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170
I. Mohammad et al.
non-keratinizing carcinoma and basaloid squa­mous cell carcinoma (Table7.1) [7].
In newly diagnosed NPC, radiotherapy or con-
current chemoradiotherapy has been the standard
Table 7.1 Histopathological types of NPC and their features
Histopathological type of NPC Features
Keratinizing squamous cell carcinoma
This is applied to tumours showing obvious or prominent squamous differentiation at the light microscopic level. Intercellular bridges and/or varying degrees of keratinization are indicators of squamous differentiation. The tumour grows in irregular islands separated by desmoplastic stroma [8].
treatment. The use of radiation allows the man­agement of both the primary site and nodal metastasis, especially the retropharyngeal lymph nodes as the rst echelon node in
Non-keratinizing squamous cell carcinoma
• Differentiated type
• Undifferentiated type
Basaloid squamous cell carcinoma
Keratinizing squamous cell carcinoma (H&E 400×) As implied by the name, the characteristics of squamous differentiation are ambiguous in
this subtype. Malignant cells are arranged in a variety of ways, ranging from solid to trabecula to singly. The more prevalent undifferentiated subtype is characterized by large cells with round-to-oval vesicular nuclei, prominent central nucleoli and sparse eosinophilic cytoplasm. Indistinct cell borders result in a syncytial appearance. They coexist with a variable proportion of lymphoplasma cells, and the stroma lacks any desmoplastic response [8].
Non-keratinizing nasopharyngeal carcinoma, undifferentiated subtype (H&E 400×) Basaloid squamous cell carcinoma is rare to occur in the nasopharynx. This type is
characterized by the presence of basaloid malignant cells mixed in with squamous cell carcinoma cells [8].
7 Surgical Management ofNasopharyngeal Carcinoma
Fig. 7.1 General management outline of NPC
Suspicious history
Imaging Treatment
Examination and
nasolaryngoscopy
171
Histopathological
diagnosis (HPE)
NPC.Radiotherapy is the loco-regional treatment for all stages of NPC, without distant metastasis [9]. General management ow of NPC is as in Fig.7.1.

