Junaid Ashraf, Salma Kiani, Assad Hafeez, Umar Farooq, Mansoor ul Haq, M Naeem Khan.
Epignathus with interesting features.
J Coll Physicians Surg Pak Jan ;12(3):179-81.

A case of epignathus in one-day-old neonate is reported. She was delivered to a 34 years old woman with elective caesarean section. Details of diagnosis, remarkable features of tumor, and management modality is presented. She was managed immediately with enucleation of teratoma and respiratory assistance to relieve asphyxia. The tumor had fully developed limb suggesting element of parasitic twining. Prognostic aspects and literature review is also discussed.

Case Report: A 34 years old woman, gravida 3, para 2, presented to obstetric outpatient department was diagnosed as having polyhydramnios and obscure fetal anatomy. Patient`s pregnancy progress and medical history were unremarkable. An ultrasound confirmed polyhydramnios with normal placenta, a single active fetus with oblique lie. Fetal length and abdominal perimeter were compatible with 31 to 34 weeks of gestation. A large semisolid and partly cystic mass like lesion was also seen on ultrasound originating from oro-frontal area. This measured 12 x 8 cms. There was also a limb like lesion noted to be protruding out of the oral cavity, as seen on ultrasound. This suggested possibility of a small acardiac twin conjoined to the fetal face. However, it was difficult to confirm this finding because the fetal structures were not properly developed and there was also a possibility of a teratoma. Doppler ultrasound of the mass demonstrated a resistive index of 0.70, which was within normal limits. Obstetric and pediatric surgical opinion, having obtained, it was decided to allow the pregnancy to progress with close weekly fetal monitoring. However, at 35 weeks of gestation patient went into labor and a female baby of 1.2 kg was delivered with a caesarean section in the presence of pediatricians and paediatric surgeons. The tumor was jetting out of oral cavity with complete obstruction to breathing. There was a complete limb like structure about 10 inches long and 6 inches in circumference with a complete skin cover. There were five toes at the distal end of the limb. In addition, there was a lobulated intraoral tumor, which was predominantly solid and had a few cystic areas. Oral cavity had been forcibly opened by the protruding tumor mass, dislocating the temporomandibular joints. With some difficulty, tumor was hooked out of its broad-based origin from oropharyngeal area. It was then noted that the child also had posterior cleft palate. She was resuscitated with endotracheal intubation and assisted in breathing till she had started breathing on her own. Subsequently she developed bradycardia and oozing from the tumor bed, survived for 24 hours and despite resuscitative measures expired. Her prematurity and our failure of intensive management were attributed to be the major factors of her death. On gross examination of the tumor, it was noted that the portion that was jetting out of the oral cavity was a complete limb with marked lymph edema, a complete set of skeleton and fully formed digits arising from the distal end of the limb. Lymphatic obstruction was caused by the constricting effect of the alveolar margin of oral cavity. As soon as the limb was incised the lymph gushed out in large quantity and the limb became deflated. The rest of the tumor was semisolid and cystic and in parts appeared to contain neural tissue. On microscopy, the tumor was reported to be a mature teratoma with elements of ectoderm, mesoderm and endoderm. There were areas of immature cells in the tumor especially at its base, but no obvious malignancy was found.

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