Int. J. Oral Maxillofac. Surg. 2014; 43: 433–436 http://dx.doi.org/10.1016/j.ijom.2013.09.004, available online at http://www.sciencedirect.com
Case Report Cleft and Craniofacial Disorders
Emergency management of a congenital teratoma of the oral cavity at birth and three-year follow-up
S. Dakpe´1,*, B. Demeer2,3, C. Cordonnier4, B. Devauchelle1 1
Department of Maxillofacial Surgery and Stomatology, University Hospital Amiens, France; 2Department of Prenatal Diagnosis, Gynaecology and Obstetrics, University Hospital Amiens, France; 3Department of Medical Genetics, Rare Disease Centre, University Hospital Amiens, France; 4 Department of Anatomopathology, University Hospital Amiens, France
S. Dakpe´, B. Demeer, C. Cordonnier, B. Devauchelle: Emergency management of a congenital teratoma of the oral cavity at birth and three-year follow-up. Int. J. Oral Maxillofac. Surg. 2014; 43: 433–436. # 2014 International Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved. Abstract. Teratomas are congenital malformations that are rarely located in the head and neck region. We report a case of congenital teratoma of the oral cavity, which was causing an airway obstruction and was treated at the time of birth. This teratoma was discovered at 27 gestational weeks by ultrasonography. A multidisciplinary team was consulted for antenatal diagnosis; the options of therapeutic abortion or management of the birth with the prevention of respiratory distress were debated. However, preterm labour at 32 gestational weeks accelerated the parental and the medical decisions. The parents agreed to the birth. The various disciplines coordinated their work, and the predefined treatment plan for clearing the airway obstruction was applied to manage the birth. The reestablishment of patency of the airway was performed during delivery and removal of the tumour was performed immediately afterwards. The follow-up of this case over 3 years is also presented.
From the Greek root ‘tera’, meaning monstrous, teratomas are congenital malformations that occur in one out of every 4000 births; they are rarely located in the head or neck (2%). The discovery of an obstructing teratoma of the upper aerodigestive tract during the antenatal period is a challenging clinical scenario. As a direct consequence of the rarity of teratomas of the head and neck, most clinicians have very limited experience with the management of these tumours.1 The case presented here demonstrates that severe respiratory obstructions can be managed 0901-5027/040433 + 04 $36.00/0
and resection should be performed without wasting any time. Furthermore, successful management of severe respiratory obstructions caused by these tumours requires the timely coordination of various disciplines, including maxillofacial surgeons, neonatalogists, anaesthetists, and high-risk obstetrics specialists at the time of the birth. We report the successful emergency management of a congenital teratoma of the oral cavity in a premature infant. The paediatric development of this patient has remained normal to date.
Keywords: teratoma; antenatal diagnosis; EXIT procedure; cleft palate; neonatal surgery; epignathus. Accepted for publication 3 September 2013 Available online 24 January 2014
Case report
Following a second trimester morphological foetal ultrasound examination, a 28-year-old woman, who was gravida 3 para 2, was referred to our medical centre for a suspicion of an oral tumour, which was confirmed. A large mass of 35 mm 20 mm was detected, with coexisting cystic and solid tissues. The oral cavity appeared to be blocked by this mass. Subsequently, a hydramnios developed (Fig. 1A and B). Amniocentesis was performed and revealed a normal 46, XX foetus. Foetal
# 2014 International Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.
434
Dakpe´ et al.
