- Email: [email protected]
Frontoethmoidal encephalocele. Report of a case
NEUCIR-324; No. of Pages 6
ARTICLE IN PRESS n e u r o c i r u g i a . 2 0 1 8;x x x(x x):xxx–xxx
NEUROCIRUGÍA www.elsevier.es/neurocirugia
Case Report
Frontoethmoidal encephalocele. Report of a case Angel Horcajadas a,∗ , Antonio Palma b , Babar M. Khalon a a b
Neurosurgery, King Saud Medical City, Riyadh, Saudi Arabia Maxillofacial, King Faisal Specialist Hospital, Riyadh, Saudi Arabia
a r t i c l e
i n f o
a b s t r a c t
Article history:
Encephaloceles are uncommon in western countries and most cases are located in the occip-
Received 14 November 2017
ital bone. Frontal encephaloceles may involve the ethmoid bone, nasal bones and/or the
Accepted 18 February 2018
orbits. Surgical repair is complex and usually requires a multidisciplinary approach. The
Available online xxx
goal of the surgery is to reconstruct the normal anatomy, to achieve a good cosmetic repair and to avoid a cerebrospinal fluid leak. We present a case of a patient with a large congeni-
Keywords:
tal frontoethmoidal encephalocele. Autologous calvarian bone grafts were used to repair of
Encephalocele
encephalocele defect and for the reconstruction of the frontonasal area. The defect closure
Congenital malformation
and the cosmetic result were satisfactory, and the only complication detected was the infec-
Frontoethmoidal encephalocele
tion of a previously performed ventriculoperitoneal shunt. A description of the technique
Nasal encephalocele
and a review of the literature are presented. ˜ ˜ S.L.U. All rights © 2018 Sociedad Espanola de Neurocirug´ıa. Published by Elsevier Espana, reserved.
Encefalocele frontoetmoidal. A propósito de un caso r e s u m e n Palabras clave:
Los encefaloceles son infrecuentes en los países occidentales y su localización más fre-
Encefaloceles
cuente es occipital. Los encefaloceles frontales pueden afectar hueso etmoidal, frontal y/o
Malformación congénita
órbitas. La reparación quirúrgica es compleja y habitualmente precisa de un abordaje mul-
Encefalocele frontoetmoidal
tidisciplinar. El objetivo de la cirugía es reconstruir la anatomía del paciente con un buen
Encefalocele nasal
resultado estético, y evitar la fístula de líquido cefalorraquídeo. Se presenta un caso de un gran encefalocele frontoetmoidal. El encefalocele fue reparado y la reconstrucción ósea se realizó con hueso autólogo de la capota craneal. El cierre y el resultado cosmético fueron buenos y la única complicación fue una infección posquirúrgica. Se describe la técnica y se revisa la literatura publicada al respecto. ˜ ˜ S.L.U. Todos © 2018 Sociedad Espanola de Neurocirug´ıa. Publicado por Elsevier Espana, los derechos reservados.
∗
Corresponding author. E-mail address: [email protected] (A. Horcajadas). https://doi.org/10.1016/j.neucir.2018.02.006 ˜ ˜ S.L.U. All rights reserved. 1130-1473/© 2018 Sociedad Espanola de Neurocirug´ıa. Published by Elsevier Espana,
Please cite this article in press as: Horcajadas A, et al. Frontoethmoidal encephalocele. Report of a case. Neurocirugia. 2018. https://doi.org/10.1016/j.neucir.2018.02.006
NEUCIR-324; No. of Pages 6
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Introduction Encephaloceles represent a group of disorders in which a skull defect allows for extracranial herniation of leptomeninges, brain, and CSF.1 They can be classified as primary if they are present at birth or secondary if they are acquired following trauma or surgery.2 The occipital bone is the most common location3 and frontoethmoidal encephaloceles are less frequent. A case of fronto-ethmoidal encephalocele is presented as well as the surgical technique performed and a review of the literature up to the present date.
