Skull Base Surgery and Transfacial Surgical Approaches

Skull Base Surgery and Transfacial Surgical Approaches

Module Summary

The skull base possesses some of the most complicated anatomy encountered in head and neck surgery. Surgical approaches to the skull base necessitate an intimate understanding of the neurovascular and bony anatomy of the viscero and neurocranium. Treatment of skull base pathology begins with understanding the common pathologies encountered, their imaging characteristics, presenting symptoms and lastly, the surgical approaches to gain access to the skull base. Reconstruction and rehabilitation following treatment are vital components that warrant consideration. 
 

Module Learning Objectives 
  1. Describe important anatomical relationships in the skull base.
  2. Review with the types of pathology that arise in, and involve the skull base.
  3. List the components of a skull base-focused physical exam.
  4. Choose appropriate imaging to evaluate skull base pathology.
  5. Describe various approaches to the skull base.
  6. List common complications seen in skull base surgery.
  7. Stage common skull base pathology based on imaging.

Anatomy

Learning Objectives 
  1. Understand the boundaries, contents and fissures/foramina of the cranial base 
    • Anterior cranial fossa
      • bony landmarks and boundaries
      • contents
      • fissures/foramina
    • Middle cranial fossa
      • bony landmarks and boundaries
      • contents
      • fissures/foramina
    • Orbit
      • bones that comprise the orbit 
      • fissures
      • relationships of ethmoidal arteries to optic nerve and skull base
    • Parapharyngeal space
      • contents of pre-styloid space
      • contents of post-styloid space
    • Masticator space
      • boundaries
      • contents
    • Infratemporal fossa
      • boundaries
      • contents
    • Pterygopalatine fossa
      • boundaries
      • contents
    • Cavernous sinus
      • contents
    • Internal Carotid Artery
      • segments
  2. Understand the layers of the scalp and relationship with the frontal branch of the facial nerve
  3. Understand the vascular anatomy of the scalp and its relationship to vascular flaps
  4. Supraorbital, supratrochlear, superficial temporal, deep temporal arteries
  5. Understand the 3-dimensional anatomy of the maxillary and sphenoid bones and course of the internal carotid artery
References 
  1. Janfaza P. Surgical Anatomy of the Head and Neck. 1R edition. Philadelphia: Lippincott Williams & Wilkins. 2011.
  2. Master Techniques in Otolaryngology- Head and Neck Surgery: Skullbase Surgery. 1st edition. Philidelphia: Wolters Kluwer; 2015.
  3. Patel CR, Fernandez-Miranda JC, Wang WH, Wang EW. Skull base anatomy. Otolaryngol Clin North Am. 2016;49(1):9-20.
  4. Pinheiro-Neto CD, Fernandez-Miranda JC, Wang EW, et al. Anatomical correlates of endonasal surgery for sinonasal malignancies. Clin Anat. 2012;25(1):129-34.

Pathogenesis

Learning Objectives 

Understand the range of pathology that effects the skull base

  • Intracranial origin
    • Meningioma
    • Pituitary adenoma 
  • Sinonasal origin
    • Inverted papilloma
    • Juvenile angiofibroma
    • Esthesioneuroblastoma (olfactory neuroblastoma)
    • Adenocarcinoma
    • Sarcoma
    • Melanoma
    • Rhabdomyosarcoma
    • Squamous cell carcinoma
    • Basal cell carcinoma
    • Nasopharyngeal carcinoma
    • Sinonasal undifferentiated carcinoma (SNUC)
    • Neuroendocrine carcinoma
    • Adenoid cystic carcinoma
  • Other/both
    • Ossifying fibroma
    • Fibrous dysplasia
    • Schwannoma
    • Chordoma
    • Chondrosarcoma
References 
  1. Bresson D, Herman P, Polivka M, Froelich S. Sellar lesions/pathology. Otolaryngol Clin North Am 2016;49(1):63-93. 
  2. Weymuller E, Davis G, Malignancies of the Paranasal Sinuses. In: Flint P, editor. Cummings Otolaryngology-Head and Neck Surgery. 5th edition. Philadelphia: Elsavier, 2010.

