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A multidisciplinary team approach for management of a giant congenital cervical teratoma
International Journal of Pediatric Otorhinolaryngology 65 (2002) 241 /247 www.elsevier.com/locate/ijporl
Case report
A multidisciplinary team approach for management of a giant congenital cervical teratoma Jean-Yves Sichel a,*, Ron Eliashar a, Ido Yatsiv b, John Moshe Gomori c, Michel Nadjari d, Chaim Springer e, Yossef Ezra f a
Department of Otolaryngology/Head and Neck Surgery, Hadassah University Hospital, Jerusalem, Israel b Department of Pediatric Intensive Care Unit, Hadassah University Hospital, Jerusalem, Israel c Department of Radiology, Hadassah University Hospital, Jerusalem, Israel d Department of Ultrasound Unit, Hadassah University Hospital, Jerusalem, Israel e Department of Pediatric Pulmonology, Unit Hadassah University Hospital, Jerusalem, Israel f Department of Obstetrics and Gynecology, Hadassah University Hospital, Jerusalem, Israel Received 22 February 2002; received in revised form 8 May 2002; accepted 12 May 2002
Abstract Congenital cervical teratomas are associated with a high rate of perinatal mortality due to airway obstruction. We describe a multidisciplinary management of a neonate with prenatal diagnosis of giant cervical teratoma. An ‘operation on placenta ;>support’ (OOPS) technique was carried out during delivery, and intubation was successfully performed with no perinatal anoxic damage. Postnatal computed tomography and angiography showed a huge teratoma covering both sides of the neck with agenesis of the big blood vessels on the left side. A rapidly developing third space phenomenon and deterioration in the general status of the neonate, required early surgical intervention. During surgical excision, the left carotid artery and internal jugular vein, the left lobe of the thyroid gland and the left recurrent laryngeal nerve were not detected. The left vagus, accessory and hypoglossal nerves were positioned between the skin and the tumor, at a distance from their normal anatomical location. Pathologic examination confirmed the diagnosis indicating immature teratoma with no signs of malignancy. The postoperative period was complicated by neurological deterioration, pharyngo-cutaneous fistula and paresis of the left hypoglossal nerve. However, all the symptoms resolved spontaneously. Tracheotomy was performed when the baby was 6 weeks old due to paralysis of the left vocal cord and to severe laryngo-tracheomalacia. She was decannulated when she was 3 years old. Today, she is suffering only from dysphonia. This report confirms the efficacy of a multidisciplinary team-approach and the usefulness of the OOPS technique in prenatally diagnosed cervical masses. It emphasizes the extraordinary characteristics of this case, mainly the development of a third space phenomenon and the unusual surgical findings. # 2002 Elsevier Science Ireland Ltd. All rights reserved. Keywords: Congenital; Teratoma; Neck; OOPS procedure; Surgical treatment
* Corresponding author. Tel.: /972-2-6776469; fax: /972-2-6779800 E-mail address: [email protected] (J.-Y. Sichel). 0165-5876/02/$ - see front matter # 2002 Elsevier Science Ireland Ltd. All rights reserved. PII: S 0 1 6 5 - 5 8 7 6 ( 0 2 ) 0 0 1 5 4 - 4
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1. Introduction Teratomas are rare tumors composed of tissue originating from all three embryonic layers. The histologic appearance of these tumors is most often benign [1]. Immaturity in congenital cases is not considered a sign of malignancy [2,3]. The term ‘malignant’ is kept for cases with histological signs of malignancy or with distant metastases. Congenital teratomas are usually found in the sacrococcygeal region, only 5% are located in the head or neck [4]. When found in this region, high perinatal mortality occurs [5 /7], mainly as a result of neonatal upper airway obstruction. The widespread utilization of antenatal ultrasonography enables prenatal diagnosis and thus improves perinatal management and outcome. On ultrasound, a semi-solid, semi-cystic cervical mass is suggestive of a teratoma [8]. Operation on placenta support (OOPS), which maintains the foeto-maternal circulation after delivery, provides time for airway management in prenatally diagnosed congenital cervical masses [9 /12]. The umbilical cord is clamped only after the airway has been secured by intubation or by tracheotomy thus providing the baby with oxygenated blood flow. We present a multidisciplinary team-approach to a case with a giant congenital cervical teratoma. The OOPS procedure was used to secure the airway. Post delivery management and surgical excision of the mass were problematic and are discussed.
