- Email: [email protected]
Current management of congenital anterior cranial base encephaloceles
International Journal of Pediatric Otorhinolaryngology 131 (2020) 109868
Contents lists available at ScienceDirect
International Journal of Pediatric Otorhinolaryngology journal homepage: www.elsevier.com/locate/ijporl
Current management of congenital anterior cranial base encephaloceles a
b
a
T
a
Harrison M. Thompson , Rodney J. Schlosser , Erika McCarty Walsh , Do-Yeon Cho , Jessica W. Graysona, Thomas T. Karnezisb, Peter L. Millera, Bradford A. Woodwortha,∗ a b
Departments of Otolaryngology – Head and Neck Surgery, University of Alabama at Birmingham, Birmingham, AL, USA Medical University of South Carolina, Charleston, SC, USA
A R T I C LE I N FO
A B S T R A C T
Keywords: Encephalocele Congenital encephalocele Basal encephalocele Cerebrospinal fluid leak Anterior cranial base Endoscopic repair Endoscopic endonasal approach Skull base reconstruction Nasoseptal flap
Objectives: Congenital encephaloceles provide unique diagnostic and reconstructive challenges for the pediatric rhinologist. The objectives of the current study were to evaluate contemporary treatment strategies for congenital encephaloceles focusing on presentation, surgical technique, and outcomes. Methods: Multi-institutional retrospective chart review of congenital encephaloceles (2003–2019). Data regarding demographics, presenting symptoms, associated abnormalities, surgical technique, size, location, and complications were collected. Results: Fourteen patients with 15 congenital encephaloceles were treated using endoscopic techniques (avg 6.0 years, range 2 months–22 years) with mean follow up of 23 months. The majority presented with nasal obstruction (n = 13); only one child had cerebrospinal fluid (CSF) rhinorrhea. Associated anomalies included nasal deformities, congenital hypopituitarism, and Morning Glory syndrome. Average encephalocele size was 2.44 cm (range 0.5–3.6 cm) with mean skull base defect size of 8.6 x 7.7 mm. Locations included the foramen cecum (n = 9), central sphenoid (n = 3), midline anterior cranial fossa (n = 1), orbital plate of frontal bone (n = 1), and ethmoid roof (n = 1). Because of favorable expansion from encephaloceles, it was unnecessary to postpone surgeries to allow nasal cavity growth. Three individuals had prior operations, including surgeries for “nasal polyp” or “adenoid cyst”. Two patients had post-operative complications (meningitis and CSF leak) effectively treated with no further sequelae. Conclusions: In the current study, congenital encephaloceles in children as young as 2 months were successfully repaired using endoscopic techniques. Endoscopic approaches remain a safe and effective intervention for management of these lesions.
1. Introduction Congenital encephaloceles of the anterior skull base are rare lesions with an incidence reported between 1 in 5000 to 1 in 40,000 live births [1–3]. Suwanwela further divided these lesions into the external sincipital (frontoethmoidal) and entirely intranasal basal encephaloceles [4]. Given that basal encephaloceles have less apparent external manifestations, their presentation can be more insidious, varying from nasal airway obstruction to frank meningitis at a wide range of ages [5–10]. In addition to their subtle presentation, basal encephaloceles have been associated with other conditions, including hypertelorism, facial clefts, and Morning Glory syndrome (congenital optic disc dysplasia) [3,11,12]. Traditionally, anterior skull base encephaloceles were managed with open craniofacial approaches, frequently with combined craniotomy and intranasal techniques [13–15]. However, there is a high
∗
rate of complications (> 20%) associated with open approaches to the anterior skull base, including cerebrospinal fluid leak, meningitis, intracranial hemorrhage, and pneumocephalus [16]. In the adult population, endoscopic endonasal approaches to the anterior skull base have become the standard of care for a wide variety of pathologies [17–20]. Multiple authors have reported successful closure rates (> 90%) of anterior skull base defects using endoscopic techniques with few complications [21–24]. While the paradigm continues to shift towards endoscopic only approaches for pediatric lesions of the anterior skull base, a major concern is the compact anatomy that may preclude endoscopic repair, especially in very young children. However, several studies suggest that endoscopic resection and repair is feasible and has a more favorable complication profile than open approaches, even in children as young as 8 months old [25–31]. Despite data indicating safety and feasibility, many contemporary authors continue to utilize primarily open or combined approaches in pediatric skull base surgery
Corresponding author. UAB – Department of Otolaryngology, FOT 1155|1720 2nd Avenue S Birmingham, AL, 35294-3412, USA. E-mail address: [email protected] (B.A. Woodworth).
https://doi.org/10.1016/j.ijporl.2020.109868 Received 28 October 2019; Received in revised form 6 January 2020; Accepted 6 January 2020 Available online 08 January 2020 0165-5876/ © 2020 Published by Elsevier B.V.
International Journal of Pediatric Otorhinolaryngology 131 (2020) 109868
H.M. Thompson, et al.
4. Discussion
[8,14,15,32]. The aim of the present study is to review the management of these rare lesions based on our collective experience and pooled data from a multi-institutional patient series.
