineralization, which could be considered age-related, and ligamentous resistance and compliance, together with the mechanism of the trauma determine the resulting lesion [1-5]. Thus, metaphyseal fractures and slipping epiphysis typically occur in children. Both carpal dislocations and fracture-dislocations typically occur in young adults. Fractures, dislocations and fracture-dislocations of the distal extremity of radius and ulna usually occur in the elderly.

Joint impingement is a painful syndrome caused by the friction of joint tissues, which is both the cause and the effect of altered joint biomechanics. From the anatomical and clinical viewpoints, these syndromes are classified as bone impingement, soft tissue impingement and entrapment neuropathy, depending on what joint portion impinges on the others. We considered the most important impingement syndromes of the upper and the lower limbs from the clinical viewpoint. As for the upper limb, supraspinatus impingement is a frequent cause of shoulder pain in both athletes and the normal population; the painful subacromial arch is a typical sign of the rotator cuff impingement syndrome and of outlet and non-outlet impingement as well. As for the elbow, we considered both medial and lateral impingement. The carpal tunnel syndrome is the most common peripheral entrapment neuropathy of the upper limb; it is caused by compression of the median nerve at the wrist. We considered the main causes of carpal tunnel narrowing and the relative clinical findings. As for the lower limb, we considered the iliotibial band friction syndrome, which is the most common overuse syndrome of the knee and the ankle impingement syndrome. The latter includes anterolateral impingement (with chronic anterolateral and lateral pain and ankle instability), sinus tarsi impingement, anterior impingement (with pain during foot dorsiflection and posterior impingement. The tarsal tunnel syndrome is the most important ankle entrapment neuropathy causing burn pain and paresthesias in the toes and sole of the foot.

The ankle impingement syndrome is a frequent condition in both athletes and the normal population. We investigated this painful syndrome from both a clinical and a diagnostic viewpoint. Depending on what ankle tissue impinges on the other, it is possible to distinguish bone impingement, soft tissue impingement and peripheral nerve entrapment. For each of these pathologic conditions we investigated the diagnostic role of conventional radiography, Computed Tomography and Magnetic Resonance Imaging. The evidence of osteophytes, exostosis and presence of the os trigonum on plain films make clinical diagnosis easy in both anterior and posterior bone impingement. CT can provide useful information about the component of the posterior ankle. MRI always adds important information about chondral or subchondral bone injuries, synovial reaction and adjacent soft tissue involvement. The anterolateral impingement syndrome is caused by repeated injuries in plantar flexion and ankle intrarotation. MRI well detects the meniscoid injury thanks to high contrast sequences; it can also distinguish this syndrome from painful chondral and/or bony lesions at this level. MRI is also the method of choice to study sinus tarsi impingement, especially thanks to fat suppression sequences which increase MR diagnostic capabilities in this important anatomic area. Deep peroneal nerve entrapment, the medial plantar nerve entrapment syndrome and the tarsal tunnel syndrome are the most important entrapment neuropathies of the ankle. US and MRI are very useful to study the tendon and soft tissue abnormalities causing the anterior tarsal tunnel syndrome. CT and particularly MRI can easily detect many pathologic conditions causing the medial plantar nerve entrapment and the tarsal tunnel syndromes.

Background Ultrasound (US), Computed Tomography (CT) and Magnetic Resonance Imaging (MRI) are widely used in the clinical diagnosis of parotid gland tumors and their efficacy in identifying benign lesions is well documented. However, problems arise when facing some malignant lesions. Only few cases of salivary gland low grade malignant tumors have been previously reported in the literature complete with the radiological features. Case presentation We here describe a case of epithelial-myoepithelial carcinoma (EMC) of the parotid gland, a low grade malignant tumor, with spread to an intraparotid lymph node and with CT and MRI findings mimicking a benign lesion. Conclusion All the images revealed sharply outlined profiles and a homogeneous enhancement of the nodule, suggesting a benign tumor and demonstrating that a radiological evaluation of the lesion alone may be unsatisfactory and misleading in the diagnosis of salivary gland tumours, especially in the case of low grade malignant tumors, such as EMC.

We describe MR findings in patients with a clinical diagnosis of corticobasal degeneration (CBD). Five patients with different extrapyramidal symptoms were submitted to MRI. All patients were clinically followed-up for a minimum period of at least 12 months. MRI study was performed on a 0.5 T system (Philips NT 5 II), using conventional SE, TSE and t-FLAIR sequences on axial and coronal planes. In all cases MR showed bilateral parietal cortical atrophy. t-FLAIR sequences demonstrated cortical signal alteration on both parietal and frontal cortex in four cases and on the parietal cortex alone in 1 case. During the follow-up clinical evolution gave rise to the suspicion of CBD. MRI demonstrated parietal cortex atrophy even in those cases in which symptoms at onset were not specific for CBD, thus helping the diagnosis. Moreover MRI, using t-FLAIR sequences suggested CBD diagnosis even in the earlier phases of the disease, in which cortical damage has not yet become evident as atrophy.

Background Congenital cystic adenomatoid malformation (CCAM) of the lung is an uncommon fetal development anomaly of the terminal respiratory structures. The large cyst type usually occurs in stillborn infants or newborn infants with respiratory distress. Cases of CCAM have been previously described in adulthood, more often type I with multiloculated cystic lesions. Case presentation We report a case of type I CCAM presenting as a single, expansive cystic mass in the middle pulmonary lobe in a 38-year-old man, revealed by persistent cough and haemoptysis. Computed tomographic scan showed a single cyst with air fluid level, occupying the lateral segment of the lobe. When the type I CCAM is a single cyst, other cystic pulmonary lesions must be excluded. The intrapulmonary localization and the absence of cartilage in the cyst wall are conclusive findings of CCAM. The pathogenesis, management and differential diagnosis with other lung malformations are discussed along with a review of the literature. Conclusion The literature data confirm that surgical resection is the treatment of choice in all cases of CCAM and in the cases of cystic pulmonary lesions with uncertain radiological findings, in order to perform a histological examination of the lesion and to prevent infection and the potential neoplastic transformation.