ORIGINAL ARTICLE

Skull Base Surgery: Indian Perspective Hemen Jaju, MCh, DNB Abstract: Skull base tumors occur at an interface of multiple surgical specialties, which need to work in tandem to treat these cases. The number of estimated new cancer cases in India is approximately 1 million in a year, and skull base tumors are estimated to be 3500 a year. We present an overview of our experience of operating 550 patients with these tumors during a period of 15 years in a crosssection of society with varying economic background and with our limited resources. We have illustrated 5 patients who have been operated on by us. We have proposed how we could further our cause in treating these patients in our scenario. Key Words: Skull base surgery, craniofacial, surgery, skull base tumor, skull base resection (J Craniofac Surg 2014;25: 1636–1639)

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kull base surgery is a specialty at the interface of various surgical subspecialties including craniofacial, neurosurgery, otorhinolaryngology, head and neck, maxillofacial, and reconstructive surgeries. Anatomy of skull base is complex, making surgery challenging, especially because of its varied pathology. Treatment needs intensive perioperative care and coordination with various subspecialties, thus adding to the challenge. Excisional and reconstructive surgery has a major role to play in the treatment of these lesions.1–10 India has a large subset of patients with head and neck cancers. This has been linked to a habitual use of chewing betel nut, raw tobacco chewing, and smoking and tobacco derivatives that are consumed in high proportions on a regular basis.11 Some of the tumors of these tobacco-related malignancies also have an extension through the skull base. Head and neck cancers constitute approximately 30% to 35% of all cancers. Tumors involving the skull base are 3% to 4% of all head and neck tumors and less than 1% of all cancers of the body. With an estimated incidence of cancer at 94 of 100,000 population, approximately 1 million new cancer cases are detected and treated in India annually.11 At a conservative estimate of 0.3% of What Is This Box? A QR Code is a matrix barcode readable by QR scanners, mobile phones with cameras, and smartphones. The QR Code links to the online version of the article.

From the Jaju Plastic Surgery Center, The Gujarat Cancer and Research Institute, Ahmedabad, Gujarat, India. Received May 15, 2014. Accepted for publication June 13, 2014. Address correspondence and reprint requests to Hemen Jaju, MCh, DNB, Jaju Plastic Surgery Center, Near Somlalit school, B/h Hindu colony, Stadium, Navrangpura, Ahmedabad, Gujarat, 380009, India; E-mail: [email protected] The author reports no conflicts of interest. Copyright © 2014 by Mutaz B. Habal, MD ISSN: 1049-2275 DOI: 10.1097/SCS.0000000000001135

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these being skull base tumors, it would mean that approximately 3500 new cases of skull base tumors are detected in India every year. Our state of Gujarat has a population of 60 million and is estimated to have an incidence of approximately 250 new cases of skull base tumors every year. These are absolute estimates and need to be established with concrete data. A registry does not exist in India yet for skull base tumors.

MATERIALS AND METHODS We work in a dual surgical set up: a public (at a regional cancer institute—The Gujarat Cancer and Research Institute) and private facility. Specialist oncosurgeons, ENT surgeons, radiotherapists, medical oncologists, and neurosurgeons from all over the state of Gujarat refer patients to us. The referring doctors primarily investigate patients, and we see those who are keen for surgery. Patients may have to travel long distances (up to 400 km or 250 miles) to reach our center. Sometimes, patients are diagnosed with this pathology, and the primary healthcare physician is unaware of the possible treatment options for these patients; hence, patients present to us late. We also have a unique socioeconomic structure and payer mix. Patients are mostly self-paid. The public system bears major expenses of patients with low socioeconomic status. Patients treated in private practice are self-paid or paid by insurance companies. It is challenging for families to shell out money for advanced treatments such as radiation and chemotherapy. The patients we treat are also more advanced in surgical staging than those treated in the developed world. At the regional cancer surgery unit, approximately 25,000 new cancer cases are seen every year. Of these, approximately 30% are head and neck cancers, and of these, 1500 head and neck cancers get surgery every year. Of these surgical cases (at the regional cancer institute), approximately 35 to 40 are of skull base surgery. In our practice (in the institute and the private setup), we see 120 to 130 new patients with skull base tumors, and of these, approximately 50 to 55 are operated on every year. Primary investigations include a computed tomography scan and an magnetic resonance imaging of the paranasal sinuses and skull base, a tissue biopsy, and workup for metastasis. All patients are assessed by a team of craniofacial surgeon (who also does the reconstruction), neurosurgeon, medical oncologist, radiotherapist, and anesthesiologist. The patient may then be taken up for surgery after careful team-based planning. The surgery is performed by the craniofacial (and reconstructive) surgeon and neurosurgeon jointly. The surgery is through the transfacial and transcranial/subcranial approach. After excision and confirmation of negative margins where possible, the reconstruction is done using local tissues (frontogaleal flap, temporalis flap, skin flaps) or free flaps (usually the vertical rectus abdominis myocutaneous flap). In our last 100 cases, the mean operating time for surgery using local flaps is approximately 250 minutes, and those for free flaps is a mean of 380 minutes. The incidence of free flap failures in our cases is 3.1% and is comparable with the international standard of care.12–14 Very early lesions may be amenable to endoscopic resection. We limit the endoscopic approach to the early midline lesions. In the postoperative period, the patient is kept in a surgical intensive care unit for 2 to 3 days and then in the postoperative

