The Journal of Craniofacial Surgery • Volume 26, Number 1, January 2015
FIGURE 2. Aneurysmal bone cyst on the proximal segment of SSRO.
the first 2 decades similar to the case reported in this article. Usually, no sex predilection is found. The mandible is more prone to being affected, with incident rates of 3:1 to 11:9. More than 90% of jaw ABCs occur in the posterior regions of the jaws.4 In the present case, the lesion involved the mandibular condyle extending to involve the coronoid, sigmoid notch, and the ramal region. It is the 11th reported case of the ABC involving mandibular condyle in existing literature. The ABC in the jaw region can have considerably varied clinical features ranging from asymptomatic lesions occasionally discovered as radiolucencies on routine radiography to sometimes expansive and destructive patterns. The main symptom according to the published data is a painless swelling. Varying degree of limited mouth opening can occur if the lesion involves mandibular condyle or ramus. In our case, both painless swelling and restricted range of mandibular motion in all directions were present. Aneurysmal bone cyst frequently appears as a well-circumscribed bloated multilocular lesion on the radiograph. In the reported case, all the typical radiographic features of ABC were present. The accepted treatment of ABC is surgical resection as high rate of recurrence is seen with enucleation.5 The access to the condylar region is possible through transparotid or anteroparotid transmasseteric, preauricular, Risdon, or submandibular and intraoral approach. The combination of preauricular and submandibular incision is most commonly used for condylar pathology resection and reconstruction. In our case, this approach was not used because the patient denied consent for any cutaneous approach for the resection of the lesion. Intraoral resection of the lesion without any reconstruction was the only available choice. Sagittal split ramus osteotomy has been described in the literature for approaches to mandibular cysts,6 myxomas,7 keratocyst odontogenic tumor, odontomas,8,9 and deeply impacted third molars.10 The procedure is simple and leads to increased accessibility to the lesion without sacrificing bone and causing damage to inferior alveolar nerve. Because of the overlap of proximal and distal segment after SSRO, the union/healing of the osteotomized segment is better if adequate osteosynthesis is used. In our case, the lesion was above the lingula, and adequate bone was present for SSRO. The advantage other than of carrying out resection of the pathology intraorally and avoiding any extraoral scar was that even without reconstruction, enough height of the mandible was preserved lingually, and the uninvolved buccal cortex was refixed using titanium miniplate and screws. The drawback is limited access to dissect out soft tissue attachments from the condylar head and removal of the thinned-out lesion in toto. Aneurysmal bone cyst was excised in toto, and no clinical or radiological recurrence was noticed after 3 years of follow-up. The approach is technique sensitive but can be used to address condylar pathologies, avoiding any facial scar or nerve damage.
REFERENCES 1. Zadik Y, Aktaş A, Drucker S, et al. Aneurysmal bone cyst of mandibular condyle: a case report and review of the literature. J Craniomaxillofac Surg 2012;40:e243–e248
Brief Clinical Studies
2. Cottalorda J, Bourelle S. Modern concepts of primary aneurysmal bone cyst. Arch Orthop Trauma Surg 2007;127:105–114 3. Kumar VV, Malik NA, Kumar DB. Treatment of large recurrent aneurysmal bone cysts of mandible: transosseous intralesional embolization as an adjunct to resection. Int J Oral Maxillofac Surg 2009;38:671–676 4. Sun ZJ, Zhao YF, Yang RL, et al. Aneurysmal bone cysts of the jaws: analysis of 17 cases. J Oral Maxillofac Surg 2010;68:2122–2128 5. Choi BJ, Choi SC, Kwon YD, et al. Aneurysmal bone cyst causing a pathologic fracture of the mandibular condyle. J Oral Maxillofac Surg 2011;69:2995–3000 6. Rittersma J, van Gool AV. Surgical access to multicystic lesions, by sagittal splitting of the lower jaw. J Maxillofac Surg 1979;7:246–250 7. Wong GB. Large odontogenic myxoma of the mandible treated by sagittal ramus osteotomy and peripheral ostectomy. J Oral Maxillofac Surg 1992;50:1221–1224 8. Casap N, Zeltser R, Abu-Tair J, et al. Removal of odontoma by sagittal split osteotomy. J Oral Maxillofac Surg 2006;64:1833–1836 9. Blinder D, Peleg M, Taicher S. Surgical considerations in cases of large mandibular odontomas located in the mandibular angle. Int J Oral Maxillofac Surg 1993;22:163–165 10. Toffanin A, Zupi A, Cicognini A. Sagittal split osteotomy in removal of impacted third molar. J Oral Maxillofac Surg 2003;61:638–640
Simultaneous Transsphenoidal and Transventricular Endoscopic Approaches for Giant Pituitary Adenoma With Hydrocephalus Ender Koktekir, MD,* Hakan Karabagli, MD,* Kayhan Ozturk, MD† Abstract: The surgical management of giant pituitary adenomas is challenging. Although most pituitary adenomas, even those with suprasellar extension, can be resected using the transsphenoidal surgery alone, the transcranial approach is still needed for approximately 1% to 4% of these tumors. The transcranial approach is usually used in large adenomas with hourglass configuration and adenomas with firm consistency impeding the adjunctive measures, which are used for delivering the suprasellar part of the tumor into the sellar area and thereby obscure the tumor resection by transsphenoidal route. In this report, we describe the successful use of transventricular endoscope as an adjunctive measure to remove giant pituitary adenoma from transsphenoidal route and discuss the limitations of this new technique. We concluded that this technique would be used safely in selected cases. Case selection and surgical strategies should be based on preoperative magnetic resonance imaging findings, ventricular size, and the availability of experienced surgeons. From the Departments of *Neurosurgery and †Otolaryngology, Selcuk University, Konya, Turkey. Received July 5, 2014. Accepted for publication August 19, 2014. Address correspondence and reprint requests to Ender Koktekir, MD, Department of Neurosurgery, Selcuk Universitesi Tip Fakultesi Neurosirurji Anabilim Dali, Alaeddin Keykubat Kampusu 42050, Konya, Turkey; E-mail: [email protected] The authors report no conflicts of interest. Copyright © 2014 by Mutaz B. Habal, MD ISSN: 1049-2275 DOI: 10.1097/SCS.0000000000001298
© 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 26, Number 1, January 2015
Brief Clinical Studies
Key Words: Hydrocephalus, pituitary adenoma, neuroendoscopy, giant, transsphenoidal approach
T
he endoscopic neurosurgical approaches to the intracranial lesions have evolved greatly during the last 2 decades.1–3 Recently, the transsphenoidal endoscopic approach to the pituitary adenomas has gained popularity because of its minimal invasiveness and ability to demonstrate the “corner of the tumor.” Especially with the aid of adjunctive measures such as jugular venous compression, the Valsalva maneuver, and the injection of air or saline through a lumbar catheter, most pituitary adenomas—even those with suprasellar extension—can be resected using this approach.2,4 However, the surgical management of giant pituitary adenomas that have firm consistency and an hourglass configuration may still present with considerable difficulties. Moreover, combined or staged transcranial and transsphenoidal approaches are still needed for the resection of this kind of tumor.4–7 The successful use of a transventricular endoscope for delivery to the suprasellar part of the pituitary adenoma into the sellar region has been reported in a few case reports.6,8–11 In the present study, we discuss the limitations of this new technique and describe the subtotal resection of a giant invasive suprasellar pituitary adenoma with a simultaneous endoscopic transventricular and transsphenoidal approach.
