Madan Mohan Gupta

Case History: Two-year-old female born from nonconsanguineous marriage presented with cough with expectoration and high-grade fever for six days.

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Case History: Two-year-old female with cough with expectoration and high-grade fever.

Cough, Fever

 A 75-year-old male with complaints of occasional headache for last two years. Laboratory examination, ophthalmic examination and family history were unremarkable. Neurophysician sent patient for routine brain MRI.

 T2WI axial and sagittal images show a small 12x8 mm well-defined, well-circumscribed heterogenous signal intensity lesion to adjacent grey matter with tiny focal area of CSF intensity in left lateral ventricle along inter ventricular septum in the region of foramen of monro (arrow).

 FLAIR axial and coronal images show a small well-defined, well-circumscribed hyperintense signal intensity lesion to adjacent grey matter in left lateral ventricle along inter ventricular septum in the region of foramen of monro (arrow).

 T1W axial image shows isointense signal intensity lesion to adjacent grey matter with focal cystic area in left lateral ventricle along inter ventricular septum in the region of foramen of monro.

 Diffusion weighted axial image shows no restricted diffusion within the lesion (arrow).

 T1 weighted FAT SAT axial imges show minimal to mild enhancement on post contrst study within lesion.

On MRI, a small 12x8 mm well-defined, well-circumscribed lesion was noted in the left lateral ventricle along inter ventricular septum in the region of foramen of monro. It showed heterogenous signal with adjacent grey matter on T2WI (Figure 1) with a tiny focal cystic area, which followed CSF signal, hyperintense signal on FLAIR weighted images (Figure 2) and isointense to white matter on T1WI (Figure 3) and did not show restricted diffusion on diffusion weighted images (Figure 4). No susceptibility artifact was noted. On post contrast study, the lesion showed minimal to mild enhancement (Figure 5). No evidence of obstructive hydrocephalus was seen.

: Subependymoma are uncommon, benign (WHO grade I) tumors, which are slow growing and non-invasive. Subependymoma tend to present in middle-aged to older individuals. There is a slight male predilection (M: F 2.3:1). Typically, patients are asymptomatic and small lesions are discovered incidentally. In some cases, especially when the tumors are larger, presentation is with symptoms of raised intracranial pressure due to obstructive hydrocephalus.


CT shows isodense intraventricular mass compared to adjacent brain, which does not usually enhance. Surrounding vasogenic edema is usually absent. MRI-T1 iso shows hypointense to white matter, T2-heterogenous to adjacent white and grey matter, with susceptibility related signal drop out due to calcifications occasionally seen no adjacent parenchymal edema. On post contrast-T1 fat sat images there is usually no enhancement, although at times may demonstrate mild enhancement


1. Ragel BT, Osborn AG, Whang K, et al. Subependymomas: an analysis of clinical and imaging features. 

. 2006;58: 881–890.
2. Russell JH, Gaillard F, Drummond KJ. A mass in the fourth ventricle. 

. 2009;16: 425,482.
3. Koral K, Kedzierski RM, Gimi B, et al. Subependymoma of the cerebellopontine angle and prepontine cistern in a 15-year-old adolescent boy. 

. 2008;29:190-191.
4. Tonn J, Westphal M, Rutka JT. Oncology of CNS Tumors. Springer Verlag. 2009.
5. Keating RF, Goodrich JT, Packer RJ. Tumors of the pediatric central nervous system. George Thieme Verlag. 2001.
6. Smith A, Smirniotopoulos J, Horkanyne-Szakaly I. From the Radiologic Pathology Archives: Intraventricular Neoplasms: Radiologic-Pathologic Correlation. 

Articles

Case History: 75-year-old male with complaint of occasional headaches for last two years.

Subependymoma of Lateral Ventricle

 A 3-month-old child brought by parents with complaints of abnormal growth of head size. As the patient’s appearance and head size was not normal, a detailed family and medical history was taken. Review of medical history was unremarkable. The mother reported normal labor and delivery. There were no anomalies in any siblings or near relatives. On general examination, the patient was had a wide nasal bridge, hypertelorism, and exophthalmos. Intra oral examination revealed high arch palate, hypo plastic maxilla causing prognathism of the mandible. The patient was subjected to radiographic investigations.

