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Successful in utero treatment of an oral teratoma via operative fetoscopy: case report and review of the literature

Published:April 09, 2012DOI:https://doi.org/10.1016/j.ajog.2012.04.008
      The prenatal diagnosis of a nasopharyngeal teratoma carries a very grave prognosis. Although these tumors constitute only 9% of all teratomas, all previous cases diagnosed antenatally have been associated with either fetal demise or emergent surgery at birth. Of the fetuses that survive to birth, delivery can be associated with airway obstruction and multiple postnatal surgeries. These complications could be averted if the tumor could be safely treated in utero. We hereby report the successful treatment of an oral teratoma via operative fetoscopy, with the birth of a healthy infant at term.

      Key words

      Neonatal teratomas are rare tumors that arise from all 3 embryonic germ layers: endoderm, mesoderm, and ectoderm.
      • Weaver R.G.
      • Meyerhoff W.L.
      • Gates G.A.
      Teratomas of the head and neck.
      The incidence of all teratomas is approximately 1/4000.
      • Weaver R.G.
      • Meyerhoff W.L.
      • Gates G.A.
      Teratomas of the head and neck.
      Most tumors arise in the midline, the most common sites being the sacrococcygeal region, anterior mediastinum, testicles, and ovaries in that order.
      • Tapper D.
      • Lack E.E.
      Teratomas in infancy and childhood A 54-year experience at the Children's Hospital Medical Center.
      • Hassett M.J.
      • Glick P.L.
      • Karamanoukian H.L.
      • Rossman J.E.
      • Wilcox D.T.
      • Azizkhan R.G.
      Pathophysiology of congenital diaphragmatic hernia XVI: elevated pulmonary collagen in the lamb model of congenital diaphragmatic hernia.
      Teratomas of the head and neck region constitute 1-9% of all teratomas.
      • Hassan S.
      • Sidek D.S.
      • Shah Jihan W.D.
      • Phutane G.
      • Mutum S.S.
      Massive lingual teratoma in a neonate.
      The incidence of teratomas of the head and neck is between 1/35,000 and 1/200,000 live births.
      • Izadi K.
      • Smith M.
      • Askari M.
      • Hackam D.
      • Hameed A.A.
      • Bradley J.P.
      A patient with an epignathus: management of a large oropharyngeal teratoma in a newborn.
      • Vandenhaute B.
      • Leteurtre E.
      • Lecomte-Houcke M.
      • et al.
      Epignathus teratoma: report of three cases with a review of the literature.
      Pure oral teratomas, or epignathi, occur in the sphenoid region on the palate or pharynx (Rathke's pouch)
      • Gull I.
      • Wolman I.
      • Har-Toov J.
      • et al.
      Antenatal sonographic diagnosis of epignathus at 15 weeks of pregnancy.
      and may rarely be found in the nasal cavity of the hard palate. Epignathi are thought to result from migration abnormalities of primordial cells settling in the mediastinum or hypothalamic regions and have a female to male ratio of 3:1.
      Prenatal diagnosis of an oral teratoma can be made with ultrasound as early as 12 weeks