Fig. 1. (A) Ultrasound examination at gestational week 28 showing a large mass with coexisting cystic and solid tissues. The exteriorized mass measured 35 mm 20 mm. Hydramnios was visualized (lateral view). (B) 3D ultrasound examination at gestational week 28 showing a large mass; the oral cavity appeared to be totally blocked by this mass. (C) Premature infant (32 gestational weeks) with a large teratoma blocking the oral cavity after nasofibroscopic intubation during the EXIT procedure. (D) Cleft palate and tumoural remnant located in the right oropharynx on day 1. Closure of the large cleft palate was performed at 19 months with a calvarial periosteal graft. (E) Cleft palate and tumoural remnant located in the right oropharynx on day 7. (F) Face of the patient at 36 months. (G) Endobuccal view of the patient at 36 months.
magnetic resonance imaging (MRI) was performed at 29 gestational weeks to attempt to accurately define the extent and nature of the tumour. The MRI showed a mass measuring 7 cm 5 cm occupying the oropharyngeal area, although the limits of the mass with the hard palate could not be clearly visualized. The left maxillary bone was poorly defined, with a bulky aspect of soft tissue on the left hemiface. The epiglottis was well defined, whereas the upper aerodigestive tract above was not. The presence of a hydramnios suggested an upper airway obstruction of the upper aerodigestive tract. Due to many uncertain issues arising from the difficulty to exactly define the extent of the mass, its nature, and the postnatal prognosis, several options were discussed with the antenatal multidisciplinary team consisting of obstetricians, paediatricians, anaesthetists, and maxillofacial surgeons. Finally the antenatal counselling team advised the parents of two possibilities: therapeutic abortion or continuing the pregnancy with close follow-up and an MRI before a planned delivery at 37 gestational weeks. For the latter possibility, the treatment procedures had to be discussed. Considering the diameter of the oral mass, a caesarean section would be scheduled to avoid the obstetric complication of pelvic dystocia. Owing to the presence of a suspected airway obstruction, a treatment plan for airway
management would be put in place as follows. Firstly, on the day of the caesarean section, the multidisciplinary team, familiar with maternal high-risk caesarean sections, neonatal surgery and resuscitation, and foetal and maternal anaesthesia would be gathered in a designated operating theatre. Secondly, in the operating room, all the various equipments required needed to be prepared, keeping foetal instruments separate from maternal ones. Thirdly the caesarean section would be performed using an ex utero intrapartum (EXIT) procedure. After delivery but before the umbilical cord was clamped, the paediatric intensive care unit (ICU) planned to intubate the premature infant on placental support. If they failed, intubation with a fibre-optic flexible bronchoscope would be attempted. If this last option failed, a tracheotomy would be performed by the surgeons. If the tracheotomy became complicated, an attempt would be made to clear the airway of the obstruction by removing the mass. Afterwards, depending on the clinical birth data, the decision to whether or not remove the tumour immediately after birth in an adjoining operating room would be made. Preterm labour ensued at 32 gestational weeks, which accelerated both the parental and the medical decision-making processes. The parents agreed to the birth of the infant as previously described. The different disciplines coordinated
themselves and followed the defined procedure. The mother was transferred from the obstetric emergency department to a paediatric surgery theatre. The caesarean section was initiated, followed by an EXIT procedure, and the treatment plan for the neonate was applied. During the hysterotomy, a 3 l hydramnios was evacuated. Once the foetus was extracted, a nasofibroscopic intubation was attempted. The intubation failed through the right nostril, but was successful through the left one (Fig. 1C). Indeed, during removal of the tumour, we discovered that it demonstrated a pedicle on the right side of the palate. Once the airway was secured, the umbilical cord was cut. While the obstetricians performed the caesarean closure, the premature infant was entrusted to the neonatalogist for further examinations. It was decided to remove the protruding tumour immediately. The tumour was attached in the right nasopharynx area and had migrated into the cleft. The premature girl (2500 g, 43 cm) had macrostomia and a large cleft palate. After removal of the tumour, the neonate was transferred to the neonatal ICU. A clinical examination of the oral cavity showed a residual ovoid mass of 1 cm 0.5 cm remaining in the right oropharynx, in the area where the mass had been attached (Fig. 1D and E). She was extubated on day 2. At 7 days, bottle feeding was started. At 10 days, she was discharged from the neonatal ICU. Histopathological analysis confirmed that the tumour was a mature teratoma. The mass weighed 61 g and measured 8 cm 5 cm 3.8 cm. The glial component was evaluated at 10%, and the immature component was evaluated at 1%. Different tissues were clearly identified as cutaneous tissue, tooth sketching, bronchial wall tissue, and bone tissue (Fig. 2A–F). At 7 months, the infant underwent a further operation to remove the residual ovoid tumour mass. At 19 months, the large cleft palate was closed with a periosteal graft in a calvaria procedure. To date, at 36 months old, this child has exhibited normal development (Fig. 1F and G). Her monitoring consists of regular paediatric and oral examinations and standard blood analyses for levels of alphafetoprotein (AFP). Discussion
