case presentation · fetal pulmonary blood flow is characterized as a low-flow high-resistance...

Case Presentation 4yF with trisomy 21 p/w cough, congestion, wheezing for several days. Intermittent difficulty breathing for last two months. No fever, chills, N/V/D/C. PMH: trisomy 21, seizures; full term vaginal delivery PSH: repair VSD and coarctation DOL#5, reaugmentation coarctation DOL#150, repair chylothorax DOL#152, plication of left hemidiaphragm for paresis DOL#158, gastrostomy DOL#172 Meds: albuterol, pulmicort, prednisolone, azithromycin

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Physical and Labs • T98.8, HR103, BP104/62, RR22, Sat97 • 33inch (<3%), 22.7lb (<3%) • NAD • Coarse breath sounds left side • Abdomen soft NT ND • Well healed sternotomy, left posterolateral

thoracotomy and gastrostomy scars

• CBC 17/13/39/319


Chest XR www.downstatesurgery.org

Presenter

Presentation Notes

Similar to two prior studies 3/18/13 and 6/15/13 Different than birth 9/24/09

Chest CT www.downstatesurgery.org

Presenter

Presentation Notes

Cleared by cardiology after EKG (right bundle branch block) and echo (no residual VSD, minimal gradient across coarctation)

Operation

• Left subcostal incision • Left posterolateral diaphragmatic defect identified • Small and large bowel reduced • Hernia sac excised • Chest tube placed • Defect closed with interrupted 0 and 2-0 silk • No malrotation identified


Postoperative Course

• POD#0 Admitted to PICU • POD#3 Return of bowel function • POD#4 Chest and nasogastric tubes removed • POD#5 Discharged home


Congenital Diaphragmatic Hernia

Christopher Turner 05.15.14


Overview

• History • Epidemiology • Embryology • Diagnosis • Management • Outcomes


Presenter

Presentation Notes

Prenatal, ICU, operative, fetal

History

1754, McCauley: First description 1925, Hedblom: First review (33/44 mortality, 75%) 1940, Ladd and Gross: First single series with consistent survival (7/16 mortality, 44%) 1946, Gross: First report of successful operative repair in infant younger than 24h

Immediate neonatal surgery standard of care until the 1980s


Ladd and Gross NEJM 1940


Epidemiology

• Incidence 1:2000-5000 • 1/3 die in utero, “hidden mortality” • 90% posterolateral • 85% left, 13% right, 2% bilateral • 50% isolated defect • 27.5% cardiovascular, 17.7% urogenital, 15.7%

musculoskeletal, 9.8% central nervous


Embryology

4th week 8th week


Presenter

Presentation Notes

Anterior: septum transversum Dorsolateral: pleuroperitoneal folds Dorsal: crura from esophageal membrane Posterior : body wall mesoderm Precusors to diaphragm begin to form during the fourth week of gestation As the PPF develops during the sixth week of gestation, concurrently, the pleuroperitoneal membranes close and separate the pleural and abdominal cavities by the eight week of gestation

Pathophysiology

• Diaphragmatic hernia – Posterolateral or Bochdalek – Anterior or Morgagni – Central septum transversum

• Pulmonary hypoplasia • Pulmonary hypertension


Presenter

Presentation Notes

Pulmonary hypoplasia is characterized by a decrease in bronchial divisions, bronchioles, and alveoli. The alveoli and terminal saccules exhibit abnormal septations that impair the air-capillary interface limiting gas exchange. Interestingly, the contralateral lung also exhibits the structural abnormalities of pulmonary hypoplasia.

Fetal Circulation

• Ductus venosus • Foramen ovale • Ductus arteriosus


Presenter

Presentation Notes

Fetal pulmonary blood flow is characterized as a low-flow high-resistance circuit due to medial and adventitial hypertrophy of the vasculature. Normally the pulmonary vascular resistance quickly decreases as the distal small pulmonary arteries and arterioles remodel over the first few months of life, resulting in a low-resistance high-flow circuit. However, this process appears to be arrested in CDH newborns, and the fetal circulation persists resulting in PHTN. In fact the abnormal fetal pulmonary circulation in CDH fetuses appears to occur early in gestation.