Congenital encephaloceles of the anterior skull base may cause significant morbidity and mortality. However, the risks and benefits of surgical repair must be weighed individually and should include a thorough assessment of presenting symptoms, patient age, and nasal cavity size, as well as comfort level of the surgeon with the endoscopic approach. Because of the rarity of these lesions, it is difficult to determine the optimal time of repair based on data gleaned from case series in the literature. Repair of the defect is critical if CSF leak or meningitis (the most dreaded complication of encephalocele) is the presenting scenario, but the decision to intervene in the existence of more benign symptoms such as nasal obstruction or deformity is not equally illuminating. Historically, basal encephaloceles were thought to confer less risk of meningitis than external lesions, prompting some providers to delay surgical repair until children developed larger nasal cavities [9,33,34]. In the current study, nasal obstruction was by far the most common presenting symptom (identified as excessive nasal secretions and snorting in infants unable to convey symptoms) and was usually discovered at a very young age. Given the two major complications following surgery were meningitis and CSF leak (patients 3 and 4, respectively), it is unclear whether the benefit outweighed the risk in these two patients. Surgical intervention was chosen primarily due to nasal obstruction. Patient 3 had post-operative meningitis after removal of the anterior encephalocele, which was considered necessary to allow cosmetic surgery for her midline nasal cleft before enrolling in school. All three patients with central sphenoid encephaloceles (patients 1, 3, and 4) involved the third ventricle, which may have contributed to the post-operative CSF leak seen in patient 4 particularly in conjunction with her severe post-operative emesis. Despite the complications, patients were managed successfully with appropriate interventions (revision endoscopic repair and intravenous antibiotics) and had no sequelae from their complications. It should also be noted our complication rate of 14% compares favorably to rates of over 20% reported in some series of open procedures [16]. While craniofacial anomalies (e.g. telecanthus) are very common in frontoethmoidal encephaloceles, the rate of abnormalities in patients with basal encephaloceles is difficult to determine from existing case series [35]. It should be noted patient 3 had 2 encephaloceles that did not physically contribute to her midline nasal cleft, but rather were associated with an overall failure of development in utero. On the other hand, several patients presented with telecanthus and nasal obstruction when originating at the foramen cecum. Larger encephaloceles in this region, particularly in younger children, pushed the boundaries of a small nasal cavity and the mass effect led to splaying of the nasal bones. In addition, Patient 8 had orbital displacement due to the encephalocele exiting at the orbital plate of the frontal bone. It is unclear what effect surgical removal will have on their telecanthus/deformity over time, but was considered to (at least) terminate the physical pressure leading to the phenomenon. In addition, facial growth has been demonstrated to realign after surgery in infancy, thus immediate removal of large encephaloceles causing craniofacial anomalies should be considered [36]. The present study suggests that children as young as 2 months of age can be surgically managed using endoscopic techniques. It should be noted that, in the younger patient population, these were larger encephaloceles that caused significant mass effect with severe septal deviations and lateralization of the turbinates. The ensuing exposure was considered excellent despite small nostrils and improved the working space for endoscopic removal and repair. However, a transphenoidal encephalocele was repaired in a 3 year old (Patient 1) although no mass effect was present besides replacement of the posterior septum. Therefore, the decision for surgery based on the age of the patient should depend on careful evaluation of the nasal cavity dimensions and surgeon experience. Our study highlights the importance of remaining vigilant for the
2. Methods Institutional Review Board approval was obtained at both the University of Alabama at Birmingham and the Medical University of South Carolina prior to the initiation of this study. A retrospective review of children and young adults with congenital encephaloceles of the anterior skull base presenting from January 1, 2003 to January 4, 2019 was performed. Criteria for inclusion were obvious congenital etiology (e.g. patent neuropore at foramen cecum, bifid sphenoid or crista galli) without previous head trauma or spontaneous etiology (intracranial hypertension). The preoperative examination included a thorough medical history, physical examination, and radiographic imaging. We performed standard computed tomographic and magnetic resonance imaging on all patients. Intraoperative computer-assisted surgical navigation was utilized in every operation. Surgical technique was similar between surgeons (RJS and BAW). Using 0°, 30°, and 70° adult and pediatric endoscopes, encephaloceles were meticulously reduced with bipolar cautery or radiofrequency coblation. Cautery was avoided along the inside of the nasal vestibule to avoid the risk of adverse outcomes such as nasal stenosis. Sinuses were opened as necessary for exposure and placement of instruments. Removal of mucosa was performed at the site of the bony defect. Wide removal of mucosa along the septum or beneath the nasal bones where meningoencephaloceles had expanded in the submucosal plane was not necessary and could contribute to nasal stenosis and prolonged healing. Multilayer repair was then performed with grafts (allografts/xenografts) +/− pedicled nasoseptal flaps. Repairs were supported with Gelfoam (Pfizer Inc, New York, NY) and Merocel sponge in a non-latex gloved finger. Merocels were left in place for 13–14 days and were well tolerated by all patients. Patients that could not have merocels removed in-office were taken back to the operating room at 14 days for repeat endoscopy and removal. Lumbar drains were not routinely used. Post-operative follow-up was performed on all patients with in-office endoscopy. Routine follow-up imaging was not performed if the patient tolerated endoscopy and the skull base repair was visualized. 3. Results Fourteen patients (average age 6.0 years, range 2 months–22 years) with 15 encephaloceles were identified and included in the study (8 females/6 males). The most common presenting symptom was nasal obstruction (Table 1). Three patients had previously undergone surgery at other institutions for mistaken nasal polyps (n = 2) and an adenoid cyst (n = 1). Associated anomalies included craniofacial deformities (n = 5), Morning Glory Syndrome (n = 1), and congenital hypopituitarism (n = 1). All patients were managed via a purely endoscopic endonasal approach, except for one patient who required a combined approach using a brow craniotomy. The craniotomy was performed in this instance because the encephalocele exited through the orbital plate of the frontal bone into the superior orbit. The majority of lesions originated from the foramen cecum with a mean encephalocele size of 2.44 cm in diameter measured from skull base defect to the distal tip (range 0.5–3.6 cm). Mean skull base defect size in length (rostral to caudal) x width (transverse) was 8.6 x 7.7 mm. One patient developed a CSF leak, which was felt to be related to postoperative severe emesis. This patient was taken back to the OR for endoscopic endonasal revision of the nasoseptal flap and additional supportive packing without complications on follow-up. Postoperative meningitis developed in one individual, but resolved with 3 weeks of intravenous antibiotic treatment. No patients were found to have recurrence of encephalocele. 2
International Journal of Pediatric Otorhinolaryngology 131 (2020) 109868
H.M. Thompson, et al.