      • Duncan J.M.
      • Meaney P.
      • Simpson P.
      • Berg R.A.
      • Nadkarni V.
      • Schexnayder S.
      Vasopressin for in-hospital pediatric cardiac arrest: results from the American Heart Association National Registry of Cardiopulmonary Resuscitation.
      and in the second trimester.
      • Gull I.
      • Wolman I.
      • Har-Toov J.
      • et al.
      Antenatal sonographic diagnosis of epignathus at 15 weeks of pregnancy.
      • Bruhwiler H.
      • Mueller M.D.
      • Rabner M.
      [Ultrasound diagnosis of epignathus in the 17th week of pregnancy Case report and review of the literature].
      • Clement K.
      • Chamberlain P.
      • Boyd P.
      • Molyneux A.
      Prenatal diagnosis of an epignathus: a case report and review of the literature.
      However, most tumors are diagnosed in the third trimester, suggesting in utero growth or subsequent development.
      • Daskalakis G.
      • Efthimiou T.
      • Pilalis A.
      • et al.
      Prenatal diagnosis and management of fetal pharyngeal teratoma: a case report and review of the literature.
      On ultrasound, the mass emerges from the fetal mouth and may produce hyperextension of the head. Oral teratomas are also associated with cleft palate and other oral malformations in 6-20% of patients.
      • Acosta-Rojas R.
      • Becker J.
      • Munoz-Abellana B.
      • Ruiz C.
      • Carreras E.
      • Gratacos E.
      Twin chorionicity and the risk of adverse perinatal outcome.
      • Noguchi T.
      • Jinbu Y.
      • Itoh H.
      • Matsumoto K.
      • Sakai O.
      • Kusama M.
      Epignathus combined with cleft palate, lobulated tongue, and lingual hamartoma: report of a case.
      The natural history of these tumors in utero include possible development of polyhydramnios, presumably secondary to pharyngeal obstruction,
      • Raveh J.
      “Keystone” approach for intracranial nasofrontal dermoid sinuses.
      as well as unexpected fetal death.
      • Daskalakis G.
      • Efthimiou T.
      • Pilalis A.
      • et al.
      Prenatal diagnosis and management of fetal pharyngeal teratoma: a case report and review of the literature.
      Perinatal management involves either intrapartum intubation (ex utero intrapartum treatment [EXIT procedure
      • Chiu H.H.
      • Hsu W.C.
      • Shih J.C.
      • Tsao P.N.
      • Hsieh W.S.
      • Chou H.C.
      The EXIT (ex utero intrapartum treatment) procedure.
      or resection of the tumor at the time of the cesarean section and prior to cutting the umbilical cord (operation on placental support [OOPS]
      • Skarsgard E.D.
      • Chitkara U.
      • Krane E.J.
      • Riley E.T.
      • Halamek L.P.
      • Dedo H.H.
      The OOPS procedure (operation on placental support): in utero airway management of the fetus with prenatally diagnosed tracheal obstruction.
      ). Despite all efforts, survival rates are typically in the 30–40% range. The purpose of this communication is to report successful in utero resection of a nasopharyngeal teratoma via operative fetoscopy.