CLINICAL REPORT A 56-year-old woman was admitted to the emergency department with a complaint of sudden-onset loss of consciousness. She had a history of headache and progressive vision loss in both eyes for 3 months. On admission, her Glasgow Coma Scale score was 12 (E3V4M5). Although she had no focal deficits, visual fields and acuity could not be evaluated because of the level of consciousness. Contrast-enhanced cranial magnetic resonance imaging (MRI) revealed a sellar, homogenously enhancing lesion (48 46 32 mm) with necrotic areas and significant suprasellar extension causing obstructive acute hydrocephalus (Fig. 1). On T2-weighted MRI, the tumor was found to be isointense-hyperintense to gray matter (Fig. 2). The radiologic features were consistent with a giant pituitary adenoma with both soft and firm consistency. Basal pituitary function evaluation and laboratory testing were normal except for hypocortisolism and hypothyroidism. Hydrocortisone and thyroxine replacement were administered. Because of the patient's neurologic status, she was operated on immediately. On the basis of the preoperative imaging findings, we decided to use the combined endoscopic transventricular and transsphenoidal approaches. During the procedure, intratumoral bleeding occurred, and an intraventricular catheter was placed into the frontal horn of the right lateral ventricle for 5 days. The patient's Glasgow Coma Scale rose to 15 immediately after the operation, but her visual acuity was 2/10 in the right eye and 6/10 in the left eye. The patient was discharged on the 10th postoperative day without any signs of hydrocephalus. Histopathologic examination revealed that the lesion was an invasive pituitary adenoma. Six-month and 1-year follow-
FIGURE 1. Preoperative coronal contrasted T1-weighted MRI showed the homogenously enhanced pituitary adenoma with hydrocephalus.
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FIGURE 2. Preoperative axial T2-weighted MRI showed the isointense-hyperintense lesion.
ups confirmed the neurologic stabilization of the patient. A control MRI showed the subtotal resection of the tumor (Fig. 3).
Surgical Procedure Two surgical teams were involved in the operation and worked simultaneously. Each of the teams has its own scrub nurses, sets of instrumentation, and monitors. The first surgical team was composed of an ear-nose-throat (ENT) surgeon and a neurosurgeon to complete the transsphenoidal stage. The other surgical team was composed of 1 neurosurgeon who was responsible for the transventricular approach. The patient was placed in the supine position with 3-point head fixation. The head was flexed 15 degrees with a neutral position. The transsphenoidal team stood on the right side of the patient, whereas the transcranial surgeon was seated at the head of the operating table (Fig. 4). The ENT surgeon initiated the endoscopic transsphenoidal approach. After creating the nasoseptal flap, the floor of the sella was exposed. Then, the neurosurgeon joined the operation, and the thin sellar floor was opened. After the opening of the dura, the tumor was removed from below. A soft tumor with hemorrhagic components was suctioned out. Because of the failure of the Valsalva maneuver and to avoid the excessive traction of the tumor from below, the transventricular approach was initiated at this stage through the right frontal burr hole (just anterior to the coronal suture) that had been created previously by the neurosurgeon. The ventricle was punctured with an endoscope cannula, and under endoscopic visualization, it was attempted to push the tumor to the sellar area from above with blunt dissection and a Fogarty catheter to the sellar area (Fig. 5). We thought that most of the tumor had dropped to the sellar area because we were able to identify the light source of the transsphenoidal endoscope. However, because of the soft nature of the tumor and intratumoral bleeding, the left lateral margins of the tumor could not be pushed. The removal of the tumor was halted at this stage because of poor endoscopic visualization. The ventricular catheter was placed at the frontal horn of the right lateral ventricle, and the transsphenoidal approach was finalized with a nasoseptal flap.
DISCUSSION Although most pituitary adenomas are amenable to transsphenoidal excision, the surgical management of the giant adenomas is challenging. Single-stage operation with combined approaches or multistaged operations have been suggested.4,5,8 Some authors advocate a 2-staged transsphenoidal approach or transcranial approach to remove the suprasellar part after the removal of intrasellar component by the transsphenoidal approach.4,12 However, previous studies have shown that
FIGURE 3. Postoperative coronal contrasted T1-weighted MRI showed the subtotal resection of tumor.