Table 1 Summary patient data. Pt
Age
Sex
Size(cm)
Defect (lxw)
Repair
Presentation
Location
1 2 3
3y 3y 5y
F F F F F M F F M F M M M M
SIS, SIS, SIS SIS SIS, SIS, SIS, SIS, SIS, SIS, CM CM CM TF MG
NO, Morning Glory Syndrome NO NO, Midline Nasal Cleft
22 y 6m 12 y 15 y 2m 7m 3y 8m 6m 16 y 2y
8x8 4x4 15x15 15x15 12x12 4x4 10x8 4x4 5x5 6x6 8x8 10x10 6x8 5x5 8x8
NSF NSF
4 5 6 7 8 9 10 11 12 13 14
3.2 2.5 3 3.5 1.5 2.6 3.6 1 2 2 2.6 2.3 2.8 0.5 3.5
NSF NSF NSF NSF NSF NSF
NO, Congenital Hypopituitarism NO, Telecanthus NO Incidental NO, Telecanthus NO NO, Nasal Swelling NO, Orbital Displacement NO, Telecanthus CSF Leak, Meningitis NO, Telecanthus
Central Sphenoid Foramen Cecum Midline ACF Central Sphenoid Central Sphenoid Foramen Cecum Foramen Cecum Foramen Cecum Foramen Cecum Foramen Cecum Foramen Cecum Orbital Plate of Frontal Bone Foramen Cecum Ethmoid Roof Foramen Cecum
Key: y– Years, m – Months, F - Female, M − Male, NO - Nasal Obstruction, ACF - Anterior Cranial Fossa, lxw (Length (rostral to caudal) vs. Width (transverse)), SIS – Porcine Submucosal Small Intestine (Biodesign®, Cook Medical, Bloomington, In), CM – Collagen Matrix, NSF- Nasoseptal Flap, MG – Mucosal Graft, TF - Temporalis Fascia.
Disclosures Bradford A. Woodworth, MD is a consultant for Cook Medical, Baxter, and Smith and Nephew. References [1] G.B. Hughes, G. Sharpino, W. Hunt, H.M. Tucker, Management of the congenital midline nasal mass: a review, Head Neck Surg. 2 (3) (1980) 222–233. [2] R. Blumenfeld, E.M. Skolnik, Intranasal encephaloceles, Arch Otolaryngol Chic Ill 1960 82 (5) (1965) 527–531. [3] M. Tirumandas, A. Sharma, I. Gbenimacho, et al., Nasal encephaloceles: a review of etiology, pathophysiology, clinical presentations, diagnosis, treatment, and complications, Childs Nerv Syst ChNS Off J Int Soc Pediatr Neurosurg 29 (5) (2013) 739–744, https://doi.org/10.1007/s00381-012-1998-z. [4] C. Suwanwela, N. Suwanwela, A morphological classification of sincipital encephalomeningoceles, J. Neurosurg. 36 (2) (1972) 201–211, https://doi.org/10. 3171/jns.1972.36.2.0201. [5] P. Castelnuovo, M. Bignami, A. Pistochini, P. Battaglia, D. Locatelli, I. Dallan, Endoscopic endonasal management of encephaloceles in children: an eight-year experience, Int. J. Pediatr. Otorhinolaryngol. 73 (8) (2009) 1132–1136, https://doi. org/10.1016/j.ijporl.2009.04.023. [6] C.M. Myer, R.T. Cotton, Nasal obstruction in the pediatric patient, Pediatrics 72 (6) (1983) 766–777. [7] G. Kalkan, S. Paksu, N. Asilioglu, M. Kiliç, Nasopharyngeal encephalocele: a rare cause of upper airway obstruction, Hong Kong Med J Xianggang Yi Xue Za Zhi Hong Kong Acad Med 19 (2) (2013) 186–187. [8] S. Sachdeva, R. Kapoor, P. Paul, R. Yadav, Recurrent meningitis with upper airway obstruction in a child: frontonasal encephalocele- a case report, J Clin Diagn Res JCDR 8 (8) (2014), https://doi.org/10.7860/JCDR/2014/9262.4749 PD01-02. [9] B.A. Woodworth, R.J. Schlosser, R.A. Faust, W.E. Bolger, Evolutions in the management of congenital intranasal skull base defects, Arch. Otolaryngol. Head Neck Surg. 130 (11) (2004) 1283–1288, https://doi.org/10.1001/archotol.130.11.1283. [10] R. Macfarlane, J.T. Rutka, D. Armstrong, et al., Encephaloceles of the anterior cranial fossa, Pediatr. Neurosurg. 23 (3) (1995) 148–158. [11] C.S. Chen, D. David, A. Hanieh, Morning glory syndrome and basal encephalocele, Childs Nerv Syst ChNS Off J Int Soc Pediatr Neurosurg 20 (2) (2004) 87–90, https:// doi.org/10.1007/s00381-003-0869-z. [12] J. Caprioli, R.L. Lesser, Basal encephalocele and morning glory syndrome, Br. J. Ophthalmol. 67 (6) (1983) 349–351. [13] S.P. Hao, H.S. Wang, T.N. Lui, Transnasal endoscopic management of basal encephalocele–craniotomy is no longer mandatory, Am. J. Otolaryngol. 16 (3) (1995) 196–199. [14] R. Gun, F. Tosun, A. Durmaz, et al., Predictors of surgical approaches for the repair of anterior cranial base encephaloceles, Eur Arch Oto-Rhino-Laryngol Off J Eur Fed Oto-Rhino-Laryngol Soc EUFOS Affil Ger Soc Oto-Rhino-Laryngol - Head Neck Surg 270 (4) (2013) 1299–1305, https://doi.org/10.1007/s00405-012-2174-5. [15] R. Rahbar, V.A. Resto, C.D. Robson, et al., Nasal glioma and encephalocele: diagnosis and management, The Laryngoscope 113 (12) (2003) 2069–2077, https://doi. org/10.1097/00005537-200312000-00003. [16] J.T. Kryzanski, D.J. Annino, H. Gopal, C.B. Heilman, Low complication rates of cranial and craniofacial approaches to midline anterior skull base lesions, Skull Base Off J North Am Skull Base Soc Al 18 (4) (2008) 229–241, https://doi.org/10.1055/ s-2007-1003924. [17] J.W. Grayson, M.R. Chaaban, K.O. Riley, B.A. Woodworth, Smell sparing unilateral intracranial dermoid resection, Allergy Rhinol Provid RI 5 (1) (2014) 39–40,
Fig. 1. Radiographic and Endoscopic Imaging A T2-weighted coronal MRI scan demonstrating a congenital encephalocele emanating from a patent foramen cecum (A). Transnasal endoscopic view of the encephalocele positioned just posterior to the nasal vault (B). The encephalocele is ablated using a Coblator (C and D). An underlay free graft (E) and overlay septal flap is used for repair (F).