Foetus-in-foetu should be discussed as part of the differential diagnosis.2,3 This condition was first described as a mass
Congenital teratoma of the oral cavity
435
Fig. 2. Histopathological examination; haematoxylin, eosin, and saffron. (A) Subcutaneous tissue (magnification 50): 1, stratified epidermis; 2, epithelial skin appendages; 3, hair follicle; 4, adipocytes. (B) Tooth bud (magnification 25): 1, adamantine epithelium; 2, enamel. (C) Bronchial epithelium (magnification 25): 1, cuboidal ciliated epithelium; 2, sebaceous glands; 3, cartilage tissue. (D) Chondrocytes (magnification 25). (E) Bone and haematopoietic tissues (magnification 100): 1, lamellar bone; 2, medullary tissue; 3, adipocytes. (F) Immature glial tissue (magnification 100).
along the vertebral axis. Distinguishing between teratoma and foetus-in-foetu can be difficult due to their similar clinical and radiological features. Although some authors have regarded this entity as a complicated form of teratoma, the distinction between teratoma and foetus-in-foetu must be made on the basis of the existence of a spinal column.3 Other components of the classical differential diagnosis include lymphangioma, cystic hygroma, branchial cyst, cephalocele, and rhabdomyosarcoma.2 Knowledge of antenatal diagnostic data facilitated the undertaking of this case. Indeed, anticipation of an airway problem required simultaneous planning for the caesarean delivery and paediatric intensive care, and the presence of a surgeon able to perform a neonatal tracheotomy in the delivery room. As is the case in our hospital, when the obstetric and paediatric theatres are located in two distant places, it is better for optimal organization to transfer the mother to the paediatric theatre for the caesarean section so that tumour removal from the premature infant can be performed in an adjoining room after securing the airway. This approach served to minimize the risks, which were already high for this child. Procedures used to perform airway control, including placental support4 or the EXIT procedure,5 have been described previously. In this procedure, the foeto-placental circulation was
preserved until the airway was secured. Skarsgard et al.4 suggested that foetal saturation can be kept at a level as high as 60–70% as long as the infant remains under placental circulation. In every case where it is possible, a tracheotomy to provide an unobstructed airway should be performed only as a last resort. Indeed, a tracheotomy could lead to complications such as infection, stenosis, swallowing dysfunction, or decannulation, and a prolonged stay in the ICU. Most of the reported clinical cases have not been subject to immediate surgical treatment after airway management.1 If the infant is stable, the removal of the obstruction from the oral cavity must be undertaken as rapidly as possible to limit the ICU stay. Tissues of ectodermal, endodermal, and mesodermal origin can appear as components of teratomas.6 Moreover, teratomas can be mature, immature, or mixed-staged tumours. In many cases, the presence of immature tissue indicates a diagnosis of malignancy.7 Nevertheless, these immature components can consist of foetal tissues that are observed due to prematurity,6 as in the current case, and these components would not have been found in a full-term infant. The 1% of immature tissue described was considered as a component in keeping with foetal prematurity. The follow-up evaluations more than 3 years later have confirmed this diagnosis,
and the patient has demonstrated no recurrence or metastasis and has a stable level of AFP. The relevance of the AFP level has not been established for facial teratomas. However, this level is commonly monitored in cases of teratoma,2 and AFP has been detected in a high proportion of tumours and has served as a useful marker for tumour recurrence or progression. There is no normal value in cases of prematurity, but a follow-up assessment showing decreased levels is a good indicator. Tsuchida and Hasegawa published normal ranges for AFP levels according to age in 1978, and for cases of extracephalic teratomas in early infancy, they showed a clear correlation between the histological classification of the teratoma and the level of AFP. In particular, the level of AFP was within the normal range in cases with mature teratomas, but the level was higher than the upper limit of the normal range in cases with malignant teratomas.8 Furthermore, it is clear that in cases of non-malignant teratomas, the AFP level should decrease after tumour excision.9 Any increase in this level should arouse suspicion of recurrence or malignant transformation.2 Ultrasound examinations are routine procedures. In France, women undergo three such examinations in cases of uncomplicated pregnancy. These examinations visualize with increasing precision the location, size, extent of the tumour, the
436
Dakpe´ et al.