© 2014 Mutaz B. Habal, MD
Copyright © 2014 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
The Journal of Craniofacial Surgery • Volume 26, Number 1, January 2015
FIGURE 4. A schematic diagram illustrating the setting in the operating theatre. TV, transventricular; TS, transsphenoidal; SC, scrub nurse.
operative mortality rates of giant pituitary adenomas can be as high as 14% for the transsphenoidal approach and 25% for the transcranial approach.5 For these reasons, some authors have suggested using the single-stage combined approach to perform total tumor removal as much as possible because the residual tumors may contribute to the critical postoperative bleeding, edema, and increased mass effect.5,9,13 The use of endoscopy in neurosurgery has greatly increased in the last 2 decades. The endonasal route provides the access and treatment of wide spectrum of skull base pathology located from the frontal sinuses to the level of the odontoid process.1,2,13 However, its use in intracranial surgery is limited with the treatment of hydrocephalus and as an assisting instrument in microscopic surgery. The transventricular endoscopic approach to the giant pituitary adenoma in conjunction with the transsphenoidal approach is a newer method and has been already described in a few case reports.5,6,9–11 The key point of these reports was the ability of the transventricular endoscope to push the suprasellar part of the tumor, which is not accessible with traditional techniques, and drop it into the intrasellar region because of the firm consistency of the tumor. However, the feasibility of this technique for soft tumors has not been elucidated. The MRI is the only diagnosis modality that can give information on tumor consistency in pituitary adenomas. Soft tumors are usually seen as hypointense on T1-weighted MRI and as hyperintense on T2-weighted MRI. Moreover, the softness of the tumor correlates to a high signal on T2-weighted MRI.9,11 In our case, the tumor had both hyperintense and isointense signals on T2-weighted MRI. Our operation experience was also consistent with the MRI findings. Because of the firm consistency, the suprasellar part was not descending into the sella even with the aid of the Valsalva maneuver. During the operation, we used the intraventricular endoscope to push the suprasellar part into the sellar area. However, because of the soft
Brief Clinical Studies
consistency and intratumoral bleeding, poor endoscopic visualization occurred with the attempt at tumor dissection around the structures. The major advantages of combined approaches are the ability to remove the hidden tumors that cannot be reached using 1 approach and the manipulation of the tumor from 2 directions simultaneously.4–6,11 Different combined surgical options, including microscopic transsphenoidal and transcranial techniques, endoscopic transsphenoidal and microscopic transcranial techniques, microscopic transsphenoidal and endoscopic transventricular techniques, and fully endoscopic transventricular and transsphenoidal techniques, have been described.4,6,10,13,14 The major disadvantage of simultaneous surgery is the necessity of 2 surgical teams and 2 operating fields. In simultaneous microscopic approaches, 2 microscopes and 1 fluoroscope are also required, which in turn means that a very large operating room is necessary. Although the use of an endoscope in the transsphenoidal stage decreases some of these requirements, the head position used for the pterional approach is not suitable for the transsphenoidal stage. Our patient was operated via a fully endoscopic transventricular and transsphenoidal approach. During the operation, we did not need to adjust the head position, and the operating room was comfortable for both surgical teams, permitting freer movement. On the other hand, it should be emphasized that the limitations of transventricular endoscopy included the requirement of ventricle enlargement to increase the working space, the need for an experienced surgeon, poor visualization, and the lack of ability to achieve adequate hemostasis if bleeding occurred. There are 2 case reports in the literature about the results of the fully endoscopic simultaneous transcranial and transsphenoidal approaches on giant pituitary adenomas. Greenfield et al14 and Romano et al11 both used this technique. In both cases, gross total removal of the tumor was achieved, resulting in neurologic improvement. The authors emphasized the possibility of a transventricular endoscope as an assistant instrument to drop the suprasellar part of tumors with firm consistency into the sellar area if traditional techniques have failed. In the present case, we also preferred the simultaneous fully endoscopic transventricular and transsphenoidal approaches because the patient had hydrocephalus and we thought that the suprasellar part of the tumor was not amenable to resection using only the transsphenoidal approach. Although we could not achieve gross total removal because of intratumoral bleeding and tumor morphology (serious lateral extension and soft consistency), our patient's neurologic status improved significantly after surgery, and long-term follow-up was uneventful. However, we cannot determine the effect of our surgery on visual acuity because the patient's preoperative visual status could not be evaluated.
CONCLUSIONS The use of a transventricular endoscope to deliver the suprasellar part of the pituitary adenoma into the sellar area seems to be a reasonable technique when traditional measures fail. Although its usage in firm consistency tumors has been reported as safe and effective, this alternative option should be used with care in pituitary adenomas, and the case selection should be based on preoperative MRI findings, ventricular size, and the availability of experienced surgeons. Although our case suggested that this approach could also be used in pituitary adenomas with soft consistency, we emphasize that the goal of the surgery in this kind of tumor should not be gross total removal because of the possibility of intraoperative complications. FIGURE 5. A–D, Intraventricular stage. E and F, Transsphenoidal stage. A, Intraventricular view of the tumor capsule. B, View of the tumor in the third ventricle after partial resection. C, Arrow indicating the light of the endoscope in the sella. D, View of the lateral ventricle after subtotal resection of the tumor. E, View of the tumor in the sella. F, Arrow indicating the light of the endoscope in the suprasellar area.
