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 Table of Contents  
Year : 2015  |  Volume : 4  |  Issue : 2  |  Page : 119-122

Giant epignathus tumor associated with disseminated intravascular coagulation: A case report and literature review

1 Department of Pediatric Critical Care Medicine, Shands Children's Hospital, University of Florida Health, Gainesville, Florida, USA
2 Department of Pediatric Surgery, Driscoll Children's Hospital, Corpus Christi, Texas, USA

Date of Web Publication6-Apr-2015

Correspondence Address:
Dr. Ravi Shankar Samraj
Pediatric Critical Care Medicine, University of Florida Health, Shands Children's Hospital, 1600 Archer Road, Gainesville, Florida - 32608
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2249-4847.154116

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Epignathus is a rare congenital head and neck tumor (1 in 35,000-200,000 live births) with many associated complications. We report a rare association of giant epignathus associated with disseminated intravascular coagulation. To our knowledge, only one case of this association has been reported so far. A review of different clinical presentations of epignathus, classification, its reported associations and available management strategies is included.

Keywords: Airway obstruction, disseminated intravascular coagulation, epignathus, ex utero intrapartum treatment procedure, neonate

How to cite this article:
Samraj RS, Almond S. Giant epignathus tumor associated with disseminated intravascular coagulation: A case report and literature review. J Clin Neonatol 2015;4:119-22

How to cite this URL:
Samraj RS, Almond S. Giant epignathus tumor associated with disseminated intravascular coagulation: A case report and literature review. J Clin Neonatol [serial online] 2015 [cited 2022 Dec 9];4:119-22. Available from: https://www.jcnonweb.com/text.asp?2015/4/2/119/154116

  Introduction Top

Epignathus is an uncommon tumor originating from the oropharyngeal region. [1] These tumors are associated with significant mortality and morbidity in the perinatal period. There has been only one reported association of epignathus with disseminated intravascular coagulation (DIC). We present a case of a large epignathus tumor [Figure 1] whose postnatal course was complicated with severe DIC, and discuss the management strategies which led to a successful outcome.
Figure 1: Giant epignathus ruptured at the distal end with profuse bleeding as noted on the operating table

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  Case Report Top

B.T. was a 2.8 kg, 31-week gestation, male born via caesarean section (C-section) to 23-year-old G2P2 female. Prenatal ultrasound showed a huge soft tissue neck mass diagnosed as cystic hygroma. Amniocentesis showed a normal male karyotype. At the delivery, the epignathus ruptured leading to hypotension, anemia and metabolic acidosis. The baby was intubated, resuscitated and the bleeding controlled by a circumferential pressure dressing. APGARs at 1 and 5 min were 2 and 7, respectively. The baby was then transferred to our care.

On arrival, he was intubated, sedated and resuscitation continued with fluids, packed red blood cells and inotropes. The epignathus (19 cm × 35 cm) remained wrapped and extended posteriorly from the left ear lobe, anteriorly to the chin, superiorly from the corner of the left eye and inferiorly to the clavicle. Pertinent laboratory studies were as follows: Hematocrit 38%, platelet count 137,000/μl, prothrombin time 33.2 s, partial thromboplastin time 45 s, international normalized ratio 3.3, serum glutamic oxaloacetic transaminase 3860 u/L and serum glutamic-pyruvic transaminase 380 u/L. Fibrinogen levels were low, and D-dimer was positive. Alpha-fetoprotein (AFP) levels were 23,892 ng/ml and beta human chorionic gonadotropin was 4 mIU/ml.

Preoperative management included continued resuscitation, correction of the coagulopathy and ventilatory management. The original pressure dressing was not removed preoperatively. Studies included a chest X-ray and computed tomography (CT) scan of the head, face, neck and chest. This revealed a large heterogeneous mass extending to the submandibular and left parapharyngeal space with significant airway displacement [Figure 2] and [Figure 3]. There was no intracranial or intraorbital extension. Preoperative evaluation and stabilization took approximately 36 h. Activated factor VII was started 2 h prior to operation and continued post-operatively.
Figure 2: Three-dimensional reconstruction of the computed tomography scan with contrast showing a large heterogeneous mass extending to the left submandibular and parapharyngeal space

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Figure 3: Origin of the tumor from left tonsillar fossa causing significant airway displacement