possible existence of hydramnios, and associated lesions. The principal associated lesions include cleft palate (the most commonly observed with a mechanical origin), bifid tongue and bifid uvula in 6% of all cases, and extrafacial malformations such as left ventricular hypoplasia, lung hypoplasia, and anal imperforation.2 The ultrasound examination data completed by MRI are essential for clinicians to evaluate the tumour and to enable informed parental choices regarding the possible termination of the pregnancy. As previously noted by other authors,1 the development of antenatal diagnostic tools has permitted early and accurate screening. Today, foeto-endoscopic surgery is performed, but the in utero treatment of an oral teratoma via foetoscopy remains an exceptional feat, reserved for selected cases and performed by a team trained in foetoscopy.10 These improvements sometimes result in doctors being confronted with difficult therapeutic decisions. Notwithstanding the ethical considerations, this clinical case shows that congenital teratomas, when located in the upper aerodigestive tract, can result in airway obstruction that must be managed immediately at the time of birth. Successful treatment is predicated on the rapid coordination of multiple specialities.
Funding
None.
Competing interests
None declared.
5.
Ethical approval
Not required.
6.
Patient consent
Written consent (parents) has been obtained to publish the clinical photographs.
7.
Acknowledgements. We sincerely acknowledge Sylvie Testelin, Pierre Tourneux, Andre´ Leke, Catherine Gondry, Se´gole`ne Lanta, Jean Gondry, Cica Gbaguidi, Jacky Dekens, and Salah Mattoug.
8.
9.
References 1. Anderson PJ, David DJ. Teratomas of the head and neck region. J Craniomaxillofac Surg 2003;31:369–77. 2. Benouaiche L, Couly G, Michel B, Devauchelle B. Diagnosis and management of cervicofacial congenital teratomas: about 4 cases, literature review and restatement. Ann Chir Plast Esthet 2007;52:114–23. 3. Brand A, Alves MC, Saraiva C, Loı´o P, Goula˜o J, Malta J, et al. Fetus in fetu— diagnostic criteria and differential diagnosis—a case report and literature review. J Pediatr Surg 2004;39:616–8. 4. Skarsgard ED, Chitkara U, Krane EJ, Riley ET, Halamek LP, Dedo HH. The OOPS procedure (operation on placental support): in utero airway management of the fetus with
10.
prenatally diagnosed tracheal obstruction. J Pediatr Surg 1996;31:826–8. Bouchard S, Johnson MP, Flake AW, Howell LJ, Myers LB, Adzick NS, et al. The EXIT procedure: experience and outcome in 31 cases. J Pediatr Surg 2002;37:418–26. Vandenhaute B, Leteurtre E, LecomteHoucke 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. Batsakis JG, el-Naggar AK, Luna MA. Teratomas of the head and neck with emphasis on malignancy. Ann Otol Rhinol Laryngol 1995;104:496–500. Tsuchida Y, Hasegawa H. The diagnostic value of alpha-fetoprotein in infants and children with teratomas: a questionnaire survey in Japan. J Pediatr Surg 1983;18: 152–5. Sumiyoshi S, Machida J, Yamamoto T, Fukano H, Shimozato K, Fujimoto Y, et al. Massive immature teratoma in a neonate. Int J Oral Maxillofac Surg 2010;39:1020–3. Kontopoulos EV, Gualtieri M, Quintero RA. Successful in utero treatment of an oral teratoma via operative fetoscopy: case report and review of the literature. Am J Obstet Gynecol 2012;207:e12–5.
