Ketan Rarhod, MD

 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


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3. Lowe LH, Booth TN, Joglar JM, et al. Midface anomalies in children. 

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4. Altintas AG, GÃ¼l aksoy FG, Altintas CS, et al. Evaluation of findings in Crouzon's syndrome. 

Articles

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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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 35-year-old patient presented with complaint of gradually progressive, painless swelling in left parotid region for 8 months. No history of fever and laboratory examination, past and family history was unremarkable. On examination, there was a smooth well marginated nontender swelling in left parotid gland. No evidence of enlarged cervical lymph nodes was seen.

Further noncontrast CT scan was performed, which revealed well-defined, well-circumscribed smooth marginated solid cystic lesion in superficial and deep lobes of left parotid gland, predominantly in posteroinferior part of gland, medially the lesion extends into stylomandibular tunnel (Figure 1). On postcontrast CT, irregular nodular enhancement of solid part was noted and in close relation to left external carotid artery and IJV, and antero-inferiorly retromandibular vein was compressed by the lesion (Figure 2). On noncontrast MRI, T2WI showed heterogenous signal intensity, T1WI images showed isointense signal within lesion and coronal showed slight hyperintense signal (Figure 3). There was no evidence of any enlarged cervical or intraparotid lymph nodes.

On basis of unilateral solid cystic lesion in middle-aged patient with irregular nodular enhancement of solid part of lesion with isointense signal on T1 and slight hyperintense signal on STIR images suggestive of two differential diagnoses were:

1. Cystic pleomorphic adenoma (due to unilateral solid cystic parotid lesion in middle-aged patient, enhancing solid part and no adjacent intraparotid and cervical lymph nodes)
2. Warthin tumor (due to irregular nodular enhancing solid part with isointense signal on STIR MRI)

Further ultrasound guided FNAC was taken, aspirated material appeared chocolate brown. On smear examination, oncocytes and lymphocytes cells were seen (Figure 4). Patient was operated on and histopathology revealed cystic spaces lined by a bilayer of oncocytic cells and abundant lymphocytes in the sub epithelial stroma (Figure 5).

 Noncontrast CT (A, axial and B, coronal) images show well-defined, well-circumscribed smooth marginated solid cystic lesion (arrow) involving superficial and deep lobes of left parotid gland predominantly in posteroinferior part of gland, further medially the lesion extends into stylomandibular tunnel (small arrow).

 Postcontrast CT (A, C, axial and B, D, coronal) images show irregular nodular enhancement of solid part (small arrow) and the lesion lies in close relation with left external carotid artery and IJV (large arrow) and antero-inferiorly retromandibular vein was compressed by the lesion.

 On noncontrast MRI, T2WI axial (A) shows heterogenous signal intensity, T1WI axial (B) shows isointense and STIR images (C, axial and D, coronal) images show slightly hyperintense signal intensity within lesion. No evidence of any enlarged cervical or intraparotid lymph nodes.

 On USG guided FNAC, aspirated material appears chocolate brown. On smear examination, oncocytes and lymphocytes cells are seen.

 Histopathology shows cystic spaces lined by a bilayer of oncocytic cells and abundant lymphocytes in the sub epithelial stroma.

 A warthin tumor (or papillary cystadenoma lymphomatosum) is a benign sharply demarcated tumour of the parotid gland. It is bilateral in 10-15 percent of cases. They are the second most common benign parotid tumour (after pleomorphic adenoma) and are the most common bilateral or multifocal benign parotid tumour. It typically occurs in the elderly (6th decade). There is a recognized male predilection. Patients typically present with painless parotid swelling. It favors parotid tail region for its location. It has a greater tendency to undergo cystic change than any other salivary gland tumor. On ultrasound, it shows as a relatively well-defined, ovoid, hyper echoic mass with anechoic internal cystic areas. On CT, it can be often seen bilaterally and appear as a cystic lesion posteriorly within the parotid with a focal tumor nodule relatively well-defined cystic changes with no calcification. On MRI, T1WI show low to intermediate signal with cyst containing cholesterol components containing focal high signal and on T2WI heterogenous signal intensity, on STIR images, hyperintense signal intensity within lesion. On postcontrast CT or MRI, solid part shows enhancement. Differential diagnosis includes adenoma, benign (BLEL) in HIV, carcinoma, adenoid, parotid nodal metastasis and parotid non-Hodgkin lymphoma.


