ABSTRACT
BACKGROUND: A persistent craniopharyngeal canal (PCC) is a rare cause of cerebrospinal fluid rhinorrhea in children. The condition often coexists with other midline facial defects, such as cleft palate. Children with PCC may also have pituitary dysfunction or neoplasms, such as craniopharyngiomas within the canal. CASE DESCRIPTION: A 5-year-old girl presented with bacterial meningitis and active cerebrospinal fluid rhinorrhea from her left nostril. Imaging showed a large nasopharyngeal meningoencephalocele, communicating with the subarachnoid space through a persistent craniopharyngeal canal. An endonasal approach was chosen to excise the PCC and meningoencephalocele and to repair the resulting skull base defect. CONCLUSIONS: The extended endonasal approach can be used to treat PCC with nasopharyngeal encephaloceles in young children. The approach is suitable to address both conditions at the same time. The extended endonasal approach avoids potentially morbid transfacial approaches and can help in earlier recovery after surgery.
Subject(s)
Encephalocele/surgery , Meningocele/surgery , Natural Orifice Endoscopic Surgery , Neuroendoscopy , Cerebrospinal Fluid Rhinorrhea/diagnostic imaging , Cerebrospinal Fluid Rhinorrhea/surgery , Child, Preschool , Encephalocele/diagnostic imaging , Female , Humans , Meningocele/diagnostic imaging , Nasal Cavity/surgery , Skull Base/diagnostic imaging , Skull Base/pathology , Skull Base/surgery , Sphenoid Bone/diagnostic imaging , Sphenoid Bone/pathology , Sphenoid Bone/surgeryABSTRACT
Encephaloceles are known as rare craniofacial developmental anomalies which are herniation of cranial contents through a skull defect. Sphenoidal meningoencephalocele represents an important cause of reVersible visual loss. We report a rare case of pituitary adenoma accompanying sphenoidal meningoencephalocele with clival extension. Although the definitive diagnosis of cystic lesions in the sellar region before surgery is difficult, accurate diagnosis of these lesions is important to determine the type of treatment and predict prognostic outcome.
Subject(s)
Craniofacial Abnormalities , Diagnosis , Encephalocele , Pituitary Neoplasms , SkullABSTRACT
Encephaloceles are known as rare craniofacial developmental anomalies which are herniation of cranial contents through a skull defect. Sphenoidal meningoencephalocele represents an important cause of reVersible visual loss. We report a rare case of pituitary adenoma accompanying sphenoidal meningoencephalocele with clival extension. Although the definitive diagnosis of cystic lesions in the sellar region before surgery is difficult, accurate diagnosis of these lesions is important to determine the type of treatment and predict prognostic outcome.