      Case Report

      A 37 year old woman, G4 P1031, with an uncomplicated prenatal course was diagnosed at 20 weeks' gestation using two-dimensional and three-dimensional ultrasound as having a fetus with a pedunculated 4.1 × 2.4 × 2.8 cm mass protruding from the fetal mouth. The tumor had cystic and solid components. Cleft lip or palate did not appear to be present, but they could not be entirely ruled out with ultrasound. A small feeding arterial vessel was identified at the stalk of the tumor using color and pulsed Doppler, although the actual anatomical site of origin of the mass could not be determined (Figure 1, A and B). Polyhydramnios, hydrops, or abnormal arterial or venous Dopplers were not present at the time of diagnosis. Fetal magnetic resonance imaging (MRI) confirmed an exophytic complex mass arising from the left paramedian aspect of the midface of the fetus. No obvious intracranial extension was apparent. Findings were suggestive of an oral teratoma.
      Figure thumbnail gr1
      FIGURE 1Ultrasound and Doppler images
      A, Ultrasound image of the fetus at 20 1/7 weeks' gestation. A complex mass is seen protruding from the fetal mouth. B, Doppler interrogation of the stalk of the mass shows a distinct arterial vessel feeding the mass.
      Kontopoulos. In utero treatment of oral teratoma via operative fetoscopy. Am J Obstet Gynecol 2012.
      Options discussed with the patient included expectant management, which could result in increased tumor size, polyhydramnios, hydrops, or fetal demise. If the fetus survived to an appropriate gestational age, delivery could require an EXIT
      • Chiu H.H.
      • Hsu W.C.
      • Shih J.C.
      • Tsao P.N.
      • Hsieh W.S.
      • Chou H.C.
      The EXIT (ex utero intrapartum treatment) procedure.
      procedure or an OOPS
      • Skarsgard E.D.
      • Chitkara U.
      • Krane E.J.
      • Riley E.T.
      • Halamek L.P.
      • Dedo H.H.
      The OOPS procedure (operation on placental support): in utero airway management of the fetus with prenatally diagnosed tracheal obstruction.
      procedure. Termination of pregnancy was also offered. The patient was particularly concerned about the possible existence of oral clefting, which, if present, would make her consider the option of pregnancy termination more seriously. Diagnostic and possibly operative fetoscopy was also discussed as an alternative. The purpose of diagnostic fetoscopy was to assess the lips and palate and rule out clefting. In addition, if it were considered feasible, an attempt would be made to either devascularize or to resect the lesion altogether.
      After serious consideration, the patient requested to undergo fetoscopy. The risk of pregnancy loss was quoted as 5%.
      • Quintero R.A.
      Twin-twin transfusion syndrome.
      The procedure was approved by the University of Miami and Jackson Memorial Hospital under the innovative therapy pathway. Amniocentesis revealed a normal 46, XX karyotype.
      The patient was taken to the main operating room. She received Remifentanil intravenously at a rate of 0.1 μg/kg per minute, increased by 0.025 μg/kg per minute increments up to a maximum dose of 0.15 μg/kg per minute. Remifentanil crosses the placenta readily and provides fetal analgesia. Under local anesthesia, a minimal skin incision was made. A 3.8 mm trocar (Richard Wolf Inc, Vernon Hills, IL) was inserted percutaneously under ultrasound guidance into the amniotic cavity. The fetal mouth was assessed externally and internally using a 25° diagnostic endoscope (Richard Wolf Inc.). There was no evidence of cleft lip or palate, findings that were relayed to the patient intraoperatively.
      The teratoma was noted to have a distinct stalk, semirotated clockwise, stemming from the hard palate anteriorly and slightly off the midline to the left (Figure 2), confirming the ultrasound and MRI findings. Consideration was given at this point to proceed with excision of the mass. The tumor was resected at the base using a 600 micrometer contact YAG laser fiber on continuous mode with 10 W of energy. A minimal amount of bleeding from the base of the tumor was controlled with a noncontact YAG laser fiber. The tumor fell inside the amniotic cavity, where it was left. The procedure lasted 68 minutes. There were no maternal or fetal complications.
      Figure thumbnail gr2
      FIGURE 2Endoscopic image inside the fetal mouth
      Endoscopic image inside the fetal mouth during operative fetoscopy showing the stalk of the mass (S) attached to the hard palate (P).
      Kontopoulos. In utero treatment of oral teratoma via operative fetoscopy. Am J Obstet Gynecol 2012.
      The postoperative course was uncomplicated. Serial weekly ultrasounds showed normal fetal mouth and profile, without recurrences. The amniotic fluid volume remained normal throughout the pregnancy. The patient went into spontaneous labor at 38 5/7 weeks. A female infant was born vaginally without complications, weighed 8 lb, and had Apgars of 9 and 9 at 1 and 5 minutes, respectively. Inspection of the lips and mouth at birth showed no evidence of clefting, injury, or residual mass (Figure 3). Surgical pathology showed necrotic tissue with cartilaginous differentiation, suggestive of a nasopharyngeal teratoma. Postnatal MRI at 1 month of life showed no evidence of recurrent mass in the brain or face.
      Figure thumbnail gr3
      FIGURE 3Image of infant at birth
      Image of infant at birth, showing no residual tumor, no clefting, and no evidence of injury from the antenatal fetoscopic surgery.
      Kontopoulos. In utero treatment of oral teratoma via operative fetoscopy. Am J Obstet Gynecol 2012.