REFERENCES 1. Cappabianca P, Cavallo LM, Esposito F, et al. Extended endoscopic endonasal approach to the midline skull base: the evolving role of transsphenoidal surgery. Adv Tech Stand Neurosurg 2008;33:151–159
© 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 26, Number 1, January 2015
2. Cavallo LM, Prevedello DM, Solari D, et al. Extended endoscopic endonasal transsphenoidal approach for residual or recurrent craniopharyngiomas. J Neurosurg 2009;111:578–589 3. Zada G, Laws ER Jr. Simultaneous transsphenoidal and intraventricular endoscopic approaches for macroadenoma with extensive suprasellar extension: surgery from below, above or both? World Neurosurg 2010;74:109–110 4. Alleyne CH Jr, Barrow DL, Oyesiku NM. Combined transsphenoidal and pterional craniotomy approach to giant pituitary tumors. Surg Neurol 2002;57:380–390 5. Leung GK, Law HY, Hung KN, et al. Combined simultaneous transcranial and transsphenoidal resection of large-to-giant pituitary adenomas. Acta Neurochir 2011;153:1401–1408 6. Ojha BK, Husain M, Rastogi M, et al. Combined trans-sphenoidal and simultaneous trans-ventricular-endoscopic decompression of a giant pituitary adenoma: case report. Acta Neurochir (Wien) 2009;151:843–847 7. Youseff AS, Agazzi S, van Loveren HR. Transcranial surgery for pituitary adenomas. Neurosurgery 2005;57:168–175 8. D'Haens J, Van Rompaey K, Stadnik T, et al. Fully endoscopic transsphenoidal surgery for functioning pituitary adenomas: a retrospective comparison with traditional transsphenoidal microsurgery in the same institution. Surg Neurol 2009; 72:336–340 9. Leung GK, Yuen MM, Chow WS, et al. An endoscopic modification of the simultaneous “above and below” approach to large pituitary adenoma. Pituitary 2012;15:237–241 10. Nishioka H, Hara T, Usuf M, et al. Simultaneous combined supra-infrasellar approach for giant/large multilobulated pituitary adenomas. World Neurosurg 2012;77:533–539 11. Romano A, Chibbarao S, Marsella M, et al. Combined endoscopic transsphenoidal-transventricular approach for resection of a giant pituitary macroadenoma. World Neurosurg 2010;74:161–164 12. Abe T, Iwata T, Kawamura N, et al. Staged transsphenoidal surgery for fibrous nonfunctioning pituitary adenomas with suprasellar extension. Neurol Med Chir (Tokyo) 1997;37:830–837 13. D'Ambrosio AL, Syed ON, Grobelny BT, et al. Simultaneous above and below approach to giant pituitary adenomas: surgical strategies and long term follow up. Pituitary 2009;12:217–225 14. Greenfield JP, Leng LZ, Chaudhry U, et al. Combined simultaneous endoscopic transsphenoidal and endoscopic transventricular resection of a giant pituitary macroadenoma. Minim Invasive Neurosurg 2008;51:306–309
Dandy-Walker Syndrome With Severe Velopharyngeal Dysfunction: A Contraindication for Le Fort I Surgery? Kamil H. Nelke, DMD,*† Wojciech Pawlak, DMD, PhD,† Hanna Gerber, MD, DMD† Abstract: Dandy-Walker syndrome is a rare congenital brain deformation. Most symptoms are related with fourth ventricle and skull base malformations. Quite often, symptoms develop from infancy or progress rapidly. Cerebellar dysfunction, lack of muscle coordination, and skull deformities involving eye movement might be present. There are several Dandy-Walker syndrome complex types. We present a 23-year-old patient who had a severe dentofacial deformity with mandibular prognathism and extremely undeveloped maxillary bone resulting in palatopharyngeal and velopharyngeal dysfunction with complete lack of soft palate function resulting in
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increased speech tone and volume. Performing Le Fort I osteotomy in this case is greatly controversial and might result in even greater loss of function or even its total lack. Velopharyngeal complex is very important, and every surgeon should consider its value while planning Le Fort I osteotomies. Key Words: Dandy-Walker syndrome, orthognathic surgery, Le Fort I osteotomy, palatopharyngeal dysfunction, velopharyngeal complex
O
rthognathic surgery is a surgery of choice in many dentofacial deformities. Some cases consisted of maxillary movement and soft palate involvement, and pronunciation and sound volume might be changed. In addition, a great impact on the upper aerodigestive tract is well-known, and it is especially involved in the improvement of upper respiratory tract function and prophylaxis of sleep apnea. Yet, so far, changes in the velopharyngeal complex and future outcomes of patients are still not yet fully known and understood; however, many studies are focused mostly on patients with cleft. Problems related to hypernasality and increased turbulence are well known.1 Dandy-Walker syndrome (DWS) is a complex syndrome related mostly with brain and skull deformities, which also quite often consisted of other features, such as facial deformity, inappropriate eye movement (nystagmus), deficiency in axial musculature movement, or others.2 Skull cysts are also common. In severe forms, distinctive skeletal changes along with bony dysplasias can be found. Occurrence rate is approximately 1:30,000 newborns.3 It is important to know that DWS is a type of a disease described as ciliopathies. Le Fort I osteotomy has an effect on hypernasality, articulation, voice, and nasal emission. Movement and relation between soft palate and the surrounding muscles depending on the dentofacial deformity might change greatly. Maxillary advancement is a surgery of choice in patients with maxillary deficiency that is mostly performed in young adults.
DANDY-WALKER SYNDROME AND LE FORT I OSTEOTOMY A 23-year-old patient who had DWS with an underdeveloped middle part of facial skeleton that consisted of extremely wide and narrowed maxillary bone with impacted and underdeveloped teeth as well as increased mandibular prognathism was admitted to the department (Figs. 1, 2). Dandy-Walker syndrome findings in this patient consisted of elongated facial profile, hypertelorismus with widely seated nose with a nasal hump. After previous cranial surgeries, brain function was sustained and both psychosomatic and intelligence levels were normal. The patient was referred to improve From the Departments of *Human Anatomy, and †Maxillofacial Surgery, Wroclaw Medical University, Wroclaw, Poland. Received May 30, 2014. Accepted for publication August 19, 2014. Address correspondence and reprint requests to Kamil H. Nelke, DMD, Department of Maxillofacial Surgery, Uniwersytecki Szpital Kliniczny, Klinka Chirurgii Szczękowo-Twarzowej, ulica Borowska 213, 50–556 Wrocław, Uniwersytet Medyczny we Wrocławiu, Wroclaw, Poland; E-mail: [email protected] The authors report no conflicts of interest. Ethics Statement: This study required no approval from an institutional committee because it is not related directly with human care and treatment of animals used in this study. The current study is fully adapted to and corresponding with the Helsinki Declaration. Copyright © 2014 by Mutaz B. Habal, MD ISSN: 1049-2275 DOI: 10.1097/SCS.0000000000001302
© 2014 Mutaz B. Habal, MD
Copyright © 2014 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
FIGURE 2. Aneurysmal bone cyst on the proximal segment of SSRO.
