1. GANGLIONEUROMA MAXILLA RARE CASE, INNOVATIVE SURGERY
Dr.RAJGOVIND SHARMA
Dr.Prashant Sharma
Dr.Harsh Deora
Division of surgical oncology,
SMS MEDICAL COLLEGE,JAIPUR

2. BACKGROUND Ganglioneuroma(GN)- Arise from PNS
Originate from neural crest sympathogonia of sympathetic nervous system
Neuroblastic tumours: (Shimada,1999)
Neuroblastoma(primarily blasts)
Ganglioneuromas(mature ganglion cells)
Ganglioneuroblastomas(both)

3. Frequency: rare tumours-1 in 1,00,000 (mostly children)
Sex: male: female:: 1:1.5
Age: Children and young adults.
Mean age is 7 yrs(O Adam, Jurnalul pediatrului,2007)
LOCATION:
Adrenal
Para spinal retroperitonium
Posterior mediastinum
RARE: Head and neck, urinary bladder, bowel wall, gall bladder.

4. SUMMARY: BENIGN TUMORS, SURGICAL EXCISION IS CURATIVE
CLASSIFICATION
TWO SYSTEMS
1. Pediatric oncology group(POG):
Based on differentiation of cellular and stromal elements
Eg: Neuroblastoma<50% differentiated elements.
2. Shimada Classification
Histology: stroma grade and differentiation
Age: <1yrs; 1-5yr; >5yrs
SUMMARY: BENIGN TUMORS, SURGICAL EXCISION IS CURATIVE

5. CASE REPORT 15 yr old female Presented with c/o Gradually increasing
swelling rt.side of face x 12y
swelling rt.temporal region x 2y
No asso symptoms of
Vision loss
Deglutation difficulties
Neuro-sensory loss

6. CASE REPORT On examination 1. Larger swelling 8 x 6cm
Firm
Nontender
Local temperature not raised
Nonmobile
Fixed to underlying bone
Skin over swelling stretched
2. Smaller temporal swelling:
6 x 4 cm size
Firm
Nontender
Local temperature not raised
Mobile and not fixed to underlying bone & muscle
Skin over swelling normal

7. CASE REPORT Intraoral examination:
Lips, buccal mucosa, gums, teeth, tongue movements. Retro molar trigone normal on appearance and palpation.
Swelling seen protruding into the oral cavity at Rt. Upper GBS.
Vision: 6/24 right eye and 6/6 left eye.

8. CT SCAN 3D RECONSTRUCTION
TEMPORAL BONE SPARED
LYTIC LESION INVOLVING MAXILLA

10. FINAL TISSUE Dx: GANGLIONEUROMA
BIOPSY
TRANS-ORAL TRU-CUT : SPINDLE CELL NEOPLASM
MARKER STUDY: POSTIVE STAIN OF S-100 & SYNAPTOPHYSIN
FINAL TISSUE Dx: GANGLIONEUROMA

11. OPERATIVE PROCEDURE
Total maxillectomy + excision of mass in temporal region + reconstruction by Rt. Temporalis muscle flap + nylone darning of orbital floor.
Per op findings:
1. A 9 x 6 x 5 CM soft, whitish tumor invading the anterior maxillary wall, sparing the orbit.
2. A second tumor 6 x 8 x 2 cm in the temporal fossa, in subcutaneous plane, well defined, superficial to temporalis muscle.

12. WEBER FERGUSSON INCISION

13. Procedure
Skin flap raised laterally over the tumor and underlying bone cut keeping capsule intact.

14. Anterior wall of maxilla separated from lateral wall of nose medially, palatine bone below, floor of orbit above and zygomatic bone laterally.

15. Main specimen taken out taking care not to damage the orbital apex and eye ball
A 6 cm incision taken over the temporal swelling just above the cranial extent of the tumor

16. MAIN TUMOR SPECIMEN, GROSSLY COMPLETE RESECTION ACHIEVED
SMALLER TEMPORAL SWELLING

17. Nylone Darning of orbital floor done.
Temporalis muscle lifted of underlying bone with help of periosteum elevator and inserted under zygomatic tunnel so as to support the eye ball.
Nylone Darning of orbital floor done.
(Prolene darning has also been tried)
TEMPORAL MUSCLE FLAP

18. POD-2
TUBE FEEDING STARTED
AT FOLLOW UP, AFTER SUTURE REMOVAL

19. Repeat vision testing: right eye 6/24, left eye 6/6
Repeat vision testing: right eye 6/24, left eye 6/6. Eye closure of right eye was almost complete with 0.3 cm gap remaining (ectropion). NO DIPLOPIA CONJUGATE EYE MOV NORMAL

20. DISSCUSSION
MAXILLECTOMIES FOR MAXILLARY NEOPLASMS
Due to resection of tumors involving orbit, nasal cavity, palate, paranasal sinuses, intraoral mucosa
Cause major functional consequences
Deglutition
Speech
Orbital function
Aesthetics

21. Maxillectomy – A Historical Perspective
Total maxillectomies performed by Dupuytren and Gensoul in 1820 and 1824?
First recorded maxillectomy by Liston in 1841
Extensive review published by Ohngren in 1933

22. MAXILLARY BONE Two horizontal and three vertical buttresses
Insertion for most muscles of facial expression and mastication
Geometrical structure with 6 walls (hexahedron)
Roof supports orbital contents
Floor forms anterior hard palate.

23. Classification System (Santamaria & Cordeiro)
Type I (Limited maxillectomy)
One or two walls, preservation of palate
Type II (Subtotal maxillectomy)
Lower 5 walls, preservation of orbital floor
Type III (Total maxillectomy)
Resection of all six walls
Orbital preservation (IIIa) vs exoneration (IIIb)
Type IV (Orbitomaxillectomy)
Upper 5 walls, preservation of palate
Santamaria & Cordeiro, Plast Recon Surg
Santamaria & Cordeiro, Plast Recon Surg

24. Santamaria & Cordeiro, 2000. Plast Recon Surg

25. Reconstruction- the past
Skin grafts
Cervicofacial flaps
Pectoralis myocutaneous flap
Usually requires two stage procedure

26. Local and Regional Flaps
Palatal mucoperichondrial island flap
Up to 15 cm2 surface area
Strong enough for through-and-through defects
Can rotate 180 deg on pedicle
Buccal fat pad
Rich vascular supply
Best for defects up to 4 cm in diameter
Can be used in combination with free bone grafts
Submental island
7-15 cm in size
Well hidden donor site scar
Temporalis
Good for orbital support

27. Free Flaps Indicated for large defects
Matching to three-dimensional shape of defect
Provide bone, palatal and nasal lining, skin, soft tissue
Requires vascular pedicle cm long
Multiple different options
Myocutaneous
Osteomyocutaneous
Combination with
free bone grafts

28. Prosthetics (Obturation)
Advantages
Shorter operative time
Shorter postop hospital stay
Better visualization of maxillectomy cavity for surveilance
Disadvantages
Hypernasal speech
Regurgitation of foods and
liquids into nasal cavity
Difficulty maintaining hygeine
Need for repeated adjustments

29. CONCLUSION
GN are rare benign tumors requiring preoperative tissue diagnosis,surgical excision is curative.
Maxillectomy and midface defects result in major functional and aesthetic abnormalities
Reconstruction depends on the size and individual components of the resected tissue
Nylone(or prolene) darning can be employed for orbit support in cases where intra-op infection is a concern.