Case report The case is presented of an Arabic female premature newborn (gestational age of 30 weeks) with a large frontal mass. The mass was bulging between the two orbits and occupying the lowest part of the frontal area and the superior part of the nasal area. Skin was intact (Fig. 1), though thin and presented some degree of exudation. Neurologically there were no motor deficits and the baby moved spontaneously the four limbs. She was active and taking orally without any problem. MRI showed a large encephalocele through a frontoethmoidal bone defect, consisting of most of the right cerebral hemisphere herniated through the defect. CT scan showed a bone defect in the zone of frontal bone, lamina cribrosa and anterior part of ethmoidal bone (Fig. 2). The weight of the baby at birth was 1.500 g and there was no CSF leak so a elective surgery was decided. A ventriculoperitoneal shunt was needed after 1 month due to the development of hydrocephalus. Surgery was performed when the patient was 7 months old and reached 3000 g.
Surgical technique A 3D model of the skull from a CT scan was made to identify clearly the bone defect and to plan the surgery (Fig. 3). The CT scan was exported as a DICOM file and processed with virtual planning software (ProPlan CMF).The morphology of the defect was established and measured, and the relation with the soft tissue coverage was identified using a virtual superposition of the soft tissues. A 3D stereolithographic model of the skull was printed and sterilized, and taken to the operative room in order to help as an anatomical reference during the surgery. The surgery was performed by a multidisciplinary team. Steps of the surgery can be seen in Fig. 5. A coronal skin incision was performed exposing the frontal bone, upper orbital edges and the frontal part of the encephalocele. A bifrontal craniotomy was done from the edge of the bone defect to expose the floor of the anterior cranial fossa surrounding the sac (Fig. 4). After that, the Maxillofacial surgeon made lateral rhinotomy incision over the encephalocele and a circumferential dissection of the encephalocele’s sac was performed, freeing it from the surrounding tissue, including the nasal bones and the
left orbital rim, preserving the lacrimal sac and the medial cantal ligament attachment. Once the sac was completely dissected and isolated from the surrounding tissues, and the intracranial and facial edges of the sac were connected, removal of the functionless brain tissue and suture of the dural sac in a watertight fashion were performed. ® Tachosil and a pericranial flap were used to reinforce the closure and fill the dead space. Two full thickness cortical block bone grafts were harvested from the posterior border of the craniotomy, in the parietal bone, according to the size and shape recommend by the virtual planning software in order to reconstruct the glabella and the nasal bone. The grafts were wired with absorbable sutures (PDS 3–0) to the remaining nasal bones. Reposition of the craniotomized frontal bone was performed and stabilized again with reservable sutures. Skin flap over the nasal area was remodeled to achieve a cosmetic closure. The patient was transferred to the Pediatric ICU under mechanical ventilation, and delayed extubation was performed without complication after 24 h.
Result No intraoperative complications were suffered, surgical site was intact, and the evolution of the patient was satisfactory from the cosmetic and functional point of view. There was no CSF leak. The patient developed a shunt infection that required external ventricular drainage and antibiotic treatment. Fig. 6 shows the cosmetic result and postoperative CT scan where the reconstruction of frontal and nasal bone can be seen.
Discussion Encephaloceles are one of the so called neural tube defects. There is an herniation of the meninges and brain matter through a structural defect in the skull.1 Encephaloceles are classified based on the location and type of skull defect in three main types: frontoethmoidal, occipital, cranial vault and basal type (Table 1). The most frequent location is the occipital cranial vault (80% of the cases).3 Frontoethmoidal encephaloceles, collectively known as nasal encephaloceles, have three subtypes according to the bone associated with the defect: nasofrontal, nasoethmoidal, and naso-orbital.4,5 The nasoethmoidal subtype is most frequently reported in the literature2,6–8 and the naso-orbital subtype is the least common.2,9 Estimated incidence in North America and Western countries is 1–3 per 10,000 live births and the most common location is occipital. Most cases of nasal encephaloceles have been found in Southeast Asian countries with very rare cases reported in Europe, North America, and the Middle Eas.4,10–12 Etiology of encephalocele is not well known. Many have defined it as a multifactorial condition, with a combination of genetic and environmental factors.2 Most of the cases are present at birth as masses over the nose, glabella and/or forehead, usually covered by skin.3 Sometimes encephaloceles are hidden and may present with symptoms as snoring, nasal obstruction, CSF leak or recurrent
Please cite this article in press as: Horcajadas A, et al. Frontoethmoidal encephalocele. Report of a case. Neurocirugia. 2018. https://doi.org/10.1016/j.neucir.2018.02.006
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Fig. 1 – Preoperative images of the frontoethmoidal encephalocele.
