Video link: https://www.youtube.com/watch?v=jMa5meoBKCg&feature=youtu.be * Downloadable English and Filipino versions of the article: please scroll to the bottom
MAIN ARTICLE:
On 4 November 2015, an infant with airway compression was safely delivered at the Philippine General Hospital, University of the Philippines Manila, using the Ex Utero Intrapartum Treatment (EXIT) to Airway procedure. It was the first time that a completed and successful EXIT was done in the Philippines. The baby, Crislin, was diagnosed months ago with an 11.2 x 10.5 x 9.8 cm cystic hygroma or lymphatic malformation which affected the cheek, chin, and neck from ear to chest, with high probability of airway obstruction. Cystic hygroma or lymphatic malformation is an abnormal growth formed by a collection of locules or cystic cavities filled with straw-colored lymphatic fluid and dark venous blood. This occurs when the lymphatic vessels of the lymph and venous systems do not form properly. In the case of baby Crislin, non-intervention could have meant death on delivery for her, as happened last year to a baby born in another hospital who had a similar condition who did not undergo the EXIT procedure, and whose airway could not be established. This is because excessive airway compression by the mass in the neck can prevent normal breathing, as well as any attempt to insert a breathing tube through the mouth into the throat in time to take the vital first breath of life. The EXIT procedure was designed to take advantage of the natural life-support the umbilical cord gives while the baby remains connected to the mother through the placenta, by delaying delivery of the baby – delivering only the head and neck to buy time to enable an airway to be established before the baby needs to take its first breath. When the mother Chrishilda’s pre-operative imaging studies revealed Crislin’s problem, a multidisciplinary team was formed a month prior to the operation to plan an EXIT procedure. The team was spearheaded by the Section of Maternal and Fetal Medicine and was a joint effort of the Departments of Obstetrics and Gynecology, Otorhinolaryngology, Anesthesiology, Pediatrics, Radiology, and OR Complex staff. Three major steps of the EXIT to Airway procedure for Congenital High Airway Obstruction (CHAOS) were detailed over numerous meetings to help baby Crislin: [1] transabdominal ultrasound-guided decompression of the mass prior to ceasarian operation to lessen airway compression (this failed due to thick intracystic fluid); [2] direct laryngeal intubation (insertion of a breathing tube into the throat through the mouth) after the head and neck were exposed through a caesarian incision (failed again due to laryngeal edema or swollen voice box and tracheal deviation or windpipe which had shifted from its normal position in the middle of the neck); and, lastly, [3] direct aspiration of cystic fluid and creation of a surgical airway directly through the neck into the trachea (tracheostomy) to ensure breathing, before delivery of the baby. Fortunately, this did not fail. To perform the EXIT to Airway, a C-section is first started on the mother’s abdomen and uterus, while the anesthesiologist maintains total uterine relaxation (a necessity which, unfortunately, exposes the mother to bleeding due to loss of uterine muscle tone) as any contractions could expel the baby or separate the placenta prematurely while maintaining optimal placental perfusion. The baby’s head and neck are then partially exposed through the small opening, but the rest of the baby is still inside the uterus, connected to the mother through the umbilical cord and placenta. Airway and pediatric anesthesiologists and a pediatric otolaryngologisthead and neck surgeon attempt to access the airway through the mouth, and failing that, the latter creates a surgical opening through the neck and inserts a tracheostomy tube directly in the windpipe so the baby can breathe. Only then is the baby delivered and umbilical cord cut. Immediate post-operative neonatal laryngoscopy showed tumor compression in the throat with an edematous voice box and a deviated windpipe, confirming the team’s pre-operative diagnosis and that the EXIT procedure had truly been lifesaving. The rest of the cesarean section was uneventful, as the uterus regained its tone with timely medications, and both mother and child recovered from their surgeries. As of press time, both mommy Chrishilda and baby Crislin are at the PostAnesthesia Care Unit (PACU) and Neonatal Intensive Care Unit (NICU), respectively, and doing well. Crislin needs definitive medications and surgery on the lymphatic malformation in the near future for which she needs to be prepared. The EXIT team thanks the UP-PGH community for its cooperation and prayers. It also acknowledges the Department of Health and the PGH Medical Foundation for their generous sponsorship which made the operation possible. The members of the EXIT team were led by: Dr. Valerie Guinto, Dr. Ireene Cacas (Maternal-Fetal Medicine), Dr. Jose Florencio Lapeña (Pediatric Otolaryngology Head and Neck Surgery), Dr. Loreto Fellizar, Dr. Carlo Catabijan, Dr. Eric Bandola and Dr. Kai Espejo (Anesthesiology), Dr. Rosanna Fragante (Radiology), Dr. Resti Ma. Bautista, Dr. Fay De Ocampo (Neonatology), Chief Nurse Juliet Mangahis (OR Complex), and Perinatology Fellows led by Dr. Pauline LimReyes, with Fellows, Residents and Nurses from the participating departments. Written by: Dr. JFLapeña and IPPAO-UPM
UP-PGH EXIT Team Contact Persons: Dr. Valerie T. Guinto Mobile: 0918-9857199/0917-8443347 Landline: 7897700 (7539) Email: laireguinto@gmail.com
Dr. Jose Florencio Lapeña, Jr. Department of Otorhinolaryngology Ward 10, Philippine General Hospital Taft Avenue, Ermita, Manila 1000 Philippines Phone: (632) 554 8467 TeleFax: (632) 524 4455 Mobile + 63 943 343 4963 Email: lapenajf@upm.edu.ph Complete EXIT Team: Anesthesia Team:
ORL Team:
Interventional Radiology: Dr. Rosanna Fragante and her team OB-Gyne Team:
*Mommy Chrishilda gave the PGH EXIT team a signed permission to photograph and upload the proceedings. Video created by: Clarissa L. Velayo, MD, PhD