7.2 Salvage Neck Dissection

Salvage neck dissection is an approach of choice for neck residue or recurrent nasopharyngeal car­cinoma after primary radiotherapy treatment (RT). NPC is easily metastasized to cervical lymph node, and the incidence of residual or recurrent NPC is 4.6–18% [1].
By denition, a recurrence of disease is when the cervical lymph nodes reappear 3months after initial complete regression, while residual of dis­ease is dened as a lymph node without complete regression by 3 months after primary therapy [10]. In current practice, the recurrence of regional NPC is conrmed by ne needle aspira­tion (FNA) cytology. Before receiving salvage treatment for regional failure, patients will receive complete physical examination, nasopha­ryngoscopy and biopsy of the suspicious naso­pharyngeal lesions, repeat imaging and restaging of the tumour to exclude local disease and distant metastases.
According to Wang etal., level II is the most common site of nodal metastasis, followed by levels V, III, IV and I.From the study, the author also suggests that if there is no metastasis in level I and parotid region after careful examination, routine dissection of level I and parotid gland is not necessary [11]. In salvage neck dissection, the operation involves modied radical neck dis-
section, radical neck dissection or extended radi­cal neck dissection (which may include parotidectomy, skin, phrenic nerve, vagus nerve, submaxillary and hypoglossal nerve).
7.2.1 Roles ofFlaps inSalvage Neck Dissection
There are few options for ap reconstructions in salvage neck dissection. These include anterior­lateral thigh (ALT) free ap and pedicled pecto­ralis major myocutaneous ap (PMMF) to repair large facial defects more than 4.0cm in diameter. However, microsurgical free ap reconstruction is a challenging procedure in a patient who received post-operative irradiation because the treatment depletes the area of potential recipient neck vessels for microvascular anastomosis [12]. According to a study by [13], the free ap recon­struction is feasible and safe in patients with prior irradiation as the failure rate was 6.3% in the prior irradiation and neck dissection group, 4.8% in the neck dissection group and 5.2% in the irradiation group compared to 2.1% in the non­irradiation and neck dissection group.
In the case series of Lin etal., they preferred ALT ap due to it being a reliable perforator ap with constant anatomy based on the descending branch of the lateral circumex femoral artery, long vascular pedicle that is suitable for micro­vascular anastomosis to the transcervical recipi­ent vessels and availability of large and long ap that can be harvested without any complications [12]. Song etal. had reported the good outcome of all the aps, which involved PMMFs, free
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I. Mohammad et al.
forearm ap, free bular bone ap and pedicled tongue ap, that were used in their salvage sur­gery in NPC cases. The NPC cases were origi­nally treated with radiotherapy and subsequently developed neck recurrence or second primary tumour in the radiated area [14]. In the study, the PMMF was created in spindle or crescent shape as required by the donor site and was tunnelled under the medial one-third of the clavicle, and the ap was transferred to the cervical area and sutured to the cervical defect.
7.3 External Access forNasopharyngectomy: Lip Split, Maxillary Swing, Midfacial Degloving
The advent of endoscopic transnasal approaches has made open approaches to the nasopharynx less popular. However, it is not entirely obsolete, especially in the circumstances where an endo­scopic approach is deemed unable to adequately clear the tumour or in centres with facilities where dedicated skull base team is limited. This chapter aims to give an idea on the selection cri­teria, advantages and disadvantages and the brief surgical steps.
The nasopharynx is bordered anteriorly by posterior nasal apertures and nasal septum, poste­riorly by the buccopharyngeal fascia which lies anterior to the longus capitis muscle, superiorly by the pharyngeal mucosa overlying the basi­sphenoid and inferiorly by the oropharynx at the level of soft palate. The lateral limit is formed by the opening of the Eustachian tube (ET). Thus, the goal of resection is to include the cartilagi­nous ET medial to the medial pterygoid muscle.
Numerous approaches have been described, and we would like to classify them as below:
1. Transoral-transpalatine approach
2. Transmandibular-transcervical approach
3. Anterolateral: Maxillary swing approach
4. Lateral: Infratemporal approach type C
5. Combined approach: Subtemporal­preauricular infratemporal fossa approach and facial translocation
7.3.1 Patient Selection
andPreoperative consideration
Open nasopharyngectomy unavoidably renders signicant morbidity to a patient both function­ally and aesthetically. It is only reasonable to be performed should the procedure change the course of disease either by total eradication of tumour or by reducing the bulk. Detailed knowl­edge of the complex anatomy of the nasopharynx and precise understanding of the vital structures like skull base and internal carotid artery (ICA) are therefore indispensable before embarking on this procedure.