Fig. 2 – MRI and 3D CT scan, most of the right hemisphere is herniated through the bone defect. The bone defect involves frontal bone, cribrosa laminae and ethmoidal bone. Orbits are intact.
meningitis.4,6 This is the main reason why the age at diagnosis has been reported to range from 0 to 40 years old.1,10,13,14 The presence of hydrocephalus is a main factor in the management of encephaloceles.8,13 Untreated or late treated cases present a high risk of postoperative CSF leak.13,15,16 Associated hydrocephalus should be treated and solved before surgical treatment. MRI and CT scan are necessary to plan the surgery. MRI is essential to understand the anatomy of the herniated brain and its relations with the surrounding structures. AngioMRI is also useful to evaluate the position of the anterior cerebral arteries, because sometimes they are herniated in the sac. CT scan with 3D reconstruction helps to define accurately the bone defect and to plan the surgery. In the case presented the 3D model made for the planning helped to understand the anatomy of the cranial defect and to plan the surgical reconstruction. Angiography is only recommended when presence of significant vessels is suspected inside the encephalocele. Surgery is the only treatment of frontoethmoidal encephaloceles. Skin is usually intact so elective surgery is indicated. Planning of surgery and timing of operation is crucial in management of anterior encephaloceles.13,17–20 Surgery is usually long and blood loss and hypothermia are frequent, which remain the two most important intraoperative complications. If there is no active CSK leak or
infection, delayed surgery is preferred and some authors recommend surgery at 8–10 months of age.8,13,16 On the other hand, frontoethmoidal encephaloceles should be treated at an early age to avoid further facial distortion during growth.20 Due to the nature of the illness, a multidisciplinary approach is strongly recommended.2,6,21 Endoscopic transnasal approaches6,7 have been successful in many cases of nasal encephaloceles but most authors recommend the “combined procedure” which combines a bicoronal and a nasofrontal flap approach with facial reconstruction.2,11 This approach provides three main advantages to surgeons and patients: a more successful closure of the meningoencephalocele, with lower risk of CSF leak, the possibility of telecanthus correction if necessary, and in some cases the reduction of facial scars.11 In many studies, the surgical procedure includes frontal coronal scalp flap incision to gain exposure to the craniofacial bones combined with the transfacial incision. Some authors prefer to limit the repair of the encephalocele through the transfacial approach, without the need of a coronal approach and bifrontal craniotomy, making the procedure less agressive.20 This is possible if the sac and the bone defect are of a moderate size. If the orbits are involved in the deformity causing hypertelorism, a correction technique is necessary. Usually there is no need
Please cite this article in press as: Horcajadas A, et al. Frontoethmoidal encephalocele. Report of a case. Neurocirugia. 2018. https://doi.org/10.1016/j.neucir.2018.02.006
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n e u r o c i r u g i a . 2 0 1 8;x x x(x x):xxx–xxx
Fig. 3 – 3D model built from 3D CT scan.
of this functionless tissue is possible. Postoperative followup is essential because of the risk of CSF rhinorrhea and infection.22 CSF leaks are the most common postoperative complication. There is an overall low mortality rate associated with encephaloceles, with a 3% surgery-related mortality.12 Some patients may need secondary cosmetic surgery, like rhinoplasty or eyelid repair, at a later stage, depending on the involvement of soft tissues. The case presented is a big frontoethmoidal encephalocele. Surgical planning using a 3D model was extremely useful to prepare the surgery and to achieve a good reconstruction. A multidisciplinary approach was planned to approach the case. The cosmetic result and the closure were very good and no CSF leak was found after the surgery. The shunt infection presented after surgery was undoubtedly related to it and required shunt removal and external ventricular drainage. The age and the weight at surgery were maybe in the limit and it could contribute to the development of this complication.