identification of encephaloceles in pediatric patients. Even though basal encephaloceles have few external manifestations, they frequently lead to symptoms. In our series, 12 of 14 patients presented with nasal obstruction. Despite the presence of congenital hypopituitarism in patient 4, no CT or MRI scan was performed at the outside institution before proceeding with an adenoidectomy for an “adenoid cyst”. Patient 2 presented with nasal obstruction at age 3 and was treated with surgery for presumptive nasal polyposis (even though a singular unilateral occurrence) before the correct diagnosis was made. Therefore, it is critical for otolaryngologists to have a high degree of suspicion in pediatric patients presenting with nasal or nasopharyngeal mass at a young age, especially in the setting of telecanthus or congenital disorders of the pituitary(see Fig. 1). 5. Conclusion Endoscopic endonasal repair of congenital anterior encephaloceles is safe and effective, even in children less than one year of age and will continue to emerge as a preferred option for therapeutic intervention for the vast majority of these lesions. 3
International Journal of Pediatric Otorhinolaryngology 131 (2020) 109868
H.M. Thompson, et al.
[27] T. Van Den Abbeele, M. Elmaleh, P. Herman, M. François, P. Narcy, Transnasal endoscopic repair of congenital defects of the skull base in children, Arch. Otolaryngol. Head Neck Surg. 125 (5) (1999) 580–584. [28] D. Locatelli, F. Rampa, I. Acchiardi, M. Bignami, A. Pistochini, P. Castelnuovo, Endoscopic endonasal approaches to anterior skull base defects in pediatric patients, Childs Nerv Syst ChNS Off J Int Soc Pediatr Neurosurg 22 (11) (2006) 1411–1418, https://doi.org/10.1007/s00381-006-0114-7. [29] A. Kassam, A.J. Thomas, C. Snyderman, et al., Fully endoscopic expanded endonasal approach treating skull base lesions in pediatric patients, J. Neurosurg. 106 (2 Suppl) (2007) 75–86, https://doi.org/10.3171/ped.2007.106.2.75. [30] M. Sasani, A.F. Ozer, A.L. Aydin, Endoscopic treatment of trans-sellar trans-sphenoidal encephalocele associated with morning glory syndrome presenting with nontraumatic cerebrospinal fluid rhinorrhea, J. Neurosurg. Sci. 53 (1) (2009) 31–35. [31] F. Di Rocco, V. Couloigner, P. Dastoli, C. Sainte-Rose, M. Zerah, G. Roger, Treatment of anterior skull base defects by a transnasal endoscopic approach in children, J. Neurosurg. Pediatr. 6 (5) (2010) 459–463, https://doi.org/10.3171/ 2010.8.PEDS09325. [32] H.K. Dutta, P. Deori, Anterior encephaloceles in children of Assamese tea workers, J. Neurosurg. Pediatr. 5 (1) (2010) 80–84, https://doi.org/10.3171/2009.8. PEDS0912. [33] E.W. Hoving, C. Vermeij-Keers, Frontoethmoidal encephaloceles, a study of their pathogenesis, Pediatr. Neurosurg. 27 (5) (1997) 246–256. [34] R.L. Rapport, R.C. Dunn, F. Alhady, Anterior encephalocele, J. Neurosurg. 54 (2) (1981) 213–219, https://doi.org/10.3171/jns.1981.54.2.0213. [35] D.J. David, New perspectives in the management of severe cranio-facial deformity, Ann. R. Coll. Surg. Engl. 66 (4) (1984) 270–279. [36] N.U. Rahman, Nasal encephalocoele. Treatment by trans-cranial operation, J. Neurol. Sci. 42 (1) (1979) 73–85.
https://doi.org/10.2500/ar.2014.5.0074. [18] N.U. Hatch, K.O. Riley, B.A. Woodworth, Endoscopic removal of a bullet penetrating the middle cranial fossa, Skull Base Rep. 1 (1) (2011) 47–50, https://doi. org/10.1055/s-0031-1275633. [19] V. Jones, F. Virgin, K. Riley, B.A. Woodworth, Changing paradigms in frontal sinus cerebrospinal fluid leak repair, Int Forum Allergy Rhinol 2 (3) (2012) 227–232, https://doi.org/10.1002/alr.21019. [20] B.A. Woodworth, R.J. Harvey, J.G. Neal, J.N. Palmer, R.J. Schlosser, Endoscopic management of frontal sinus mucoeceles with anterior table erosion, Rhinology 46 (3) (2008) 231–237. [21] A.H. Marshall, N.S. Jones, I.J. Robertson, Endoscopic management of basal encephaloceles, J. Laryngol. Otol. 115 (7) (2001) 545–547. [22] C.A. Banks, J.N. Palmer, A.G. Chiu, B.W. O'Malley, B.A. Woodworth, D.W. Kennedy, Endoscopic closure of CSF rhinorrhea: 193 cases over 21 years, Otolaryngol–Head Neck Surg Off J Am Acad Otolaryngol-Head Neck Surg 140 (6) (2009) 826–833, https://doi.org/10.1016/j.otohns.2008.12.060. [23] M.T. Purkey, B.A. Woodworth, S. Hahn, J.N. Palmer, A.G. Chiu, Endoscopic repair of supraorbital ethmoid cerebrospinal fluid leaks, ORL J Oto-Rhino-Laryngol Its Relat Spec. 71 (2) (2009) 93–98, https://doi.org/10.1159/000193219. [24] G.M. Oakley, R.R. Orlandi, B.A. Woodworth, P.S. Batra, J.A. Alt, Management of cerebrospinal fluid rhinorrhea: an evidence-based review with recommendations, Int Forum Allergy Rhinol. September (2015), https://doi.org/10.1002/alr.21627. [25] M. Abdel-Aziz, H. El-Bosraty, M. Qotb, et al., Nasal encephalocele: endoscopic excision with anesthetic consideration, Int. J. Pediatr. Otorhinolaryngol. 74 (8) (2010) 869–873, https://doi.org/10.1016/j.ijporl.2010.04.015. [26] S.J. Kanowitz, J.M. Bernstein, Pediatric meningoencephaloceles and nasal obstruction: a case for endoscopic repair, Int. J. Pediatr. Otorhinolaryngol. 70 (12) (2006) 2087–2092, https://doi.org/10.1016/j.ijporl.2006.08.007.