dual enlargement of the tumor accompanied by polyhydramnios. At 36 weeks of pregnancy an elective caesarian section was performed. A multidisciplinary team including obstetricians, anesthesiologists, intensive care pediatricians, otolaryngologists, pediatric pulmonologists and an ultrasonographist participated. Anesthesia was induced by thiopenthal and intubation was facilitated by intravenous succinylcholine. Maintenance was done by isoflurane in oxygen and atracurium as needed. Under ultrasonic guidance, 300 ml of fluid were aspirated from the cystic component of the tumor in order to enable easier extraction of the neonate without traumatizing the tumor. After the baby was delivered by a caesarian section, she was placed on an adjacent table without clamping the umbilical cord. The placenta was left in situ (OOPS procedure). The tumor was found to cover both sides of the neck, predominately on the left side. It extended from the auricle to the clavicle (Fig. 1). Direct laryngoscopy showed the larynx, hypopharynx and floor of mouth severely compressed by the tumor. Only the tip of the epiglottis was visualized. After several attempts, and within 3 min from delivery, endotracheal intubation was successfully performed under guidance of a fiberoptic bronchoscope. After the airway was secured, the cord was clamped and the placenta extracted. The
2. Case report A 27-year-old multiparous woman underwent routine antenatal ultrasonography at 24 weeks gestation. The fetal sonogram showed a 6 /5 cm mass on the left side of the neck. The mass was composed of a cystic portion and a solid portion containing blood vessels. A teratoma was suspected. There was no polyhydramnios and the tracheal cavity was open. These findings and their potential consequences were discussed with the parents who preferred to continue the pregnancy. Follow-up ultrasonic examinations showed a gra-
Fig. 1. The neonate immediately after delivery during intubation attempt. The huge cervical teratoma is evident. The child is still on feto-maternal circulation (OOPS procedure). The intact umbilical cord is visible (arrowhead).
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Fig. 2. An axial CT-scan with contrast material performed on the 1st day of life. The right internal jugular vein (black arrow), the right common carotid artery (black arrowhead), the trachea (visualized by the endotracheal tube /white arrow) and the esophagus (identified by the feeding tube near the trachea) are seen. No other organs are recognizable in the anterior neck. The teratoma is located on both sides of the neck. It is partially solid and partially cystic with some calcifications.
child was then transferred to the pediatric intensive care unit. Physical examination, chest radiography and abdominal and cardiac ultrasonography performed on the same day did not reveal any malformations except the mass on the neck. A contrast computed tomography (CT) scan of the head and neck revealed a partially cystic, partially solid mass with calcifications, covering both sides of the neck. Except for the right common carotid artery, the trachea and the esophagus, it was hard to recognize any normal anatomy in the anterior neck (Fig. 2). On the left side, neither the common carotid artery nor the internal jugular vein could be identified. Angiography confirmed the absence of the left common carotid artery (Fig. 3). On the following day (day 1 after delivery), the cystic part of the tumor began to grow rapidly and the teratoma measured 18 cm on its largest diameter. The baby’s general condition was deteriorating. Abdominal ultrasonography, chest radiography and blood tests revealed ascites, bilateral pleural effusion, hypoalbuminemia (23 g/l); thrombocytopenia (43,000/mm3) and a coa-
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gulopathy (INR /2.93). A third space phenomenon due to the giant teratoma was suspected. On the next day (day 2 after delivery), because of further deterioration, excision of the tumor seemed to be a last resort to cure this phenomenon and to save the baby’s life. Thrombocytes and fresh frozen plasma were given prior to the operation. As a subclavian line was being inserted in preparation for the general anesthesia, a pneumothorax developed and was immediately evacuated by a chest drain. The surgical excision was performed in part with the aid of an operating microscope. The teratoma was first dissected from the right side of the neck. On that side, the carotid artery and the hypoglossal and vagus nerves were easily identified and preserved. On the left side however, the hypoglossal, vagus and accessory nerves were found to be under the skin, lateral to the cystic part of the tumor. They were very elongated and far off from their normal anatomical position. A relatively small artery, possibly the remnant of the left common carotid artery, was found adjacent to the left vagus nerve. In the midline, the mass was adherent to the strap muscles, the larynx, and the pharynx. The hypo-
Fig. 3. Angiography performed on the 1st day of life. The ascending aorta (A), the innominate artery (1), the right subclavian artery (2), the right common carotid artery (3), the right vertebral artery (4), the left subclavian artery (5), and the left vertebral artery (6) are easily recognized. A big vessel is seen going to the tumor (7), possibly the left transverse cervical artery. No left common carotid artery can be identified.