Conclusions Fig. 4 – Line of craniotomy.
to perform a full orbital mobilization, and an hemiorbital advancement is usually enough.21 Any neural or meningeal herniations are cut out and the dura is subsequently closed and repaired.7,10,12 The herniated brain does not contain any significant structures13 so removal
Encephaloceles are a rare type of neural tube defect caused by herniation of brain matter through areas of arrested bone development in the skull. Frontoethmoidal encephaloceles are rare and repair of the defect is mandatory. Approach and timing of surgery have to be carefully planned and cases should be individualized. Decision making has to take into account the age and weight of the patient, the status of the skin and the anatomy of the herniation. MRI and 3D reconstruction of
Please cite this article in press as: Horcajadas A, et al. Frontoethmoidal encephalocele. Report of a case. Neurocirugia. 2018. https://doi.org/10.1016/j.neucir.2018.02.006
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Fig. 5 – Surgical technique. 1: bicoronal incision and craneotomy. 2–3: dissection of the dural sac from the surrounding structures in the nasoorbital area. 4: resection of the encephalocele. 5: dissection of the duramater from brain and suture. 6: pericranial flap for covering duramater and filling dead space. 7: replacement of frontal bone adding a piece of autologous bone to reconstruct the glabella. 8: use of another piece of autologous bone from the skull to reconstruct the nasal bone.
Fig. 6 – Postoperative. In the right side 3D CT reconstruction showing the two pieces of bone used for reconstruction (yellow arrows) and the places of skull where they were taken out (green arrows).
Table 1 – Classification of encephaloceles. Frontoethmoidal
Basal
Encephalocele of the cranial vault
Nasofrontal Nasoethmoidal Naso-orbital Transethmoidal Transsphenoidal Sphenoethmoidal Sphenomaxillary Frontosphenoidal/spheno-orbital Interfrontal Anterior fontanelle Interparietal Posterior fontanelle Temporal Occipital
skull CT are necessary to plan the surgery. A multidisciplinary approach is strongly recommended and surgical plan must be tailored in each patient. Complications, especially infections, are quite common.
references
1. Mahajan C, Rath GP, Dash HH, Bithal PK. Perioperative management of children with encephalocele: an institutional experience. J Neurosurg Anesthesiol. 2011;23:352–6. 2. Tirumandas M, Sharma A, Gbenimacho I, Shoja MM, Tubbs RS, Oakes WJ, et al. Nasal encephaloceles: a review of etiology, pathophysiology, clinical presentations, diagnosis, treatment, and complications. Childs Nerv Syst. 2013;29:739–44. 3. Barnes L. Surgical pathology of the head and neck, vol. 3. New York: Marcel Dekker; 2001.
Please cite this article in press as: Horcajadas A, et al. Frontoethmoidal encephalocele. Report of a case. Neurocirugia. 2018. https://doi.org/10.1016/j.neucir.2018.02.006
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4. Fatterpekar GM, Naidich TP, Som PM. The teaching files: brain and spine imaging. Philadelphia: Elsevier/Saunders; 2012. 5. Wacharasindhu S, Asawutmangkul U, Srivuthana S. Endocrine abnormalities in patients with frontoethmoidal encephalomeningocele. A preliminary study. Horm Res. 2005;64:64–7. 6. Abdel-Aziz M, El-Bosraty H, Qotb M, El-Hamamsy M, El-Sonbaty M, Abdel-Badie H, et al. Nasal encephalocele: endoscopic excision with anesthetic consideration. Int J Pediatr Otorhinolaryngol. 2010;74:869–733. 7. Baradaran N, Nejat F, Baradaran N, El Khashab M. Cephalocele: report of 55 cases over 8 years. Pediatr Neurosurg. 2009;45:461–6. 8. Mahapatra AK, Agrawal D. Anterior encephaloceles: a series of 103 cases over 23 years. J Clin Neurosci. 2006;13:536–9. 9. Kindler P. Morning glory syndrome: unusual congenital optic disc anomaly. Am J Ophthalmol. 1970;69:376–84. 10. Morina A, Kelmendi F, Morina Q, Dragusha S, Ahmeti F, Morina D. Treatment of anterior encephaloceles over 24 years in Kosova. Med Arh. 2011;65:122–4. 11. Oucheng N, Lauwers F, Gollogly J, Draper L, Joly B, Roux FE. Frontoethmoidal meningoencephalocele: appraisal of 200 operated cases. J Neurosurg Pediatr. 2010;6:541–9. 12. Warf BC, Stagno V, Mugamba J. Encephalocele in Uganda: ethnic distinctions in lesion location, endoscopic management of hydrocephalus, and survival in 110 consecutive children. J Neurosurg Pediatr. 2011;7:88–93.
