Dr Varsha V S,Dr Jayasree C, Dr Lakshmi S Nair, Dr Perraju Bendapudi, Dr Ashwin S Prabhu, Dr Smrithi Madhava Menon. DrC Jayakumar
Department of Pediatrics & Neonatology, AIMS Kochi.
Term/AGA/Female ba was born LSCS to a 33 years old Primi mother ,Gestational diabetes Mellitus on insulin. Third trimester anomaly scan showed a bilobulated thick walled cystic lesion on upper part of the ba’s neck .Ba cried immediately after birth and shifted to mother’s side. Examination revealed cystic swelling behind right ear with palpable bony structure in it. No other external anomalies noted .Ba developed respiratory distress and was shifted to NICU. Ba was started on HFNC with flow of 6L with FiO2 of 21%.
Auxology Weight: 2.89kg, HC: 35cm, Length: 45cm
Systemic examination done showed tachypnea, subcostal retraction and grunting.
Labs CBC: TC: 10.39K/uL/ N/L: 43.2%/ 40.9%, Hb: 20g/dl, Platelet count: 2.48Lac. RFT/ TFT/ Serum electrolytes: normal.
Blood C/S: Sterile.
X-ray chest: Fluid in fissure s/o TTNB
Differentials considered were
1. Cystic hygroma,
2. Lymphangioma,
3. Teratoma.
4. Branchial cyst,
5. Cervical congenital thyroid goitre.
USG neck: Features like large cystic locules with clear contents in the subcutaneous plane and another small locule with aberrant bony structure. S/o cystic hygroma
MDCT neck: Showed 2 cysts with clear fluid in the right mastoid region with ossification forming tubular and flat bone like structure seen adjacent to it. S/o cystic teratoma.
When ba has improved from breathlessness excision of soft tissue tumour (? Teratoma) under GA and tissue biopsy was done
HPE section showed: Dilated cystic spaces lined cuboidal to columnar to focally ciliated epithelium and areas resembling tubal epithelium with papillary projections. The walls show skeletal muscle, smooth muscle, fat with hibernoma like foci, glial tissue, dilated vessels, nerves, adrenal rest, fallopian tube and primitive gonadal tissue. Suggestive of Teratoma.
Neurosonogram, USG abdomen and ECHO done were normal. Hence other associated congenital anomalies are ruled out.
Thyroid profile and AFP levels were within normal range.
Ba was started on direct breast feeds and adlib pallada feeding and was discharged with regular follow up.
Discussion: Cervical teratoma.
Cervical teratomas are rare fetal tumors with a frequency of 1 in 20,000 to 1 in 40,000 live births, with a male to female ratio of 1:3.These are usually benign tumours formed the three germ layers (ectoderm, mesoderm and endoderm).
Most common site of congenital teratoma is coccyx region.
Cervical teratomas represent only about 6% of congenital teratomas.
They are usually unilateral with well-defined margins and are multiloculated. They can cause polyhydramnios and preterm labor. Postnatally can cause respiratory distress with progressive swelling leading to disfigurement.
Investigations: Antenatal diagnosis can be made ultrasound scan in the 2nd trimester featuring hydramnios and cervical mass with calcification. Cervical ultrasound and CT scan show cystic or heterogenous mass with both solid and liquid components with calcification. MRI helps in distinguishing different types of tissues, lesion limit and mass effect on the adjacent structures. Abnormal levels of beta HCG and Alfa fetoprotein will raise the suspicion of malignant component.
Complications: Respiratory distress in new-born, facial disfigurement, pulmonary hypoplasia, giant cervical teratoma can cause fetal hydrops.
Management: Surgical resection is the standard of care for cervical teratomas. However, the top priority in a neonate with a cervical teratoma is airway management.
Prognosis mostly depends on the risk of neonatal respiratory distress, its extension and potential malignancy.
Carry home message is surgical excision should be done to an asymptomatic small teratoma since these can grow gradually and can cause respiratory compromised and due to malignant potential