Computed tomography and magnetic reso­nance imaging are complementary in assessing the tumour volume and local and intracranial­intradural extension, to evaluate the ICA and the potential damage to it. Angiography and balloon occlusion test are needed if resection of ICA is deemed necessary. A pre-procedure tracheos­tomy is necessary in almost all the open approaches including the transoral approach. This is to avoid airway compromise due to oedema in the immediate post-operative period. Most patients will require alternative feeding method in the post-operative period. Therefore, they need to be counselled on nasogastric tube feeding or even PEG tube feeding if resection is anticipated to be extensive, which will signi­cantly delay the oral feeding. If a transpalatal approach is used, preoperative designing and planning for an acrylic palatal splint or obturator need to be considered.
An honest discussion on the risks of the surgery with the patient cannot be overemphasized. This includes potential residual tumour, subsequent
7 Surgical Management ofNasopharyngeal Carcinoma
173
adjuvant chemoradiotherapy, fatal injury to ICA, haemorrhage from the pterygoid plexus and cere­brospinal uid leak. In patients undergoing naso­pharyngectomy as a salvage procedure after radiotherapy, it is utmost importance to carefully explain the risk of osteoradionecrosis not only at the mandible but more dangerously over the sphe­noid body. Other risks are injury to the tooth, pala­tal numbness and velopharyngeal insufciencies.
Intraoperatively, local anaesthesia to the skin and mucosa can be given with 1% lidocaine with 1:100,000 adrenaline. Patient is positioned in the standard supine position with neck extension. Drains are required when the neck is accessed. Prophylactic antibiotics can be administered to reduce the risk of infection. Intraoperative steroid
a
(IV dexamethasone) will aid in reducing airway oedema. Ventilation is best considered using a tracheostomy as mentioned above.
Image-guided navigation has become almost a
must in recent days.
7.3.2 Transoral-Transpalatine Approach (Fig.7.2)
This is an approach for malignant tumours with limited clivus and craniocervical junc­tion extension and size measuring less than 2 cm. This approach is contraindicated for tumours larger than 2 cm and with intracranial- intradural involvement, exten-
b
c
Fig. 7.2 Transoral transpalatine approach. (a) Transoral incision. (b) Mucosal incision along the palatal aspect of the alveolar ridge. (c) Mucosal ap raised and hinged laterally
174
I. Mohammad et al.
sion lateral to the lateral pterygoid muscle, involvement of cavernous sinus, infratempo­ral fossa extension and peritubal extension [1517].
7.3.2.1 Procedure
1. The patient is placed in the supine position with their head extended as much as possible.
2. Mayeld clamp xation is required if image­guided navigation is employed.
3. Tracheostomy is performed.
4. Crockard wide-eld retractors and dissecting instruments that have been specially designed for working through narrow long-distance transoral approach are needed.
5. Mouth gag is inserted, and the oral cavity and soft and hard palate are inltrated with 1:200,000 adrenaline.
6. The soft palate is split to one side of the midline.
7. The hard palate mucosa is incised along the ipsilateral junction of the hard and soft palate within 5mm of the maxillary dentition.
8. This mucosal incision is extended along the palatal aspect of the alveolar ridge up to the level of the rst molar and then taken in an arc across the palate to the opposite side.
9. A mucoperiosteal ap is elevated exposing the bony hard palate. The greater palatine neurovascular bundle is coagulated and divided.
10. Muscular insertions of the soft palate from the hard palate are divided to increase exposure.
11. A self-retaining retractor is then inserted to keep the two halves of the soft palate out of the eld.
12. Dissection through the posterior nasopharyn­geal wall gives access to the lower clivus.
13. Removal of some palatal vomerine bone gives access to the sphenoid oor.
14. Perform a Le Fort I osteotomy and then split the palate in the midline, displacing each maxilla laterally, known as an ‘open-door maxillotomy’, which gives access to clivus and craniocervical junction.
15. A suitable mucosal ap is raised and tucked laterally or inferiorly.
16. The length of this ap varies according to the extent of the lesion being removed. The larger the ap, the better the chance of being able to approximate it at the end of the procedure.
17. If access to the clivus is required, the prever­tebral fascia and muscles have to be opened and this is best undertaken in the midline. The important anatomical landmark is the anterior arch of the atlas.
18. Most resection is usually performed in a piecemeal fashion.
19. Dural tears require watertight closure, which is possible with tissue sealants.
20. The dead space created by tumour resection can be lled with autograft soft tissue secured in place by brin glue and covered by pedi­cled local mucosal or mucoperichondrial aps.
21. The prevertebral fascia, musculature and pharyngeal mucosa are reapproximated.
22. The soft palate is approximated in a three­layered closure to minimize a stula.
23. Bone plates and screws are applied if the maxilla has been split.
24. Removal of the tracheostomy is determined by the clinical progress of the patient in terms of swallowing and resolution of oedema.
7.3.2.2 Advantages
Transoral transpalatine technique is a simple and direct approach to the nasopharynx.
It is also extendable through the Le Fort
I-palatal split approach.
7.3.2.3 Disadvantages