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pharyngeal lumen had to be violated in several places during resection of the tumor. The thyroid and cricoid cartilages together with the trachea were distorted by the teratoma. The thyroid cartilage had an inverse angulation and was open anteriorly. The left lobe of the thyroid gland was absent and the left recurrent laryngeal nerve could not be found. The right lobe was adherent to the tumor and was preserved with its feeding vessels. Excess skin was excised. During surgery, an episode of severe bradycardia and hypotension occurred lasting about 5 min and necessitating resuscitation. The cause of this event was a default of the chest drain, which caused a temporary tension pneumothorax. The solid part of the excised tumor weighed 330 g. Histologic examination confirmed the diagnosis of immature congenital teratoma with no evidence of malignancy. Skin, cartilage, muscle, choroid plexus, placenta, fibrous tissue, and primitive neuroectoderm tissue were found in the tumor. During the immediate postoperative period, the neurological status deteriorated. The pupils were non-reactive. This condition resolved within 24 h. The third space phenomenon resolved and did not recur. A pharyngo-cutaneous fistula developed a week after surgery. It was treated conservatively and closed after 3 weeks. Paresis of the left hypoglossal nerve was also noted and resolved spontaneously within several weeks. Extubation was first attempted 3 weeks after the operation, but it failed due to severe stridor and respiratory distress. Fiberoptic laryngoscopy showed severe laryngotracheomalacia and paralysis of the left vocal cord. A tracheotomy was performed after 4 unsuccessful attempts of extubation. After the tracheotomy, the child began to eat with no evidence of aspiration. Thyroid-scan showed a functioning right thyroid lobe. The child was discharged when she was 3 months old and continued to develop normally after that (Fig. 4). When she was 3 years old she was decannulated. She is now 4 years old with no evidence of any recurrence. The only remaining problem is dysphonia which is being treated by a speech therapist.
Fig. 4. The girl at the age of 9 months. The tracheotomy is still in place.
3. Discussion 3.1. Perinatal management Cervical teratomas may cause severe airway obstruction. In a review of 163 congenital cases, Jordan and Gauderer [6], described three groups: a) 27 stillborns and moribund live newborns. b) 99 newborns with respiratory distress. c) 34 newborns without respiratory distress. Mortality rate was 100, 43.4 and 2.7%, respectively. Dramatic improvement in the management of neonates suffering from congenital neck masses has occurred during the past decade. This development is associated with the widespread use of prenatal ultrasonography enabling prenatal diagnosis of these masses. When upper airway impairment is anticipated, the basis for successful management is to perform an early elective cesarean delivery (before spontaneous labor begins) and to leave the baby on foeto-maternal circulation until the airway is secured. Essentially two procedures may be used: EXIT (EX Utero Intrapartum Treatment) and OOPS (Operation On Placenta Support). In the EXIT procedure, after a low transverse uterine incision, the head and at least one hand of the fetus are delivered. The rest of the body along with the umbilical cord
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and the placenta remain in-utero. This allows examination of the airway with a laryngoscope and a bronchoscope, intubation or tracheotomy if necessary, and monitoring of the fetus with a pulse oximeter and EKG [9 /11,13 /16]. In the OOPS procedure, the baby is completely delivered, placed on a table adjacent to the mother with preservation of the umbilical cord, which is clamped only after the airway has been secured by intubation or by tracheotomy [2,17,18]. In both procedures, maximum uterine relaxation is important in order to avoid separation of the placenta from the uterus and thus, maintenance of foeto-maternal circulation is ensured [9]. In addition to cases with cervical teratomas, these techniques have been used with patients suffering from cervical lymphangioma [9 /11,14,19], cervicofacial hemangioma [10,15], laryngeal atresia [16,19], tongue rhabdomyosarcoma [19], congenital goiter [19], and for removing a tracheal clip used in-utero when treating congenital diaphragmatic hernia [9]. The EXIT and OOPS procedures are not entirely undangerous for the mother. Uterine relaxation necessary to avoid placental separation may increase hemorrhage from the uterus. This complication should be discussed with the parents before deciding on the procedure. In addition, they should be informed that a caesarian delivery may be necessary in future pregnancies [11]. In addition to the OOPS and EXIT procedures, other techniques have been proposed for managing neonates with complicated airways. Extra corporeal membrane oxygenation (ECMO), cannulation of the umbilical vessels [10 /12], or open fetal surgery [20], have all been suggested, but to the best of our knowledge, were never performed in patients with congenital cervical teratomas.
3.2. Postnatal management Perinatal management was uneventful in our case when the OOPS procedure was used. However, the postnatal period was quite complicated. Several hours after delivery, the tumor began to grow rapidly and a third space phenomenon with
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hypoalbuminemia, ascites, pleural effusion, thrombocytopenia and coagulopathia developed. Similar phenomena have been described before, but only in-utero: rapid growth of a fetal sacrococcygeal teratoma was associated with polyhydramnios, placentomegaly, fetal hydrops and a possible maternal mirror syndrome. This was attributed to ‘vascular steal’ of the tumor from the placenta [20,21]. Another report described an in-utero thrombocytopenia (36,000/mm3) in a fetus with faciocervical teratoma. In this case, the pregnancy was interrupted. The author assumed that this phenomenon was caused by a highly vascular lesion creating a low-pressure shunt which caused sequestration of the fetal blood [22]. We did not find any report on thrombocytopenia or third space phenomenon in live neonates suffering from a congenital cervical teratoma. This condition forced us to operate in a non-optimal situation and was resolved after removal of the tumor. Surgical excision of the tumor was not easy due to adherence of the tumor to surrounding structures, essentially in the midline. Even though most benign congenital cervical teratomas are usually encapsulated and do not infiltrate into the surrounding tissue [23], adherence and even invasion have been described [2,24/26]. It has been attributed to possible action of proteolytic enzymes produced by salivary and pancreatic tissue within the tumor [27,28]. Another interesting finding was observed during the surgical excision. Unexpectedly, several structures were found at a distance from their normal anatomical location. Actually, the left vagus, accessory and hypoglossal nerves were found between the skin and the tumor. However, after they were identified, dissection from the teratoma was quite easy. Postoperatively, the child suffered from temporary paresis of the hypoglossal nerve and from a left vocal cord paralysis. We attributed the latter finding to an injury of the recurrent laryngeal nerve, which was not identified during the dissection. Since the left lobe of the thyroid gland could not be found, we tend to believe that the origin of the teratoma was in the thyroid gland.