13. Mahapatra AK, Suri A. Anterior encephaloceles: a study of 92 cases. Pediatr Neurosurg. 2002;36:113–8. 14. Wang IJ, Lin SL, Tsou KI, Hsu MC, Chiu WT, Tsai SH, et al. Congenital midline nasal mass: cases series and review of the literature. Turk J Pediatr. 2010;52:520–4. 15. Hoving EW, Vermeij-Keers C. Frontoethmoidal encephaloceles, a study of their pathogenesis. Pediatr Neurosurg. 1997;27:246–56. 16. Mahapatra AK, Tandon PN, Dhawan IK, Khazanchi RK. Anterior encephaloceles: a report of 30 cases. Childs Nerv Syst. 1994;10:501–4. 17. Bhagwati S, Mahapatra A. Encephaloceles and anomalies of the scalp. In: Choux M, Di Rocco C, Hockley A, editors. Pediatric neurosurgery. London: Churchill Livingstone; 1999. p. 101–20. 18. Dhawan IK, Tandon PN. Excision, repair and corrective surgery for fronto-ethmoidal meningocoele. Childs Brain. 1982;9:126–36. 19. David DJ. New perspectives in the management of severe cranio-facial deformity. Ann R Coll Surg Eng. 1984;66:270–9. 20. Hoving EW. Nasal encephaloceles. Childs Nerv Syst. 2000;16:702–6. 21. Arshad AR, Selvapragasam T. Frontoethmoidal encephalocele: treatment and outcome. J Craniofac Surg. 2008;19:175–83. 22. Holmes AD, Meara JG, Kolker AR, Rosenfeld JV, Klug GL. Frontoethmoidal encephaloceles: reconstruction and refinements. J Craniofac Surg. 2001;12:6–18.
Please cite this article in press as: Horcajadas A, et al. Frontoethmoidal encephalocele. Report of a case. Neurocirugia. 2018. https://doi.org/10.1016/j.neucir.2018.02.006
ARTICLE IN PRESS n e u r o c i r u g i a . 2 0 1 8;x x x(x x):xxx–xxx
NEUROCIRUGÍA www.elsevier.es/neurocirugia
Case Report
Frontoethmoidal encephalocele. Report of a case Angel Horcajadas a,∗ , Antonio Palma b , Babar M. Khalon a a b
Neurosurgery, King Saud Medical City, Riyadh, Saudi Arabia Maxillofacial, King Faisal Specialist Hospital, Riyadh, Saudi Arabia
a r t i c l e
i n f o
a b s t r a c t
Article history:
Encephaloceles are uncommon in western countries and most cases are located in the occip-
Received 14 November 2017
ital bone. Frontal encephaloceles may involve the ethmoid bone, nasal bones and/or the
Accepted 18 February 2018
orbits. Surgical repair is complex and usually requires a multidisciplinary approach. The
Available online xxx
goal of the surgery is to reconstruct the normal anatomy, to achieve a good cosmetic repair and to avoid a cerebrospinal fluid leak. We present a case of a patient with a large congeni-
Keywords:
tal frontoethmoidal encephalocele. Autologous calvarian bone grafts were used to repair of
Encephalocele
encephalocele defect and for the reconstruction of the frontonasal area. The defect closure
Congenital malformation
and the cosmetic result were satisfactory, and the only complication detected was the infec-
Frontoethmoidal encephalocele
tion of a previously performed ventriculoperitoneal shunt. A description of the technique
Nasal encephalocele
and a review of the literature are presented. ˜ ˜ S.L.U. All rights © 2018 Sociedad Espanola de Neurocirug´ıa. Published by Elsevier Espana, reserved.