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At the operation, a central venous catheter was placed, the mass unwrapped and the patient re-prepped and draped. Bleeding from the ruptured epignathus was controlled with pressure and skin flaps developed anteriorly and posteriorly. The tumor was removed in three parts [Figure 4]. The tumor origin was the left tonsillar fossa. The oral cavity was closed; skin flaps trimmed and closed over two close-suction drains [Figure 5].
Figure 4: Pathology specimen showing immature teratoma weighing 580 g, measuring 21 cm × 15 cm × 9 cm with solid and cystic components with areas of calcification, hemorrhage and necrosis

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Figure 5: Postoperative appearance after skin reconstruction

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Pathology showed an immature teratoma with no malignant germ cell components. Neuroectodermal and glial tissues were prominent. Focal glial fibrillary acidic protein was positive and placental like alkaline phosphatase negative.

The postoperative course was complicated by respiratory failure, SIRS, myocardial dysfunction, liver dysfunction and feeding difficulty. He was extubated on postoperative day 17. Dysphagia study was done, and a gastrostomy tube (G tube) placed on postoperative day 78. He was discharged at 11 weeks of age on G tube feeds with normal pulmonary, cardiac, and hepatic function.

  Discussion Top

Teratomas are tumors with components derived from the multipotent cells of more than one primitive embryonic layer (ectoderm, endoderm, mesoderm). It is composed of multiple tissues foreign to the normal organ in which it arises and can be mature, immature or malignant. Most commonly they arise from sacrococcygeal region, ovary, testis, mediastinum, and brain. Teratomas rarely arise from the oropharyngeal region.

Oropharyngeal tumors were classified by Ehrich (1945) as episphenoid, epipalatine or epignathus tumors (epi - above, gnathos - jaw). Epignathus tumor is rare (1 in 35,000-200,000 live births) [1] and is thought to arise from the craniopharyngeal canal. While most arise from the sphenoid bone or hard palate [1] , tumors arising from the hard palate, soft palate, pharynx, tongue, and jaw are reported. In contrast to teratomas, epignathus tumors rarely arise from regions other than nasopharynx and rarely undergo malignant transformation.

In the antenatal period, epignathus presents with polyhydramnios, [2] placental hydrops and/or fetal heart failure. [3] Associated anomalies (6%) include facial clefts, [1] bronchial cysts, hypertelorism, and congenital heart defects. [4] Less common anomalies include:

Pierre Robin sequence, meningoencephalocele, duplication of the pituitary gland and aplasia of the corpus callosum. [5]

Prognosis depends on the extent of airway obstruction [6] and associated anomalies. [7] Sonography and magnetic resonance imaging (MRI) are useful in prenatal diagnosis. CT and MRI are useful postnatally for narrowing the diagnosis and delineating tumor extent. MRI allows better differentiation from cystic hygromas, [8] whereas CT scan is useful in ruling out intracranial extension. [9]

Prenatal diagnosis and preparation are imperative for ensuring a good outcome. At the delivery, personnel and instruments for an ex utero intrapartum treatment procedure should be available. [10] After securing the airway, associated anomalies are ruled out and the extent of the tumor determined. Most tumors have no intracranial extension so they can be resected using an intraoral approach. Complete resection is common as most tumors are encapsulated or pseudoencapsulated. [9] In our case, excision was performed in three parts due to the bulky nature of the tumor. A transpalatal, endoscopic, endonasal approach has been used for tumors with intracranial extensions. [11] Serial measurements of serum AFPare necessary as failure of the expected gradual fall of this marker postoperatively may indicate the existence of residual malignant elements or development of metastasis. [12] Malignant degeneration is rare though described [13] and completely excised tumors do not recur. Although distant metastases have not been reported, an epignathus can be locally invasive. [14]

The first successful resection of an epignathus was reported by Ochsner and Ayres in 1951. [15] Since then, many associated conditions have been described, including a single case associated with disseminated intravascular coagulation (DIC). [7] This case was a 32 week gestation baby with a giant epignathus arising from the hard palate and nasopharynx. There was no prenatal care. The postnatal period was complicated by the inability to intubate, requiring emergency tracheostomy. In addition, the baby had severe respiratory distress syndrome, asphyxia, renal failure and profound bleeding secondary to DIC. A subtotal resection was done at 8 h of life. However there was further clinical deterioration and the baby expired at 11½ h after birth.