4
Contents lists available at ScienceDirect
International Journal of Pediatric Otorhinolaryngology journal homepage: www.elsevier.com/locate/ijporl
Current management of congenital anterior cranial base encephaloceles a
b
a
T
a
Harrison M. Thompson , Rodney J. Schlosser , Erika McCarty Walsh , Do-Yeon Cho , Jessica W. Graysona, Thomas T. Karnezisb, Peter L. Millera, Bradford A. Woodwortha,∗ a b
Departments of Otolaryngology – Head and Neck Surgery, University of Alabama at Birmingham, Birmingham, AL, USA Medical University of South Carolina, Charleston, SC, USA
A R T I C LE I N FO
A B S T R A C T
Keywords: Encephalocele Congenital encephalocele Basal encephalocele Cerebrospinal fluid leak Anterior cranial base Endoscopic repair Endoscopic endonasal approach Skull base reconstruction Nasoseptal flap
Objectives: Congenital encephaloceles provide unique diagnostic and reconstructive challenges for the pediatric rhinologist. The objectives of the current study were to evaluate contemporary treatment strategies for congenital encephaloceles focusing on presentation, surgical technique, and outcomes. Methods: Multi-institutional retrospective chart review of congenital encephaloceles (2003–2019). Data regarding demographics, presenting symptoms, associated abnormalities, surgical technique, size, location, and complications were collected. Results: Fourteen patients with 15 congenital encephaloceles were treated using endoscopic techniques (avg 6.0 years, range 2 months–22 years) with mean follow up of 23 months. The majority presented with nasal obstruction (n = 13); only one child had cerebrospinal fluid (CSF) rhinorrhea. Associated anomalies included nasal deformities, congenital hypopituitarism, and Morning Glory syndrome. Average encephalocele size was 2.44 cm (range 0.5–3.6 cm) with mean skull base defect size of 8.6 x 7.7 mm. Locations included the foramen cecum (n = 9), central sphenoid (n = 3), midline anterior cranial fossa (n = 1), orbital plate of frontal bone (n = 1), and ethmoid roof (n = 1). Because of favorable expansion from encephaloceles, it was unnecessary to postpone surgeries to allow nasal cavity growth. Three individuals had prior operations, including surgeries for “nasal polyp” or “adenoid cyst”. Two patients had post-operative complications (meningitis and CSF leak) effectively treated with no further sequelae. Conclusions: In the current study, congenital encephaloceles in children as young as 2 months were successfully repaired using endoscopic techniques. Endoscopic approaches remain a safe and effective intervention for management of these lesions.
1. Introduction Congenital encephaloceles of the anterior skull base are rare lesions with an incidence reported between 1 in 5000 to 1 in 40,000 live births [1–3]. Suwanwela further divided these lesions into the external sincipital (frontoethmoidal) and entirely intranasal basal encephaloceles [4]. Given that basal encephaloceles have less apparent external manifestations, their presentation can be more insidious, varying from nasal airway obstruction to frank meningitis at a wide range of ages [5–10]. In addition to their subtle presentation, basal encephaloceles have been associated with other conditions, including hypertelorism, facial clefts, and Morning Glory syndrome (congenital optic disc dysplasia) [3,11,12]. Traditionally, anterior skull base encephaloceles were managed with open craniofacial approaches, frequently with combined craniotomy and intranasal techniques [13–15]. However, there is a high
∗
rate of complications (> 20%) associated with open approaches to the anterior skull base, including cerebrospinal fluid leak, meningitis, intracranial hemorrhage, and pneumocephalus [16]. In the adult population, endoscopic endonasal approaches to the anterior skull base have become the standard of care for a wide variety of pathologies [17–20]. Multiple authors have reported successful closure rates (> 90%) of anterior skull base defects using endoscopic techniques with few complications [21–24]. While the paradigm continues to shift towards endoscopic only approaches for pediatric lesions of the anterior skull base, a major concern is the compact anatomy that may preclude endoscopic repair, especially in very young children. However, several studies suggest that endoscopic resection and repair is feasible and has a more favorable complication profile than open approaches, even in children as young as 8 months old [25–31]. Despite data indicating safety and feasibility, many contemporary authors continue to utilize primarily open or combined approaches in pediatric skull base surgery
Corresponding author. UAB – Department of Otolaryngology, FOT 1155|1720 2nd Avenue S Birmingham, AL, 35294-3412, USA. E-mail address: [email protected] (B.A. Woodworth).
https://doi.org/10.1016/j.ijporl.2020.109868 Received 28 October 2019; Received in revised form 6 January 2020; Accepted 6 January 2020 Available online 08 January 2020 0165-5876/ © 2020 Published by Elsevier B.V.
International Journal of Pediatric Otorhinolaryngology 131 (2020) 109868
H.M. Thompson, et al.
4. Discussion
[8,14,15,32]. The aim of the present study is to review the management of these rare lesions based on our collective experience and pooled data from a multi-institutional patient series.