Address: Ste´phanie Dakpe´ Department of Maxillofacial Surgery and Stomatology University Hospital Place Victor Pauchet 80000 Amiens France Tel.: +33 322 668 328; fax: +33 322 668 329 E-mails: [email protected], [email protected]
Case Report Cleft and Craniofacial Disorders
Emergency management of a congenital teratoma of the oral cavity at birth and three-year follow-up
S. Dakpe´1,*, B. Demeer2,3, C. Cordonnier4, B. Devauchelle1 1
Department of Maxillofacial Surgery and Stomatology, University Hospital Amiens, France; 2Department of Prenatal Diagnosis, Gynaecology and Obstetrics, University Hospital Amiens, France; 3Department of Medical Genetics, Rare Disease Centre, University Hospital Amiens, France; 4 Department of Anatomopathology, University Hospital Amiens, France
S. Dakpe´, B. Demeer, C. Cordonnier, B. Devauchelle: Emergency management of a congenital teratoma of the oral cavity at birth and three-year follow-up. Int. J. Oral Maxillofac. Surg. 2014; 43: 433–436. # 2014 International Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved. Abstract. Teratomas are congenital malformations that are rarely located in the head and neck region. We report a case of congenital teratoma of the oral cavity, which was causing an airway obstruction and was treated at the time of birth. This teratoma was discovered at 27 gestational weeks by ultrasonography. A multidisciplinary team was consulted for antenatal diagnosis; the options of therapeutic abortion or management of the birth with the prevention of respiratory distress were debated. However, preterm labour at 32 gestational weeks accelerated the parental and the medical decisions. The parents agreed to the birth. The various disciplines coordinated their work, and the predefined treatment plan for clearing the airway obstruction was applied to manage the birth. The reestablishment of patency of the airway was performed during delivery and removal of the tumour was performed immediately afterwards. The follow-up of this case over 3 years is also presented.
From the Greek root ‘tera’, meaning monstrous, teratomas are congenital malformations that occur in one out of every 4000 births; they are rarely located in the head or neck (2%). The discovery of an obstructing teratoma of the upper aerodigestive tract during the antenatal period is a challenging clinical scenario. As a direct consequence of the rarity of teratomas of the head and neck, most clinicians have very limited experience with the management of these tumours.1 The case presented here demonstrates that severe respiratory obstructions can be managed 0901-5027/040433 + 04 $36.00/0
and resection should be performed without wasting any time. Furthermore, successful management of severe respiratory obstructions caused by these tumours requires the timely coordination of various disciplines, including maxillofacial surgeons, neonatalogists, anaesthetists, and high-risk obstetrics specialists at the time of the birth. We report the successful emergency management of a congenital teratoma of the oral cavity in a premature infant. The paediatric development of this patient has remained normal to date.
Keywords: teratoma; antenatal diagnosis; EXIT procedure; cleft palate; neonatal surgery; epignathus. Accepted for publication 3 September 2013 Available online 24 January 2014
Case report
Following a second trimester morphological foetal ultrasound examination, a 28-year-old woman, who was gravida 3 para 2, was referred to our medical centre for a suspicion of an oral tumour, which was confirmed. A large mass of 35 mm 20 mm was detected, with coexisting cystic and solid tissues. The oral cavity appeared to be blocked by this mass. Subsequently, a hydramnios developed (Fig. 1A and B). Amniocentesis was performed and revealed a normal 46, XX foetus. Foetal
# 2014 International Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.
434
Dakpe´ et al.