Encefalocele frontoetmoidal. A propósito de un caso r e s u m e n Palabras clave:
Los encefaloceles son infrecuentes en los países occidentales y su localización más fre-
Encefaloceles
cuente es occipital. Los encefaloceles frontales pueden afectar hueso etmoidal, frontal y/o
Malformación congénita
órbitas. La reparación quirúrgica es compleja y habitualmente precisa de un abordaje mul-
Encefalocele frontoetmoidal
tidisciplinar. El objetivo de la cirugía es reconstruir la anatomía del paciente con un buen
Encefalocele nasal
resultado estético, y evitar la fístula de líquido cefalorraquídeo. Se presenta un caso de un gran encefalocele frontoetmoidal. El encefalocele fue reparado y la reconstrucción ósea se realizó con hueso autólogo de la capota craneal. El cierre y el resultado cosmético fueron buenos y la única complicación fue una infección posquirúrgica. Se describe la técnica y se revisa la literatura publicada al respecto. ˜ ˜ S.L.U. Todos © 2018 Sociedad Espanola de Neurocirug´ıa. Publicado por Elsevier Espana, los derechos reservados.
∗
Corresponding author. E-mail address: [email protected] (A. Horcajadas). https://doi.org/10.1016/j.neucir.2018.02.006 ˜ ˜ S.L.U. All rights reserved. 1130-1473/© 2018 Sociedad Espanola de Neurocirug´ıa. Published by Elsevier Espana,
Please cite this article in press as: Horcajadas A, et al. Frontoethmoidal encephalocele. Report of a case. Neurocirugia. 2018. https://doi.org/10.1016/j.neucir.2018.02.006
NEUCIR-324; No. of Pages 6
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ARTICLE IN PRESS n e u r o c i r u g i a . 2 0 1 8;x x x(x x):xxx–xxx
Introduction Encephaloceles represent a group of disorders in which a skull defect allows for extracranial herniation of leptomeninges, brain, and CSF.1 They can be classified as primary if they are present at birth or secondary if they are acquired following trauma or surgery.2 The occipital bone is the most common location3 and frontoethmoidal encephaloceles are less frequent. A case of fronto-ethmoidal encephalocele is presented as well as the surgical technique performed and a review of the literature up to the present date.
Case report The case is presented of an Arabic female premature newborn (gestational age of 30 weeks) with a large frontal mass. The mass was bulging between the two orbits and occupying the lowest part of the frontal area and the superior part of the nasal area. Skin was intact (Fig. 1), though thin and presented some degree of exudation. Neurologically there were no motor deficits and the baby moved spontaneously the four limbs. She was active and taking orally without any problem. MRI showed a large encephalocele through a frontoethmoidal bone defect, consisting of most of the right cerebral hemisphere herniated through the defect. CT scan showed a bone defect in the zone of frontal bone, lamina cribrosa and anterior part of ethmoidal bone (Fig. 2). The weight of the baby at birth was 1.500 g and there was no CSF leak so a elective surgery was decided. A ventriculoperitoneal shunt was needed after 1 month due to the development of hydrocephalus. Surgery was performed when the patient was 7 months old and reached 3000 g.
Surgical technique A 3D model of the skull from a CT scan was made to identify clearly the bone defect and to plan the surgery (Fig. 3). The CT scan was exported as a DICOM file and processed with virtual planning software (ProPlan CMF).The morphology of the defect was established and measured, and the relation with the soft tissue coverage was identified using a virtual superposition of the soft tissues. A 3D stereolithographic model of the skull was printed and sterilized, and taken to the operative room in order to help as an anatomical reference during the surgery. The surgery was performed by a multidisciplinary team. Steps of the surgery can be seen in Fig. 5. A coronal skin incision was performed exposing the frontal bone, upper orbital edges and the frontal part of the encephalocele. A bifrontal craniotomy was done from the edge of the bone defect to expose the floor of the anterior cranial fossa surrounding the sac (Fig. 4). After that, the Maxillofacial surgeon made lateral rhinotomy incision over the encephalocele and a circumferential dissection of the encephalocele’s sac was performed, freeing it from the surrounding tissue, including the nasal bones and the
left orbital rim, preserving the lacrimal sac and the medial cantal ligament attachment. Once the sac was completely dissected and isolated from the surrounding tissues, and the intracranial and facial edges of the sac were connected, removal of the functionless brain tissue and suture of the dural sac in a watertight fashion were performed. ® Tachosil and a pericranial flap were used to reinforce the closure and fill the dead space. Two full thickness cortical block bone grafts were harvested from the posterior border of the craniotomy, in the parietal bone, according to the size and shape recommend by the virtual planning software in order to reconstruct the glabella and the nasal bone. The grafts were wired with absorbable sutures (PDS 3–0) to the remaining nasal bones. Reposition of the craniotomized frontal bone was performed and stabilized again with reservable sutures. Skin flap over the nasal area was remodeled to achieve a cosmetic closure. The patient was transferred to the Pediatric ICU under mechanical ventilation, and delayed extubation was performed without complication after 24 h.