The major drawback is narrow working eld and long working distance and no access later­ally to the parapharyngeal and infratemporal spaces. Apart from this, it requires tracheos­tomy. The rate of palatal dehiscence and orona­sal stula is around 30–40%. Patients are also prone to the risk of meningitis due to wound contamination.
7 Surgical Management ofNasopharyngeal Carcinoma
175
7.3.3 Transmandibular­transcervical approach
This approach (Fig.7.3) is suitable for tumours that are encroaching into the anterior part of the infratemporal fossa. It is contraindicated if the tumour has intracranial intradural invasion more than 2cm or involving the parasellar and poste­rior infratemporal region [18, 19].
7.3.3.1 Procedure
1. The patient is placed in the supine position
and a tracheostomy is done.
2. Skin incision made from mastoid tip extend-
ing to the midline of the lip to split it. Subplatysmal ap is raised and directed beneath the submandibular gland.
3. The tendons of the digastric and stylohyoid
muscles can be released from their hyoid attachment and reected superiorly along with the submandibular gland to increase exposure of the mandible.
4. The great vessels in the carotid sheath and
adjacent cranial nerves are then identied.
5. Dissection is continued as near to the skull
base following the internal and external carotid arteries.
6. A mandibular osteotomy is performed after pre-plating is done. The authors prefer a paramedian mandibulotomy.
7. The floor of the mouth is dissected. The tongue is retracted to the opposite side, the mucosa incised and the mylohyoid muscle divided together with the anterior belly of the digastric muscle. The lingual and hypoglossal nerves are identified and preserved.
8. The mandible is retracted laterally to reach the parapharyngeal space, which then becomes continuous between the mouth and neck. The external carotid artery can be ligated distal to the lingual artery to increase the retraction of the mandible.
9. This nally allows an access to the nasopha­ryngeal part of the parapharyngeal space and the anterolateral infratemporal fossa.
10. Additionally, the incision can be extended upward towards the hard palate and ptery­goid plates ending about 1cm medial to the gingival margin should a more median access be required.
11. Blunt dissection lateral to and behind the superior and middle constrictor muscles pro­duces a surgical working space.
a
Fig. 7.3 Transmandibular transcervical approach. (a) Extended incision from the mastoid tip rising anteriorly to split the lip. (b) A median mandibular osteotomy exposing
b
the oor of the mouth by diving the anterior belly of digastric and mylohyoid muscle. Lingual and hypoglossal nerves are in view
176
ac
I. Mohammad et al.
12. Additional posterolateral division of the sty­loid musculature and glossopharyngeal nerve and superior dissection along the retropharyngeal- prevertebral plane release the oropharynx, making retraction easier to the contralateral side.
13. Next, to access the cartilaginous part of the ET, the ICA should be accurately identied followed by transection of tensor and levator muscle.
14. Transection of the ET releases the nasophar­ynx from the skull base at the level of the pharyngeal tubercle.
15. Access to nasopharynx and upper sphenoid body can be further increased by removing the posterior portion of the hard palate. If the lesion reaches the posterior aspect of the infratemporal fossa, combination with a lat­eral approach may be required to allow en bloc resection.
16. Any dural defect is repaired in watertight fashion.
17. The remaining pharyngeal sleeve is reat­tached to the prevertebral muscles at the skull base; the pharynx is realigned by sutur­ing the ET at intratubal splint. The palatal muscles are reapproximated, the mucoperi­osteal palatal ap is repositioned and the mucosa is sutured.
18. A pre-planned palatal splint is then applied at hard palate. The oor of the mouth is recon-
structed, and the mandible is xed with the pre-tted miniplates.
19. Large drains though a separate stab incision in the lower neck are placed.
20. The lower lip is closed to achieve accurate alignment of the vermilion border.
7.3.3.2 Advantages
The transmandibular-transcervical technique gives good direct access to the epipharynx and the parapharyngeal and anterior infratemporal space. It also enables good vascular control.
7.3.3.3 Disadvantages
This approach places the wound at a hazard of potential infection through oral contamination, possible mandibular non-union and prolonged need for tracheostomy. Apart from that, restora­tion of normal or adequate swallowing is often signicantly delayed (mean 7weeks). Therefore, temporary PEG is often needed.
7.4 Anterolateral Approach:
Maxillary Swing
This approach (Fig.7.4) is suitable for nasopha­ryngeal tumours with limited extension to the anterior infratemporal region. However, it is con­traindicated for tumours encroaching the petrous ICA, petrous apex, parasellar and/or posterior
b
Fig. 7.4 Anterolateral: Maxillary swing approach. (a) Weber-Ferguson-Longmire incision made, extending lat­erally to the zygoma. (b) Osteotomy site to separate max­illa from zygoma, medially along the inferior orbital rim
and in midline. (c) Transoral view showing palatal split. Entire maxilla swung laterally together with cheek ap and masseter muscle
7 Surgical Management ofNasopharyngeal Carcinoma
177
infratemporal region (combination with lateral approach required) [20].