Incidence

Learning Objectives 

Understand the relative incidence of common sinonasal malignancies 

  • Squamous cell carcinoma
  • Adenocarcinoma
  • Esthesioneuroblastoma
  • Adenoid cystic carcinoma
  • Melanoma
  • Nasopharyngeal carcinoma
References 
  1. Di Maio S, Ramanathan D, Garcia-Lopez R, et al. Evolution and future skull base surgery: The paradigm of skull base meningiomas. World Neurogsurg. 2012;78(3-4):220-221. 
  2. Ezzat S, Asa SL, Couldwell WT, et al. The prevalence of pituitary adenomas: A systematic review. Cancer. 2004;101(3):613-619.
  3. Fernandez-Miranda JC, Gardner PA, Snyderman CH, et al. Clival chordomas: A pathological, surgical, and radiotherapeutic review. Head Neck. 2014;36(6):892-906.

Genetics

Learning Objectives 

Understand the risk factors for development of skull base pathologies

  • Nasopharyngeal carcinoma
    • Keratinizing
    • Non-keratinizing
  • Sinonasal squamous cell carcinoma
  • Intestinal type adenocarcinoma
References 
  1. Chua ML, Wee JT, Hui EP, Chan AT. Nasopharyngeal carcinoma. Lancet. 2016;387:1012-1024.

Patient Evaluation

Learning Objectives 
  • Understand the most common presenting symptoms of skull base pathologies and their etiologies
    • Visual acuity loss and diplopia
    • Epistaxis
    • Cranial neuropathies 
    • Headaches/Facial Pressure
    • Nasal obstruction/Congestion
  • Explain how to perform and interpret a comprehensive cranial nerve exam 
  • Discuss decision making in deciding whether to biopsy a skull base lesion in clinic and the utility of endoscopic examination
  • Explain the importance of ancillary non-imaging tests in skull base pathology
    • Neuro-ophthalmologic testing
    • Pituitary function testing
References 
  1. Hong GK, Payne SC, Jane JA Jr. Anatomy, physiology, and laboratory evaluation of the pituitary gland. Otolaryngol Clin North Am. 2016;49(1):21-32.

Measurement of Functional Status

Learning Objectives 
  1. Understand the consequences of loss of each cranial nerve. 
  2. Be familiar with quality of life tools of sinonasal symptoms (SNOT-22).
References 
  1. Hopkins C, Gillett S, Slack R, Lund VJ, Browne JP. Psychometric validity of the 22-item Sinonasal Outcome Test. Clin Otolaryngol. 2009;34(5):447-54.
  2. Pant H, Bhatki AM, Snyderman CH, et al. Quality of life following endonasal skull base surgery. Skull Base. 2010;20(1):35-40.

Imaging

Learning Objectives 
  1. Understand the strengths of various imaging modalities, how to utilize these to establish a differential diagnosis and radiographic staging of skull base pathology
    • CT
      • Bony delineation, destruction
      • Calcification
    • MRI
      • Soft tissue definition
    • Angiography
    • PET
  2. Understand the imaging characteristics of common skull base pathologies
    • Primary bone lesions
      • Ossifying fibroma
      • Fibrous dysplasia
    • Inverted papilloma
    • Schwannoma
    • Chordoma
    • Meningioma
    • Esthesioneuroblastoma
    • Sinonasal malignancy
    • Juvenile Angiofibroma
References 
  1. Nunes RH, Abello AL, Zanation AM, Sasaki-Adams D, Huang BY. Imaging in endoscopic cranial skull base and pituitary surgery. Otolaryngol Clin North Am. 2016;49(1):33-62.
  2. KAwaguchi M, Kato H, Tomita Het al. Imaging Characteristics of Malignant Sinonasal Tumors. Journal of clinical medicine 2017;6(12).

