Median Facial Cleft with a Frontoethmoidal Encephalocele Treated with Craniofacial Bipartition and Free Radial Forearm Flap: A Case Report

Abstract

We describe a patient with a median facial cleft with a frontoethmoidal encephalocele, hypertelorism, hydrocephalus, and cerebrospinal fluid (CSF) leakage referred to our department due to numerous complications after previous surgical treatments. An 8-yearold girl, born with median cleft syndrome, underwent neurosurgical repair of the encephalocele at another hospital and cleft lip/palate repair later in the same year. Her hydrocephalus was treated with a ventriculoperitoneal shunt, but she underwent numerous shunt revisions due to recurrent intracerebral infections. In 2008, she was rehospitalized due to a gramnegative meningitis and cerebral abscess. She underwent surgery where part of her frontal bone was removed due to osteomyelitis. She was referred to our department due to persistent CSF leakage, recurrent infections, and significant dura defect. In addition, she had hypertelorism and a strongly reduced vision. We performed a monobloc and facial bipartition osteotomy where 15 mm of her frontal and nasal bone was removed after facial bipartiton. The dura defect was closed using a free fasciocutanous flap. The patient had no CSF leakage or infections postoperatively, and her hypertelorism was reduced. The case represents the first monobloc and facial bipartition osteotomy performed in Norway as a part of the treatment of median cleft syndrome with a nasoethmoidal encephalocele.

Occipital Encephalocele with Multiple Birth Defects: A Case Report

“…Such midline defects are associated with other midline lesions. 8 This fetus presented with bilateral cleft lip and cleft palate. Such variable association of midline defects with occipital encephalocele can be attributed to multifactorial aetiology.…”

Clinical Study of Cranioplasty Combined With Ipsilateral Ventriculoperitoneal Shunt in the Treatment of Skull Defects With Hydrocephalus

“…Due to the lack of normal skull protection, patients with skull defects with hydrocephalus often have brain tissue deformation, displacement, and excessive bulging (as shown in Fig. 1C), which has a greater impact on the prognosis of patients, so early and effective cranioplasty and placement of a ventriculoperitoneal shunt are important factors to improve the prognosis of patients 10 . However, whether the 2 operations can be carried out simultaneously is still controversial.…”

“…Decompressive craniotomy is an effective measure to treat refractory intracranial hypertension (intracranial pressure [ICP] >20 mm Hg) 1–7 . However, postoperative complications such as hydrocephalus, subdural effusion, encephalocele, intracranial hemorrhage, postoperative infection, and sinking skin flap syndrome often occur, which can affect the prognosis of the patients 1,3–4,7–12 and bring challenges and difficulties to neurosurgeons. Studies have shown that the probability of hydrocephalus after decompressive craniectomy is approximately 11.9% to 36% 5,17 .…”

“…When hydrocephalus develops after decompressive craniectomy, patients need cranioplasty and ventriculoperitoneal shunts to relieve the hydrocephalus 9,16,18 . In the past, ventriculoperitoneal shunts were placed clinically to treat hydrocephalus in 1 stage, and cranioplasty was performed to restore the skull structure in the second stage 10 . However, some studies 16 have shown that after a 1-stage ventriculoperitoneal shunt placement, patients may suffer severe subsidence of the scalp flap at the position of the skull defect, which increases the difficulty of second-stage cranioplasty and postoperative complications such as the formation of hemorrhage and intracranial infection.…”

“…9,16,18 In the past, ventriculoperitoneal shunts were placed clinically to treat hydrocephalus in 1 stage, and cranioplasty was performed to restore the skull structure in the second stage. 10 However, some studies 16 have shown that after a 1stage ventriculoperitoneal shunt placement, patients may suffer severe subsidence of the scalp flap at the position of the skull defect, which increases the difficulty of second-stage cranioplasty and postoperative complications such as the formation of hemorrhage and intracranial infection. In recent years, some studies 2,11,19,20 have shown that 1-stage cranioplasty combined with ventriculoperitoneal shunting is more beneficial and can not only avoid secondary surgical trauma but also reduce the occurrence of complications, including cerebral tissue collapse due to excessive cerebrospinal fluid drainage, brain displacement, subdural effusion, and subdural hemorrhage.…”