NEC and Aortic Coarctation

Espino

Pediatric Surgery

3.26.15

PGY 1 - 3 casesAndrew TraceyJason Wood

PGY 2 - 20 casesTori WhitlowAmar Shah

PGY 4 - 37 casesSasa Espino

Total Cases - 60

Duty hour violations 0

Deaths and Complications

Attending/ Resident DATE PATIENT Dx/PROCEDURE COMPLICATION

OiticicaLanning 02.26 morbid obesity/

gastric plication readmission

Haynes/Espino 02.27

Aspiration/Lap Nissen, g tube

placementg tube dislodgment,

death

Haynes/Espino 03.06 NEC/

Exlap, silo placementNEC, h/o aortic

coarctation

Complication

Date:03.06.2015

Fac/Res:Haynes/Espino

Procedure: Exlap, bowel

resection, coarc repair

Complication: ACS/NEC

Background◈ HPI

4 month old born at 26w 2d after PPROMIntubated DOL 1, given surfactant, extubated DOL 2Weaned to RA DOL 9Failure to thrive and reflux → UGI showed hiatal herniaLap Nissen and G tube placement 02.05.15Reintubation - RSV, Klebsiella PNAFound to have prominent systolic ejection murmur,

HTNTTE showed aortic coarctation w high gradientTransferred to MCV 02.09.15

◈ PMH - Premature birth, GERD, hiatal hernia

◈ PSH - Lap Nissen, g tube placement, hiatal hernia repair, circumcision

◈ FH - Mom - epilepsy, CHD surgery - likely either coarc or PDA given lateral thoracotomy

BackgroundPhysical ExamGeneral - sedated, ETT in placeHEENT - No notable edema. mucous membranes pink and moistNeck - No JVDChest - Symmetric hemithoraces. CTABCV - Regular rhythm. Normal S1 and S2. harsh systolic murmur III-VI. No

gallop. PMI is nondisplaced with a normal impulse. Extremities are warm with <2 second capillary refill. Distal pulses are brisk and equal in the upper and lower extremities.

Abd - Soft with normoactive bowel sounds. Liver edge at the RCM

TransThoracic Echocardiogram

Coarctation of the aorta, juxtaductal. Severe concentric left ventricular hypertrophy. Hyperdynamic left ventricular function. Normal tricuspid aortic valve. Collateral vessels noted in the area of the coarctation. Intact atrial septum. No patent ductus arteriosus. Peak gradient through the coarctation area is 88mmHg.

Cardiac Cath - 02.12.15Left Heart Catheterization with Descending Aortogram of the Coarctation: The coarctation is not discrete as it appeared on the echocardiogram. There is a brachiocephalic trunk with the bilateral carotids arising from it. The isthmus of the aorta appears narrowed and the coarctation appears to be 1 cm in length with a diameter of 3-4 mm. Gradient appears to be 40-45 peak mmHg pre to post coarctation

Post Procedure:

- Arterial line in L femoral access site. 3-4h after procedure found to have poor perfusion at L leg. Nonpalpable, nondopplerable pedal pulse. Arterial line removed. Heparin gtt started

- HTN w SBP in 200s - Nicardipine gtt started 02.13.15 for UE BP control. Goal SBP <140

Abdominal Distention02.15.15 - concern for Abdominal Compartment Syndrome

Abdominal distention

Decreased UOP, increase in creatinine

Worsening oxygenation

Hypotension

Pediatric Surgery consult:

Exploratory laparotomy, partial small bowel resection, placement of abdominal wall silo, central line

- 115cm of necrotic and perforated small bowel resected

- 45cm of viable SB beyond ligament of Treitz and 5 cm of viable TI

- All colon and rectum viable

- Covered with pre-formed silo

Necrotizing Enterocolitis02.17.15- re-exploration, removal of silo, distal jejunum resection, terminal ileum resection, replacement of silo

Findings: approx 13 cm of distal jejunum w necrotic patches on antimesenteric border. approx 8 cm terminal ileum w necrotic patches on antimesenteric border. necrotic jejunum perforated during manipulation of bowel. cecum w slightly dusky appearance but no frank necrosis. remainder of colon viable.

02.27.15 - abd washout, ileostomy creation, abdominal closure

Findings: significant adhesions, no further perforation or necrosis

Aortic Coarctation03.10.15 - Repair of coarctation with extended end to end anastomosis via L thoracotomy

Ductus ligation and division

Right radial artery cutdown.