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4. Conclusion Giant congenital cervical teratoma is a challenging lesion. Surgical excision of a large lesion such as in our case is mostly anecdotal and has been described in literature only recently. Evidently the reason lies in the rarity of these tumors and in the fact that most fetuses without prenatal diagnosis did not survive the delivery. The present case demonstrates that the embryological development of the neck may be disturbed by a tumor. Important structures such as cranial nerves or great blood vessels may therefore be situated in non-anatomical sites. Surgical procedures in such cases must be performed with great care. The possibility of rapid deterioration of the general condition due to third space phenomenon should be considered in such huge lesions. Successful management depends on a multidisciplinary team approach and on careful planning of the delivery, the surgical procedure, and post-operative management.
References [1] R.G. Azizkhan, G.M. Haase, H. Applebaum, P.W. Dillon, A.G. Coran, P.A. King, et al., Diagnosis, management, and outcome of cervicofacial teratomas in neonates: a children cancer group study, J. Pediatr. Surg. 30 (1995) 312 /316. [2] M.A. Rothschild, P. Catalano, M. Urken, M. Randwein, P. Som, K. Norton, et al., Evaluation and management of congenital cervical teratoma, Arch. Otolaryngol. Head Neck Surg. 120 (1994) 444 /448. [3] J.G. Batsakis, A. El-Naggar, M.A. Luna, Pathology consultation. Teratomas of the head and neck with emphasis on malignancy, Ann. Otol. Rhinol. Laryngol. 104 (1995) 496 /500. [4] E.E. Lack, Extragonadal germ-cell tumors of the head and neck region: review of 16 cases, Hum. Pathol. 16 (1985) 56 /64. [5] A.L. Gagnon, M.W. Bebbington, A. Kamami, A. Solimano, Prenatally diagnosed fetal neck teratoma. Case report and novel management options, Fetal. Diagn. Ther. 13 (1998) 266 /270. [6] R.B. Jordan, M.W.L. Gauderer, Cervical teratomas: an analysis. Literature review and proposed classification, J. Pediatr. Surg. 23 (1988) 583 /591. [7] S.R. Gundry, J.R. Wesley, M.D. Klein, M. Barr, A.G. Coran, Cervical teratomas in the newborn, J. Pediatr. Surg. 18 (1983) 382 /386.
[8] J.C. Trecet, V. Claramunt, J. Larraz, E. Ruiz, M. Zuzuarregui, F.J. Ugalde, Prenatal ultrasound diagnosis of fetal teratomas of the neck, J. Clin. Ultrasound 12 (1984) 509 /511. [9] G.B. Mychaliska, J.F. Bealer, J.L. Graf, M.A. Rosen, N.S. Adzick, M.R. Harrison, Operation on placenta support: the Ex Utero Intrapartum treatment procedure, J. Pediatr. Surg. 32 (1997) 227 /231. [10] R.M.S. Stocks, R.S. Egerman, G.E. Woodson, C.M. Bower, J.W. Thompson, G.J. Wiet, Airway management of neonates with antenatally detected head and neck anomalies, Arch. Otolaryngol. Head Neck Surg. 123 (1997) 641 /645. [11] K.W. Liechty, T.M. Crombleholme, A.W. Flake, M.A. Morgan, C.D. Kurth, A.M. Hubbard, N.S. Adzick, Intrapartum airway management for giant fetal neck masses: the EXIT (ex utero intrapartum treatment) procedure, Am. J. Obstet. Gynecol. 