Pathology

Learning Objectives 

Understand classic pathologic findings of common skull base lesions

  • Ossifying fibroma
  • Fibrous dysplasia
  • Inverted papilloma
  • Schwannoma
  • Chordoma
  • Melanoma
  • Squamous cell carcinoma
  • Adenoid cystic carcinoma
  • Juvenile angiofibroma
References 

Tsai EC, Santoreneos S, Rutka JT. Tumors of the skull base in children: review of tumor types and management strategies. Neurosurg Focus. 2002;12(5):e1.
Bresson D, Herman P, Polivka M, Froelich S. Sellar lesions/pathology. Otolaryngol Clin North Am. 2016;49(1):63-93.

Treatment

Learning Objectives 

Discuss the merits and disadvantages of primary and adjuvant treatment options

  • Surgery
  • Radiation
    • Intensity-modulated
    • Stereotactic 
    • Proton Beam 
  • Chemotherapy
References 
  1. Pfister, DG. Version 2.2017. NCCN Clinical Practice Guidelines in Oncology. Head and Neck Cancers. 
  2. Scangas GA, Eloy JA, Lin DT. The Role of Chemotherapy in the Management of Sinonasal and Ventral Skull Base Malignancies. Otolaryngologic clinics of North America. 2017;50:433-441.
  3. Wang K, Zanation AM, Chera BS. The Role of Radiation Therapy in the Management of Sinonasal and Ventral Skull Base Malignancies. Otolaryngologic clinics of North America. 2017;50:419-432.
     

Medical Therapies

Learning Objectives 
  1. Understand the medical treatment of common post-operative derangements following skull base surgery.
    • SIADH
    • Diabetes insipidus
  2. Understand the medical management of postoperative cerebrospinal fluid leak
References 
  1. Fraser S, Gardner PA, Koutourousiou Met al. Risk factors associated with postoperative cerebrospinal fluid leak after endoscopic endonasal skull base surgery. J Neurosurg. 2018 Apr;128(4):1066-1071.

Surgical Therapies

Learning Objectives 
  1. Understand the various open approaches utilized to access the skull base and craniofacial skeleton
    • Anterior cranial base
      • Bicoronal approach and layers of the scalp
      • Transcranial anterior craniofacial resection 
    • Middle cranial fossa and infratemporal fossa
      • Pre-auricular (sub-temporal) approaches
      • Post-auricular (transtemporal) approaches
      • Transfacial approaches
      • Orbitozygomatic osteotomies
    • Maxillectomy
      • Lateral rhinotomy
      • Weber-Ferguson
    • Orbital exenteration 
  2. Be familiar with the most commonly used techniques for skull base reconstruction
    • Soft tissue
      • Vascularized flaps
        • Pericranial flap
        • Temporalis muscle flap
        • Temporoparietal fascia flap
        • Nasoseptal
      • Free tissue transfer
      • Allografts for dural reconstruction
    • Craniofacial skeleton
      • Titanium plate and mesh
      • Autologous bone
References 
  1. Master Techniques in Otolaryngology- Head and Neck Surgery: Skullbase Surgery. 1st edition. Philadelphia: Wolters Kluwer; 2015.
  2. Hadad G, Bassagasteguy L, Carrau RLet al. A novel reconstructive technique after endoscopic expanded endonasal approaches: vascular pedicle nasoseptal flap. The Laryngoscope. 2006; 116:1882-1886.

Rehabilitation

Learning Objectives 

Be familiar with rehabilitation needs following skull base surgery

  • Eye care following facial nerve injury
  • Trismus
  • Cerebrospinal fluid leak precautions
  • Swallowing rehabilitation 
References 
  1. Netterville JL, Civantos FJ. Rehabilitation of cranial nerve deficits after neurotologic skull base surgery. The Laryngoscope. 1993 Nov;103(11 Pt 2 Suppl 60):45-54.