7.4.1 Procedure

1. Patient is placed in a supine position and draped.
2. The authors prefer a preliminary tracheostomy.
3. Skin incision: Weber-Ferguson-Longmire incision is made.
4. The vertical incision limb is through the upper lip and is continued between the cen­tral incisors and onto the hard palate until the junction of hard and soft palate.
5. The incision is then curved laterally to run behind the maxillary tuberosity.
6. The facial incision is made through the sub­cutaneous, muscular and periosteal layers to expose the planned osteotomy line.
7. The osteotomy is begun by separating the zygoma from the maxilla, moving medially along the infraorbital rim until the frontal process is divided from the anterior maxil­lary wall. Then the medial maxillary wall is separated from the midline nasal complex in an antero-posterior fashion.
8. The posterior wall is released using an osteo­tome inserted through the antrum.
9. The palatal bone is incised, and a midline osteotomy is fashioned.
10. The pterygoid plates are separated from maxillary tuberosity by using a curved osteotome.
11. Finally, the maxilla can be swung laterally attached to the cheek ap and the masseter muscle.
12. The nasopharynx including the cartilaginous ET bilaterally becomes well exposed. This allows en bloc resection of the lesion.
13. Additional ipsilateral turbinectomy gives tis­sue for free graft that can be placed on exposed bone in the epipharynx. Nasal pack­ing keeps the graft in place.
14. Maxilla is returned to its anatomical position and xed to the zygoma and contralateral maxilla with miniplates.
15. Alveolar ridges are approximated by using a preoperatively fashioned dental splint.
16. The facial and intraoral incisions are closed in layers.
17. A grommet should be inserted if cartilagi­nous ET was resected.
18. Oral feeding can be started after 72h.
19. Any non-resorbable nasal packs should be removed on the seventh post-operative day and the dental plate after about a month or when all the mucosal wounds have healed.

7.4.2 Advantages

Maxillary swing is relatively a simple technique, which gives wide exposure and good cosmetic and functional results.

7.4.3 Disadvantages

However, it gives very limited parapharyngeal and infratemporal exposure. Lack of access to the neck renders lack of vascular control. Patient will also suffer from post-operative trismus from pter­ygoid myositis or brosis. In some patients, the maxilla can become necrosed.
7.5 Lateral Infratemporal Fossa
Approach Type C
This procedure (Fig.7.5) is indicated in infratem­poral fossa (ITF) tumours that extend into the temporal bone with involvement of the epiphar­ynx such as juvenile angiobroma class III b–IV (Fisch) and nasopharyngeal carcinoma (NPC). This procedure is contraindicated in lesions extending into the sella, contralateral middle fossa or anterior skull base [21, 22].