the first 2 decades similar to the case reported in this article. Usually, no sex predilection is found. The mandible is more prone to being affected, with incident rates of 3:1 to 11:9. More than 90% of jaw ABCs occur in the posterior regions of the jaws.4 In the present case, the lesion involved the mandibular condyle extending to involve the coronoid, sigmoid notch, and the ramal region. It is the 11th reported case of the ABC involving mandibular condyle in existing literature. The ABC in the jaw region can have considerably varied clinical features ranging from asymptomatic lesions occasionally discovered as radiolucencies on routine radiography to sometimes expansive and destructive patterns. The main symptom according to the published data is a painless swelling. Varying degree of limited mouth opening can occur if the lesion involves mandibular condyle or ramus. In our case, both painless swelling and restricted range of mandibular motion in all directions were present. Aneurysmal bone cyst frequently appears as a well-circumscribed bloated multilocular lesion on the radiograph. In the reported case, all the typical radiographic features of ABC were present. The accepted treatment of ABC is surgical resection as high rate of recurrence is seen with enucleation.5 The access to the condylar region is possible through transparotid or anteroparotid transmasseteric, preauricular, Risdon, or submandibular and intraoral approach. The combination of preauricular and submandibular incision is most commonly used for condylar pathology resection and reconstruction. In our case, this approach was not used because the patient denied consent for any cutaneous approach for the resection of the lesion. Intraoral resection of the lesion without any reconstruction was the only available choice. Sagittal split ramus osteotomy has been described in the literature for approaches to mandibular cysts,6 myxomas,7 keratocyst odontogenic tumor, odontomas,8,9 and deeply impacted third molars.10 The procedure is simple and leads to increased accessibility to the lesion without sacrificing bone and causing damage to inferior alveolar nerve. Because of the overlap of proximal and distal segment after SSRO, the union/healing of the osteotomized segment is better if adequate osteosynthesis is used. In our case, the lesion was above the lingula, and adequate bone was present for SSRO. The advantage other than of carrying out resection of the pathology intraorally and avoiding any extraoral scar was that even without reconstruction, enough height of the mandible was preserved lingually, and the uninvolved buccal cortex was refixed using titanium miniplate and screws. The drawback is limited access to dissect out soft tissue attachments from the condylar head and removal of the thinned-out lesion in toto. Aneurysmal bone cyst was excised in toto, and no clinical or radiological recurrence was noticed after 3 years of follow-up. The approach is technique sensitive but can be used to address condylar pathologies, avoiding any facial scar or nerve damage.
REFERENCES 1. Zadik Y, Aktaş A, Drucker S, et al. Aneurysmal bone cyst of mandibular condyle: a case report and review of the literature. J Craniomaxillofac Surg 2012;40:e243–e248
Brief Clinical Studies
2. Cottalorda J, Bourelle S. Modern concepts of primary aneurysmal bone cyst. Arch Orthop Trauma Surg 2007;127:105–114 3. Kumar VV, Malik NA, Kumar DB. Treatment of large recurrent aneurysmal bone cysts of mandible: transosseous intralesional embolization as an adjunct to resection. Int J Oral Maxillofac Surg 2009;38:671–676 4. Sun ZJ, Zhao YF, Yang RL, et al. Aneurysmal bone cysts of the jaws: analysis of 17 cases. J Oral Maxillofac Surg 2010;68:2122–2128 5. Choi BJ, Choi SC, Kwon YD, et al. Aneurysmal bone cyst causing a pathologic fracture of the mandibular condyle. J Oral Maxillofac Surg 2011;69:2995–3000 6. Rittersma J, van Gool AV. Surgical access to multicystic lesions, by sagittal splitting of the lower jaw. J Maxillofac Surg 1979;7:246–250 7. Wong GB. Large odontogenic myxoma of the mandible treated by sagittal ramus osteotomy and peripheral ostectomy. J Oral Maxillofac Surg 1992;50:1221–1224 8. Casap N, Zeltser R, Abu-Tair J, et al. Removal of odontoma by sagittal split osteotomy. J Oral Maxillofac Surg 2006;64:1833–1836 9. Blinder D, Peleg M, Taicher S. Surgical considerations in cases of large mandibular odontomas located in the mandibular angle. Int J Oral Maxillofac Surg 1993;22:163–165 10. Toffanin A, Zupi A, Cicognini A. Sagittal split osteotomy in removal of impacted third molar. J Oral Maxillofac Surg 2003;61:638–640
Simultaneous Transsphenoidal and Transventricular Endoscopic Approaches for Giant Pituitary Adenoma With Hydrocephalus Ender Koktekir, MD,* Hakan Karabagli, MD,* Kayhan Ozturk, MD† Abstract: The surgical management of giant pituitary adenomas is challenging. Although most pituitary adenomas, even those with suprasellar extension, can be resected using the transsphenoidal surgery alone, the transcranial approach is still needed for approximately 1% to 4% of these tumors. The transcranial approach is usually used in large adenomas with hourglass configuration and adenomas with firm consistency impeding the adjunctive measures, which are used for delivering the suprasellar part of the tumor into the sellar area and thereby obscure the tumor resection by transsphenoidal route. In this report, we describe the successful use of transventricular endoscope as an adjunctive measure to remove giant pituitary adenoma from transsphenoidal route and discuss the limitations of this new technique. We concluded that this technique would be used safely in selected cases. Case selection and surgical strategies should be based on preoperative magnetic resonance imaging findings, ventricular size, and the availability of experienced surgeons. From the Departments of *Neurosurgery and †Otolaryngology, Selcuk University, Konya, Turkey. Received July 5, 2014. Accepted for publication August 19, 2014. Address correspondence and reprint requests to Ender Koktekir, MD, Department of Neurosurgery, Selcuk Universitesi Tip Fakultesi Neurosirurji Anabilim Dali, Alaeddin Keykubat Kampusu 42050, Konya, Turkey; E-mail: [email protected] The authors report no conflicts of interest. Copyright © 2014 by Mutaz B. Habal, MD ISSN: 1049-2275 DOI: 10.1097/SCS.0000000000001298
© 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 26, Number 1, January 2015
Brief Clinical Studies
Key Words: Hydrocephalus, pituitary adenoma, neuroendoscopy, giant, transsphenoidal approach
T
he endoscopic neurosurgical approaches to the intracranial lesions have evolved greatly during the last 2 decades.1–3 Recently, the transsphenoidal endoscopic approach to the pituitary adenomas has gained popularity because of its minimal invasiveness and ability to demonstrate the “corner of the tumor.” Especially with the aid of adjunctive measures such as jugular venous compression, the Valsalva maneuver, and the injection of air or saline through a lumbar catheter, most pituitary adenomas—even those with suprasellar extension—can be resected using this approach.2,4 However, the surgical management of giant pituitary adenomas that have firm consistency and an hourglass configuration may still present with considerable difficulties. Moreover, combined or staged transcranial and transsphenoidal approaches are still needed for the resection of this kind of tumor.4–7 The successful use of a transventricular endoscope for delivery to the suprasellar part of the pituitary adenoma into the sellar region has been reported in a few case reports.6,8–11 In the present study, we discuss the limitations of this new technique and describe the subtotal resection of a giant invasive suprasellar pituitary adenoma with a simultaneous endoscopic transventricular and transsphenoidal approach.