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Case History: 35-year-old patient with gradually progressive, painless swelling in left parotid region, unremarkable history.

Unilateral Warthin Tumor Mimics Cystic Pleomorphic Adenoma 

 A 45-year-old female presented with complaint of occasional headache for last three years. Family and past history were unremarkable. Neurological and systemic examinations were normal. Blood analysis was within normal limits and EEG did not show abnormal discharges.

. T2W axial (A) and sagittal (B) MRI image show well defined intermediate to high signal intensity lesion in quadrigeminal cistern on right side (large white arrow) abutting inferior coliculus. The lesion does not cause mass effect or obstruct hydrocephalous and normal corpus callosum (small white arrow) in sagittal image.

. T1W axial MRI image shows high signal intensity lesion in quadrigeminal cistern on right side (arrow) which follow fat signal.

. FLAIR coronal MRI images show high signal intensity lesion in quadrigeminal cistern (arrow).

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

. T1W fat sat pre-contrast (A) axial MRI image shows hypointense signal, suggests suppression of fat signal of the lesion (arrow) and T1W fat sat post-contrast (B) axial image shows no enhancement within the lesion (arrow).

Patient was referred for MRI brain by neurophysician.MRI was performed using 1.5 Tesla Siemens Magnetom Essenga spin echo T2 weighted (T2WI), T1WI, fluid attenuation inversion recovery (FLAIR), diffusion weighted (DWI), pre-contrast T1W fat sat and post-contrast T1W fat sat with slice thickness 5 mm. On MRI, there was a lesion in quadrigeminal plate cistern on the right side which showed intermediate to high signal on T2WI (Figure 1), high signal onT1WI (Figure 2), FLAIR (Figure 3), no restriction on diffusion weighted imaging (Figure 4), low signal on pre-contrast T1 fat sat (Figure 5) and showed no enhancement on post-contrast T1W fat sat (Figure 6). Sagittal MR revealed a normal corpus callosum (Figure 7). The diagnosis lipoma in quadrigeminal plate cistern on right side was made. The patient was managed conservatively and the patient's headaches subsided with conservative management.

 Lipoma in quadrigeminal plate cistern on right side

 Intracranial lipomas (ICLs) are rare intracranial lesions. They represent a group of congenital malformations of the brain parenchyma, are located more frequently in the pericallosal cistern and associated with other parenchymal or brain vascular malformations in up to half of cases. Intracranial lipomas are slow growing benign congenital lesions accounting for 0.1-0.5 percent of all primary brain tumors and are usually detected as incidental findings.

 Intracranial lipomas are histologically benign lesions that are congenital hamartomatous malformations derived from the embryologic meninx primitiva. ICLs are usually an isolated finding, but, uncommonly, may be associated with other intracranial malformations such as callosal dysgenesis with pericallosal lipomas. Lipomas of the quadrigeminal plate are usually asymptomatic,

 but may cause significant symptoms of mass effect in about 20 percent of patients.

 Pressure on the ventricular system can result in consequent neurologic deficits, obstructive hydrocephalus or raised intracranial pressure.

 Radiological characteristics of quadrigeminal cistern lipoma is always definitive on imaging, hence histopathological confirmation is almost never required.

 On CT scan, the lipoma is characterized by attenuation values of fat density (-50 to -100 Hounsfield units) and absence of post-contrast enhancement.

 On MRI, the lipoma has short T1 and T2 and therefore, a hyperintense signal is present on T1-weighted images and a relatively low to moderate intensity signal on T2-weighted images. The differential diagnoses of lipomas in the quadrigeminal cistern include: arachnoid cysts, tectal plate cyst, tectal masses (gliomas), supracerebellar abscess, dermoid and epidermoid cysts, ruptured P4 segment aneurysm of the posterior cerebral artery and, rarely, pineal region mass.

