The Transpalatal Approach to Repair of Congenital Basal Skull Base Cephaloceles

Abstract

Basal skull base herniations, including meningoceles and encephaloceles, are rare and may present with characteristic facial and neurologic features. The traditional craniotomy approach has known morbidity, and nasal endoscopy may not allow for control of large posterior basal defects, especially in newborns. We present two cases of successful repair of basal transsphenoidal meningoceles using an oral-transpalatal approach. The first patient with an intact palate presented with respiratory distress, and a palatectomy was performed for access to the skull base. The second patient had a large basal herniation that was reduced through a congenital midline cleft palate, and a calvarial bone graft was used to repair the defect. A literature search revealed 10 previous successful cases using the transpalatal repair, which allows for excellent access, low morbidity, and a team-oriented method to skull base surgery.

Combined Endoscopic Endonasal and Transpalatal Repair of Congenital Anterior Cranial Fossa Agenesis

“…Anterior skull base congenital meningoceles and encephaloceles do not always present in isolation, and several distinctive associated clinical features have been reported. 1,[3][4][5] These associations include hypertelorism, broad nasal dorsum, and cleft lip and palate. It is believed that the cleft palate is a result of the herniated sac which interferes with midline fusion of the secondary palate between the 6th and 12th week of gestation, as disruption of the skull base formation occurs before the 10th week.…”

“…[11][12][13] The transpalatal approach has been used and described in over 12 cases and can also be used in patients with an intact palate. 5 In such instances, a U-shaped incision is made over the hard palate, while keeping the soft palatal flap bipedicled at each anterior tonsillar pillar. The soft palate flap is retracted anteriorly, and the posterior aspect of the hard palate can be removed to improve further anterior visualization.…”

Craniofacial Bone Tissue Engineering: Current Approaches and Potential Therapy

“…Large cranial defects can result from a variety of conditions, including congenital defects [ 1 , 2 , 3 , 4 ], tumor resection [ 3 , 5 , 6 ], infection [ 5 , 6 ], and severe trauma [ 3 , 4 , 6 , 7 ]. Critical-size cranial defects can leave a large area of the brain unprotected with a significant cosmetic deformity [ 4 , 7 ].…”

A hybrid simulation model for pre-operative planning of transsphenoidal encephalocele

“…A basal encephalocele is defined as a neural element herniation, including the pituitary gland or optic chiasm, through an anatomical defect in the base of the skull [1][2][3][4]. They represent 1.5-5% of all cephaloceles, and the transsphenoidal encephalocele is the rarest form [2,5,6], with an incidence estimate of 1 in 700,000 live births [5][6][7]. Because of its rarity, the approaches to repair this congenital anomaly remain a challenge.…”

“…Transnasal endoscopic techniques have rapidly evolved, and their advantages over the open method include improved visualization and decreased morbidity. Nevertheless, most reports focus on basal encephaloceles in newborns and infants who have a much smaller nasal cavity and more difficult access [7]. In cases of transsphenoidal encephaloceles with significant skull defects, most authors prefer transpalatal approaches to avoid the recurrence of the herniation through safe and wide access [3,4].…”