7.5.1 Procedure

1. A postauricular C-shaped incision is made that extends superiorly into the temporal region and inferiorly into the neck.
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I. Mohammad et al.
a
Fig. 7.5 Lateral infratemporal fossa approach, type C. (a) Skin incision from the temporal region extending infe­riorly to the neck. (b) Skin ap together with pinna
2. The temporalis muscle, mastoid and zygoma are exposed. A periosteal ap is elevated, and the external auditory canal is transected and closed in a blind sac.
3. The pinna and skin ap are reected anteriorly.
4. For vascular control, the neck is dissected so that carotid and jugular vessels are accessible.
5. The main trunk of the facial nerve is identi­ed together with its frontal branch.
6. The zygomatic arch is exposed and divided anterior to the temporomandibular joint (TMJ) and just behind the orbital rim pre­serving the frontal branch.
7. The zygomatic arch is reected inferiorly attached to masseter muscle.
8. The temporalis muscle and fascia are ele­vated from the temporal fossa and reected inferiorly, exposing the superolateral quad­rant of ITF.
9. A subtotal petrosectomy is done in which the sigmoid sinus, ICA and middle fossa dura are skeletonized with preservation of the labyrinth.
10. The TMJ capsule is exposed followed by excision of the articular disc and displacing the condyle inferiorly.
b
reected anteriorly and the main trunk of facial nerve is identied to divide zygomatic branch anterior to the tem­poromandibular joint without damaging frontal branch
11. Additional space can be established by release of the sphenomandibular and stylo­mandibular ligament.
12. The glenoid fossa is resected to give exten­sive exposure of ITF, and this can be enlarged further by division of the mandibular nerve and middle meningeal artery.
13. The pterygoid process with lateral and medial plates is removed, giving access to the anterior third of the ET and nasopharynx.
14. The nasopharyngeal cavity is entered through incision of the pharyngobasilar membrane and nasopharyngeal mucosa. Subsequent dissection is done to complete the resection.
15. To close the defect, the temporalis muscle can be rotated into it. The alternative is to use the latissimus dorsi ap.
16. The TMJ is reconstructed by interposing temporalis muscle between the condyle of the mandible and the middle fossa dura.
17. The skin and subcutaneous tissue are closed in layers.
18. Drain is placed and secured.
19. Normal oral feeding can be started on the rst post-operative day.
ac
7 Surgical Management ofNasopharyngeal Carcinoma
179

7.5.2 Advantages

The type C lateral ITF approach gives a wide and direct access to the infratemporal region, includ­ing the parasellar and temporal regions. It also provides short working distance.

7.5.3 Disadvantages

The drawbacks of this procedure are transient post-operative trismus, malocclusion and hypaes­thesia of the lower half of the face and ipsilateral tongue (V3). As the ET is resected, it results in permanent conductive hearing loss. Temporary frontal facial paresis occurs in 30%.

7.6 Subtemporal-Preauricular Infratemporal Fossa Approach

This is a combined approach (Fig.7.6) requiring an established team comprising a lateral skull base ENT surgeon and a neurosurgeon. This
approach is not often performed, but it is done for large ITF tumours (T4) encroaching the naso­pharynx, the cavernous sinus and the middle cranial fossa. It is contraindicated in bilateral optic chiasm or ICA involvement, or in lesions extending to the posterior cranial fossa for which a combination with a retrosigmoid or transotic approach is necessary [23].

7.6.1 Procedure

1. This procedure requires preoperative tracheostomy.
2. The ipsilateral scalp, face, neck, lower abdomen and thigh are prepared and draped.
3. An extended Blair incision is made and a cervico-facial ap raised.
4. The facial nerve trunk is identied, and its major branches are dissected peripherally.
5. The parotid gland is raised from the masse­teric fascia.
6. The temporal branches of external carotid are ligated.
b
Fig. 7.6 Subtemporal preauricular infratemporal fossa approach. (a) An extended Blair incision. (b) Osteotomy site for the zygomatic arch including in a fronto-temporal craniotomy; also to include glenoid fossa and oor of the middle fossa lateral to the foramen spinosum and ovale.
(c) Removal of more bone medial to glenoid fossa and greater wing of the sphenoid giving access to the Eustachian tube. Removal of the pterygoid process giving access to the anterior Eustachian tube and posterolateral aspect of nasopharynx