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Facial Defect
Question :
Girish N, Sreedhara murthy B N, Archana K
Department of Pediatrics, Kempegowda Institute of Medical Sciences Hospital and Research Centre, Bangalore, India.

Address for correspondence: Dr.Girish N, #1753per,1st C main road, 2nd stage, Rajajinagar, Bangalore 560010, Karnataka, India. Email:drgirish79@gmail.com

A female infant weighing 2.6 kg was born at term by lower caesarean section through third degree consanguinous marriage to a 27years old primigravida. She had no significant antenatal problems. The antenatal ultrasonography at 32 weeks of gestation showed a multiloculated cyst in the right temporal and cheek region with defect in the parietal bone. At birth, the child was noted to have a soft fluctuant swelling in the right maxillary region extending horizontally from the right nasolabial fold to the right ear which was displaced posteriorly and vertically extending from the right temporal region to the lower border of mandible. CT scan of head and neck showed a large multiloculated mixed density mass lesion containing fat, ossific, solid and cystic components involving the right side of the neck and face extending superiorly from the right peritemporal level to perimandibular region inferiorly. Right maxillary bone is epicenter of the lesion. The mass lesion does not show any neck and intracranial extension. Alpha fetoprotein {AFP} levels were markedly elevated.
 
Answer :
The baby underwent surgery for excision of the swelling and histopathological examination revealed the lesion to be a mature cystic teratoma. Teratomas are neoplasm composed of three germinal layers of the embryo that form tissues not normally found in which they arise. Histologically they may be mature, immature or malignant. These are most common in the saccrococcygeal region and are rare in head and neck, which account for less than 6 percent. {1} Sacrococcygeal teratomas occur in one in 20000-40000 live births, four times more frequently in girls than in boys. {2} Facial teratoma is a very rare tumour, generally benign. {3} Complete surgical excision should be performed as early as possible in the neonatal period to prevent ongoing facial distortion and to prevent local recurrence and the need for further surgery. Postoperative monitoring for recurrences should include AFP levels in difficult cases. Clinical outcome of teratomas depend on their histological grade, localization and quality of surgical treatment. {4}
References
1. Rai M, Hegde P, Devaraju UM. Congenital facial teratoma. J Maxillofac Oral Surg. 2011. Published online 25 February 2011
2. Okafor CI, Okafor CO, Odike MA. Congenital teratoma of the face. J Obstet Gynaecol. 2004` 24: 828-829
3. Paulus P, Crêvecoeur H, Piette E, Lejuste P, Hustin J. Huge teratoma of the face. J Craniomaxillofac Surg. 2009` 37: 352-325
4. Rowe MI. Tumors and twins. In: James AON, Jay LG, Eric IF, Arnold GC, eds. Essentials of Pediatric Surgery. St. Louis, Mosby Yearbook. 1995: 302-303

E-published: August 2011 Vol 8 Issue 8 Art No. 54