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


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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.


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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.

Author | Madan Mohan Gupta

Articles

Cough, Fever

Subependymoma of Lateral Ventricle

Crouzon Syndrome

Complete Fistula of Third Branchial Cleft