177 (1997) 870 /874. [12] M.F. Kelly, L. Berenholz, K.A. Rizzo, R. Greco, P. Wolfson, D.A. Zwillenberg, Approach for oxygenation of the newborn with airway obstruction due to a cervical mass, Ann. Otol. Rhinol. Laryngol. 99 (1990) 179 /182. [13] G.H. Shih, G.L. Boyd, R.D. Vincent, G.W. Long, J.C. Hauth, K.E. Georgeson, The EXIT procedure facilitates delivery of an infant with a pretracheal teratoma, Anesthesiology 89 (1998) 1573 /1575. [14] E.D. Skarsgard, U. Chitkara, E.J. Krane, E.T. Riley, L.P. Halamek, H.H. Dedo, The OOPS procedure (operation on placenta support): in utero airway management of the fetus with prenatally diagnosed tracheal obstruction, J. Pediatr. Surg. 31 (1996) 826 /828. [15] M.Z. Schwartz, H. Silver, S. Schulman, Maintenance of the placental circulation to evaluate and treat an infant with massive head and neck hemangioma, J. Pediatr. Surg. 28 (1993) 520 /522. [16] J.M. DeCou, D.C. Jones, H.D. Jacobs, R.J. Touloukian, Successful ex utero intrapartum treatment (EXIT) procedure for congenital high airway obstruction syndrome (CHAOS) owing to laryngeal atresia, J. Pediatr. Surg. 33 (1998) 1563 /1565. [17] P.J. Catalano, M.L. Urken, M. Alvarez, K. Norton, J. Wedgewood, I. Holzman, H. Biller, New approach to the management of airway obstruction in ‘high risk’ neonates, Arch. Otolaryngol. Head Neck Surg. 118 (1992) 306 /309. [18] M.F. Kelly, L. Berenholz, K.A. Rizzo, R. Greco, P. Wolfson, D.A. Zwillenberg, Approach for oxygenation of the newborn with airway obstruction due to a cervical mass, Ann. Otol. Rhinol. Laryngol. 99 (1989) 179 /182. [19] V.M.M. Ward, K. Langford, G. Morrison, Prenatal diagnosis of airway compromise: EXIT (ex utero intrapartum treatment) and fetal airway surgery, Int. J. Pediatr. Otorhinolaryngol. 53 (2000) 137 /141. [20] Y. Kitano, A.W. Flake, T.M. Crombleholme, M.P. Johnson, S. Adzick, Open fetal surgery for life-threatening
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fetal malformations, Semin. Perinatalogy 23 (1999) 448 / 461. N.S. Adzick, T.M. Crombleholme, M.A. Morgan, T.M. Quinn, A rapidly growing fetal teratoma, Lancet 349 (1997) 538. G. Brown, L. Baxi, A. Monteagudo, T. Tharakan, A. Saad, S. Sharma, Fetal faciocervical teratoma with anemia and thrombocytopenia, J. Reproduct. Med. 40 (1995) 80 / 82. M.M. April, R.F. Ward, J.M. Garelick, Diagnosis management, and follow-up of congenital head and neck teratomas, Laryngoscope 108 (1998) 1398 /1401. W.I. Kuhel, M. Yagoda, P. Pereson, Benign cervical teratoma in the adult: report of a rare case with dense
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fibrosis involving adjacent vital structures, Oto. Laryngol. Head Neck Surg. 115 (1996) 152 /155. N.B. Bikhazi, T.P. Eriksson, M.I. Singer, Pathological quiz case 2, Arch. Otolaryngol. Head Neck Surg. 124 (1998) 927. M.M. Carr, P. Thorner, J.H. Phillips, Congenital teratomas of the head and neck, J. Otolaryngol. 26 (1997) 246 / 252. W.I. Kuhel, Adherence of benign cervical teratomas to surrounding soft tissue. Letter to the Editor, Arch. Otolaryngol. Head Neck Surg. 125 (1999) 236 /237. J. Southgate, P.R. Slade, Teratomatoid cyst of the mediastinum with pancreatic enzyme secretion, Thorax 37 (1983) 476 /477.