Result No intraoperative complications were suffered, surgical site was intact, and the evolution of the patient was satisfactory from the cosmetic and functional point of view. There was no CSF leak. The patient developed a shunt infection that required external ventricular drainage and antibiotic treatment. Fig. 6 shows the cosmetic result and postoperative CT scan where the reconstruction of frontal and nasal bone can be seen.
Discussion Encephaloceles are one of the so called neural tube defects. There is an herniation of the meninges and brain matter through a structural defect in the skull.1 Encephaloceles are classified based on the location and type of skull defect in three main types: frontoethmoidal, occipital, cranial vault and basal type (Table 1). The most frequent location is the occipital cranial vault (80% of the cases).3 Frontoethmoidal encephaloceles, collectively known as nasal encephaloceles, have three subtypes according to the bone associated with the defect: nasofrontal, nasoethmoidal, and naso-orbital.4,5 The nasoethmoidal subtype is most frequently reported in the literature2,6–8 and the naso-orbital subtype is the least common.2,9 Estimated incidence in North America and Western countries is 1–3 per 10,000 live births and the most common location is occipital. Most cases of nasal encephaloceles have been found in Southeast Asian countries with very rare cases reported in Europe, North America, and the Middle Eas.4,10–12 Etiology of encephalocele is not well known. Many have defined it as a multifactorial condition, with a combination of genetic and environmental factors.2 Most of the cases are present at birth as masses over the nose, glabella and/or forehead, usually covered by skin.3 Sometimes encephaloceles are hidden and may present with symptoms as snoring, nasal obstruction, CSF leak or recurrent
Please cite this article in press as: Horcajadas A, et al. Frontoethmoidal encephalocele. Report of a case. Neurocirugia. 2018. https://doi.org/10.1016/j.neucir.2018.02.006
NEUCIR-324; No. of Pages 6
ARTICLE IN PRESS n e u r o c i r u g i a . 2 0 1 8;x x x(x x):xxx–xxx
3
Fig. 1 – Preoperative images of the frontoethmoidal encephalocele.
Fig. 2 – MRI and 3D CT scan, most of the right hemisphere is herniated through the bone defect. The bone defect involves frontal bone, cribrosa laminae and ethmoidal bone. Orbits are intact.