Fig. 1. (A) Ultrasound examination at gestational week 28 showing a large mass with coexisting cystic and solid tissues. The exteriorized mass measured 35 mm 20 mm. Hydramnios was visualized (lateral view). (B) 3D ultrasound examination at gestational week 28 showing a large mass; the oral cavity appeared to be totally blocked by this mass. (C) Premature infant (32 gestational weeks) with a large teratoma blocking the oral cavity after nasofibroscopic intubation during the EXIT procedure. (D) Cleft palate and tumoural remnant located in the right oropharynx on day 1. Closure of the large cleft palate was performed at 19 months with a calvarial periosteal graft. (E) Cleft palate and tumoural remnant located in the right oropharynx on day 7. (F) Face of the patient at 36 months. (G) Endobuccal view of the patient at 36 months.
magnetic resonance imaging (MRI) was performed at 29 gestational weeks to attempt to accurately define the extent and nature of the tumour. The MRI showed a mass measuring 7 cm 5 cm occupying the oropharyngeal area, although the limits of the mass with the hard palate could not be clearly visualized. The left maxillary bone was poorly defined, with a bulky aspect of soft tissue on the left hemiface. The epiglottis was well defined, whereas the upper aerodigestive tract above was not. The presence of a hydramnios suggested an upper airway obstruction of the upper aerodigestive tract. Due to many uncertain issues arising from the difficulty to exactly define the extent of the mass, its nature, and the postnatal prognosis, several options were discussed with the antenatal multidisciplinary team consisting of obstetricians, paediatricians, anaesthetists, and maxillofacial surgeons. Finally the antenatal counselling team advised the parents of two possibilities: therapeutic abortion or continuing the pregnancy with close follow-up and an MRI before a planned delivery at 37 gestational weeks. For the latter possibility, the treatment procedures had to be discussed. Considering the diameter of the oral mass, a caesarean section would be scheduled to avoid the obstetric complication of pelvic dystocia. Owing to the presence of a suspected airway obstruction, a treatment plan for airway
management would be put in place as follows. Firstly, on the day of the caesarean section, the multidisciplinary team, familiar with maternal high-risk caesarean sections, neonatal surgery and resuscitation, and foetal and maternal anaesthesia would be gathered in a designated operating theatre. Secondly, in the operating room, all the various equipments required needed to be prepared, keeping foetal instruments separate from maternal ones. Thirdly the caesarean section would be performed using an ex utero intrapartum (EXIT) procedure. After delivery but before the umbilical cord was clamped, the paediatric intensive care unit (ICU) planned to intubate the premature infant on placental support. If they failed, intubation with a fibre-optic flexible bronchoscope would be attempted. If this last option failed, a tracheotomy would be performed by the surgeons. If the tracheotomy became complicated, an attempt would be made to clear the airway of the obstruction by removing the mass. Afterwards, depending on the clinical birth data, the decision to whether or not remove the tumour immediately after birth in an adjoining operating room would be made. Preterm labour ensued at 32 gestational weeks, which accelerated both the parental and the medical decision-making processes. The parents agreed to the birth of the infant as previously described. The different disciplines coordinated
themselves and followed the defined procedure. The mother was transferred from the obstetric emergency department to a paediatric surgery theatre. The caesarean section was initiated, followed by an EXIT procedure, and the treatment plan for the neonate was applied. During the hysterotomy, a 3 l hydramnios was evacuated. Once the foetus was extracted, a nasofibroscopic intubation was attempted. The intubation failed through the right nostril, but was successful through the left one (Fig. 1C). Indeed, during removal of the tumour, we discovered that it demonstrated a pedicle on the right side of the palate. Once the airway was secured, the umbilical cord was cut. While the obstetricians performed the caesarean closure, the premature infant was entrusted to the neonatalogist for further examinations. It was decided to