Case report
A multidisciplinary team approach for management of a giant congenital cervical teratoma Jean-Yves Sichel a,*, Ron Eliashar a, Ido Yatsiv b, John Moshe Gomori c, Michel Nadjari d, Chaim Springer e, Yossef Ezra f a
Department of Otolaryngology/Head and Neck Surgery, Hadassah University Hospital, Jerusalem, Israel b Department of Pediatric Intensive Care Unit, Hadassah University Hospital, Jerusalem, Israel c Department of Radiology, Hadassah University Hospital, Jerusalem, Israel d Department of Ultrasound Unit, Hadassah University Hospital, Jerusalem, Israel e Department of Pediatric Pulmonology, Unit Hadassah University Hospital, Jerusalem, Israel f Department of Obstetrics and Gynecology, Hadassah University Hospital, Jerusalem, Israel Received 22 February 2002; received in revised form 8 May 2002; accepted 12 May 2002
Abstract Congenital cervical teratomas are associated with a high rate of perinatal mortality due to airway obstruction. We describe a multidisciplinary management of a neonate with prenatal diagnosis of giant cervical teratoma. An ‘operation on placenta ;>support’ (OOPS) technique was carried out during delivery, and intubation was successfully performed with no perinatal anoxic damage. Postnatal computed tomography and angiography showed a huge teratoma covering both sides of the neck with agenesis of the big blood vessels on the left side. A rapidly developing third space phenomenon and deterioration in the general status of the neonate, required early surgical intervention. During surgical excision, the left carotid artery and internal jugular vein, the left lobe of the thyroid gland and the left recurrent laryngeal nerve were not detected. The left vagus, accessory and hypoglossal nerves were positioned between the skin and the tumor, at a distance from their normal anatomical location. Pathologic examination confirmed the diagnosis indicating immature teratoma with no signs of malignancy. The postoperative period was complicated by neurological deterioration, pharyngo-cutaneous fistula and paresis of the left hypoglossal nerve. However, all the symptoms resolved spontaneously. Tracheotomy was performed when the baby was 6 weeks old due to paralysis of the left vocal cord and to severe laryngo-tracheomalacia. She was decannulated when she was 3 years old. Today, she is suffering only from dysphonia. This report confirms the efficacy of a multidisciplinary team-approach and the usefulness of the OOPS technique in prenatally diagnosed cervical masses. It emphasizes the extraordinary characteristics of this case, mainly the development of a third space phenomenon and the unusual surgical findings. # 2002 Elsevier Science Ireland Ltd. All rights reserved. Keywords: Congenital; Teratoma; Neck; OOPS procedure; Surgical treatment
* Corresponding author. Tel.: /972-2-6776469; fax: /972-2-6779800 E-mail address: [email protected] (J.-Y. Sichel). 0165-5876/02/$ - see front matter # 2002 Elsevier Science Ireland Ltd. All rights reserved. PII: S 0 1 6 5 - 5 8 7 6 ( 0 2 ) 0 0 1 5 4 - 4
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1. Introduction Teratomas are rare tumors composed of tissue originating from all three embryonic layers. The histologic appearance of these tumors is most often benign [1]. Immaturity in congenital cases is not considered a sign of malignancy [2,3]. The term ‘malignant’ is kept for cases with histological signs of malignancy or with distant metastases. Congenital teratomas are usually found in the sacrococcygeal region, only 5% are located in the head or neck [4]. When found in this region, high perinatal mortality occurs [5 /7], mainly as a result of neonatal upper airway obstruction. The widespread utilization of antenatal ultrasonography enables prenatal diagnosis and thus improves perinatal management and outcome. On ultrasound, a semi-solid, semi-cystic cervical mass is suggestive of a teratoma [8]. Operation on placenta support (OOPS), which maintains the foeto-maternal circulation after delivery, provides time for airway management in prenatally diagnosed congenital cervical masses [9 /12]. The umbilical cord is clamped only after the airway has been secured by intubation or by tracheotomy thus providing the baby with oxygenated blood flow. We present a multidisciplinary team-approach to a case with a giant congenital cervical teratoma. The OOPS procedure was used to secure the airway. Post delivery management and surgical excision of the mass were problematic and are discussed.
dual enlargement of the tumor accompanied by polyhydramnios. At 36 weeks of pregnancy an elective caesarian section was performed. A multidisciplinary team including obstetricians, anesthesiologists, intensive care pediatricians, otolaryngologists, pediatric pulmonologists and an ultrasonographist participated. Anesthesia was induced by thiopenthal and intubation was facilitated by intravenous succinylcholine. Maintenance was done by isoflurane in oxygen and atracurium as needed. Under ultrasonic guidance, 300 ml of fluid were aspirated from the cystic component of the tumor in order to enable easier extraction of the neonate without traumatizing the tumor. After the baby was delivered by a caesarian section, she was placed on an adjacent table without clamping the umbilical cord. The placenta was left in situ (OOPS procedure). The tumor was found to cover both sides of the neck, predominately on the left side. It extended from the auricle to the clavicle (Fig. 1). Direct laryngoscopy showed the larynx, hypopharynx and floor of mouth severely compressed by the tumor. Only the tip of the epiglottis was visualized. After several attempts, and within 3 min from delivery, endotracheal intubation was successfully performed under guidance of a fiberoptic bronchoscope. After the airway was secured, the cord was clamped and the placenta extracted. The
2. Case report A 27-year-old multiparous woman underwent routine antenatal ultrasonography at 24 weeks gestation. The fetal sonogram showed a 6 /5 cm mass on the left side of the neck. The mass was composed of a cystic portion and a solid portion containing blood vessels. A teratoma was suspected. There was no polyhydramnios and the tracheal cavity was open. These findings and their potential consequences were discussed with the parents who preferred to continue the pregnancy. Follow-up ultrasonic examinations showed a gra-
Fig. 1. The neonate immediately after delivery during intubation attempt. The huge cervical teratoma is evident. The child is still on feto-maternal circulation (OOPS procedure). The intact umbilical cord is visible (arrowhead).