Prenatal Diagnosis

• 50-70% diagnosed in pregnancy • US diagnosis as early as 11wks • Findings include polyhydramnios, bowel loops

in chest, echogenic chest mass, intrathoracic stomach, cardiac shift

• Features to risk stratify – Lung-to-head ratio – Liver herniation into chest


Presenter

Presentation Notes

100% survival LHR>1.35 61% survival LHR 1.35-0.6 No survival LHR<0.6

Ultrasound www.downstatesurgery.org

Presenter

Presentation Notes

Gastric bubble at that the level of the four-chamber heart suggests CDH. This is the level used to calculate LHR.

MRI www.downstatesurgery.org

Presenter

Presentation Notes

A large CDH with herniation of the small bowel and stomach is found within the left hemithorax, dextroposition of the fetal heart, and no evidence of liver herniation. Excellent modality for morphologic and volumetric measurements of the fetal lung. It is especially advantageous for oligohydramnios and maternal obesity.

Clinical Presentation

• Tachypnea, chest wall retractions, grunting, cyanosis, pallor

• Scaphoid abdomen, increased chest diameter, decreased breath sounds, presence of bowel sounds in chest, displacement of heart sounds

• Typically confirmed with CXR showing bowel loops in chest

• 20% may present outside neonatal period


CXR www.downstatesurgery.org

Prenatal Care

• Advanced US to determine associated anomalies and signs of fetal compromise

• Chromosomal screening • Maternal counseling • Referral to tertiary perinatal center

• Prenatal glucocorticoids not currently

recommended


Stabilization and Monitoring

• Tracheal intubation • CPAP contraindicated • Large bore NGT or OGT • H2 blockers or PPIs • Pulse oximetry probes placed preductally (right

arm or earlobe) and postductally (lower extremities) indicate presence of RL shunt and PHTN

• Consider arterial and venous catheters


Presenter

Presentation Notes

Preductal values indicate flow to the brain via carotid arteries, critical. Postductal values indicate flow to the body, less critical.

ICU Management

• Pressure support with minimal sedation • Limit tidal volume to 4-6cc/kg and peak airway

pressure to 25-30 cm H2O • Maintain preductal pO2>60 and pCO2<60 • Alkalosis with NaHCO3 or THAM improves PHTN • Dopamine or dobutamine may be needed • Systemic antibiotics with ampicillin and

gentamicin • ECMO for persistent hypoxemia or hypotension


Presenter

Presentation Notes

More aggressive than ARDSnet Volutrauma and barotrauma kills CDH babies Permissive hypercapnia Pulmonary vascular resistance is increased by hypoxia and acidosis

Surgery

• Repair is delayed until cardiopulmonary stability

• Open abdominal approach is gold standard • Thoracic or minimally invasive also options • Diaphragmatic replacements include non-

absorbable synthetic, absorbable biosynthetic, autologous tissue, tissue engineered

• 50% require replacement • 50% will recur


Presenter

Presentation Notes

Advantages to laparotomy include easier reduction of intrathoracic viscera, the ability to mobilize the posterio rim of diaphram, easier management of intestinal rotational anomalies, and avoidance of thoracotomy associated musculoskeletal sequelae. The vast majority of neonatal repairs are through a subcostal incision 90%. Nonabsorbable synthetic patch = goretex (polytetrafluoroethylene) or marlex (polypropylene) Absorbably biosynthetic patch = surgisis (SIS, porcine intestinal submucosa) or permacol (porcine dermal collagen) or alloderm (human dermal collagen)

Fetal Therapies

• Harrison et al. Correction of Congenital Diaphragmatic Hernia in utero: A Prospective Trial. Journal of Pediatric Surgery, 1997.

• Harrison et al. A Randomized Trial of Fetal Endoscopic Tracheal Occlusion for Severe Fetal Congenital Diaphragmatic Hernia. New England Journal of Medicine, 2003.


Fetal Endoscopic Tracheal Occlusion (FETO)

• Clinical observation: infants with congenital high airway obstruction develop hyperplastic lungs

• Animal model: tracheal ligation in the fetus accelerates lung growth beyond normal limits and prevents pulmonary hypoplasia associated with CDH


Animal Model of Tracheal Occlusion www.downstatesurgery.org

Presenter

Presentation Notes

(A) Gross necropsy finding in a newborn lamb with experimental congenital diaphragmatic hernia (CDH). The scissors are placed through the defect on the left diaphragm. Abdominal viscera are present in the left hemi-thorax. The lungs are small and not visible. (B) Gross necropsy finding in a newborn lamb with experimental CDH and complete tracheal ligation. The forceps are holding the left diaphragm. Both lungs are markedly enlarged. The left lung has completely reduced the herniated abdominal viscera and has grown into the abdomen, through the diaphragmatic defect. Extensive lung morphometry confirmed hyperplasia in the enlarged lungs. Permission for reproduction requested from: Wilson JM, DiFiore JW, Peters CA: Experimental fetal tracheal ligation prevents the pulmonary hypoplasia associated with fetal nephrectomy: possible application for congenital diaphragmatic hernia. J Pediatr Surg 28:1433-1439; discussion 1439-1440, 1993.