remove the protruding tumour immediately. The tumour was attached in the right nasopharynx area and had migrated into the cleft. The premature girl (2500 g, 43 cm) had macrostomia and a large cleft palate. After removal of the tumour, the neonate was transferred to the neonatal ICU. A clinical examination of the oral cavity showed a residual ovoid mass of 1 cm 0.5 cm remaining in the right oropharynx, in the area where the mass had been attached (Fig. 1D and E). She was extubated on day 2. At 7 days, bottle feeding was started. At 10 days, she was discharged from the neonatal ICU. Histopathological analysis confirmed that the tumour was a mature teratoma. The mass weighed 61 g and measured 8 cm 5 cm 3.8 cm. The glial component was evaluated at 10%, and the immature component was evaluated at 1%. Different tissues were clearly identified as cutaneous tissue, tooth sketching, bronchial wall tissue, and bone tissue (Fig. 2A–F). At 7 months, the infant underwent a further operation to remove the residual ovoid tumour mass. At 19 months, the large cleft palate was closed with a periosteal graft in a calvaria procedure. To date, at 36 months old, this child has exhibited normal development (Fig. 1F and G). Her monitoring consists of regular paediatric and oral examinations and standard blood analyses for levels of alphafetoprotein (AFP). Discussion
Foetus-in-foetu should be discussed as part of the differential diagnosis.2,3 This condition was first described as a mass
Congenital teratoma of the oral cavity
435
Fig. 2. Histopathological examination; haematoxylin, eosin, and saffron. (A) Subcutaneous tissue (magnification 50): 1, stratified epidermis; 2, epithelial skin appendages; 3, hair follicle; 4, adipocytes. (B) Tooth bud (magnification 25): 1, adamantine epithelium; 2, enamel. (C) Bronchial epithelium (magnification 25): 1, cuboidal ciliated epithelium; 2, sebaceous glands; 3, cartilage tissue. (D) Chondrocytes (magnification 25). (E) Bone and haematopoietic tissues (magnification 100): 1, lamellar bone; 2, medullary tissue; 3, adipocytes. (F) Immature glial tissue (magnification 100).
along the vertebral axis. Distinguishing between teratoma and foetus-in-foetu can be difficult due to their similar clinical and radiological features. Although some authors have regarded this entity as a complicated form of teratoma, the distinction between teratoma and foetus-in-foetu must be made on the basis of the existence of a spinal column.3 Other components of the classical differential diagnosis include lymphangioma, cystic hygroma, branchial cyst, cephalocele, and rhabdomyosarcoma.2 Knowledge of antenatal diagnostic data facilitated the undertaking of this case. Indeed, anticipation of an airway problem required simultaneous planning for the caesarean delivery and paediatric intensive care, and the presence of a surgeon able to perform a neonatal tracheotomy in the delivery room. As is the case in our hospital, when the obstetric and paediatric theatres are located in two distant places, it is better for optimal organization to transfer the mother to the paediatric theatre for the caesarean section so that tumour removal from the premature infant can be performed in an adjoining room after securing the airway. This approach served to minimize the risks, which were already high for this child. Procedures used to perform airway control, including placental support4 or the EXIT procedure,5 have been described previously. In this procedure, the foeto-placental circulation was
preserved until the airway was secured. Skarsgard et al.4 suggested that foetal saturation can be kept at a level as high as 60–70% as long as the infant remains under placental circulation. In every case where it is possible, a tracheotomy to provide an unobstructed airway should be performed only as a last resort. Indeed, a tracheotomy could lead to complications such as infection, stenosis, swallowing dysfunction, or decannulation, and a prolonged stay in the ICU. Most of the reported clinical cases have not been subject to immediate surgical treatment after airway management.1 If the infant is stable, the removal of the obstruction from the oral cavity must be undertaken as rapidly as possible to limit the ICU stay. Tissues of ectodermal, endodermal, and mesodermal origin can appear as components of teratomas.6 Moreover, teratomas can be mature, immature, or mixed-staged tumours. In many cases, the presence of immature tissue indicates a diagnosis of malignancy.7 Nevertheless, these immature components can consist of foetal tissues that are observed due to prematurity,6 as in the current case, and these components would not have been found in a full-term infant. The 1% of immature tissue described was considered as a component in keeping with foetal prematurity. The follow-up evaluations more than 3 years later have confirmed this diagnosis,