 The lipoma can be differentiated with other negative attenuation value lesions (epidermoid or dermoid tumors) that two tumors will demonstrate heterogeneous attenuation values.8 Intracranial lipomas rarely become symptomatic and surgery is seldom required. The usual neurological symptoms are intracranial hypertension and hydrocephalus, which can be treated by shunt procedure.

 Incidental and asymptomatic lipoma of the quadrigeminal region do not require surgical intervention,

 as our patient also presented with headaches but it appeared to be unrelated to the lipoma, as no compressive, obstructive or evidence of raised intracranial pressure were seen, hence the patient was managed conservatively.


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Case History: 45-year-old female with complaint of occasional headache for three years, had normal neurological and systemic exams.

Quadrigeminal Plate Cistern Lipoma 

 A 65 year old patient presented with complaint of sudden onset of left sided hemiparesis. He also had complaints of a headache for the past five months. He has a known case of diabetes mellitus and IHD and has had regular treatment. Review of family history was unremarkable. On examination, there was severe loss of motor function on the left side of his body. The patient was then subjected to radiographic investigations.

. Non contrast CT brain shows a large well-defined broad dural based extra axial solid (arrow) cystic lesion in right parietal region causing buckling of underlying cortex and white matter oedema.

. Post-contrast CT brain shows a large well-defined broad dural based extra axial solid cystic lesion in right parietal region causing buckling of underlying cortex and white matter oedema with intense homogenous enhancement of solid portion of the lesion (arrow) and multiple intratumoral vascular channels (small arrow).

. Non-contrast MRI brain shows a large well-defined broad dural based extra axial solid cystic lesion in right parietal region causing buckling of underlying cortex (arrow) and white matter oedema with midline shift of 6 mm towards left side. The lesion appears hyperintense on T2WI with internal dural based solid portion and septations and hypointense on T1WI.

. Post-contrast MRI coronal T1 fat sat images intense homogenous enhancement of broad dural based solid portion of the lesion (arrow) and multiple intratumoral vascular channels (small arrow) and internal enhancing septae.

Non-contrast brain CT revealed a large well-defined broad dural based extra axial solid cystic lesion in right parietal region causing buckling of underlying cortex (Figure 1). On post-contrast study, solid portion of the lesion shows intense homogenous enhancement and multiple intratumoral vascular channels (Figure 2). Further brain MRI showed large extra axial broad dural based solid cystic mass in right parietal causing buckling of underlying cortex and white matter edema with midline shift of 6 mm towards left side (Figure 3).On post-contrast MRI, similar enhancement of the solid part was seen as on the CT and multiple intratumoral vessels and enhancing septae were seen (Figure 4).

 Cystic meningioma is applied to both meningiomas with intratumoral degenerative cyst formation as well as those with peritumoral arachnoid cysts or reactive intraparenchymal cysts. Patients present clinically in the same way as patients with non-cystic meningiomas, with symptoms related to increased intracranial pressure (ICP), focal neurology or seizures.

 divided cystic meningiomas into four subtypes according to the location of the cyst with respect to the brain and meningioma:
Type 1: Intratumoral cyst(s), located centrally within the meningioma
Type 2: Intratumoral cyst(s), located peripherally within the meningioma but still surrounded by tumor
Type 3: Cyst(s) located within the adjacent brain
Type 4: Cyst(s) located between the meningioma and brain (arachnoid cyst)

The tumor itself has imaging features identical to non-cystic meningiomas. The cysts are of variable size and can be entirely surrounded by tumor (types 1 or 2) or clearly between tumor and brain (type 4) or within the adjacent brain (type 3). On imaging it is sometimes difficult to distinguish between these types.

The differential is dependent on the location of the tumor. For tumors around the base of skull, cystic schwannoma (eg, acoustic schwannoma, trigeminal schwannoma) are the main differential, as they share the bright contrast enhancement, and are commonly cystic when large.


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