      Comment

      To our knowledge, this is the first successful treatment of a fetal oral teratoma in utero. The experience in this case suggests that fetoscopy can be of use in the detailed assessment of the lesion as well as potentially allowing resection of the mass in utero in selected cases. In utero resection is aimed at avoiding the development of polyhydramnios, preterm labor/delivery, or potential fetal demise that could occur with expectant management. Furthermore, in utero resection allows for vaginal delivery and eliminates the need to perform an EXIT or OOPS procedure, with its attendant logistic and medical disadvantages. In utero treatment could potentially be associated with improved healing as well.
      Oral teratomas have been classified into 4 subgroups: (1) dermoids or hairy polyps that are the most common and contain only epidermal and mesodermal elements; (2) teratoids that consist of ecto-, meso-, and endodermal elements but that are incompletely organized; (3) true teratomas that contain all 3 germ layers but with greater histological organization and recognizable early organ differentiation; and (4) epignathus that are highly differentiated tumors with well-formed organs and limbs, which are rare and generally incompatible with life.
      • Cohen A.F.
      • Mitsudo S.
      • Ruben R.J.
      Nasopharyngeal teratoma in the neonate.
      • Freitas Rda S.
      • Alonso N.
      • Azzolini Tde F.
      • et al.
      Epignathus: two cases.
      • Zhang G.Z.
      • Din G.C.
      • Zhao Y.F.
      Giant epignathus teratoma: report of a case.
      • Liang C.C.
      • Lai J.P.
      • Lui C.C.
      Cleft palate with congenital midline teratoma.
      Nasopharyngeal teratomas, as in our case, are associated with an exceptionally high risk of neonatal mortality, particularly from airway obstruction. Polyhydramnios, hydrops, and cleft palate may also occur. Other tumors, such as pure oral teratomas, have also been reported. Of 15 reported cases of pure oral teratomas, fetal death occurred in 2 cases, 1 patient terminated the pregnancy, 2 other patients had cleft palate, and 1 neonate had nerve palsy.
      • Cay A.
      • Bektas D.
      • Imamoglu M.
      • Bahadir O.
      • Cobanoglu U.
      • Sarihan H.
      Oral teratoma: a case report and literature review.
      • April M.M.
      • Ward R.F.
      • Garelick J.M.
      Diagnosis, management, and follow-up of congenital head and neck teratomas.
      • Uchida K.
      • Urata H.
      • Suzuki H.
      Teratoma of the tongue in neonates: report of a case and review of the literature.
      • Wakhlu A.
      • Wakhlu A.K.
      Head and neck teratomas in children.
      • Bras G.
      • Butts D.
      • Hoyte D.A.
      Gliomatous teratoma of the tongue Report of a case.
      • Dudgeon D.L.
      • Isaacs Jr, H.
      • Hays D.M.
      Letter: multiple teratomas of the head and neck.
      • Ashley D.J.
      Origin of teratomas.
      • Zakaria M.A.
      Epignathus (congenital teratoma of the hard palate): a case report.
      • Ekici E.
      • Soysal M.
      • Kara S.
      • Dogan M.
      • Gokmen O.
      Prenatal diagnosis of epignathus causing acute polyhydramnios.
      Prior to the year 2000, this type of tumor was considered essentially incompatible with life.
      • Izadi K.
      • Smith M.
      • Askari M.
      • Hackam D.
      • Hameed A.A.
      • Bradley J.P.
      A patient with an epignathus: management of a large oropharyngeal teratoma in a newborn.
      In fact, a review article in 2008 stated that only 8 cases existed in which the teratoma had been successfully excised.
      • Kumar B.
      • Sharma S.B.
      Neonatal oral tumors: congenital epulis and epignathus.
      Perinatal management of oral teratomas includes delivery by cesarean section and either resection (OOPS
      • Skarsgard E.D.
      • Chitkara U.
      • Krane E.J.
      • Riley E.T.
      • Halamek L.P.
      • Dedo H.H.
      The OOPS procedure (operation on placental support): in utero airway management of the fetus with prenatally diagnosed tracheal obstruction.
      ) or an EXIT
      • Chiu H.H.
      • Hsu W.C.
      • Shih J.C.
      • Tsao P.N.
      • Hsieh W.S.
      • Chou H.C.
      The EXIT (ex utero intrapartum treatment) procedure.
      procedure with or without tracheostomy. Chiu et al
      • Chiu H.H.
      • Hsu W.C.
      • Shih J.C.
      • Tsao P.N.
      • Hsieh W.S.
      • Chou H.C.
      The EXIT (ex utero intrapartum treatment) procedure.
      reported 2 cases with prenatal diagnosis of upper airway obstruction. One case was an epignathus successfully treated with an EXIT procedure. Postnatal management of oral teratomas involves surgical resection, which is often curative, without recurrences if the initial surgery is successful.
      • Hossein A.
      • Mohammad A.
      Huge teratoma of the nasopharynx.
      Our case suggests that in utero fetoscopic management of fetuses with oral teratomas may be feasible in selected cases. In utero endoscopic assessment or treatment may allow parents to make a better educated decision about their management of the pregnancy. If done early enough, as in our case, fetoscopic removal of the teratoma may avoid further growth of the mass, distortion of the facial structures, micrognathia, development of polyhydramnios, airway obstruction, the need for an EXIT or OOPS procedure, and the need for cesarean section delivery. Further experience with in utero fetoscopic treatment may be difficult because of the rarity of the condition but deserves consideration.

      Acknowledgment

      We would like to thank our Fetal Therapy Coordinator, Ms Michaela Tregembo, RN; our sonographers, Ms Jill Osit and Ms Masohmeh Haghayeh; our operating room staff Ms Jazlene Daniel and Ms Jennifer King; our anesthesiologist Dr Bruce Saltzman; Dr Gaurav Saigal from pediatric radiology; and Dr Maria Rodriguez from surgical pathology for their invaluable assistance. We would also like to thank the University of Miami Miller School of Medicine and Jackson Memorial Hospital for allowing us to offer this novel treatment to our patient.

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