| Abstract|| |
Facial teratomas are uncommon tumours in children that distort the face and may be associated with functional problems. They are less common than cervical teratomas though they are often grouped together and considered an emergency due to their tendency to cause respiratory compromise. They tend to be large and cause cosmetic issues; hence usually noticed early and medical help sought promptly by parents. The close proximity of facial teratomas to structures like eyes, parotid gland, facial nerve, vessels and brain makes them challenging and requires a patient and meticulous exploration during surgery. We present a case of an 11 month old girl with left sided temporal teratoma. Well planning of the incision and complete excision of the tumour with careful sparing of the facial nerves and parotid gland yielded good result.
Keywords: Abnormalities, congenital, face, mature teratoma
|How to cite this article:|
Yhoshu E, Chaudhary G, Ahmed I, Gupta MK. Congenital lateral facial teratoma: A case report and review of literature. Afr J Paediatr Surg 2021;18:99-103
| Introduction|| |
Teratomas are among the common tumours of infants and children with an incidence of 1: 4000 live births and a female predominance. Head and neck or craniocervical teratomas are uncommon tumours comprising 0.47%–6% of all teratomas, with an incidence of 2.5–5/100,000 live births. Craniocervical teratomas are different in that they are large and cause facial distortions, produce respiratory distress, cause obstetric complications like polyhydramnios, preterm labour and are associated with other abnormalities such as congenital cardiac disease, cleft lip and palate, bifid tongue and nose, mandibular hypoplasia.
Lateral facial tumours are extremely rare and are usually associated with bony and neurological deformities. Total surgical excision continues to be the best mode of treatment and early intervention can prevent the risk of malignant transformation. Here, we want to describe our management and follow-up of a lateral facial teratoma case with review of the literature.
| Case Report|| |
An 11-month-old girl was brought with swelling on the left side of the face present since birth. Parents had shown her to a doctor, but due to financial constraints, they could not get her operated. According to the parents, the child had no pain, difficulty in chewing food, opening mouth, difficulty in hearing or other neurological deficit. On examination, the swelling was occupying the lateral part of the face and measuring about 10 cm × 7 cm, extending superiorly to left temporo-parietal region, medially to mid-cheek and lateral part of upper eye lid, and posteriorly pushing the ear backwards and downwards laterally [Figure 1]. The overlying skin was normal with no neurological deficit.
Routine blood tests were normal, and serum alpha foetoprotein (AFP) was 7.3 ng/ml (n = 0–10 ng/ml). Ultrasound showed a solid-cystic mass with septations in the left temporoparietal region. Contrast-enhanced magnetic resonance imaging (MRI) of the brain and face showed a large extracranial well-defined rounded mixed intensity mass lesion measuring approximately 8 cm × cm 6 × 9 cm along the left side of skull vault[Figure 2]a and [Figure 2]b. Internally lesion was multicystic with areas of fat intensity. There was scalp defect with curvilinear communication of the mass lesion with extradural space. On computed tomography (CT) correlation, there were no areas of calcification seen within the lesion.
|Figure 2: Picture details of the patient: (a) Magnetic resonance imaging of patient-Transverse sections showing mixed density mass lesion left side temporal area. (b) Magnetic resonance imaging – Coronal sections showing multicystic mass with areas of fat density in left temporal. (c) Pre-o-perative marking of the incision line (left lateral facial) over the mass. (d) Intra-operative well encapsulated mass being dissected. (e) Whole-mass being mobilised off the floor with parotid in its floor. (f) Post-closure of wound showing the stitch line with sunken temporal area and drain in situ|
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Intraoperatively, a left lateral facial incision was made over the mass extending to the forehead [Figure 2]c. There was a large solid cystic mass about 9 cm × 12 cm × 5 cm in the left temporal region and extending to the cheek, causing displacement of the left ear posteriorly with no visible deficit in the skull. Posteriorly the mass was densely adherent to the anterior wall of the parotid which was gradually teased off. The mass was excised in-toto and excess skin was excised, and closure was done after placing a suction drain in the residual cavity [Figure 2]d,[Figure 2]e,[Figure 2]f. Post-operatively, the patient received intravenous analgesic and antibiotics. She recovered well with no neurological deficit or wound infection and was discharged after the drain was removed on the postoperative day 5.