FETO www.downstatesurgery.org

Presenter

Presentation Notes

Current in utero techniques involve endoscopic insertion of an occlusive balloon in the fetal trachea without maternal laparotomy or general anesthesia. These ballons are inserted percutaneously with ultrasound guidance between 24-28 weeks gestations and are deflated at 34 weeks. This strategy of temporary tracheal occlusionavoids the need for an EXIT procedure at delivery, although emergent airway access may be needed at delivery for any patient who undergoes in utero tracheal occlusion.

FETO www.downstatesurgery.org

Presenter

Presentation Notes

Open fetal surgery has been abandoned

FETO Clinical Trials

• US, 2003: n=24, LHR<1.4 and liver in chest, survival 73% vs 77%, terminated early

• Europe, 2009: n=210, LHR<1 and liver in chest, survival 49% vs 24%

• Brazil, 2012: n=41, LHR<1 and liver in chest, survival 50% vs 4.8%

• Multicenter RCT currently in process


Presenter

Presentation Notes

Open fetal surgery has been abandoned (A) Gross necropsy finding in a newborn lamb with experimental congenital diaphragmatic hernia (CDH). The scissors are placed through the defect on the left diaphragm. Abdominal viscera are present in the left hemi-thorax. The lungs are small and not visible. (B) Gross necropsy finding in a newborn lamb with experimental CDH and complete tracheal ligation. The forceps are holding the left diaphragm. Both lungs are markedly enlarged. The left lung has completely reduced the herniated abdominal viscera and has grown into the abdomen, through the diaphragmatic defect. Extensive lung morphometry confirmed hyperplasia in the enlarged lungs. Permission for reproduction requested from: Wilson JM, DiFiore JW, Peters CA: Experimental fetal tracheal ligation prevents the pulmonary hypoplasia associated with fetal nephrectomy: possible application for congenital diaphragmatic hernia. J Pediatr Surg 28:1433-1439; discussion 1439-1440, 1993.

Outcomes, JPS 2007 www.downstatesurgery.org

Presenter

Presentation Notes

CDH study group currently working on an evidence-based risk stratification classification “A” defects were entirely surrounded by muscle, “B” defects had a small (b50%) and “C” defects a large (N50%) portion of the chest wall devoid of diaphragm tissue, and “D” patients had complete or near complete absence of the diaphragm.

Standardized Reporting System, JPS 2013

A: Entirely surrounded by muscle B: <50% devoid C: >50% devoid D: complete or near complete absence


Presenter

Presentation Notes

CDH study group currently working on an evidence-based risk stratification classification “A” defects were entirely surrounded by muscle, “B” defects had a small (b50%) and “C” defects a large (N50%) portion of the chest wall devoid of diaphragm tissue, and “D” patients had complete or near complete absence of the diaphragm.

Follow-Up Guidelines www.downstatesurgery.org

Conclusion

• CDH leads to pulmonary hypoplasia and pulmonary hypertension

• Stabilize with intubation and NGT/OGT • Volutrauma and barotrauma kills these babies • Delay operation until cardiopulmonary

stability • Outcomes predicted by size of defect and

major cardiac anomalies


SESAP Question

Which of the following statements about neonatal CDH is correct? A. Defect size is not an important factor in overall

survival B. HFOV is a contraindication for thoracoscopic repair C. Primary repair has a survival rate of 90% D. Patch repair has equivalent long term morbidity

compared with primary closure E. ECMO does not improve overall survival


Snickers Award for Most Presentations*

#1 Dr Stefan Kénel-Pierre, 15

(also most languages spoken, 6)

#2 Dr Aliu Sanni, 13 #3 Multiple, 12

* Through June 2014 according to downstatesurgery.org


case presentation · fetal pulmonary blood flow is characterized as a low-flow high-resistance...

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