Staging

Learning Objectives 

Understand staging systems used for various skull base pathologies

  • Nasopharyngeal carcinoma, sinonasal malignancies, skin cancers, minor salivary gland tumors
    • AJCC 
  • Angiofibroma
    • UPMC 
  • Esthesioneuroblastoma
    • Kadish
    • Dulguerov
References 
  1. Snyderman CH, Pant H, Carrau RL, Gardner P. A new endoscopic staging system for angiofibromas. Arch Otolaryngol Head Neck Surg. 2010;136:588-594.
  2. Kadish S, Goodman M, Wang CC. Olfactory neuroblastoma. A clinical analysis of 17 cases. Cancer. 1976;37(3):1571-6.
  3. Pfister, DG. Version 2.2017. NCCN Clinical Practice Guidelines in Oncology. Head and Neck Cancers.
  4. Dulguerov P, Calcaterra T. Esthesioneuroblastoma: the UCLA experience 1970-1990. Laryngoscope. 1992 Aug;102(8):843-9.

Case Studies

  1. A healthy 53-year-old female presents to clinic a chief complaint of 6 months of nasal congestion and anosmia. Physical exam is unrevealing. Endoscopy demonstrates a mass medial to the middle turbinate. CT demonstrates erosion of the cribriform plate. MRI demonstrates a mass filling the superior nasal cavity with extension through the cribriform plate, heterogeneous T1/T2 signal and variable contrast enhancement. What is your differential diagnosis? Biopsy of the tumor demonstrates discrete nests of tumor cells, which are small and round. How would you stage this tumor? What surgical approach or approaches would you choose? What adjuvant treatment would you anticipate administering? 
  2. A 63-year-old immunosuppressed male presents with a chief complaint of diplopia and lack of tearing from the right eye. On physical examination, he has fullness and tenderness of the right cheek. His cranial nerve exam is notable for limited extraocular movements laterally and superiorly and V2 numbness. CT reveals erosion of the medial and inferior orbit and posterior maxillary sinus wall. MRI reveals an infiltrative mass based in the maxillary sinus, which involves the medial and inferior rectus muscles, inferior orbital fissure, infratemporal fossa and pterygopalatine fossa. There is enlargement of V2 at foramen rotundum. Biopsy reveals squamous cell carcinoma. What are your treatment options? What surgical approaches would you use to remove this tumor? How would you reconstruct the defect? 
References 
  1. Snyderman, C. October 4, 2017. Esthesioneuroblastoma. https://www-uptodate-com.
  2. Pfister, DG. Version 2.2017. NCCN Clinical Practice Guidelines in Oncology. Head and Neck Cancers. 
  3. Master Techniques in Otolaryngology- Head and Neck Surgery: Skullbase Surgery. 1st edition. Philadelphia: Wolters Kluwer; 2015.

Complications

Learning Objectives 

Be familiar with common and major complications encountered in skull base surgery

  • Cranial neuropathies
  • Trismus
  • Vascular injury
  • Cerebrospinal fluid leak
  • Post-operative sinonasal bleeding
  • Enophthalmos and limitations in extraocular movement
References 
  1. Kassam AB, Prevedello DM, Carrau RLet al. Endoscopic endonasal skull base surgery: analysis of complications in the authors' initial 800 patients. J Neurosurg. 2011;114:1544-1568.
  2. Gardner PA, Snyderman CH, Fernandez-Miranda JC, Jankowitz BT. Management of Major Vascular Injury During Endoscopic Endonasal Skull Base Surgery. Otollaryngol Clin North Am. 2016; 49:819-828.

Review

Review Questions 
  1. What are the contents of superior orbital fissure and the syndrome that arises from damage to these contents?
  2. What are the contents of the infratemporal fossa?
  3. What are the risk factors for developing non-keratinizing nasopharyngeal carcinoma?
  4. What are the layers of the scalp?
  5. What are the vascular supplies to the pericranial flap and temporalis flap?
  6. How would you stage a patient with an ethmoid sinus squamous cell carcinoma that invades the medial orbital wall, without evidence of cervical or distant metastases?