Findings: no post repair gradient

Right radial artery 141/72

Descending aorta direct measurement 138/72

Necrotizing Enterocolitis- Neonatal disease characterized by an initial intestinal mucosal injury

- May ultimately progress to transmural bowel necrosis

- Most frequently encountered neonatal surgical emergency

- Pathogenesis remains obscure

- Surgical treatment directed at controlling complications

- Most common site is terminal ileum, R colon

- May be localized, segmental or entire GI tract

- Tissue demonstrates submucosal edema, hemorrhage, microvascular thrombosis leading to transmural necrosis

- Dissection of intraluminal gas through the mucosa leads to gas in bowel wall, aka pneumatosis intestinalis

Clinical Presentation- Approx 50% pts extremely low birthweight weighing <1500g

- Mean gestational age 30.4 weeks

- 1-3 of 1000 live births; 30 per 1000 low birthweight births

- Classic clinical signs:

- abdominal distention

- feeding intolerance

- bilious emesis

- occult or gross blood in stool

- Physical exam findings:

- edema, erythema, crepitus of abdominal wall suggests necrosis, perforation, intra-abd abscess

- Systemic signs:

- temperature instability, apnea, bradycardia, hypoxemia

Diagnosis- Primary diagnostic goal is to determine if irreversible, transmural intestinal necrosis is present

- Radiographic confirmation of NEC requires only plain abdominal films

- Serial abdominal films should be obtained during early course

- Classic radiographic findings:

- pneumatosis intestinalis

- thickened bowel loops

- ascites

- portal venous gas

- Presence of pneumoperitoneum mandates operative intervention

Treatment- Nonoperative

- vast majority can be managed medically

- decompression, bowel rest, IV abx (usu 7-14 days)

- correction of systemic derangements

- TPN (enteral feeding usually resumed when abx stopped)

- Operative

- resection of involved intestine (does not prevent further extension of disease in other involved areas of the bowel

- resection of necrotic bowel w proximal enterostomy and distal mucous fistula placement

- primary goal is expedient operation w preservation of as much length as possible, including the ileocecal valve

- preserve marginal areas of involved intestine w 2nd look

Complications, Results, Outcomes

- Morbidity and mortality primarily related to problems associated w prematurity

- Overall surgical complication rate 20-40%

- 26% mortality rate

Aortic Coarctation• 7% of children w CHD• 7th most common CHD• Males > Females by factor of 2• Associated w Turner’s, Sturge-Weber,

neurofibromatosis, William’s syndrome• Complications include aneurysm, dissection,

endocarditis• Unoperated coarctation has 90% mortality by

age 50. Average age of death at 35yo

Anatomy• Juxtaductal coarctation (adult type)

– Narrowing of thoracic descending aorta just beyond remnant of ductus arteriosus

– 95% of all coarc cases– Collaterals develop– 70% have bicuspid aortic valve

• Tubular hypoplasia (infantile type)– More diffuse narrowing of transverse aorta– Proximal to PDA

Plain film• Advantages

– Ease of access– Relative ease of interpretation– Sensitive

• Limitations– Nonspecific– Lack of quantitative data

• Findings– Classic “3” sign– Rib notching of posterior third of ribs 3-8– “E” sign on esophagram– Infantile cardiomegaly– Infantile pulmonary congestion

Echocardiogram, MRI, Angiography• Echocardiography - Ease of accessibility, quantitative data

w pressure gradients, LVH, anatomical measurements• MRI - Allows topographic imaging, accurate quantitative

data measurements, no ionizingradiation or contrast medium

• Angiography - gold std, definevalvular/anatomic disease,delineates collateral circulation,opportunity for therapeuticintervention

Therapeutic OptionsSurgical

– Resection, end to end anastomosis – gold standard– Indicated for pts w transcoarctation pressure gradient >30mmHg– 90% of kids normotensive w 5 year f/u– Residual HTN develops in ½ pts >40years

Catheterization– Balloon angioplasty – indicated for postop restenosis and dilatation

of native coarc– Mortality ranges from 0 to 2.5% compared w mortality of surgery 4-

25%– 50% restenosis rates– Limited by higher incidence of aortic aneurysm and recurrent coarc

- Objective: determine whether differences exist in the location of NEC in infants w congenital heart disease versus those without CHD

- Retrospective cohort study over 11 years

- n = 167 pts

- Results: no difference in location of NEC between non CHD and CHD pts- Predominant location of NEC in small intestine- No difference in location of NEC between preterm non CHD and full term CHD

Assessment◈ Delay in coarc repair◈ Aggressive BP control◈ Delay in ACS diagnosis and

treatment