Imaging Assessment of Re-Exploratory Repair of an Occipital Bone Defect-Associated Tectocerebellar Dysraphism via Hybrid Cranioplasty

2016 ◽  
Vol 51 (3) ◽  
pp. 164-166 ◽  
Author(s):  
Daniel Thomas Ginat ◽  
Russell Reid ◽  
David M. Frim
Keyword(s):  
Bone Defect ◽  
Occipital Bone ◽  
Occipital Bone Defect

Suboccipital meningocele presenting as a huge retropharyngeal mass in a patient with neurofibromatosis Type 1

1999 ◽  
Vol 91 (3) ◽  
pp. 503-505 ◽  
Author(s):  
Masanori Kurimoto ◽  
Yutaka Hirashima ◽  
Nakamasa Hayashi ◽  
Shunro Endo ◽  
Masayoshi Ohi ◽  
...  
Keyword(s):  
Bone Defect ◽  
Neurofibromatosis Type 1 ◽  
Contrast Material ◽  
Neurofibromatosis Type ◽  
Occipital Bone ◽  
Cutaneous Manifestations ◽  
Content Type ◽  
The Right ◽  
Occipital Bone Defect

✓ The authors report an extremely rare case of neurofibromatosis Type 1 (NF1) with a suboccipital meningocele presenting as a huge retropharyngeal mass. A 73-year-old woman with typical cutaneous manifestations of NF1 presented with nasal obstruction and dysphagia due to a retropharyngeal mass. Magnetic resonance imaging revealed a huge mass lesion extending from the right occipital bone defect to the retropharynx through the right paravertebral region. Computerized tomography scanning after intrathecal administration of contrast material confirmed that the mass was a meningocele protruding through a right occipital bone defect. The authors attempted to ligate this meningocele, most of which was excised via a suboccipital approach, but a second transcervical operation was required. Finally, the meningocele resolved and the patient was discharged without symptoms.

Iniencephaly: neuroradiological and surgical features

1998 ◽  
Vol 89 (2) ◽  
pp. 317-320 ◽  
Author(s):  
Pamir Erdinçler ◽  
Mehmet Y. Kaynar ◽  
Bülent Canbaz ◽  
Naci Koçer ◽  
Cengiz Kuday ◽  
...  
Keyword(s):  
Congenital Anomaly ◽  
Cervical Spine ◽  
Bone Defect ◽  
Cervical Vertebrae ◽  
Psychomotor Retardation ◽  
Occipital Bone ◽  
Content Type ◽  
Rare Congenital Anomaly ◽  
Occipital Bone Defect ◽  
Fine Print

Iniencephaly is a rare congenital anomaly characterized by spina bifida of the cervical vertebrae, fixed retroflexion of the head on the cervical spine, and occipital bone defect. There are only five reports of surviving patients with iniencephaly. The authors report the case of a newborn who presented with iniencephaly and an encephalocele that were surgically treated in our service. Neurological examination of the patient yielded normal results except for a moderate psychomotor retardation. The neuroradiological and surgical findings of the case suggested that the trigger of the anomaly was the occipital bone defect and rachischisis of the posterior vertebral arches.

Ruptured Aneurysms of the Occipital Artery Associated with Congenital Occipital Bone Defect

2017 ◽  
Vol 97 ◽  
pp. 759.e13-759.e15 ◽  
Author(s):  
Toshinari Kawasaki ◽  
Kazumichi Yoshida ◽  
Takayuki Kikuchi ◽  
Akira Ishii ◽  
Yasushi Takagi ◽  
...  
Keyword(s):  
Bone Defect ◽  
Occipital Bone ◽  
Occipital Artery ◽  
Ruptured Aneurysms ◽  
Occipital Bone Defect

Knobloch Syndrome, a Rare Cause of Occipital Encephalocele and Seizures: A Case Report

2021 ◽  
pp. 1-5
Author(s):  
Bhaskar Madivala Venkateshappa ◽  
Bharath Raju ◽  
Michael S. Rallo ◽  
Fareed Jumah ◽  
Sumatha Channapatna Suresh ◽  
...  
Keyword(s):  
Bone Defect ◽  
Autosomal Recessive ◽  
Surgical Intervention ◽  
Early Recognition ◽  
Autosomal Recessive Disorder ◽  
Occipital Bone ◽  
Skull Defect ◽  
Occipital Encephalocele ◽  
Knobloch Syndrome ◽  
Occipital Bone Defect

<b><i>Background:</i></b> Knobloch syndrome (KS) is a rare autosomal recessive disorder associated with multiple ocular and cranial abnormalities. Occult occipital skull defect or encephalocele should raise suspicion of this disease. It is never reported in neurosurgical literature, possibly due to a lack of clinician familiarity, leading to underdiagnosis and inadequate management. Our patient also had seizures, which is a sporadic presentation of this syndrome. <b><i>Case Description:</i></b> Here, we report a clinico-radiologic finding of a 7-year-old boy who presented with seizures, cataracts, and an occipital bone defect along with bilateral subependymal heterotopias and polymicrogyria. <b><i>Conclusions:</i></b> This case highlights the importance of consideration of this syndrome in children with a midline occipital bone defect with or without encephalocele and seizures. Early recognition of this presentation is critical for obtaining access to appropriate genetic counseling and subsequent monitoring and prevention of complications by surgical intervention.

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