FM NOVEMBER 2018 ISSUE - digital edition

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The tumour was subtracted
digitally and the remnant
bones on right were mirrored
to the left. Plates were
prebent as a frame to which
free fibula bone flap was to
be contoured and placed
(Figure 2).
THE CASE DEMONSTRATED
THE IMPORTANCE
OF METICULOUS
MULTIDISCIPLINARY
PLANNING AND
SUCCESSFUL EXECUTION.
Surgical procedure: The
tumour was resected
completely through an
incision placed on the swelling. Much of the skin, the left oral
commissure and the left eye could be preserved structurally. A
left total maxillectomy and right partial maxillectomy was done.
The defect was mainly left maxillary and total hard palate.
There was also a loss of dorsal nasal support. A free fibula
osteocutaneous flap was taken from the left leg of the patient.
Three osteotomies were done to make four bone segments.
Contouring was done on the prebent plate. Three segments
were used to reconstruct the upper jaw; the last segment
was placed vertically for malar eminence and orbital support.
Fixation was done with plate and screws. The nasal deformity
was corrected with dorsal augmentation from the remaining
free fibula graft. Another piece was also used as a columellar
strut. The flap was anastomosed to the facial artery and vein.
The surgery lasted for about 14 hours.
A week later, the pathology was reported as juvenile
ossifying fibroma, psammomatoid type. Juvenile ossifying
fibroma can be rarely associated with
hyperparathyroidism. The patient was
seen by an endocrinologist. Investigations
for hyperparathyroid related syndromes
were done. Serum Calcium (14.6 mg/
dl) and parathyroid hormone (426 ng/L)
were elevated. A Technitium Sestamibi scan
showed increased uptake in the left inferior
parathyroid. Ultrasonogram of the abdomen
showed hamartoma of both kidneys. Left
inferior parathyroidectomy was done as a
second surgery. The PTH level dropped
to 92 ng/L intraoperatively. The post-operative
serum calcium was 8.5 mg//dl). The patient
recovered well from the surgeries and was
discharged. The visual acuity in the left eye
remained the same. He may also require some
additional corrective cosmetic procedures for
the left eye later (Figure 3, 4).
Hyperparathyroidism jaw tumour
syndrome is a rare entity. It is inherited as an
autosomal dominant pattern with incomplete
penetrance. The syndromic associations are
parathyroid adenomas, ossifying fibroma
of the jaw, renal lesions, polycystic kidney
disease, hamartomas and Wilm’s tumour.
Absolute treatment is the removal of the
jaw tumour and the offending parathyroid
adenoma.
It is important to look for the association
of hyperparathyroidism in juvenile ossifying
fibroma of the jaw. Treatment is complete only
if parathyroidectomy is done. There is a 24%
chance of the adenoma to turn malignant.
The case demonstrated the importance of
meticulous multidisciplinary planning and
successful execution. Head and neck surgeons,
plastic surgeons, craniomaxillofacial surgeons,
radiologists, nuclear medicine specialists,
ophthalmologists and pathologists were
involved. The nursing and technical staff also
helped immensely.
Dr Krishnakumar Thankappan is Professor,
Department of Head & Neck Surgery and Oncology
and Dr Subramania Iyer is Professor and Head,
Department of Head and Neck and Plastic Surgery,
Amrita Institute of Medical Sciences, Kochi.
drkrishnakumart@gmail.com
NOVEMBER 2018 / FUTURE MEDICINE / 41