and the patient has demonstrated no recurrence or metastasis and has a stable level of AFP. The relevance of the AFP level has not been established for facial teratomas. However, this level is commonly monitored in cases of teratoma,2 and AFP has been detected in a high proportion of tumours and has served as a useful marker for tumour recurrence or progression. There is no normal value in cases of prematurity, but a follow-up assessment showing decreased levels is a good indicator. Tsuchida and Hasegawa published normal ranges for AFP levels according to age in 1978, and for cases of extracephalic teratomas in early infancy, they showed a clear correlation between the histological classification of the teratoma and the level of AFP. In particular, the level of AFP was within the normal range in cases with mature teratomas, but the level was higher than the upper limit of the normal range in cases with malignant teratomas.8 Furthermore, it is clear that in cases of non-malignant teratomas, the AFP level should decrease after tumour excision.9 Any increase in this level should arouse suspicion of recurrence or malignant transformation.2 Ultrasound examinations are routine procedures. In France, women undergo three such examinations in cases of uncomplicated pregnancy. These examinations visualize with increasing precision the location, size, extent of the tumour, the
436
Dakpe´ et al.
possible existence of hydramnios, and associated lesions. The principal associated lesions include cleft palate (the most commonly observed with a mechanical origin), bifid tongue and bifid uvula in 6% of all cases, and extrafacial malformations such as left ventricular hypoplasia, lung hypoplasia, and anal imperforation.2 The ultrasound examination data completed by MRI are essential for clinicians to evaluate the tumour and to enable informed parental choices regarding the possible termination of the pregnancy. As previously noted by other authors,1 the development of antenatal diagnostic tools has permitted early and accurate screening. Today, foeto-endoscopic surgery is performed, but the in utero treatment of an oral teratoma via foetoscopy remains an exceptional feat, reserved for selected cases and performed by a team trained in foetoscopy.10 These improvements sometimes result in doctors being confronted with difficult therapeutic decisions. Notwithstanding the ethical considerations, this clinical case shows that congenital teratomas, when located in the upper aerodigestive tract, can result in airway obstruction that must be managed immediately at the time of birth. Successful treatment is predicated on the rapid coordination of multiple specialities.
Funding
None.
Competing interests
None declared.
5.
Ethical approval
Not required.
6.
Patient consent
Written consent (parents) has been obtained to publish the clinical photographs.
7.
Acknowledgements. We sincerely acknowledge Sylvie Testelin, Pierre Tourneux, Andre´ Leke, Catherine Gondry, Se´gole`ne Lanta, Jean Gondry, Cica Gbaguidi, Jacky Dekens, and Salah Mattoug.
8.
9.
References 1. Anderson PJ, David DJ. Teratomas of the head and neck region. J Craniomaxillofac Surg 2003;31:369–77. 2. Benouaiche L, Couly G, Michel B, Devauchelle B. Diagnosis and management of cervicofacial congenital teratomas: about 4 cases, literature review and restatement. Ann Chir Plast Esthet 2007;52:114–23. 3. Brand A, Alves MC, Saraiva C, Loı´o P, Goula˜o J, Malta J, et al. Fetus in fetu— diagnostic criteria and differential diagnosis—a case report and literature review. J Pediatr Surg 2004;39:616–8. 4. Skarsgard ED, Chitkara U, Krane EJ, Riley ET, Halamek LP, Dedo HH. The OOPS procedure (operation on placental support): in utero airway management of the fetus with
10.
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Address: Ste´phanie Dakpe´ Department of Maxillofacial Surgery and Stomatology University Hospital Place Victor Pauchet 80000 Amiens France Tel.: +33 322 668 328; fax: +33 322 668 329 E-mails: [email protected], [email protected]