The Journal of Craniofacial Surgery • Volume 25, Number 5, September 2014

Copyright © 2014 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.

The Journal of Craniofacial Surgery • Volume 25, Number 5, September 2014

SBS: Indian Perspective

surgical floor. They have a mean hospital stay of 14 days postoperatively. Before discharge, the patient is generally evaluated by the radiotherapy department and, if needed, is called for treatment at approximately 4 weeks from the date of surgery. After surgery, the asymptomatic patient is followed up quarterly for 2 years and then 4 to 6 times monthly for the next 3 to 5 years depending on the pathology. Magnetic resonance imaging scan is performed at the follow-up visits for residual/recurrent diseases and failures. Most patients are self-paid for the postoperative investigations (approximately $80–$100), and many cannot afford these follow-up visits. Recurrent residual disease may be seen, and decision for treatment with a curative or palliative intent is taken.

RESULTS We have surgically treated more than 550 patients in the last 15 years.15 The pathology has been varied. The age of the patient at surgery has been from 4 to 90 years. Tumors may be at early or late stage at presentation. The common pathologies seen by us include squamous cell carcinoma, adenoid cystic carcinoma, adenocarcinoma, undifferentiated carcinoma, and olfactory neuroblastoma in that order. The other pathologies that are frequently seen by us include sarcoma, mucoepidermoid tumors, basal cell carcinoma (BCC), meibomian gland carcinoma, teratocarcinoma, angiosarcoma, hemangioendothelioma, angiofibroma, fibrous histiocytoma, schwannoma, melanoma, meningioma, and inverted papilloma. We present a subset of patients that only represent a very limited example of the scope of our practice. We see and treat a large variety of head and neck cancers that have favorable outcomes despite most adverse circumstances. Our free flap success rate is comparable with that of the developed countries in most advanced institutions.12–14 We attribute this to good surgical training and expertise along with good postoperative personalized care. The following patients have either been operated on or were inoperable when the patient presented to us and were treated conservatively.

Patient 1 Osteosarcoma of the Maxilla, Orbit, and Cranial Base (Postretinoblastoma) A 25-year-old woman presented with a swelling in the left side of the cheek. She was previously operated on for a retinoblastoma when she was 3 years old and had been radiated. On investigation, she was diagnosed with osteosarcoma involving the left maxilla and zygoma and into the anterior and lateral skull base, also including the middle cranial fossa. After receiving 3 cycles of chemotherapy, she was taken for surgery, and the tumor was excised. The defect was covered with a free VRAM flap with skin paddles for nasal lining, palatal lining, and eye defect. The muscle was used for giving a bulk to the middle fossa base. The postoperative course was uneventful. The patient completed her chemotherapy and is disease free for 7 years now (Figs. 1A–F).

Patient 2 Teratocarcinosarcoma of the Nasoethmoid Area A 10-year-old child was referred to us with swelling in the nose and nasal bleeding for approximately 2 months. On examination, he had proptosis of the right eye and swelling on the right side of the nose. The tumor was protruding through the nasal cavity. Histology was teratocarcinosarcoma. Complete excision of the tumor was performed, which included a maxillectomy and resection of the medial orbital wall and the cribriform area, through a frontal craniotomy and facial incisions. The reconstruction was done using a galeofrontalis flap and the temporalis muscle flap. Postoperative course was uneventful, and the patient received concurrent

FIGURE 1. A–B, Osteosarcoma of the left maxilla, zygoma, and anterior and middle cranial fossa. C, Anterior and lateral craniotomy to excise the tumor. D, vertical rectus abdominis myocutaneous free flap used to give bulk to the middle cranial base. E–F, Postoperative CT scan to show the defect in the bone and the lesion after 4 years.

chemotherapy and radiation therapy after surgery. Now, the patient has been following up for 5 years without evidence of disease. There is secondary facial asymmetry, which needs surgical correction (Figs. 2A–E).