CLINICAL REPORT A 56-year-old woman was admitted to the emergency department with a complaint of sudden-onset loss of consciousness. She had a history of headache and progressive vision loss in both eyes for 3 months. On admission, her Glasgow Coma Scale score was 12 (E3V4M5). Although she had no focal deficits, visual fields and acuity could not be evaluated because of the level of consciousness. Contrast-enhanced cranial magnetic resonance imaging (MRI) revealed a sellar, homogenously enhancing lesion (48 46 32 mm) with necrotic areas and significant suprasellar extension causing obstructive acute hydrocephalus (Fig. 1). On T2-weighted MRI, the tumor was found to be isointense-hyperintense to gray matter (Fig. 2). The radiologic features were consistent with a giant pituitary adenoma with both soft and firm consistency. Basal pituitary function evaluation and laboratory testing were normal except for hypocortisolism and hypothyroidism. Hydrocortisone and thyroxine replacement were administered. Because of the patient's neurologic status, she was operated on immediately. On the basis of the preoperative imaging findings, we decided to use the combined endoscopic transventricular and transsphenoidal approaches. During the procedure, intratumoral bleeding occurred, and an intraventricular catheter was placed into the frontal horn of the right lateral ventricle for 5 days. The patient's Glasgow Coma Scale rose to 15 immediately after the operation, but her visual acuity was 2/10 in the right eye and 6/10 in the left eye. The patient was discharged on the 10th postoperative day without any signs of hydrocephalus. Histopathologic examination revealed that the lesion was an invasive pituitary adenoma. Six-month and 1-year follow-
FIGURE 1. Preoperative coronal contrasted T1-weighted MRI showed the homogenously enhanced pituitary adenoma with hydrocephalus.
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FIGURE 2. Preoperative axial T2-weighted MRI showed the isointense-hyperintense lesion.
ups confirmed the neurologic stabilization of the patient. A control MRI showed the subtotal resection of the tumor (Fig. 3).
Surgical Procedure Two surgical teams were involved in the operation and worked simultaneously. Each of the teams has its own scrub nurses, sets of instrumentation, and monitors. The first surgical team was composed of an ear-nose-throat (ENT) surgeon and a neurosurgeon to complete the transsphenoidal stage. The other surgical team was composed of 1 neurosurgeon who was responsible for the transventricular approach. The patient was placed in the supine position with 3-point head fixation. The head was flexed 15 degrees with a neutral position. The transsphenoidal team stood on the right side of the patient, whereas the transcranial surgeon was seated at the head of the operating table (Fig. 4). The ENT surgeon initiated the endoscopic transsphenoidal approach. After creating the nasoseptal flap, the floor of the sella was exposed. Then, the neurosurgeon joined the operation, and the thin sellar floor was opened. After the opening of the dura, the tumor was removed from below. A soft tumor with hemorrhagic components was suctioned out. Because of the failure of the Valsalva maneuver and to avoid the excessive traction of the tumor from below, the transventricular approach was initiated at this stage through the right frontal burr hole (just anterior to the coronal suture) that had been created previously by the neurosurgeon. The ventricle was punctured with an endoscope cannula, and under endoscopic visualization, it was attempted to push the tumor to the sellar area from above with blunt dissection and a Fogarty catheter to the sellar area (Fig. 5). We thought that most of the tumor had dropped to the sellar area because we were able to identify the light source of the transsphenoidal endoscope. However, because of the soft nature of the tumor and intratumoral bleeding, the left lateral margins of the tumor could not be pushed. The removal of the tumor was halted at this stage because of poor endoscopic visualization. The ventricular catheter was placed at the frontal horn of the right lateral ventricle, and the transsphenoidal approach was finalized with a nasoseptal flap.
DISCUSSION Although most pituitary adenomas are amenable to transsphenoidal excision, the surgical management of the giant adenomas is challenging. Single-stage operation with combined approaches or multistaged operations have been suggested.4,5,8 Some authors advocate a 2-staged transsphenoidal approach or transcranial approach to remove the suprasellar part after the removal of intrasellar component by the transsphenoidal approach.4,12 However, previous studies have shown that
FIGURE 3. Postoperative coronal contrasted T1-weighted MRI showed the subtotal resection of tumor.