Lateral skull view demonstrated a scaphocephaly skull, retruded maxilla with a relatively large mandible and copper beaten appearance (Figure 1). Radiographic examination of metacarpal bones and fingers were unremarkable. Further, on non contrast CT, brain and face was performed, which revealed increased antero-posterior diameter of the skull with obliterated sagittal suture (scaphocephaly skull), small paranasal sinuses (Figure 2). Three-dimensional (3D) computed tomographic images of the skull revealed increased circumference of the skull in antero-posterior diameter with hypo plastic maxilla (Figure 3). On non contrast MRI and CT, brain did not reveal any abnormality in brain parenchyma. No evidence of hydrocephalous was noted (Figure 4).

 Lateral skull projection reveals abnormally enlarged skull (more antero-posterior diameter) due to premature closure of sagittal suture (scaphocephaly) mandibular prognathism (white arrow), maxillary hypoplasia (arrow), copper beaten appearance and enlarged hypophyseal cavity.

 Non contrast CT of brain and face bone window images (A and B) show increased antero-posterior diameter of skull (scaphocephaly skull), copper beaten appearance, small paranasal sinuses, mandibular prognathism. C and D show maxillary hypoplasia (arrow). E shows premature closure of sagittal suture in its posterior aspect (small arrow) resultant scaphocephaly.

 Three-dimensional (3D) computed tomographic VR images (A and B) of the skull with face revealed increased circumference of the skull in antero-posterior diameter.

 Non contrast CT brain (A, axial and B, coronal) images and non contrast MRI (C, T2WI axial, D, sagittal and FLAIR, coronal E and axial F) images show normal brain parenchyma. No evidence of hydrocephalus is noted.

 Crouzon syndrome (CS) is a rare genetic disorder characterized by premature closure of one or more cranial sutures and produces characteristic craniofacial and other associated abnormalities. It is characterized by premature craniosynostoses, mid facial hypoplasia and orbital defects. Here, we report a case of this rare entity. The patient presented with brachycephaly and abnormal calvarial shape. Severe cases can give a "cloverleaf skull," maxillary hypoplasia, exophthalmos, mandibular prognathism or bifid uvula along with dental and orbital abnormalities.

 It carries an autosomal dominant inheritance due to a mutation in fibroblast growth factor receptor 2 (FGFR2) gene on chromosome 10q25-26.

Chiari I malformations:3 may be present in ~ 70 percent of cases

Calcification of the stylohyoid ligament: may be present in ~ 50 percent of patients over 4 years old


1. Chen CP, Lin SP, Su YN, et al. A cloverleaf skull associated with Crouzon syndrome. 

. 2006;9:F98.
2. Entezami M, Albig M, Knoll U, et al. Ultrasound Diagnosis of Fetal Anomalies. Thieme. 2003.
3. Lowe LH, Booth TN, Joglar JM, et al. Midface anomalies in children. 

. 20:907-922.
4. Altintas AG, GÃ¼l aksoy FG, Altintas CS, et al. Evaluation of findings in Crouzon's syndrome. 

Case History: 3-month-old child with abnormal growth of head size.

Crouzon Syndrome

 A 75-year-old male presented with complaint of mucus discharge from an external opening on skin at anterior aspect of mid part of neck on left side. No history of trauma or surgery and unremarkable family history. Upon examination, there was an external opening on skin at anterior aspect of mid part of neck on the left side. Naked eye examination of the oropharynx did not reveal any visible opening at posterior to tonsillar pillar.

The patient was subjected to radiologic investigations. A fistulogram was performed but did not reveal internal opening. Further, on contrast CT, observed an elongated fusiform-shaped approximately 8-10 cm long, well-defined, smoothly marginated thin-walled, peripherally rim enhancing cystic lesion extending from the anterior surface of the mid part of the neck on the left side up to lateral wall of hypopharynx at the hypoid bone level. It had an external opening on the anterior surface of the mid part of the neck on the left side (Figure 1A). It lies anterior to carotid vessels and lateral to thyrohyoid membrane and antero-medial to sternocleidomastoid muscle (Figure 1B). Cranially, it terminated in the lateral wall of left pyriform fossa at hyoid level. It did not open into posterior to tonsillar pillar, so it ruled out second branchial fistula (Figure 2–4). On the basis of these radiological findings, final diagnosis was made of complete fistula of third branchial cleft. The patient was operated on and complete excision of the cord-like fistulous tract was done, which followed up to the lateral wall of left pyriform fossa. The excised tract measured 9 cm in length. It was found to lie over the carotid sheath and was not going up to tonsillar pillar or in between the internal and external carotid arteries, so it ruled out second branchial cleft fistula which is most common in literature (Figure 5).