Congenital encephaloceles of the anterior skull base may cause significant morbidity and mortality. However, the risks and benefits of surgical repair must be weighed individually and should include a thorough assessment of presenting symptoms, patient age, and nasal cavity size, as well as comfort level of the surgeon with the endoscopic approach. Because of the rarity of these lesions, it is difficult to determine the optimal time of repair based on data gleaned from case series in the literature. Repair of the defect is critical if CSF leak or meningitis (the most dreaded complication of encephalocele) is the presenting scenario, but the decision to intervene in the existence of more benign symptoms such as nasal obstruction or deformity is not equally illuminating. Historically, basal encephaloceles were thought to confer less risk of meningitis than external lesions, prompting some providers to delay surgical repair until children developed larger nasal cavities [9,33,34]. In the current study, nasal obstruction was by far the most common presenting symptom (identified as excessive nasal secretions and snorting in infants unable to convey symptoms) and was usually discovered at a very young age. Given the two major complications following surgery were meningitis and CSF leak (patients 3 and 4, respectively), it is unclear whether the benefit outweighed the risk in these two patients. Surgical intervention was chosen primarily due to nasal obstruction. Patient 3 had post-operative meningitis after removal of the anterior encephalocele, which was considered necessary to allow cosmetic surgery for her midline nasal cleft before enrolling in school. All three patients with central sphenoid encephaloceles (patients 1, 3, and 4) involved the third ventricle, which may have contributed to the post-operative CSF leak seen in patient 4 particularly in conjunction with her severe post-operative emesis. Despite the complications, patients were managed successfully with appropriate interventions (revision endoscopic repair and intravenous antibiotics) and had no sequelae from their complications. It should also be noted our complication rate of 14% compares favorably to rates of over 20% reported in some series of open procedures [16]. While craniofacial anomalies (e.g. telecanthus) are very common in frontoethmoidal encephaloceles, the rate of abnormalities in patients with basal encephaloceles is difficult to determine from existing case series [35]. It should be noted patient 3 had 2 encephaloceles that did not physically contribute to her midline nasal cleft, but rather were associated with an overall failure of development in utero. On the other hand, several patients presented with telecanthus and nasal obstruction when originating at the foramen cecum. Larger encephaloceles in this region, particularly in younger children, pushed the boundaries of a small nasal cavity and the mass effect led to splaying of the nasal bones. In addition, Patient 8 had orbital displacement due to the encephalocele exiting at the orbital plate of the frontal bone. It is unclear what effect surgical removal will have on their telecanthus/deformity over time, but was considered to (at least) terminate the physical pressure leading to the phenomenon. In addition, facial growth has been demonstrated to realign after surgery in infancy, thus immediate removal of large encephaloceles causing craniofacial anomalies should be considered [36]. The present study suggests that children as young as 2 months of age can be surgically managed using endoscopic techniques. It should be noted that, in the younger patient population, these were larger encephaloceles that caused significant mass effect with severe septal deviations and lateralization of the turbinates. The ensuing exposure was considered excellent despite small nostrils and improved the working space for endoscopic removal and repair. However, a transphenoidal encephalocele was repaired in a 3 year old (Patient 1) although no mass effect was present besides replacement of the posterior septum. Therefore, the decision for surgery based on the age of the patient should depend on careful evaluation of the nasal cavity dimensions and surgeon experience. Our study highlights the importance of remaining vigilant for the
2. Methods Institutional Review Board approval was obtained at both the University of Alabama at Birmingham and the Medical University of South Carolina prior to the initiation of this study. A retrospective review of children and young adults with congenital encephaloceles of the anterior skull base presenting from January 1, 2003 to January 4, 2019 was performed. Criteria for inclusion were obvious congenital etiology (e.g. patent neuropore at foramen cecum, bifid sphenoid or crista galli) without previous head trauma or spontaneous etiology (intracranial hypertension). The preoperative examination included a thorough medical history, physical examination, and radiographic imaging. We performed standard computed tomographic and magnetic resonance imaging on all patients. Intraoperative computer-assisted surgical navigation was utilized in every operation. Surgical technique was similar between surgeons (RJS and BAW). Using 0°, 30°, and 70° adult and pediatric endoscopes, encephaloceles were meticulously reduced with bipolar cautery or radiofrequency coblation. Cautery was avoided along the inside of the nasal vestibule to avoid the risk of adverse outcomes such as nasal stenosis. Sinuses were opened as necessary for exposure and placement of instruments. Removal of mucosa was performed at the site of the bony defect. Wide removal of mucosa along the septum or beneath the nasal bones where meningoencephaloceles had expanded in the submucosal plane was not necessary and could contribute to nasal stenosis and prolonged healing. Multilayer repair was then performed with grafts (allografts/xenografts) +/− pedicled nasoseptal flaps. Repairs were supported with Gelfoam (Pfizer Inc, New York, NY) and Merocel sponge in a non-latex gloved finger. Merocels were left in place for 13–14 days and were well tolerated by all patients. Patients that could not have merocels removed in-office were taken back to the operating room at 14 days for repeat endoscopy and removal. Lumbar drains were not routinely used. Post-operative follow-up was performed on all patients with in-office endoscopy. Routine follow-up imaging was not performed if the patient tolerated endoscopy and the skull base repair was visualized. 3. Results Fourteen patients (average age 6.0 years, range 2 months–22 years) with 15 encephaloceles were identified and included in the study (8 females/6 males). The most common presenting symptom was nasal obstruction (Table 1). Three patients had previously undergone surgery at other institutions for mistaken nasal polyps (n = 2) and an adenoid cyst (n = 1). Associated anomalies included craniofacial deformities (n = 5), Morning Glory Syndrome (n = 1), and congenital hypopituitarism (n = 1). All patients were managed via a purely endoscopic endonasal approach, except for one patient who required a combined approach using a brow craniotomy. The craniotomy was performed in this instance because the encephalocele exited through the orbital plate of the frontal bone into the superior orbit. The majority of lesions originated from the foramen cecum with a mean encephalocele size of 2.44 cm in diameter measured from skull base defect to the distal tip (range 0.5–3.6 cm). Mean skull base defect size in length (rostral to caudal) x width (transverse) was 8.6 x 7.7 mm. One patient developed a CSF leak, which was felt to be related to postoperative severe emesis. This patient was taken back to the OR for endoscopic endonasal revision of the nasoseptal flap and additional supportive packing without complications on follow-up. Postoperative meningitis developed in one individual, but resolved with 3 weeks of intravenous antibiotic treatment. No patients were found to have recurrence of encephalocele. 2
International Journal of Pediatric Otorhinolaryngology 131 (2020) 109868
H.M. Thompson, et al.
