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Trigeminal hypoplasia due to vertebrobasilar dolichoectasia: A new entity.

Jha A, Gupta P, Haroon M, Shah G, Gupta G, Khalid M - J Pediatr Neurosci (2015 Apr-Jun)

Bottom Line: The etiology of the disease is unknown and is usually detected incidentally.The predominant clinical manifestations arise due to the mass effect of the dilated vessels and may include cranial nerve compression, extrinsic aqueductal compression, motor and sensory disturbances.Trigeminal hypoplasia is a very uncommon condition, usually described in association with Goldenhar-Gorlin syndrome and has not yet been attributed to vertebrobasilar dolichoectasia.

View Article: PubMed Central - PubMed

Affiliation: Department of Radiodiagnosis, Jawaharlal Nehru Medical College, Aligarh Muslim University, Aligarh, Uttar Pradesh, India.

ABSTRACT
The term "vertebrobasilar dolichoectasia" refers to anomalous dilatation of the intracranial arteries associated with elongation or tortuosity of the affected vessels. The etiology of the disease is unknown and is usually detected incidentally. The predominant clinical manifestations arise due to the mass effect of the dilated vessels and may include cranial nerve compression, extrinsic aqueductal compression, motor and sensory disturbances. Trigeminal hypoplasia is a very uncommon condition, usually described in association with Goldenhar-Gorlin syndrome and has not yet been attributed to vertebrobasilar dolichoectasia. The current case report highlights this rare association of trigeminal nerve hypoplasia and vertebrobasilar dolichoectasia, leading to hemifacial and corneal anesthesia.

No MeSH data available.


Related in: MedlinePlus

(a and b) Time of flight magnetic resonance angiography showing dolichoectatic left vertebral artery. Note that there is no confluence of vertebral arteries and no basilar artery is seen. The posterior cerebral arteries are not distinctly visualized. (c) Further evaluation through the constructive interference in steady state sequences revealed bifurcation of the right vertebral artery (black arrow) with anomalous high union of the left vertebral artery with the left branch of the right vertebral artery at level of brachium conjunctivum (not shown in figure)
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Figure 5: (a and b) Time of flight magnetic resonance angiography showing dolichoectatic left vertebral artery. Note that there is no confluence of vertebral arteries and no basilar artery is seen. The posterior cerebral arteries are not distinctly visualized. (c) Further evaluation through the constructive interference in steady state sequences revealed bifurcation of the right vertebral artery (black arrow) with anomalous high union of the left vertebral artery with the left branch of the right vertebral artery at level of brachium conjunctivum (not shown in figure)

Mentions: No definite evidence of thrombosis was seen, and the rest of the brain parenchyma was normal at the time of the scan. For further work up of the disease etiology, patient underwent magnetic resonance imaging (MRI) of the head with magnetic resonance angiography (MRA). MRI of the brain showed the abnormally dilated vessel to be the left vertebral artery, with evidence of compression of the left inferior olivary nucleus causing its flattening and the brachium pontis [Figures 2a and b, 3]. Application of constructive interference in steady state sequence revealed that the ectatic vessel was associated with left-sided trigeminal nerve hypoplasia, with marked thinning of the preganglionic cisternal segment of the trigeminal nerve. The Meckel's cave and the porus trigeminus were empty [Figure 4]. Also noticed was mild displacement of the left facial nerve by the ectatic vertebral artery [Figure 4]. Rest of the visualized cranial nerves appeared normal. MRA showed that the left vertebral artery was unduly prominent, with an aberrant course having an anomalous high union with the left branch of the right vertebral artery at the level of brachium conjunctivum [Figure 5]. There was associated atrophy of the lingula and lobulus centralis on the left side leading to prominent cerebellopontine angle cistern [Figure 4]. The patient was reassured and was advised keratoplasty for correction of corneal ulcer. Being congenital in nature, the pathology was deduced to be nerve hypoplasia rather than nerve atrophy.