 Contrast CT axial images at thyroid gland level (A) show external opening of cystic lesion in anterior surface of the mid part of the neck on the left side (small arrow). In B, at thyroid cartilage level, the cystic lesion (large arrow) lies anterior to carotid vessels and lateral to thyrohyoid membrane and antero-medial to sternocleidomastoid muscle.

 Contrast CT axial images of upper neck at hyoid bone level show a well-defined smoothly marginated peripherally rim enhancing cystic lesion (large arrow in A) opens in to lateral wall of left pyriform fossa (B, small arrow). It does not open into posterior to tonsillar pillar, so it ruled out possibility of second branchial cleft fistula.

 Contrast CT sagittal images of the neck show an elongated fusiform-shaped long well-defined smoothly marginated peripherally rim enhancing cystic lesion extending from anterior surface of the middle part of the neck (long arrow in A) and opens in to lateral wall of left pyriform fossa (small arrow in B).

 Contrast CT coronal images of the neck show an elongated fusiform shaped long well-defined smoothly marginated peripherally rim enhancing cystic lesion extending from anterior surface of the middle part of the neck and opens in to lateral wall of left pyriform fossa.

 Postoperative specimen. Complete excision of cord-like tract was performed, which followed up to the lateral wall of left pyriform fossa. The excised tract measured 9 cm in length. It was found to lie over the carotid sheath and was not going up to the tonsillar pillar or in between the internal and external carotid arteries, so it ruled out second branchial cleft fistula which is most common in literature.

: Complete fistula of third branchial cleft

 Third branchial cleft cysts are rare. A third branchial fistula extends from the same skin location as a second branchial fistula; however, a third branchial fistula pierces the thyrohyoid membrane to enter the larynx, terminating on the lateral aspect of the pyriform sinus. Third branchial cleft cysts occur anywhere along that course, but they are characteristically located deep to the sternocleidomastoid muscle. The external opening occurs about two-thirds of the way down the SCM anteriorly, similar to second branchial cleft cysts. The tract ascends along the carotid sheath posteriorly to the internal carotid artery, under the glossopharyngeal nerve, and over the vagus nerve and hypoglossal nerve to open into the pyriform sinus. Second branchial cleft anomalies are most common. The majority of these have an external opening in the neck along the anterior border of sternocleidomastoid at the junction of the upper two-thirds with the lower one-third and the internal opening extends up to the posterior pillar of the tonsil. In our case, the fistulous tract had external opening in the mid part of the neck along the sternocleidomastoid muscle and terminated in the lateral wall of pyriform fossa.

 CT - rounded, sharply circumscribed, fluid density centrally, thin wall. Ultrasound - sharply demarcated, posterior acoustic enhancement, imperceptible walls. MRI-T1 - variable signal dependant on protein content, high protein content; high signal, low protein content: low signal, T2 - usually high signal, T1 C+ (Gd) - no enhancement in uncomplicated lesions.

 Paramedian thyroglossal duct cyst, cystic lymph nodes, necrotic nodal metastases, esp. SCC and papillary thyroid cancer, tuberculous adenitis.


1. Koch BL. Cystic malformations of the neck in children. 

. 2005;35:463-477.
2. Goff CJ, Allred C, Glade RS. Current management of congenital branchial cleft cysts, sinuses, and fistulae. 

. 2012;20:533-539.
3. Zaifullah S, Yunus MR, See GB. Diagnosis and treatment of branchial cleft anomalies in UKMMC: a 10-year retrospective study. 

. 2012.
4. Guldfred LA, Philipsen BB, Siim C. Branchial cleft anomalies: accuracy of pre-operative diagnosis, clinical presentation and management.

. 2012;126:598-604.
5. Lanham PD, Wushensky C. Second brachial cleft cyst mimic: case report. AJNR 

. 2005;26:1862-1864.
6. Harnsberger HR, Mancuso AA, Muraki AS, et al. Branchial cleft anomalies and their mimics: computed tomographic evaluation. 

. 1984;152:739-748.
7. Ahuja AT, King AD, Metreweli C. Second branchial cleft cysts: variability of sonographic appearances in adult cases. 

Case History: 75-year-old male presents with complaint of mucus discharge from external opening on skin at anterior aspect of mid part of neck on the left side.