Table 1 Summary patient data. Pt
Age
Sex
Size(cm)
Defect (lxw)
Repair
Presentation
Location
1 2 3
3y 3y 5y
F F F F F M F F M F M M M M
SIS, SIS, SIS SIS SIS, SIS, SIS, SIS, SIS, SIS, CM CM CM TF MG
NO, Morning Glory Syndrome NO NO, Midline Nasal Cleft
22 y 6m 12 y 15 y 2m 7m 3y 8m 6m 16 y 2y
8x8 4x4 15x15 15x15 12x12 4x4 10x8 4x4 5x5 6x6 8x8 10x10 6x8 5x5 8x8
NSF NSF
4 5 6 7 8 9 10 11 12 13 14
3.2 2.5 3 3.5 1.5 2.6 3.6 1 2 2 2.6 2.3 2.8 0.5 3.5
NSF NSF NSF NSF NSF NSF
NO, Congenital Hypopituitarism NO, Telecanthus NO Incidental NO, Telecanthus NO NO, Nasal Swelling NO, Orbital Displacement NO, Telecanthus CSF Leak, Meningitis NO, Telecanthus
Central Sphenoid Foramen Cecum Midline ACF Central Sphenoid Central Sphenoid Foramen Cecum Foramen Cecum Foramen Cecum Foramen Cecum Foramen Cecum Foramen Cecum Orbital Plate of Frontal Bone Foramen Cecum Ethmoid Roof Foramen Cecum
Key: y– Years, m – Months, F - Female, M − Male, NO - Nasal Obstruction, ACF - Anterior Cranial Fossa, lxw (Length (rostral to caudal) vs. Width (transverse)), SIS – Porcine Submucosal Small Intestine (Biodesign®, Cook Medical, Bloomington, In), CM – Collagen Matrix, NSF- Nasoseptal Flap, MG – Mucosal Graft, TF - Temporalis Fascia.
Disclosures Bradford A. Woodworth, MD is a consultant for Cook Medical, Baxter, and Smith and Nephew. References [1] G.B. Hughes, G. Sharpino, W. Hunt, H.M. Tucker, Management of the congenital midline nasal mass: a review, Head Neck Surg. 2 (3) (1980) 222–233. [2] R. Blumenfeld, E.M. Skolnik, Intranasal encephaloceles, Arch Otolaryngol Chic Ill 1960 82 (5) (1965) 527–531. [3] M. Tirumandas, A. Sharma, I. Gbenimacho, et al., Nasal encephaloceles: a review of etiology, pathophysiology, clinical presentations, diagnosis, treatment, and complications, Childs Nerv Syst ChNS Off J Int Soc Pediatr Neurosurg 29 (5) (2013) 739–744, https://doi.org/10.1007/s00381-012-1998-z. [4] C. Suwanwela, N. Suwanwela, A morphological classification of sincipital encephalomeningoceles, J. Neurosurg. 36 (2) (1972) 201–211, https://doi.org/10. 3171/jns.1972.36.2.0201. [5] P. Castelnuovo, M. Bignami, A. Pistochini, P. Battaglia, D. Locatelli, I. Dallan, Endoscopic endonasal management of encephaloceles in children: an eight-year experience, Int. J. Pediatr. Otorhinolaryngol. 73 (8) (2009) 1132–1136, https://doi. org/10.1016/j.ijporl.2009.04.023. [6] C.M. Myer, R.T. Cotton, Nasal obstruction in the pediatric patient, Pediatrics 72 (6) (1983) 766–777. [7] G. Kalkan, S. Paksu, N. Asilioglu, M. Kiliç, Nasopharyngeal encephalocele: a rare cause of upper airway obstruction, Hong Kong Med J Xianggang Yi Xue Za Zhi Hong Kong Acad Med 19 (2) (2013) 186–187. [8] S. Sachdeva, R. Kapoor, P. Paul, R. Yadav, Recurrent meningitis with upper airway obstruction in a child: frontonasal encephalocele- a case report, J Clin Diagn Res JCDR 8 (8) (2014), https://doi.org/10.7860/JCDR/2014/9262.4749 PD01-02. [9] B.A. Woodworth, R.J. Schlosser, R.A. Faust, W.E. Bolger, Evolutions in the management of congenital intranasal skull base defects, Arch. Otolaryngol. Head Neck Surg. 130 (11) (2004) 1283–1288, https://doi.org/10.1001/archotol.130.11.1283. [10] R. Macfarlane, J.T. Rutka, D. Armstrong, et al., Encephaloceles of the anterior cranial fossa, Pediatr. Neurosurg. 23 (3) (1995) 148–158. [11] C.S. Chen, D. David, A. Hanieh, Morning glory syndrome and basal encephalocele, Childs Nerv Syst ChNS Off J Int Soc Pediatr Neurosurg 20 (2) (2004) 87–90, https:// doi.org/10.1007/s00381-003-0869-z. [12] J. Caprioli, R.L. Lesser, Basal encephalocele and morning glory syndrome, Br. J. Ophthalmol. 67 (6) (1983) 349–351. [13] S.P. Hao, H.S. Wang, T.N. Lui, Transnasal endoscopic management of basal encephalocele–craniotomy is no longer mandatory, Am. J. Otolaryngol. 16 (3) (1995) 196–199. [14] R. Gun, F. Tosun, A. Durmaz, et al., Predictors of surgical approaches for the repair of anterior cranial base encephaloceles, Eur Arch Oto-Rhino-Laryngol Off J Eur Fed Oto-Rhino-Laryngol Soc EUFOS Affil Ger Soc Oto-Rhino-Laryngol - Head Neck Surg 270 (4) (2013) 1299–1305, https://doi.org/10.1007/s00405-012-2174-5. [15] R. Rahbar, V.A. Resto, C.D. Robson, et al., Nasal glioma and encephalocele: diagnosis and management, The Laryngoscope 113 (12) (2003) 2069–2077, https://doi. org/10.1097/00005537-200312000-00003. [16] J.T. Kryzanski, D.J. Annino, H. Gopal, C.B. Heilman, Low complication rates of cranial and craniofacial approaches to midline anterior skull base lesions, Skull Base Off J North Am Skull Base Soc Al 18 (4) (2008) 229–241, https://doi.org/10.1055/ s-2007-1003924. [17] J.W. Grayson, M.R. Chaaban, K.O. Riley, B.A. Woodworth, Smell sparing unilateral intracranial dermoid resection, Allergy Rhinol Provid RI 5 (1) (2014) 39–40,
Fig. 1. Radiographic and Endoscopic Imaging A T2-weighted coronal MRI scan demonstrating a congenital encephalocele emanating from a patent foramen cecum (A). Transnasal endoscopic view of the encephalocele positioned just posterior to the nasal vault (B). The encephalocele is ablated using a Coblator (C and D). An underlay free graft (E) and overlay septal flap is used for repair (F).