In our case, prenatal diagnosis of a large head and neck mass and planned C-section allowed for a controlled delivery. Airway access was obtained immediately avoiding asphyxia. Tumor bleeding was controlled by direct pressure and hypotension treated aggressively with fluids, blood transfusions and inotropes. Importantly, the resuscitation period (36 h) allowed sufficient time to identify the extent of the tumor and correct hypotension, electrolyte abnormalities and DIC. Our case highlights the rare association of epignathus with DIC, successful use of factor VII to decrease bleeding and preoperative stabilization with correction of DIC resulting in a successful outcome.

  References Top

Levine AB, Alvarez M, Wedgwood J, Berkowitz RL, Holzman I. Contemporary management of a potentially lethal fetal anomaly: A successful perinatal approach to epignathus. Obstet Gynecol 1990;76:962-6.  Back to cited text no. 1
Ekici E, Soysal M, Kara S, Dogan M, Gokmen O. Prenatal diagnosis of epignathus causing acute polyhydramnios. Acta Obstet Gynecol Scand 1996;75:498-501.  Back to cited text no. 2
Petrikovsky BM, Chervenak F, Schapfel DM. 1993-06-13-21 Epignathus© Petrikovsky. Available from: http://www.TheFetus.net. [Last accessed on 2014 Jan 05]  Back to cited text no. 3
Gilman PA. Epidemiology of human teratomas. In: Damjanov I, Knowles BB, Solter D, editors. The Human Teratomas. Experimental and Clinical Biology. Clifton, New Jersey: Humana Press; 1983. p. 94.  Back to cited text no. 4
Vandenhaute B, Leteurtre E, Lecomte-Houcke M, Pellerin P, Nuyts JP, Cuisset JM, et al. Epignathus teratoma: Report of three cases with a review of the literature. Cleft Palate Craniofac J 2000;37:83-91.  Back to cited text no. 5
Velasco Sanchez MA, Moreno HC, Salgado AS, Serena JLB, Irra EJ, Sanchez PG et al. Epignathus 2007-04-12-12 Epignathus© Sanchez. Available from: http://www.TheFetus.net. [Last accessed on 2014 Jan 05]  Back to cited text no. 6
Lionel J, Valvoda M, Abdul Hadi KA. Giant epignathus - A Case Report. Kuwait Med J 2004;36:217-20.  Back to cited text no. 7
Wiatrak BJ, Myer CM 3 rd , Bratcher GO. Report of a nasopharyngeal teratoma evaluated with magnetic resonance imaging. Otolaryngol Head Neck Surg 1990;102:186-90.  Back to cited text no. 8
Rothschild MA, Catalano P, Urken M, Brandwein M, Som P, Norton K, et al. Evaluation and management of congenital cervical teratoma. Case report and review. Arch Otolaryngol Head Neck Surg 1994;120:444-8.  Back to cited text no. 9
Woodard TD, Yong S, Hotaling YA. The ex utero intrapartum treatment (EXIT) procedure used for airway control in a newborn with cervical fetus in fetu: A rare case. Int J Pediatric Otorhinolaryngol 2006 Nov; 70:1989-94T.  Back to cited text no. 10
Prevedello DM, Kassam AB, Carrau RL, Snyderman CH, Thomas A, Gardner P, et al. Transpalatal endoscopic endonasal resection of a giant epignathus skull base teratoma in a newborn. Case report. J Neurosurg 2007;107:266-71.  Back to cited text no. 11
Okafor CI, Okafor CO, Odike MA. Congenital teratoma of the face. J Obstet Gynaecol 2004;24:828-9.  Back to cited text no. 12
Too S, Ahmad Sarji S, Yik Y, Ramanujam T. Malignant epignathus teratoma. Biomed Imaging Interv J 2008;4:e18.  Back to cited text no. 13
Keswani RK, Chugh TD, Dhall JC, Mehrotra GC. Epignathus - A case report. Br J Plast Surg 1968;21:355-9.  Back to cited text no. 14
Ochsner A, Ayres WB. Case of epignathus survival of the host after its excision. Surgery 1971;30:560.  Back to cited text no. 15


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]


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