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Fig. 2. An axial CT-scan with contrast material performed on the 1st day of life. The right internal jugular vein (black arrow), the right common carotid artery (black arrowhead), the trachea (visualized by the endotracheal tube /white arrow) and the esophagus (identified by the feeding tube near the trachea) are seen. No other organs are recognizable in the anterior neck. The teratoma is located on both sides of the neck. It is partially solid and partially cystic with some calcifications.
child was then transferred to the pediatric intensive care unit. Physical examination, chest radiography and abdominal and cardiac ultrasonography performed on the same day did not reveal any malformations except the mass on the neck. A contrast computed tomography (CT) scan of the head and neck revealed a partially cystic, partially solid mass with calcifications, covering both sides of the neck. Except for the right common carotid artery, the trachea and the esophagus, it was hard to recognize any normal anatomy in the anterior neck (Fig. 2). On the left side, neither the common carotid artery nor the internal jugular vein could be identified. Angiography confirmed the absence of the left common carotid artery (Fig. 3). On the following day (day 1 after delivery), the cystic part of the tumor began to grow rapidly and the teratoma measured 18 cm on its largest diameter. The baby’s general condition was deteriorating. Abdominal ultrasonography, chest radiography and blood tests revealed ascites, bilateral pleural effusion, hypoalbuminemia (23 g/l); thrombocytopenia (43,000/mm3) and a coa-
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gulopathy (INR /2.93). A third space phenomenon due to the giant teratoma was suspected. On the next day (day 2 after delivery), because of further deterioration, excision of the tumor seemed to be a last resort to cure this phenomenon and to save the baby’s life. Thrombocytes and fresh frozen plasma were given prior to the operation. As a subclavian line was being inserted in preparation for the general anesthesia, a pneumothorax developed and was immediately evacuated by a chest drain. The surgical excision was performed in part with the aid of an operating microscope. The teratoma was first dissected from the right side of the neck. On that side, the carotid artery and the hypoglossal and vagus nerves were easily identified and preserved. On the left side however, the hypoglossal, vagus and accessory nerves were found to be under the skin, lateral to the cystic part of the tumor. They were very elongated and far off from their normal anatomical position. A relatively small artery, possibly the remnant of the left common carotid artery, was found adjacent to the left vagus nerve. In the midline, the mass was adherent to the strap muscles, the larynx, and the pharynx. The hypo-
Fig. 3. Angiography performed on the 1st day of life. The ascending aorta (A), the innominate artery (1), the right subclavian artery (2), the right common carotid artery (3), the right vertebral artery (4), the left subclavian artery (5), and the left vertebral artery (6) are easily recognized. A big vessel is seen going to the tumor (7), possibly the left transverse cervical artery. No left common carotid artery can be identified.
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pharyngeal lumen had to be violated in several places during resection of the tumor. The thyroid and cricoid cartilages together with the trachea were distorted by the teratoma. The thyroid cartilage had an inverse angulation and was open anteriorly. The left lobe of the thyroid gland was absent and the left recurrent laryngeal nerve could not be found. The right lobe was adherent to the tumor and was preserved with its feeding vessels. Excess skin was excised. During surgery, an episode of severe bradycardia and hypotension occurred lasting about 5 min and necessitating resuscitation. The cause of this event was a default of the chest drain, which caused a temporary tension pneumothorax. The solid part of the excised tumor weighed 330 g. Histologic examination confirmed the diagnosis of immature congenital teratoma with no evidence of malignancy. Skin, cartilage, muscle, choroid plexus, placenta, fibrous tissue, and primitive neuroectoderm tissue were found in the tumor. During the immediate postoperative period, the neurological status deteriorated. The pupils were non-reactive. This condition resolved within 24 h. The third space phenomenon resolved and did not recur. A pharyngo-cutaneous fistula developed a week after surgery. It was treated conservatively and closed after 3 weeks. Paresis of the left hypoglossal nerve was also noted and resolved spontaneously within several weeks. Extubation was first attempted 3 weeks after the operation, but it failed due to severe stridor and respiratory distress. Fiberoptic laryngoscopy showed severe laryngotracheomalacia and paralysis of the left vocal cord. A tracheotomy was performed after 4 unsuccessful attempts of extubation. After the tracheotomy, the child began to eat with no evidence of aspiration. Thyroid-scan showed a functioning right thyroid lobe. The child was discharged when she was 3 months old and continued to develop normally after that (Fig. 4). When she was 3 years old she was decannulated. She is now 4 years old with no evidence of any recurrence. The only remaining problem is dysphonia which is being treated by a speech therapist.
Fig. 4. The girl at the age of 9 months. The tracheotomy is still in place.