Complete Fistula of Third Branchial Cleft

A 58-year-old patient presented with complaint of dyspnoea for eight months. No history of fever and laboratory examination, and individual and family history were unremarkable. On chest X-ray (frontal and lateral view), a large well-defined, smooth marginated homogenous opacity was seen in posterior mediastinum with obliteration of right ascending aorta border (Figure 1). Further chest CT was performed, which showed two large saccular aneurysms arising from ascending aorta and arch of aorta with mural thrombus and peripheral calcification (Figure 2). Aneurysmal segment of aorta causes mass effect in form of compression of adjacent vascular structures and compression over trachea and oesophagus and causing posterior displacement of them (Figure 3). There was no evidence of contrast leakage or impending rupture of aorta. Diffuse changes of atherosclerosis were also noted in visible aorta.

: Chest radiograph (A, frontal view and B, lateral view) shows a large, well-defined, smooth marginated homogenous opacity in posterior mediastinum with obliteration of right ascending aorta border. The lesion is forming an obtuse angle with right lung parenchyma.

: Contrast CT angiography shows two large saccular aneurysms (large and small arrows) arising from ascending aorta and arch of aorta with mural thrombus and peripheral calcification in its wall. There is no evidence of contrast leakage or impending rupture of aorta. Diffuse changes of atherosclerosis in visible aorta.

: Contrast CT angiography shows aneurysmal segment of aorta causes mass effect in form of compression of adjacent vascular structures (large arrow) and compression over trachea (small arrow) and oesophagus and causing posterior displacement of them. There is no evidence of contrast leakage or impending rupture of aorta. Diffuse changes of atherosclerosis in visible aorta.

Aneurysm of ascending aorta and arch of aorta

Aneurysms in thoracic aorta are relatively uncommon compared to their counterparts below the diaphragm. Majority of thoracic aortic aneurysms are atherosclerotic. Symptomatic presentation may be due to mass effect on airway of oesophagus. Alternatively, they may present due to a complication, including rupture, aorto-bronchial or aorto-oesophageal fistulae. Thoracic aortic aneurysms can be divided pathologically according to their relationship to the aortic wall: o True aneurysm-atherosclerotic aneurysms (most common), inflammatory/aortitis
• Rheumatoid arthritis (RA), ankylosing spondylitis, takayasu arteritis, giant cell arteritis (GCA), syphiliss
• Structural: cystic medial necrosis, Marfan disease, Ehlers-Danlos syndrome, false aneurysms
• Traumas
• Mycotic aneurysms

Radiographic features: The location and shape of thoracic aortic aneurysms is variable. Aneurysms elsewhere can be described as saccular or fusiform. In the case of fusiform dilatation, the term aneurysm should be applied when the diameter is greater than 4 cm.

Plain film: The thoracic aorta can usually be seen on both frontal and lateral chest radiographs, and aneurysms are often obvious. Additionally, mediastinal masses may mimic aortic aneurysms. Mural calcification is seen both in atherosclerotic disease as well as various causes of aortitis.

CT: Typically aneurysms appear as dilatations of the lumen. The walls may be thin or thickened by presence of mural thrombus (circumferential or more frequently eccentric). Calcified atherosclerotic disease is often identified not only in the wall of the aneurysm but in adjacent arteries. If rupture or leak has occurred haematoma/fluid may be seen adjacent to the aorta. MRI has the advantage of not requiring ionizing radiation or large volumes of iodinated contrast.

 This is particularly advantageous in young patients with connective tissue disorders.

Digital subtraction angiography (DSA): Although angiography has long been considered the gold standard for vascular imaging, it has largely been superseded by CTA and MRA, which are able to obtain 3D volumetric data, and able to assess the extra luminal soft tissues. Angiography is however used during endovascular repair.

Complications

• Rupture
• Distal embolization
• Fistula formation
• Aorto-oesophageal fistula
• Aorto-bronchial fistula Differential diagnoses

• Aortic dissection
• Penetrating atherosclerotic ulcer
• Intramural aortic haematoma
• Kommerell diverticulum

References
1. Collins J, Stern EJ. Chest radiology, the essentials. Lippincott Williams & Wilkins. 2007.
2. Atar E, Belenky A, Hadad M, Ranany E, Baytner S, Bachar GN. MR angiography for abdominal and thoracic aortic aneurysms: assessment before endovascular repair in patients with impaired renal function. 

 2006;186:386-393.
3. Masuda Y, Takanashi K, Takasu J, Morooka N, Inagaki Y. Expansion rate of thoracic aortic aneurysms and influencing factors. Chest 1992;102: 461-466.
4. Kato N, Dake MD, Miller DC, Semba CP, Mitchell RS, Razavi MK, Kee ST. Traumatic thoracic aortic aneurysm: treatment with endovascular stent-grafts. Radiology 1997;205:657-662.
5. Reeder MM, Felson B. Reeder and Felson's gamuts in radiology, comprehensive lists of roentgen differential diagnosis. Springer Verlag. 2003.
6. Agarwal P, Chughtai A, Matzinger F, Kazerooni EA. Multidetector CT of thoracic aortic aneurysms1. 