© 2014 Mutaz B. Habal, MD
Copyright © 2014 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
The Journal of Craniofacial Surgery • Volume 26, Number 1, January 2015
FIGURE 4. A schematic diagram illustrating the setting in the operating theatre. TV, transventricular; TS, transsphenoidal; SC, scrub nurse.
operative mortality rates of giant pituitary adenomas can be as high as 14% for the transsphenoidal approach and 25% for the transcranial approach.5 For these reasons, some authors have suggested using the single-stage combined approach to perform total tumor removal as much as possible because the residual tumors may contribute to the critical postoperative bleeding, edema, and increased mass effect.5,9,13 The use of endoscopy in neurosurgery has greatly increased in the last 2 decades. The endonasal route provides the access and treatment of wide spectrum of skull base pathology located from the frontal sinuses to the level of the odontoid process.1,2,13 However, its use in intracranial surgery is limited with the treatment of hydrocephalus and as an assisting instrument in microscopic surgery. The transventricular endoscopic approach to the giant pituitary adenoma in conjunction with the transsphenoidal approach is a newer method and has been already described in a few case reports.5,6,9–11 The key point of these reports was the ability of the transventricular endoscope to push the suprasellar part of the tumor, which is not accessible with traditional techniques, and drop it into the intrasellar region because of the firm consistency of the tumor. However, the feasibility of this technique for soft tumors has not been elucidated. The MRI is the only diagnosis modality that can give information on tumor consistency in pituitary adenomas. Soft tumors are usually seen as hypointense on T1-weighted MRI and as hyperintense on T2-weighted MRI. Moreover, the softness of the tumor correlates to a high signal on T2-weighted MRI.9,11 In our case, the tumor had both hyperintense and isointense signals on T2-weighted MRI. Our operation experience was also consistent with the MRI findings. Because of the firm consistency, the suprasellar part was not descending into the sella even with the aid of the Valsalva maneuver. During the operation, we used the intraventricular endoscope to push the suprasellar part into the sellar area. However, because of the soft
Brief Clinical Studies
consistency and intratumoral bleeding, poor endoscopic visualization occurred with the attempt at tumor dissection around the structures. The major advantages of combined approaches are the ability to remove the hidden tumors that cannot be reached using 1 approach and the manipulation of the tumor from 2 directions simultaneously.4–6,11 Different combined surgical options, including microscopic transsphenoidal and transcranial techniques, endoscopic transsphenoidal and microscopic transcranial techniques, microscopic transsphenoidal and endoscopic transventricular techniques, and fully endoscopic transventricular and transsphenoidal techniques, have been described.4,6,10,13,14 The major disadvantage of simultaneous surgery is the necessity of 2 surgical teams and 2 operating fields. In simultaneous microscopic approaches, 2 microscopes and 1 fluoroscope are also required, which in turn means that a very large operating room is necessary. Although the use of an endoscope in the transsphenoidal stage decreases some of these requirements, the head position used for the pterional approach is not suitable for the transsphenoidal stage. Our patient was operated via a fully endoscopic transventricular and transsphenoidal approach. During the operation, we did not need to adjust the head position, and the operating room was comfortable for both surgical teams, permitting freer movement. On the other hand, it should be emphasized that the limitations of transventricular endoscopy included the requirement of ventricle enlargement to increase the working space, the need for an experienced surgeon, poor visualization, and the lack of ability to achieve adequate hemostasis if bleeding occurred. There are 2 case reports in the literature about the results of the fully endoscopic simultaneous transcranial and transsphenoidal approaches on giant pituitary adenomas. Greenfield et al14 and Romano et al11 both used this technique. In both cases, gross total removal of the tumor was achieved, resulting in neurologic improvement. The authors emphasized the possibility of a transventricular endoscope as an assistant instrument to drop the suprasellar part of tumors with firm consistency into the sellar area if traditional techniques have failed. In the present case, we also preferred the simultaneous fully endoscopic transventricular and transsphenoidal approaches because the patient had hydrocephalus and we thought that the suprasellar part of the tumor was not amenable to resection using only the transsphenoidal approach. Although we could not achieve gross total removal because of intratumoral bleeding and tumor morphology (serious lateral extension and soft consistency), our patient's neurologic status improved significantly after surgery, and long-term follow-up was uneventful. However, we cannot determine the effect of our surgery on visual acuity because the patient's preoperative visual status could not be evaluated.
CONCLUSIONS The use of a transventricular endoscope to deliver the suprasellar part of the pituitary adenoma into the sellar area seems to be a reasonable technique when traditional measures fail. Although its usage in firm consistency tumors has been reported as safe and effective, this alternative option should be used with care in pituitary adenomas, and the case selection should be based on preoperative MRI findings, ventricular size, and the availability of experienced surgeons. Although our case suggested that this approach could also be used in pituitary adenomas with soft consistency, we emphasize that the goal of the surgery in this kind of tumor should not be gross total removal because of the possibility of intraoperative complications. FIGURE 5. A–D, Intraventricular stage. E and F, Transsphenoidal stage. A, Intraventricular view of the tumor capsule. B, View of the tumor in the third ventricle after partial resection. C, Arrow indicating the light of the endoscope in the sella. D, View of the lateral ventricle after subtotal resection of the tumor. E, View of the tumor in the sella. F, Arrow indicating the light of the endoscope in the suprasellar area.