meningitis.4,6 This is the main reason why the age at diagnosis has been reported to range from 0 to 40 years old.1,10,13,14 The presence of hydrocephalus is a main factor in the management of encephaloceles.8,13 Untreated or late treated cases present a high risk of postoperative CSF leak.13,15,16 Associated hydrocephalus should be treated and solved before surgical treatment. MRI and CT scan are necessary to plan the surgery. MRI is essential to understand the anatomy of the herniated brain and its relations with the surrounding structures. AngioMRI is also useful to evaluate the position of the anterior cerebral arteries, because sometimes they are herniated in the sac. CT scan with 3D reconstruction helps to define accurately the bone defect and to plan the surgery. In the case presented the 3D model made for the planning helped to understand the anatomy of the cranial defect and to plan the surgical reconstruction. Angiography is only recommended when presence of significant vessels is suspected inside the encephalocele. Surgery is the only treatment of frontoethmoidal encephaloceles. Skin is usually intact so elective surgery is indicated. Planning of surgery and timing of operation is crucial in management of anterior encephaloceles.13,17–20 Surgery is usually long and blood loss and hypothermia are frequent, which remain the two most important intraoperative complications. If there is no active CSK leak or
infection, delayed surgery is preferred and some authors recommend surgery at 8–10 months of age.8,13,16 On the other hand, frontoethmoidal encephaloceles should be treated at an early age to avoid further facial distortion during growth.20 Due to the nature of the illness, a multidisciplinary approach is strongly recommended.2,6,21 Endoscopic transnasal approaches6,7 have been successful in many cases of nasal encephaloceles but most authors recommend the “combined procedure” which combines a bicoronal and a nasofrontal flap approach with facial reconstruction.2,11 This approach provides three main advantages to surgeons and patients: a more successful closure of the meningoencephalocele, with lower risk of CSF leak, the possibility of telecanthus correction if necessary, and in some cases the reduction of facial scars.11 In many studies, the surgical procedure includes frontal coronal scalp flap incision to gain exposure to the craniofacial bones combined with the transfacial incision. Some authors prefer to limit the repair of the encephalocele through the transfacial approach, without the need of a coronal approach and bifrontal craniotomy, making the procedure less agressive.20 This is possible if the sac and the bone defect are of a moderate size. If the orbits are involved in the deformity causing hypertelorism, a correction technique is necessary. Usually there is no need
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Fig. 3 – 3D model built from 3D CT scan.
of this functionless tissue is possible. Postoperative followup is essential because of the risk of CSF rhinorrhea and infection.22 CSF leaks are the most common postoperative complication. There is an overall low mortality rate associated with encephaloceles, with a 3% surgery-related mortality.12 Some patients may need secondary cosmetic surgery, like rhinoplasty or eyelid repair, at a later stage, depending on the involvement of soft tissues. The case presented is a big frontoethmoidal encephalocele. Surgical planning using a 3D model was extremely useful to prepare the surgery and to achieve a good reconstruction. A multidisciplinary approach was planned to approach the case. The cosmetic result and the closure were very good and no CSF leak was found after the surgery. The shunt infection presented after surgery was undoubtedly related to it and required shunt removal and external ventricular drainage. The age and the weight at surgery were maybe in the limit and it could contribute to the development of this complication.
Conclusions Fig. 4 – Line of craniotomy.
to perform a full orbital mobilization, and an hemiorbital advancement is usually enough.21 Any neural or meningeal herniations are cut out and the dura is subsequently closed and repaired.7,10,12 The herniated brain does not contain any significant structures13 so removal
Encephaloceles are a rare type of neural tube defect caused by herniation of brain matter through areas of arrested bone development in the skull. Frontoethmoidal encephaloceles are rare and repair of the defect is mandatory. Approach and timing of surgery have to be carefully planned and cases should be individualized. Decision making has to take into account the age and weight of the patient, the status of the skin and the anatomy of the herniation. MRI and 3D reconstruction of
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Fig. 5 – Surgical technique. 1: bicoronal incision and craneotomy. 2–3: dissection of the dural sac from the surrounding structures in the nasoorbital area. 4: resection of the encephalocele. 5: dissection of the duramater from brain and suture. 6: pericranial flap for covering duramater and filling dead space. 7: replacement of frontal bone adding a piece of autologous bone to reconstruct the glabella. 8: use of another piece of autologous bone from the skull to reconstruct the nasal bone.
Fig. 6 – Postoperative. In the right side 3D CT reconstruction showing the two pieces of bone used for reconstruction (yellow arrows) and the places of skull where they were taken out (green arrows).
Table 1 – Classification of encephaloceles. Frontoethmoidal
Basal
Encephalocele of the cranial vault
Nasofrontal Nasoethmoidal Naso-orbital Transethmoidal Transsphenoidal Sphenoethmoidal Sphenomaxillary Frontosphenoidal/spheno-orbital Interfrontal Anterior fontanelle Interparietal Posterior fontanelle Temporal Occipital
skull CT are necessary to plan the surgery. A multidisciplinary approach is strongly recommended and surgical plan must be tailored in each patient. Complications, especially infections, are quite common.
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Please cite this article in press as: Horcajadas A, et al. Frontoethmoidal encephalocele. Report of a case. Neurocirugia. 2018. https://doi.org/10.1016/j.neucir.2018.02.006