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Journal of Indian Association of Pediatric Surgeons, Vol. 12, No. 4, October-December, 2007, pp. 226-227 Case Report Ex-Utero intrapartum procedure for congenital high airway obstruction syndrome in a neonate: First case in Alexandria Youssef MohammedAly Specialist of Pediatric Surgery, Alexandria Hospital for Sick Children, Health Insurance Authority, Alexandria Code Number: ip07073 Abstract Introduction: Large fetal neck masses can present a major challenge for securing an airway at birth with associated risks of hypoxia, brain injury and death. Teratomas of the oropharynx are rare, presenting 3% of teratomas in childhood, and are treated by surgical excision. If respiratory distress accompanies the lesion, priority must be given to the securing of the airway.Case History: We present a case of an infant who was diagnosed antenatally as having a huge oropharyngeal teratoma. The anaesthetic, surgical and neonatology teams were ready to perform surgical excision depending on the placental circulation immediately after securing the airway. The tumour weighed 1591 g and was 20 x 22 x 12 cm. The patient was a male and weighed 715 g. Histopathology showed Grade II teratoma. Conclusion: Large fetal neck masses can present a major challenge for securing an airway at birth with associated risks of hypoxia, brain injury and death. A multidisciplinary team approach combined with an accurate prenatal diagnosis obtained through fetal ultrasound is the key to a successful outcome. Ex utero intrapartum treatment (EXIT) that is based on the placental blood during intubation, tracheostomy or surgical excision is the standard procedure. Keywords: CHAO syndrome, Ex utero intrapartum treatment, oropharyngeal teratoma Introduction Large fetal neck masses can present a major challenge for securing an airway at birth with associated risks of hypoxia, brain injury and death. [1] The ex utero intrapartum treatment (EXIT) procedure can be used to obtain a fetal airway while feto-maternal circulation is preserved to optimise the fetal outcome. A multidisciplinary team approach, combined with an accurate prenatal diagnosis obtained through fetal ultrasound magnetic resonance imaging examination was the key to a successful outcome. The role of the pediatric surgeon is initially to secure the airways through tracheostomy if intubation is difficult followed by excision of the mass when the vital parameters of the infant are stabilized. [2] The EXIT procedure is an extremely valuable tool in providing time to secure the airway in infants with large fetal neck masses and abnormalities of the upper airway that impede resuscitation. The fetus is partially delivered while maintaining the utero-placental-fetal circulation. The EXIT procedure provides up to 1 h of good uteroplacental support, and it is the procedure of choice to secure the airway in the fetus with a giant neck mass. [3] We present our experience with EXIT procedure in a patient with a large oropharyngeal teratoma. The success of the procedure lies in multidisciplinary team approach and co-operation. [4] Case History In our study, we present a case of the subject is a male infant who was diagnosed antenatally at 28 weeks of gestation to be having a huge oropharyngeal teratoma. The anaesthetic, surgical and neonatology teams were ready to perform the EXIT procedure [Figure - 1]. We started the EXIT procedure immediately after cesarean section depending on the placental circulation to secure an airway through intubation with the use of a standard end-tidal carbon dioxide probe to confirm the correct endotracheal intubation. Subsequently, the immediate excision of the mass was done. The tumour weighed 1,591 g and was 20 x 22 x 12 cm [Figure - 2]. Histopathology showed Grade II teratoma. The postoperative course was smooth and the baby was discharged after 7 days and followed up to the age of 3 months with no recurrence. Discussion The combination of intensive maternal-fetal monitoring, cesarean section with maximal uterine relaxation and maintenance of intact fetoplacental circulation provides a controlled environment for securing the airway in infants with prenatally diagnosed airway obstruction. [5] The EXIT procedure was developed originally for management of airway obstruction after fetal surgery and indications have continued to expand for a variety of fetal anomalies. [6] Even in twin gestations, the EXIT procedure is the delivery method of choice for fetuses with giant neck masses. [7] The EXIT technique, performed for the first time in 1989 and now in many centers abroad, can be considered as a safe procedure as long as a multidisciplinary approach is carried out. [8] The EXIT procedure was successfully used to ensure the uteroplacental gas exchange and fetal hemodynamic stability during a variety of surgical procedures performed to secure the fetal airway or to ensure the successful transition to postnatal environment. [9] Conclusion A multidisciplinary team approach combined with an accurate prenatal diagnosis obtained through fetal ultrasound is the key to a successful outcome. EXIT depending on placental blood during intubation, tracheostomy or surgical excision is the standard procedure.References
Copyright 2007 - Journal of Indian Association of Pediatric Surgeons The following images related to this document are available:Photo images[ip07073f1.jpg] [ip07073f2.jpg] |
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