Fetal Neck and Chest Lesions

Extrinsic Compression

Cervical Mediastinal teratoma Lymphangioma Goiter Thyroid cysts Thyroglosal duct cyst Branchial cleft cyst Neuroblastoma Hamartoma Hemangioma Lipoma Parotid tumor Cervical MMC Epignathus Fetal Neck Masses Cervical •All 3 germ layers represented •Totipotential germ cells vs conjoined twin •40% contain thyroid tissue •Large bulky tumors •Airway compromise •Polyhydramnios in 40% •Benign lymph node metastases •Thyroid destruction •Parathyroid engulfment •Mandibular hypoplasia •Marginal mandibular nerve palsy •Laryngotracheomalacia Fetal Neck Masses

•No matter the etiology •Very large masses can cause: •Neck hyperextension •Pulmonary hypoplasia •Secondary to carina pulled into neck • pulled into apex of hemithorax TheThe EXITEXIT ProcedureProcedure Airway Algorithm

•Direct laryngoscopy •Flexible bronchoscopy •Rigid bronchoscopy •Tracheotomy with retrograde intubation •Tracheostomy •Mass resection + tracheostomy •TRI-EXIT Establishing an airway

Laryngoscopy Bronchoscopy Tracheostomy Resection Congenital High Airway Obstruction Syndrome Prenatal Diagnosis Often misdiagnosed Bilateral CCAMs Bilateral extremely large echogenic lungs Compressed heart and mediastinum Non‐immune hydrops Complete airway obstruction

MRI US CHAOS

Intrinsic Airway Obstruction •Laryngeal atresia/stenosis •Tracheal atresia/stenosis •

Prenatal Natural History • 2/3 Hydrops and IUFD • 1/3 Resolution of hydrops •Laryngeal fistula •Tracheoesophageal fistula CHAOS

• Diagnosed with bilateral CCAMs CHAOS • Dilated tracheobronchial tree

• Massively enlarged lungs

• Inverted and stretched diaphragms

• Non-immune hydrops

• Massive

• Complete airway obstruction CHAOS

CHAOS Fetal Lamb Model of CHAOS

•Fetal tracheal occlusion at mid‐gestation •Non‐immune hydrops •Massive ascites •Massively enlarged lungs •Inverted stretched diaphragms •Dilated tracheobronchial tree

•Release of tracheal occlusion •Resolution of hydrops •Normal at delivery CHAOS CHAOS

Profound capillary leak syndrome Severe RDS Hepatic synthetic defect Diaphragmatic paralysis Stretch injury Hyper‐compliant lungs Ascites‐behave like cirrhotic Gut disfunction Prune belly Intact larynx Subglottic trecheal atresia Tracheal Reconstruction for CHAOS Laryngo-Tracheoplasty - Definition of natural history

- Shunts - Open CCAM - Steroids

Fetal MRI • CPAM Volume Ratio (CVR)

- 3 dimensions volume of lesion /Head circumference Saggital - CVR= .52 x L x W x H HC

- >1.6 high risk hydrops

Transverse • Fastest rate of growth 20- 25 weeks • Plateau at 26 weeks Range 24-28 weeks • No growth once plateau reached • CAM volume lagged behind CVR • Low risk CPAM - Excellent survival (98%) • High risk CPAM n=42 − Lower survival(71.4%)

• Excellent survival in non-hydropic high risk CPAM treated with steroid (100%) 8 weeks • Hydropic CPAM treated with steroids

post steroid - Survival 45% compared to 0% in historical controls - 88.9% survival with resolution of hydrops - Persistent of hydrops predicts mortality

Fetal Pericardial Teratoma

• Rare benign congenital tumor • 40 prenatally diagnosed cases • 2:1 female to male ratio • Often associated pericardial effusion • Rapid growth between 20-30 wks • 50% develop hydrops • Polyhydramnios common • Fatal in setting of hydrops/poly • Fetal Therapy • Pericardiocentesis • Fetal surgery Fetal Pericardial Teratoma

Open Pericardium to expose Teratoma Fetal Pericardial Teratoma Resection/repair complete Improved Survival and Decreased Need for ECMO in CDH