3. Discussion 3.1. Perinatal management Cervical teratomas may cause severe airway obstruction. In a review of 163 congenital cases, Jordan and Gauderer [6], described three groups: a) 27 stillborns and moribund live newborns. b) 99 newborns with respiratory distress. c) 34 newborns without respiratory distress. Mortality rate was 100, 43.4 and 2.7%, respectively. Dramatic improvement in the management of neonates suffering from congenital neck masses has occurred during the past decade. This development is associated with the widespread use of prenatal ultrasonography enabling prenatal diagnosis of these masses. When upper airway impairment is anticipated, the basis for successful management is to perform an early elective cesarean delivery (before spontaneous labor begins) and to leave the baby on foeto-maternal circulation until the airway is secured. Essentially two procedures may be used: EXIT (EX Utero Intrapartum Treatment) and OOPS (Operation On Placenta Support). In the EXIT procedure, after a low transverse uterine incision, the head and at least one hand of the fetus are delivered. The rest of the body along with the umbilical cord
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and the placenta remain in-utero. This allows examination of the airway with a laryngoscope and a bronchoscope, intubation or tracheotomy if necessary, and monitoring of the fetus with a pulse oximeter and EKG [9 /11,13 /16]. In the OOPS procedure, the baby is completely delivered, placed on a table adjacent to the mother with preservation of the umbilical cord, which is clamped only after the airway has been secured by intubation or by tracheotomy [2,17,18]. In both procedures, maximum uterine relaxation is important in order to avoid separation of the placenta from the uterus and thus, maintenance of foeto-maternal circulation is ensured [9]. In addition to cases with cervical teratomas, these techniques have been used with patients suffering from cervical lymphangioma [9 /11,14,19], cervicofacial hemangioma [10,15], laryngeal atresia [16,19], tongue rhabdomyosarcoma [19], congenital goiter [19], and for removing a tracheal clip used in-utero when treating congenital diaphragmatic hernia [9]. The EXIT and OOPS procedures are not entirely undangerous for the mother. Uterine relaxation necessary to avoid placental separation may increase hemorrhage from the uterus. This complication should be discussed with the parents before deciding on the procedure. In addition, they should be informed that a caesarian delivery may be necessary in future pregnancies [11]. In addition to the OOPS and EXIT procedures, other techniques have been proposed for managing neonates with complicated airways. Extra corporeal membrane oxygenation (ECMO), cannulation of the umbilical vessels [10 /12], or open fetal surgery [20], have all been suggested, but to the best of our knowledge, were never performed in patients with congenital cervical teratomas.
3.2. Postnatal management Perinatal management was uneventful in our case when the OOPS procedure was used. However, the postnatal period was quite complicated. Several hours after delivery, the tumor began to grow rapidly and a third space phenomenon with
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hypoalbuminemia, ascites, pleural effusion, thrombocytopenia and coagulopathia developed. Similar phenomena have been described before, but only in-utero: rapid growth of a fetal sacrococcygeal teratoma was associated with polyhydramnios, placentomegaly, fetal hydrops and a possible maternal mirror syndrome. This was attributed to ‘vascular steal’ of the tumor from the placenta [20,21]. Another report described an in-utero thrombocytopenia (36,000/mm3) in a fetus with faciocervical teratoma. In this case, the pregnancy was interrupted. The author assumed that this phenomenon was caused by a highly vascular lesion creating a low-pressure shunt which caused sequestration of the fetal blood [22]. We did not find any report on thrombocytopenia or third space phenomenon in live neonates suffering from a congenital cervical teratoma. This condition forced us to operate in a non-optimal situation and was resolved after removal of the tumor. Surgical excision of the tumor was not easy due to adherence of the tumor to surrounding structures, essentially in the midline. Even though most benign congenital cervical teratomas are usually encapsulated and do not infiltrate into the surrounding tissue [23], adherence and even invasion have been described [2,24/26]. It has been attributed to possible action of proteolytic enzymes produced by salivary and pancreatic tissue within the tumor [27,28]. Another interesting finding was observed during the surgical excision. Unexpectedly, several structures were found at a distance from their normal anatomical location. Actually, the left vagus, accessory and hypoglossal nerves were found between the skin and the tumor. However, after they were identified, dissection from the teratoma was quite easy. Postoperatively, the child suffered from temporary paresis of the hypoglossal nerve and from a left vocal cord paralysis. We attributed the latter finding to an injury of the recurrent laryngeal nerve, which was not identified during the dissection. Since the left lobe of the thyroid gland could not be found, we tend to believe that the origin of the teratoma was in the thyroid gland.
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4. Conclusion Giant congenital cervical teratoma is a challenging lesion. Surgical excision of a large lesion such as in our case is mostly anecdotal and has been described in literature only recently. Evidently the reason lies in the rarity of these tumors and in the fact that most fetuses without prenatal diagnosis did not survive the delivery. The present case demonstrates that the embryological development of the neck may be disturbed by a tumor. Important structures such as cranial nerves or great blood vessels may therefore be situated in non-anatomical sites. Surgical procedures in such cases must be performed with great care. The possibility of rapid deterioration of the general condition due to third space phenomenon should be considered in such huge lesions. Successful management depends on a multidisciplinary team approach and on careful planning of the delivery, the surgical procedure, and post-operative management.
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