Case History: 58-year-old patient with dyspnoea for eight months. No history of fever. Lab exam and history were unremarkable.

Aneurysm of Ascending Aorta and Arch of Aorta

 An 8-year-old female presented by his parents in pediatric emergency with seizures, right side hemiplegia and mental retardation. She had hypoxic ischemic insult. On examination she had spasticity in right side of body and mental retardation.

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 MRI T2W axial images show left cerebral hemiatrophy, dilatation of ipsilateral lateral ventricle and ipsilateral mid line shift of 7 mm.

 FLAIR axial and coronal images at the level of lateral ventricle show gliotic area with atrophy of left parietal lobe with relative preservation of deep grey nuclei.

 MRI diffusion weighted and ADC mapping images does not show restricted diffusion in concerned area.

Brain MRI was performed which revealed atrophy of left cerebral hemisphere, cerebral peduncle. There was ipsilateral midline shift and ventricular dilatation along with skull vault thickening and prominent frontal sinus, suggestive of congenital type of cerebral hemiatrophy or Dyke-Davidoff-Masson Syndrome.

MRI findings: There is presence of left cerebral atrophy involving left frontal, parietal and temporal lobe with dilatation of ipsilateral lateral ventricle and ipsilateral mid line shift of 7 mm towards left side with gliotic area in left parietal lobe with ipsilateral calvarial thickening.

 Patient presented with seizures, facial asymmetry, contralateral hemiparesis and mental retardation. The underlying etiology is cerebral insult that may occur in utero or early in life. Prenatal causes include congenital anomalies, cerebral infarction, vascular malformations and infections.

Perinatal causes are birth trauma, hypoxia and intracranial bleed. Postnatal hemiatrophy can develop secondary to cerebral trauma, tumors, infections and febrile seizures. Infantile (congenital) type of DDMS, in contrast to adult (acquired) DDMS, shows enlargement of calvarium, diploic space and paranasal sinuses. These compensatory cranial changes occur to take up the relative vacuum created by the atrophied cerebral hemisphere. Shen et al. depicted three MR imaging patterns of cerebral hemiatrophy: MR imaging pattern I corresponds to diffuse cortical and subcortical atrophy; pattern II corresponds to diffuse cortical atrophy coupled with porencephalic cysts; and pattern III corresponds to previous infarction with gliosis in the middle cerebral artery (MCA) territory. In our case, pattern III was present. The atrophied cerebral hemisphere will have prominent sulcal spaces if the vascular insult occurs after birth or after end of sulcation. However, if ischemia occurs during embryogenesis when the formation of gyri and sulci is deficient, prominent sulcal spaces will be absent.

Children with medically refractive epilepsy and hemiplegia may be candidates for hemispherectomy, which is helpful in eradicating or significantly reducing seizures in 85 percent of patients.

MRI is a valuable method of examination in the analysis of cerebral hemiatrophy as it has the ability to bring to light changes in the cerebral hemispheres as well as highlighting bony structural changes and thus differentiating between congenital and acquired types of DDMS.

Madan Mohan Gupta, Radiology Resident; Harinder Yant, Radiology Resident; N. U. Bahri, MD, S. L. Chudasama, MD; and Reeta Gupta MD
Department of Radiodiagnosis, M.P. Shah Medical College, Jamnagar, Gujarat, India

1. Atalar MH, Icagasioglu D, Tas F. Cerebral hemiatrophy (Dyke-Davidoff-Masson syndrome) in childhood: Clinicoradiological analysis of 19 cases. Pediatr Int 2007;49:70-5.
2. Kochar DK, Jain N, Sharma BV. Dyke-Davidoff-Masson syndrome-Neuroimage. Neurol India 2001;49:417-8. [PUBMED]
3. Shen WC, Chen CC, Lee SK, Ho YJ, Lee KR. Magnetic resonance imaging of cerebral hemiatrophy. J Formos Med Assoc 1993;92:995-1000.
4. Zilkha A. CT of cerebral hemiatrophy. Am J Roentgenol 1980;135:259-62 .
5. Pedley TA, De Vivo DC. Seizure disorders in infants and children. In: Rudolph AM, Hoffman JI, editors. Pediatrics. 17th ed. Norwalk: Appleton-Century-Crofts; 1982. p. 216-39.