CFCC Liver 33 56% Patch 33 67%

Pre‐CDH Team CDH Team CDH Team CHCO & FCC CDH Team Longest Methods LHR Right Area/Head Circumference

UCSF AP Method Tracing Methods Observed to Expected LHR Survival with Conventional Postnatal Therapy

DePrest et al, Seminars Fetal and Neonatal Medicine 2008 Congenital

Percent Predicted Lung Volume (PPLV)

Total Lung Volume Total Thoracic Vol‐Mediastinal Vol

= PPLV

Mahieu‐Caputo et al BJOG 108: 863‐868, 2001 Paek et al Radiology 220: 63‐67, 2001 Gorincour et al Ultrasound Obstet Gynecol 26: 738‐744 Barnewolt et al J Pediatr Surg 42: 193‐197, 2007 TotalTotal LungLung VolumeVolume

• By 34 wk fetal lung growth should be complete.

• Fetal MRI to estimate total lung volume

• Goal to identify, counsel, and develop a treatment plan for high risk CDH Mahieu‐Caputo et al. BJOG 2001; 108:863 Neff et al. AJR 2007;189:1307 patient Rypens et al. Radiology 2001; 236

AssessmentAssessment ofof PostnatalPostnatal PulmonaryPulmonary HypertensionHypertension inin CDHCDH

• All prognostic indicators focus on lung

- Indirect indicators

- LHR, PPLV, TLV, Liver position

- All measure lung size or volume

• Indicators of pulmonary hypoplasia

• No prenatal measurement has tried to predict severity of postnatal pulmonary hypertension Prenatal Pulmonary Hypertension Index== PPHIPPHI

Vermis LPA

RPA

PPHI = (LPA d / Vermis l) x 10 ModifiedModified McGoonMcGoon IndexIndex == MGIMGI

LPA

Ao

RPA

MGI = (RPA d + LPA d) / Ao d CDH Prognostic Profile

• Congenital heart disease Criteria for EXIT-to-ECMO • Karyotype (and tracheal occlusion) • Liver position Liver up • O/E LHR LHR< 1.0 - <25th% 20% survival • LHR PPLV <15 - >1.0 100% survival TLV <18 - < 1.0 50% survival Normal karyotype • PPLV early and late - < 15% high risk - > 22% low risk - Late more reliable • TLV late - > 25cc favorable - < 18cc unfavorable • Modified Magoon Index? - <0.8 severe pulm htn - >1.0 mild pulm htn CDH-Composite Prognostic Index (CDH-CDI) CDHCDH--CompositeComposite PrognosticPrognostic IndexIndex (CDH(CDH--CDI)CDI) CDHCDH

TRACHEAL OCCLUSION • FETO Task Group • Leuven, Barcelona, London • FETO at 26-28 wks • Prenatal reversal of tracheal occlusion • Outcomes in 210 cases - Survival 49% • Conventional therapy - Survival 20% • NA Consortium: TOTAL • CHOP, Cincinnati, Denver • Maryland and Texas Deprest et al J Pediatr Surg 2011 • Toronto Jani et al Ultrasound Obstet Gynecol 2009 EXITEXIT--toto--ECMOECMO forfor HighHigh RiskRisk CDHCDH

• Rationale: - Minimize hemodynamic instability - Minimize barotrauma - Eliminates resuscitation - Time for pulm vascular remodeling - Smooth transition to postnatal life - Early repair to allow remodeling • Maternal risks - Deep general anesthesia - Cesarean section for subsequent pregnancy - Risk of uterine atony

Bouchard S, Johnson MP, Flake AW, Howell LJ, Myers LB, Adzick NS, Crombleholme TM: The EXIT Procedure: Experience and Outcome in 31 Cases. J Pediatr Surg 37: 428‐426, 2002 Met EXIT‐to‐ECMO Criteria Excluded for N=19 Abnormal Posture N=2

Excluded for Comfort Care EXIT‐to‐ECMO Non‐EXIT N=1 N=8 N=8

Cesarean Delivery Vaginal Delivery N=7 N=1

Cesarean Cesarean with Delivery Alone ECMO Standby N=5 N=2

ECMO ECMO* ECMO ECMO 00% (8/8) 60% (3/5)50% (1/2) 100% (1/1)

Survival Survival Survival 0% 20% (1/5) 50% (1/2)