Patient 3 Olfactory Neuroblastoma A 12-year-old girl presented with nasal bleed on the left side and partial nasal obstruction. It was diagnosed as olfactory neuroblastoma involving the medial orbital wall and the medial maxilla on the ipsilateral side. The lesion was excised with a conventional craniofacial approach. Reconstruction was done with a VRAM free flap. Postoperative radiotherapy was given. Patient was disease free at 3-year follow-up (Figs. 3A–G).

Patient 4 Squamous Cell Carcinoma of the Nasal Cavity and Ethmoids and Orbit This patient was 29 years old and presented with exophthalmos. He was previously operated on at an outside hospital for endoscopic nasal resection of the tumor, which was histologically squamous cell carcinoma. The patient presented to us 6 months after this surgery. On presentation, the disease was involving the right maxilla, orbits, ethmoids, and anterior cranial fossa. Wide excision with an intracranial and extracranial approach was performed. The tumor was removed en bloc, and the defect was covered with bilateral temporalis muscle and a galeofrontalis flap. The patient received postoperative radiation therapy and then developed radionecrosis of the frontal bone, which was surgically debrided and covered with a flap. The patient was asymptomatic for 2 years and then complained of knee pain, which, on investigation, was found to be a bony metastasis. The primary site was disease free (Figs. 4A–F).

© 2014 Mutaz B. Habal, MD

Copyright © 2014 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.

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The Journal of Craniofacial Surgery • Volume 25, Number 5, September 2014

FIGURE 2. A, Tumor extending into the right maxilla, orbit, and ethmoids and through the cribriform plate. B, Tumor protruding from the nose and right eye exophthalmos. C, Transcranial approach for en bloc excision. D, The excised tumor. E, Postoperative after 2 years.

Patient 5 Extensive BCC A patient had previously been treated in a primary healthcare center with wide excision and split thickness graft for BCC of the right malar area. He subsequently had repeat wide excision and a forehead flap. We saw the patient after he had a recurrence. The tumor involved the right maxilla and the overlying skin. Wide excision with a maxillectomy was done, and after the tumor-free margins were confirmed, a VRAM free flap was used to cover the defect. The patient was regularly followed up for 2 years and was found to be clinically disease free. He could not get an imaging investigation on follow-up. Eight years later, he presented again with a very extensive lesion involving the whole face and the anterior skull base, which was inoperable. The patient was later deemed as “end of life” and was treated by pain and palliative care team (Figs. 5A–D).

FIGURE 4. A–B, SCC involving the bilateral maxilla, nasal cavity, and ethmoids. C–D, En bloc excision of the tumor and coverage with bilateral temporalis muscle flaps. E, Osteoradionecrosis of the frontal bone. F, Debridement and flap cover. Patient was seen after 2 years.

Complications and Unfavorable Results Surgery in this complex area is not always associated with favorable outcomes.15 In our last review of 546 operated patients, we had 3 patients (0.5%) who died perioperatively (two of myocardial infarction and one due to meningitis). There were 2 patients with meningitis, and both were in the first 100 patients, when the galeal flap was not used for cover. Both these patients had cerebrospinal fluid leak. There were 27 patients who turned blind, because of either radiation-induced keratitis or optic neuritis. Diplopia, vertical dystopia, canthal drift, nasolacrimal duct blockage, and ectropion and entropion were the common eye problems seen. Esthetic deformities, soft tissue losses, and psychologic issues were also seen in patients. There was 3.1% of incidence of free flap failures.

Future This specialty needs a multidisciplinary approach with inputs from various subspecialties. This was previously labeled as a “No Man’s land,” but with increasing advances in the reconstructive options and better technology for resection and adjuvant treatment,

FIGURE 3. A–B, Olfactory neuroblastoma involving the left ethmoids and the left medial maxilla. C–D, Excision of the tumor with the defect in the anterior skull base. E, The defect filled with vertical rectus abdominis myocutaneous flap. F–G, Postoperative after 2 years and after radiotherapy.

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FIGURE 5. A, Recurrent BCC of the right cheek. B, After wide excision and maxillectomy with tumor-negative margins. C, Coverage of defect with free vertical rectus abdominis myocutaneous flap. D, 8 years later, patient presents with an inoperable lesion.

© 2014 Mutaz B. Habal, MD

Copyright © 2014 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.