Histopathology report showed mature cystic teratoma-solid cystic lesion, filled with milky fluid with an intact capsule. Tumour had squamous, ciliated columnar, intestinal epithelium, neuronal tissue, gastric tissue, salivary gland, lymphoid follicles, adipose tissue, muscle bundles, pancreatic tissue and foamy macrophages [Figure 3]a and [Figure 3]b. Repeat AFP level done at 3 months was normal (2.3 ng/ml). The patient is on follow-up for 21 months now and is doing well with no recurrent growth or neurological deficit [Figure 3]c and [Figure 3]d.
|Figure 3: (a) Photomicrograph showing a cystic tumour with squamous epithelium, intestinal and respiratory epithelium. H and E stain, ×2 view. (b) Photomicrograph showing muscle tissue. H and E stain, ×2 view. (c) Follow-up at 21 months showing the child's face with no skin laxity. (d) Closer lateral view of the face showing healed scar|
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| Discussion|| |
Extragonadal teratomas are more common in newborns and gonadal predominant in older children. Craniocervical teratomas comprise about 6% of teratomas and occur usually in the neonatal period. Differential diagnosis for craniocervical teratomas are: encephalocele, lymphangioma, cystic hygroma, vascular malformations, large branchial and thyroglossal cysts. The sites in the craniofacial area for teratomas to occur includes 'Extracranial' such as nasopharyngeal area (epignathus), base of the tongue, lateral face, scalp, cheeks and lips; and 'intracranial' such as massive tumours replacing intracranial contents and extending into orbit or neck, small tumours causing hydrocephalus and a combination of both occurring together.,,,,,
Lateral facial teratomas are extremely rare and do not pose as an emergency for airway compromise or feeding, unlike cervical teratomas. They can range from small lesions to large grotesque lesions which are apparent from birth or increase gradually and convince parents to seek early medical opinion. Their close proximity to structures such as nerves, eyes, ears and parotid gland requires clear understanding of the anatomy, relation to surrounding structures and planning with a multidisciplinary team for a satisfactory outcome. The following are similar cases described in the literature [Table 1].,,,,,, As seen in our case, facial mass in the reported cases were also noticed in the neonatal age. The size of the mass ranged from 3 cm × 2 cm to 24 cm × 18 cm × 16 cm in literature, our case mass measured 12 cm × 9 cm × 5 cm.
|Table 1: Tabulated details of lateral facial teratoma case reports in literature|
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About 16% of patients have calcifications on plain X-rays due to bone or tooth formation. Ultrasound and contrast-enhanced CT scan and MRI help in identifying the consistency-cystic or solid; extent of the lesion-extracranial or intracranial; and identifying the distortion/involvement of the surrounding tissues. Of the cases reported, 3 of them underwent CT scan alone, 3 including ours underwent both CT scan and MRI and one had done only MRI.
Complete surgical excision is the treatment of choice and can be challenging due to the large size, adherence to surrounding structures like facial muscles, parotid gland and facial nerves, and extent to intracranial region. Recurrence of follow-up is related to incomplete resection and immature teratoma has been seen to have a higher tendency for recurrence. In the three cases described by Kadlub et al., two of them underwent subtotal excision, and recurrence was inevitably seen [Table 1]. Our case underwent total excision of the left temporal mass. Whenever the excision is a challenge due to surrounding structures being involved, removal of the tumour as much as possible along with additional surgeries like cranial facial reconstruction, fat grafting, osteogenesis distraction of impaired mandibular growth were done., If tumour is dumbbell, advanced surgical techniques like neuronavigation, endoscopic sinus surgery or transbasal approaches are needed for complete clearance of teratoma. There can be neurological deficits after surgery such as Mandibular nerve hypoesthesia or partial right facial nerve palsy., These highlights the importance of appropriate counselling of the parents regarding the post-operative complications, recurrence and need for additional surgeries in follow-up.
The histopathology in craniocervical teratomas is commonly mature, though rarely it can be immature teratoma as seen in the case of Alexander et al. Our case was also reported as mature teratoma. Serum AFP can be used for follow-up in post-operative period though serial AFP values are more important than a single value.
In conclusion, though teratomas are common tumours in infancy and childhood, lateral facial teratomas are extremely rare. The key elements in the optimum management of such tumours include appropriate imaging for the extent of tumour, in-depth discussion with parents regarding the diagnosis and probable prognosis and all attempts for complete surgical excision with preservation of surrounding vital structures. A good follow-up of patient to exclude recurrence, neurological deficit and need for additional surgeries are crucial.
Written informed consent has been obtained from the parents for publication.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the parents have given their consent for images and other clinical information to be reported in the journal. The parents understand that names and initials will not be published, and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.
We are grateful to Dr Sakshi Garg Tayal (pathologist) for her efforts to contribute the biopsy pictures for the paper.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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Dr. Enono Yhoshu
Department of Pediatric Surgery, 6th Floor 'A' Block, All India Institute of Medical Sciences, Rishikesh - 249 203, Uttarakhand
Source of Support: None, Conflict of Interest: None
[Figure 1], [Figure 2], [Figure 3]