REFERENCES 1. Cappabianca P, Cavallo LM, Esposito F, et al. Extended endoscopic endonasal approach to the midline skull base: the evolving role of transsphenoidal surgery. Adv Tech Stand Neurosurg 2008;33:151–159
© 2014 Mutaz B. Habal, MD
Copyright © 2014 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
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2. Cavallo LM, Prevedello DM, Solari D, et al. Extended endoscopic endonasal transsphenoidal approach for residual or recurrent craniopharyngiomas. J Neurosurg 2009;111:578–589 3. Zada G, Laws ER Jr. Simultaneous transsphenoidal and intraventricular endoscopic approaches for macroadenoma with extensive suprasellar extension: surgery from below, above or both? World Neurosurg 2010;74:109–110 4. Alleyne CH Jr, Barrow DL, Oyesiku NM. Combined transsphenoidal and pterional craniotomy approach to giant pituitary tumors. Surg Neurol 2002;57:380–390 5. Leung GK, Law HY, Hung KN, et al. Combined simultaneous transcranial and transsphenoidal resection of large-to-giant pituitary adenomas. Acta Neurochir 2011;153:1401–1408 6. Ojha BK, Husain M, Rastogi M, et al. Combined trans-sphenoidal and simultaneous trans-ventricular-endoscopic decompression of a giant pituitary adenoma: case report. Acta Neurochir (Wien) 2009;151:843–847 7. Youseff AS, Agazzi S, van Loveren HR. Transcranial surgery for pituitary adenomas. Neurosurgery 2005;57:168–175 8. D'Haens J, Van Rompaey K, Stadnik T, et al. Fully endoscopic transsphenoidal surgery for functioning pituitary adenomas: a retrospective comparison with traditional transsphenoidal microsurgery in the same institution. Surg Neurol 2009; 72:336–340 9. Leung GK, Yuen MM, Chow WS, et al. An endoscopic modification of the simultaneous “above and below” approach to large pituitary adenoma. Pituitary 2012;15:237–241 10. Nishioka H, Hara T, Usuf M, et al. Simultaneous combined supra-infrasellar approach for giant/large multilobulated pituitary adenomas. World Neurosurg 2012;77:533–539 11. Romano A, Chibbarao S, Marsella M, et al. Combined endoscopic transsphenoidal-transventricular approach for resection of a giant pituitary macroadenoma. World Neurosurg 2010;74:161–164 12. Abe T, Iwata T, Kawamura N, et al. Staged transsphenoidal surgery for fibrous nonfunctioning pituitary adenomas with suprasellar extension. Neurol Med Chir (Tokyo) 1997;37:830–837 13. D'Ambrosio AL, Syed ON, Grobelny BT, et al. Simultaneous above and below approach to giant pituitary adenomas: surgical strategies and long term follow up. Pituitary 2009;12:217–225 14. Greenfield JP, Leng LZ, Chaudhry U, et al. Combined simultaneous endoscopic transsphenoidal and endoscopic transventricular resection of a giant pituitary macroadenoma. Minim Invasive Neurosurg 2008;51:306–309
Dandy-Walker Syndrome With Severe Velopharyngeal Dysfunction: A Contraindication for Le Fort I Surgery? Kamil H. Nelke, DMD,*† Wojciech Pawlak, DMD, PhD,† Hanna Gerber, MD, DMD† Abstract: Dandy-Walker syndrome is a rare congenital brain deformation. Most symptoms are related with fourth ventricle and skull base malformations. Quite often, symptoms develop from infancy or progress rapidly. Cerebellar dysfunction, lack of muscle coordination, and skull deformities involving eye movement might be present. There are several Dandy-Walker syndrome complex types. We present a 23-year-old patient who had a severe dentofacial deformity with mandibular prognathism and extremely undeveloped maxillary bone resulting in palatopharyngeal and velopharyngeal dysfunction with complete lack of soft palate function resulting in
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increased speech tone and volume. Performing Le Fort I osteotomy in this case is greatly controversial and might result in even greater loss of function or even its total lack. Velopharyngeal complex is very important, and every surgeon should consider its value while planning Le Fort I osteotomies. Key Words: Dandy-Walker syndrome, orthognathic surgery, Le Fort I osteotomy, palatopharyngeal dysfunction, velopharyngeal complex
O
rthognathic surgery is a surgery of choice in many dentofacial deformities. Some cases consisted of maxillary movement and soft palate involvement, and pronunciation and sound volume might be changed. In addition, a great impact on the upper aerodigestive tract is well-known, and it is especially involved in the improvement of upper respiratory tract function and prophylaxis of sleep apnea. Yet, so far, changes in the velopharyngeal complex and future outcomes of patients are still not yet fully known and understood; however, many studies are focused mostly on patients with cleft. Problems related to hypernasality and increased turbulence are well known.1 Dandy-Walker syndrome (DWS) is a complex syndrome related mostly with brain and skull deformities, which also quite often consisted of other features, such as facial deformity, inappropriate eye movement (nystagmus), deficiency in axial musculature movement, or others.2 Skull cysts are also common. In severe forms, distinctive skeletal changes along with bony dysplasias can be found. Occurrence rate is approximately 1:30,000 newborns.3 It is important to know that DWS is a type of a disease described as ciliopathies. Le Fort I osteotomy has an effect on hypernasality, articulation, voice, and nasal emission. Movement and relation between soft palate and the surrounding muscles depending on the dentofacial deformity might change greatly. Maxillary advancement is a surgery of choice in patients with maxillary deficiency that is mostly performed in young adults.
DANDY-WALKER SYNDROME AND LE FORT I OSTEOTOMY A 23-year-old patient who had DWS with an underdeveloped middle part of facial skeleton that consisted of extremely wide and narrowed maxillary bone with impacted and underdeveloped teeth as well as increased mandibular prognathism was admitted to the department (Figs. 1, 2). Dandy-Walker syndrome findings in this patient consisted of elongated facial profile, hypertelorismus with widely seated nose with a nasal hump. After previous cranial surgeries, brain function was sustained and both psychosomatic and intelligence levels were normal. The patient was referred to improve From the Departments of *Human Anatomy, and †Maxillofacial Surgery, Wroclaw Medical University, Wroclaw, Poland. Received May 30, 2014. Accepted for publication August 19, 2014. Address correspondence and reprint requests to Kamil H. Nelke, DMD, Department of Maxillofacial Surgery, Uniwersytecki Szpital Kliniczny, Klinka Chirurgii Szczękowo-Twarzowej, ulica Borowska 213, 50–556 Wrocław, Uniwersytet Medyczny we Wrocławiu, Wroclaw, Poland; E-mail: [email protected] The authors report no conflicts of interest. Ethics Statement: This study required no approval from an institutional committee because it is not related directly with human care and treatment of animals used in this study. The current study is fully adapted to and corresponding with the Helsinki Declaration. Copyright © 2014 by Mutaz B. Habal, MD ISSN: 1049-2275 DOI: 10.1097/SCS.0000000000001302
© 2014 Mutaz B. Habal, MD
Copyright © 2014 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