identification of encephaloceles in pediatric patients. Even though basal encephaloceles have few external manifestations, they frequently lead to symptoms. In our series, 12 of 14 patients presented with nasal obstruction. Despite the presence of congenital hypopituitarism in patient 4, no CT or MRI scan was performed at the outside institution before proceeding with an adenoidectomy for an “adenoid cyst”. Patient 2 presented with nasal obstruction at age 3 and was treated with surgery for presumptive nasal polyposis (even though a singular unilateral occurrence) before the correct diagnosis was made. Therefore, it is critical for otolaryngologists to have a high degree of suspicion in pediatric patients presenting with nasal or nasopharyngeal mass at a young age, especially in the setting of telecanthus or congenital disorders of the pituitary(see Fig. 1). 5. Conclusion Endoscopic endonasal repair of congenital anterior encephaloceles is safe and effective, even in children less than one year of age and will continue to emerge as a preferred option for therapeutic intervention for the vast majority of these lesions. 3
International Journal of Pediatric Otorhinolaryngology 131 (2020) 109868
H.M. Thompson, et al.
[27] T. Van Den Abbeele, M. Elmaleh, P. Herman, M. François, P. Narcy, Transnasal endoscopic repair of congenital defects of the skull base in children, Arch. Otolaryngol. Head Neck Surg. 125 (5) (1999) 580–584. [28] D. Locatelli, F. Rampa, I. Acchiardi, M. Bignami, A. Pistochini, P. Castelnuovo, Endoscopic endonasal approaches to anterior skull base defects in pediatric patients, Childs Nerv Syst ChNS Off J Int Soc Pediatr Neurosurg 22 (11) (2006) 1411–1418, https://doi.org/10.1007/s00381-006-0114-7. [29] A. Kassam, A.J. Thomas, C. Snyderman, et al., Fully endoscopic expanded endonasal approach treating skull base lesions in pediatric patients, J. Neurosurg. 106 (2 Suppl) (2007) 75–86, https://doi.org/10.3171/ped.2007.106.2.75. [30] M. Sasani, A.F. Ozer, A.L. Aydin, Endoscopic treatment of trans-sellar trans-sphenoidal encephalocele associated with morning glory syndrome presenting with nontraumatic cerebrospinal fluid rhinorrhea, J. Neurosurg. Sci. 53 (1) (2009) 31–35. [31] F. Di Rocco, V. Couloigner, P. Dastoli, C. Sainte-Rose, M. Zerah, G. Roger, Treatment of anterior skull base defects by a transnasal endoscopic approach in children, J. Neurosurg. Pediatr. 6 (5) (2010) 459–463, https://doi.org/10.3171/ 2010.8.PEDS09325. [32] H.K. Dutta, P. Deori, Anterior encephaloceles in children of Assamese tea workers, J. Neurosurg. Pediatr. 5 (1) (2010) 80–84, https://doi.org/10.3171/2009.8. PEDS0912. [33] E.W. Hoving, C. Vermeij-Keers, Frontoethmoidal encephaloceles, a study of their pathogenesis, Pediatr. Neurosurg. 27 (5) (1997) 246–256. [34] R.L. Rapport, R.C. Dunn, F. Alhady, Anterior encephalocele, J. Neurosurg. 54 (2) (1981) 213–219, https://doi.org/10.3171/jns.1981.54.2.0213. [35] D.J. David, New perspectives in the management of severe cranio-facial deformity, Ann. R. Coll. Surg. Engl. 66 (4) (1984) 270–279. [36] N.U. Rahman, Nasal encephalocoele. Treatment by trans-cranial operation, J. Neurol. Sci. 42 (1) (1979) 73–85.
https://doi.org/10.2500/ar.2014.5.0074. [18] N.U. Hatch, K.O. Riley, B.A. Woodworth, Endoscopic removal of a bullet penetrating the middle cranial fossa, Skull Base Rep. 1 (1) (2011) 47–50, https://doi. org/10.1055/s-0031-1275633. [19] V. Jones, F. Virgin, K. Riley, B.A. Woodworth, Changing paradigms in frontal sinus cerebrospinal fluid leak repair, Int Forum Allergy Rhinol 2 (3) (2012) 227–232, https://doi.org/10.1002/alr.21019. [20] B.A. Woodworth, R.J. Harvey, J.G. Neal, J.N. Palmer, R.J. Schlosser, Endoscopic management of frontal sinus mucoeceles with anterior table erosion, Rhinology 46 (3) (2008) 231–237. [21] A.H. Marshall, N.S. Jones, I.J. Robertson, Endoscopic management of basal encephaloceles, J. Laryngol. Otol. 115 (7) (2001) 545–547. [22] C.A. Banks, J.N. Palmer, A.G. Chiu, B.W. O'Malley, B.A. Woodworth, D.W. Kennedy, Endoscopic closure of CSF rhinorrhea: 193 cases over 21 years, Otolaryngol–Head Neck Surg Off J Am Acad Otolaryngol-Head Neck Surg 140 (6) (2009) 826–833, https://doi.org/10.1016/j.otohns.2008.12.060. [23] M.T. Purkey, B.A. Woodworth, S. Hahn, J.N. Palmer, A.G. Chiu, Endoscopic repair of supraorbital ethmoid cerebrospinal fluid leaks, ORL J Oto-Rhino-Laryngol Its Relat Spec. 71 (2) (2009) 93–98, https://doi.org/10.1159/000193219. [24] G.M. Oakley, R.R. Orlandi, B.A. Woodworth, P.S. Batra, J.A. Alt, Management of cerebrospinal fluid rhinorrhea: an evidence-based review with recommendations, Int Forum Allergy Rhinol. September (2015), https://doi.org/10.1002/alr.21627. [25] M. Abdel-Aziz, H. El-Bosraty, M. Qotb, et al., Nasal encephalocele: endoscopic excision with anesthetic consideration, Int. J. Pediatr. Otorhinolaryngol. 74 (8) (2010) 869–873, https://doi.org/10.1016/j.ijporl.2010.04.015. [26] S.J. Kanowitz, J.M. Bernstein, Pediatric meningoencephaloceles and nasal obstruction: a case for endoscopic repair, Int. J. Pediatr. Otorhinolaryngol. 70 (12) (2006) 2087–2092, https://doi.org/10.1016/j.ijporl.2006.08.007.
4