The Journal of Craniofacial Surgery • Volume 25, Number 5, September 2014

there is an increase in the number of surgeries performed for these lesions with better chances of survival and cure. Most of these tumors are seen in an advanced stage and would warrant an open approach with tissue replacement. With endoscopic surgical advances, some of these tumors can be treated with endoscopic surgery. However, most of the patients who are being seen by us are either failures at an attempted endoscopic resection or present at a very late stage. With a projected increase in the number of total cancer patients and advances in therapy and technologies in treating these patients in the coming 15 to 20 years, there would be a surge in the number of operable cases. The open surgical techniques are now moving more toward excision and a functionally and esthetically acceptable reconstruction with free flaps. Minimally invasive surgery is also fast developing, and an expertise in this would help in decreasing the morbidity in a select group of patients. As of now, there are very few centers in India that perform more than 20 to 25 skull base surgeries in a year. Future attempts should be made at consolidating these centers into centers of excellence. A national registry for these patients needs to be established. The family physicians and allied healthcare providers, who see these patients in the primary setting, need more education regarding the treatment possibilities and outcomes for these lesions so that patients are seen at an earlier stage. More collaboration is needed between multiple centers, which treat such patients.

Training It would benefit to have a dedicated unit of skull base surgery in India. Skull base surgeons should have a fellowship training that involves rotations in craniofacial surgery, maxillofacial surgery, oncology, and reconstructive surgery and should be able to work in tandem with the neurosurgeons, otorhinolaryngology surgeons, radiotherapists, and medical oncologists. It would be helpful to have a dedicated fellowship in a busy unit so that the trainee can have an extensive exposure in treating such patients. To conclude, skull-based tumors are not very common, and not many patients nor primary care physicians are aware of the treatment possibilities. There are very few centers in India that treat these patients in relatively large numbers on a regular basis. Thus, it would be easy to create a national online database or registry for these patients. Training and surgical skill required is highly specialized. The surgery needs to be done at a tertiary care referral unit

SBS: Indian Perspective

because of the need for team approach for postoperative care and intensive care unit care of these patients and involvement with airway, base of the brain, and other vital structures. Any flap failures and cerebrospinal fluid leak would lead to high morbidity and mortality. The expected workload may initially be high, and it needs a dedicated unit to deliver high standard of medical, nursing, and palliative care to both pediatric and adult patients.

REFERENCES 1. Ketcham AS, Wilkins RH, van Buren JM, et al. A combined intracranial facial approach to the paranasal sinuses. Am J Surg 1963;106:698–703 2. Shah JP, Sundaresan N, Galicich J, et al. Craniofacial resections for tumours involving the base of the skull. Am J Surg 1987;154:352–358 3. Shah JP. The skull base. In: Shah JP. Head and Neck Surgery. London, UK: Mosby-Wolfe, 1996:85–141 4. Ketcham AS, van Buren JM. Tumours of the paranasal sinuses: a therapeutic challenge. Am J Surg 1985;150:406–413 5. Jackson IT, Webster HR. Craniofacial tumours. Clin Plast Surg 1994;21:633–648 6. Janecka IP, Sen C, Sekhar LN, et al. Cranial base surgery: results in 183 patients. Otolaryngol Head Neck Surg 1994;110:539–546 7. Lund VJ, Howard DJ, Wei WI, et al. Craniofacial resection for tumours of the nasal cavity and paranasal sinuses: a 17-year experience. Head Neck 1998;20:97–105 8. Kraus DH, Shah JP, Arbit E, et al. Complications of craniofacial resection for tumours involving the anterior skull base. Head Neck 1994;16:307–312 9. Donald PJ. Complications in skull base surgery for malignancy. Laryngoscope 1999;109:1959–1966 10. Jackson IT, Adham MN, Marsh WR. Use of galealfrontalis myofascial flap in craniofacial surgery. Plast Reconstr Surg 1986;77:905–910 11. Mallath MK, Taylor DG, Badwe RA, et al. Cancer burden and health systems in India 1: the growing burden of cancer in India: epidemiology and social context. Lancet 2014;15:e205–e212 12. Califano J, Cordeiro PG, Disa JJ, et al. Anterior cranial base reconstruction using free tissue transfer: changing trends. Head Neck 2003;25:89–96 13. Teknos TN, Smith JC, Day TA, et al. Microvascular free tissue transfer in reconstructing skull base defects: lessons learned. Laryngoscope 2002;112:1871–1876 14. Neligan PC, Mulholland S, Irish J, et al. Flap selection in cranial base reconstruction. Plast Reconstr Surg 1996;98:1159–1168 15. Jaju H. Unfavourable results in skull base surgery. Indian J Plast Surg 2013;46:239–246

© 2014 Mutaz B. Habal, MD

Copyright © 2014 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.

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Skull base surgery: Indian perspective.

Skull base tumors occur at an interface of multiple surgical specialties, which need to work in tandem to treat these cases. The number of estimated n...
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