D&C SHANNON F. ROSATI SEPTEMBER 11, 2014

Case Presentation Male infant, born to a 34 y/o G4P3 female at 0942 on 9/1/14, at gestational age 38 weeks. Had significant respiratory distress at birth, requiring intubation. Transferred to MCV NICU at 15 hrs of life after CXR obtained which showed a L CDH for further management. Birth weight 3.95 kg. PMedHx: none PSurgHx: none Soc: no other h/o anomalies in siblings Imaging:

Pre-op

Read – CXR: CHEST: Multiple gaseous distended loops of bowel are identified herniating into the left hemithorax, with resulting left to right shift of the cardiomediastinal structures. Aerated lung is visualized along the periphery of the right hemithorax. ABDOMEN: There is a paucity of intra-abdominal bowel gas, with small amount of gas identified within the lower mid abdomen and pelvis. No definitive intraperitoneal air is identified.

HD 2 US of head obtained, normal with no sign of intracranial hemorrhage TTE obtained: Mild right atrial enlargement. Normal left atrial size. Patent foramen ovale. Normal mitral valve. Normal pulmonic valve. Right ventricle systolic pressure estimate normal. Normal left aortic arch. Normal pulmonary artery branches. Normal tricuspid aortic valve. Normal right ventricle structure and size. Normal left ventricle structure and size. Normal left ventricular systolic function. Qualitatively normal right ventricular systolic function. No patent ductus arteriosus detected.

HD 3 Went to OR for a thoracoscopic L CDH repair Repair performed with three trocars- 2 operating 3 mm instruments, 1 3mm trocar for 3mm 30 degree scope EBL: <5cc

Video

POD 0

Hospital Course Extubated evening of POD 0 Weaned off nasal cannula over next two days Feeds advanced Central line removed POD POD 3

Congenital Diaphragmatic Hernia Congenital Diaphragmatic Hernia (CDH), first described in 1754, first successful operative repair in an infant less than 24hrs old reported in 1946 Occurs in approximately 1 in every 2500 births (1600 cases in the US every year) Exact cause is unknown Approximately 50% of infants born with CDH do not survive 1/3 of affected infants have associated major defects Lung function, as determined by the degree of pulmonary hypertension and pulmonary hypoplasia, is the most important determinant of survival

Diaphragm Embryology Precursors of the diaphragm begin to form during the 4 th week of gestation with the appearance of the peritoneal folds from lateral mesenchymal tissue At the same time, the septum transversum forms from the inferior portion of the pericardial cavity and serves to delineate the thoracic from the abdominal cavities The septum transversum leads to the formation of the central tendinous area of the fully developed diaphragm The pleuroperitoneal folds extend from the lateral body wall and grow medially and ventrally until they fuse with the septum transversum and dorsal mesentery of the esophagus during the 6 th week of gestation Complete closure of the canal takes place during the 8th week of gestation

Diaphragm Embryology-Resident Question Which side of the diaphragm closes first?

Diaphragm Embryology-Resident Question Which side of the diaphragm closes first? Right side closes before the left by the 8 th week of gestation

Diaphragm Embryology-Resident Question Which side is more common for a CDH?

Diaphragm Embryology-Resident Question Which side is more common for a CDH? Defects more common on the left side (80% left, 20% right)

Diaphragm Embrology- Resident Question Name the hernia defects: A B C

Diaphragm Embrology- Resident Question Name the hernia defects: A- Eventration of the diaphragm B- Morgagni hernia C- Bochdalek hernia

Congenital Diaphragmatic Hernia- Resident Question Question: What is a Morgagni Hernia?

Congenital Diaphragmatic Hernia- Resident Question Question: What is a Morgagni Hernia? The anterior diaphragmatic hernia of Morgagni is located anteromedially on either side of the junction of the septum transversum and the thoracic wall Typically, a sac is present, and herniation of the colon, small bowel, or liver is usually discovered to the right or left of the midline Morgagni hernias account for less than 2% of diaphragmatic defects and usually present in older children or adults

Congenital Diaphragmatic Hernia- Resident Question Question: What is eventration of the diaphragm?

Congenital Diaphragmatic Hernia- Resident Question Question: What is eventration of the diaphragm? Eventration of the diaphragm is a disorder in which all or part of the diaphragmatic muscle is replaced by fibroelastic tissue The diaphragm retains its continuity and attachments to the costal margin Eventration of the diaphragm may be either congenital or acquired The congenital form may be indistinguishable from a diaphragmatic hernia with a sac, and symptoms are similar, although the presentation is typically clinically less severe

Congenital Diaphragmatic Hernia- Physiology If closure of the pleuroperitoneal canal has not occurred by the time the midgut returns to the abdomen during the 9th and 10th weeks of gestation, the abdominal viscera herniate into the ipsilateral thoracic cavity The resulting abnormal position of the bowel prevents its normal counterclockwise rotation and fixation

Congenital Diaphragmatic Hernia- Resident Question Question: Does a CDH often have a hernia sac present?

Congenital Diaphragmatic Hernia- Resident Question Question: Does a CDH often have a hernia sac present? No hernia sac is present if the event occurs before complete closure of the pleuroperitoneal canal, but a nonmuscularized membrane forms a hernia sac in 10% to 15% of CDH patients In addition to the small bowel, other intra- abdominal organs such as the spleen, stomach, colon, and liver may also herniate through the diaphragmatic defect

Congenital Diaphragmatic Hernia- Treatments and Procedures

Thoracoscopic Repair of CDH- Literature Review 1.Is it safe? 2.What are clinical indications for a minimally invasive approach? 3.How does minimally invasive repair compare to open repair?

Study performed to examine outcomes and rate of successful completion between open and thoracoscopic approaches in CDH repair Retrospective analysis from Feb 2004-Jan 2008 Two groups: open and thoracoscopic Exclusion criteria: repair after 50 wks post conception, recurrent hernia, congenital cardiac anomalies (other than PFO or PDA), preoperative identification of a Morgagni hernia, or ECMO support Analysis of 31 infants (14 thoracoscopic and 17 open)

Results: 79% of thoracoscopic operative approaches were completed successfully No differences were found between total ICU days, LOS, days on a ventilator, or days until enteral feeds were started between the 2 groups There was a significant difference between the 2 groups with respect to estimated gestational age (39 vs 36.5 wks, p=0.006) and operating room time (70 vs 145 minutes, p=0.004)

Conclusion: A thoracoscopic approach for CDH repair is a safe and feasible surgical approach in select infants

Retrospective study between Jan 2008 and Dec 2012, Goal of study was to determine clinical indications for thoracoscopic approach for CDH repair 35 patients total, 20 open, 15 thoracoscopic

Conclusions: Minimally invasive repair of CDH can be suggested as the treatment of choice for patients with a small (<3cm) size diaphragmatic defect, in neonates with stable hemodynamics and without additional anomalies, or in infants with delayed presentation of CDH, with excellent clinical outcomes

Meta-analysis comparing open and minimally invasive repair 10 studies included with study periods ranging from Primary outcome of interest was recurrence

Results: Total recurrence was higher after MIS repair (p<0.001) Subgroup analysis indicated higher recurrence after MIS for patch repairs (p<0.001), but not for primary repairs Operative time was longer for MIS (p<0.001) Post operative ventilator time and post operative mortality was higher after open surgery (p=0.04 and p<0.001)

Conclusions: Recurrence rate is higher after MIS than open repair when a patch is used Operative time is also longer with MIS Poorer outcomes after open surgery may be due to selection bias

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