Trigeminal hypoplasia due to vertebrobasilar dolichoectasia: A new entity.

Jha A, Gupta P, Haroon M, Shah G, Gupta G, Khalid M - J Pediatr Neurosci (2015 Apr-Jun)

(a and b) Time of flight magnetic resonance angiography showing dolichoectatic left vertebral artery. Note that there is no confluence of vertebral arteries and no basilar artery is seen. The posterior cerebral arteries are not distinctly visualized. (c) Further evaluation through the constructive interference in steady state sequences revealed bifurcation of the right vertebral artery (black arrow) with anomalous high union of the left vertebral artery with the left branch of the right vertebral artery at level of brachium conjunctivum (not shown in figure)
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4489062&req=5

Figure 5: (a and b) Time of flight magnetic resonance angiography showing dolichoectatic left vertebral artery. Note that there is no confluence of vertebral arteries and no basilar artery is seen. The posterior cerebral arteries are not distinctly visualized. (c) Further evaluation through the constructive interference in steady state sequences revealed bifurcation of the right vertebral artery (black arrow) with anomalous high union of the left vertebral artery with the left branch of the right vertebral artery at level of brachium conjunctivum (not shown in figure)
Mentions: No definite evidence of thrombosis was seen, and the rest of the brain parenchyma was normal at the time of the scan. For further work up of the disease etiology, patient underwent magnetic resonance imaging (MRI) of the head with magnetic resonance angiography (MRA). MRI of the brain showed the abnormally dilated vessel to be the left vertebral artery, with evidence of compression of the left inferior olivary nucleus causing its flattening and the brachium pontis [Figures 2a and b, 3]. Application of constructive interference in steady state sequence revealed that the ectatic vessel was associated with left-sided trigeminal nerve hypoplasia, with marked thinning of the preganglionic cisternal segment of the trigeminal nerve. The Meckel's cave and the porus trigeminus were empty [Figure 4]. Also noticed was mild displacement of the left facial nerve by the ectatic vertebral artery [Figure 4]. Rest of the visualized cranial nerves appeared normal. MRA showed that the left vertebral artery was unduly prominent, with an aberrant course having an anomalous high union with the left branch of the right vertebral artery at the level of brachium conjunctivum [Figure 5]. There was associated atrophy of the lingula and lobulus centralis on the left side leading to prominent cerebellopontine angle cistern [Figure 4]. The patient was reassured and was advised keratoplasty for correction of corneal ulcer. Being congenital in nature, the pathology was deduced to be nerve hypoplasia rather than nerve atrophy.

Bottom Line: The etiology of the disease is unknown and is usually detected incidentally.The predominant clinical manifestations arise due to the mass effect of the dilated vessels and may include cranial nerve compression, extrinsic aqueductal compression, motor and sensory disturbances.Trigeminal hypoplasia is a very uncommon condition, usually described in association with Goldenhar-Gorlin syndrome and has not yet been attributed to vertebrobasilar dolichoectasia.

View Article: PubMed Central - PubMed

Affiliation: Department of Radiodiagnosis, Jawaharlal Nehru Medical College, Aligarh Muslim University, Aligarh, Uttar Pradesh, India.

ABSTRACT
The term "vertebrobasilar dolichoectasia" refers to anomalous dilatation of the intracranial arteries associated with elongation or tortuosity of the affected vessels. The etiology of the disease is unknown and is usually detected incidentally. The predominant clinical manifestations arise due to the mass effect of the dilated vessels and may include cranial nerve compression, extrinsic aqueductal compression, motor and sensory disturbances. Trigeminal hypoplasia is a very uncommon condition, usually described in association with Goldenhar-Gorlin syndrome and has not yet been attributed to vertebrobasilar dolichoectasia. The current case report highlights this rare association of trigeminal nerve hypoplasia and vertebrobasilar dolichoectasia, leading to hemifacial and corneal anesthesia.

No MeSH data available.


Related in: MedlinePlus