EXIT‐to‐ECMO Non‐ EXIT Delivery Survival Survival 50% (4/8) * 2 died at birth 25% (2/8) Conclusions

• Essential role of prenatal imaging in neck and chest lesions • Combination of ultrasound, MRI and echocardiography • Serial assessment • Prognostic assessment • Treatment options •Open fetal surgery •Fetoscopic treatment? •Interstitial laser treatment •EXIT strategy •EXIT‐to‐Airway •EXIT‐to‐Resection Fetal Care Center of Cincinnati •Maternal-Fetal Medicine •Pediatric Surgery • − Mounira Habli MD •Timothy Crombleholme,MD − James Greenberg, MD − Tanya Cahill, MD −William Polzin, MD •Foong-Yen Lim,MD −Kim Brady, MD − Kurt Schibler, MD −Jim VanHook, MD •Sundeep Keswani MD − Beth Haberman, MD −Ronald Jaekle,MD •Anesthesiology − Paul Kingma, MD −David Lewis, MD –Ann Boat,MD − Amy Nathan, MD •Nursing Director •Fetal Echocardiography –Mohammad Mahmoud,MD −Erik Michelfelder, MD − Kelli Young –Bonnie Hugus −James |Cnota,MD •Coordinators −William Gottliebson, MD •Radiology − Gina Sharp, RN −Allison Divanovic,MD •Connie Bitters − Deb Voet, RN −Haleh Hadarian, MD •Linda Martin − Jenni Mason − Steve Imhoff •Urology/Nephrology –Beth Kline-Fath, MD −Pramod Reddy,MD − Judy Hostiuck −Robert Defore,MD –Maria Calvo, MD •Genetics −Elizabeth Jackson,MD –Eva Rubio, MD − Rob Hopkin,MD •ENT –Leann Linam, MD − Howard Saul,MD − Christine Spaeth, CGC −Ravi Ellarhu,MD –Kyuran Choe, MD −Paul Willging,MD − Diana Smith, CGC •Operating Room Nurses •Neurosurgery •Social Services −Karin Bierbrauer,MD •Missy Ritter − Erin Hartman, MSW −Francesco Mangano,MD •Stacy Ruth •Administrative •Cardiac Surgery •Tracy Heidrich − Rachel Jones − Emmie Beyer −Pirooz Eghtesady,MD •Latressa Ratner −Peter Manning,MD − Cheryl Snell •Curtis Johnson •Level III L&D Nurses • Good Samaritan Hospita •Nurse Midwife – Karen McGirr Colorado Fetal Care Center

•Maternal-Fetal Medicine • Fetal and Pediatric Surgery •Neonatology - Henry Galan, MD •Timothy M Crombleholme MD - Regina Reynolds, MD - Shane Reeves, MD •Stigg Somme, MD - Peter Hulac, MD - John Hobbins MD •Anesthesiology - Jason Gien, MD - Joyce Sung, MD –Geoffrey Galinkin, MD - John Kinsella, MD •Fetal Echocardiography –Debnath Chaterjee, MD - Therese Grover MD - Adel Younozsai, MD –Joy Hawkins, MD •Coordinators - Lisa Howley, MD – Carrie Dean, MD - Kelli Young, RN - Pei Ni Jone MD –Christine Wood, MD - Jann Hodge, RN - Karrie Villavicencio,MD •Radiology - Carrie Rafferty, RN •Genetics •Urology/Nephrology • Lisa Waters, RDMS - David Manchester, MD - Duncan Wilcox, MD – Mariana Meyers, MD - Naomi Meks, MD - Jeffrey Campbell, MD – Kim Dannull, MD - Vijay Velmulakonda, MD – Laura Brown, MD •Administrative •ENT – David Mersky, MD - Peggy Kelley MD • Obstetric Nursing - Kelli Young, RN, CNP - Jeremy Prager, MD •Jennifer Livingston, MD - Mary Beth Martin, RN, MBA •Neurosurgery •Jena - Gina Dooley - Michael Handler, MD •Tiffany - Corbett Wilkinson, MD •Operating Room •Cardiac Surgery • Erin Hughey,RN - James Jaggers, MD • Nicole Stuart, RN - David Campbell, MD • Heidi - Max Mitchell, MD