CONGENITAL DIAPHRAGMATIC HERNIA Maj Asrar Ahmad MBBS, FCPS

Anatomy 5 % Efficacy: later have randomized trial comparing lifestyle with pharmacologic; and a decision analysis model paper comparing lifestyle and surgical. 95 %

Embryology Efficacy: later have randomized trial comparing lifestyle with pharmacologic; and a decision analysis model paper comparing lifestyle and surgical.

Pathophysiology 61% of U.S. adults are overweight (BMI > 25 kg/m2) and obese (BMI > 30 kg/m2) [1] 22.3% -30.5% are obese [1-3] Compare to breast and colon CA combined 95,000 deaths/yr [4] seventh leading cause of death [1] Leptin produced by adipose decreases food intake (high leptin inhibits appetite stimulant NPY; also stimulates B3-adrenergic receptors…uncoupling proteinIn to generate heat instead of ATP. Initially there was speculation that obesity was caused by leptin deficiency but actually the majority of obese patients have high leptin, so we’re now thinking leptin insensitivity (like insulin insensitivity in diabetes. [2] Chronic. Obvious but important to keep in mind. You don’t become acutely obese nor can you acutely cure it (we’ll see considering surgery). Like HTN treatment Stigmatized. “Historically the public has believed that weight loss is a matter of willpower. Obese individuals have been considered weak-willed and unmotivated.

Pathophysiology Pulmonary hypoplasia Pulmonary hypertension “compression theory” - modeled in fetal lambs - rationale for early surgery “global embryopathy” - modeled in newborn rats - rationale for new therapeutic ideas Pulmonary hypertension - causes persistent fetal circulation When you’re treating obesity, every pound counts.

Incidence 1:2500-5000 live births 1100 cases in the U.S. annually 80 % Left side survival remains around ~65% “It can be estimated that if everyone were at optimal weight we would have 25% less CHD, and 35% less CHF and brain infarcts” NEJM analysis of Framingham data [3]

Diagnosis Antenatal: U/S at ~20 weeks gestation ~60% Polyhydramnios; intrathoracic stomach or liver; abdominal circumference; lung-to-head ratio Ideal Body Weight for 6ft tall man: 165-190; he’s morbidly obese (BMI > 35 with comorbidities, or BMI > 40). According to Framingham (risks: age, HTN, low HDL) patient has 20% 10-year risk of developing CHD. Goal LDL < 130; can do TLC <7% cal from saturated fats, < 200mg/d total cholesterol intake, physical activity 30min/d, most days of week, appropriate weight control).

Presentation Shortness of breath Scaphoid abdomen Three general presentations: Severe respiratory distress at the time of birth. Respiratory deterioration hours after delivery Benefit from correction of hypoxemia and pulmonary hypertension Feeding difficulties, chronic respiratory disease, pneumonia 10-20 % intestinal obstruction

Initial Management Oxygenate but avoid barotrauma Intubate Sedate NGT for decompression LDL and total chol decrease after 20% reduction in body weight Linear decrease in serum glucose, insulin, and TG with increasing weight loss 0.3-1.0mmHg decrease in BP for every 1.0 kg lost.

Medical Management Medical emergency not surgical Pulmonary vasodilators Inotropes High frequency oscillatory ventilation ECMO Surfactant Antenatal steroids? National Academy of Science: “successful long-term weight control means losing at least five percent of body weight…and keeping it below our definition of successful weight loss for at least one year.” Studies: Mortatlity rates lower in those trying to lose weight, even if they did not actually lose weight. [3] 2. Any amount of intentional weight loss aw a 10% reduction in CV dz, 20% reduction in all-cause mort., 30-40% reduction in DM mort., 40-50% reduction in mort. from cancers related to obesity. [3] 3. Even small weight loss are associated with improvements in HTN, hypercholestorlemia, and DM2. 4. 7% weight loss and increase physical activity reduced risk of developing DM2 despite the fact that most participants regained 1/3 of their lost weight in the next 4 years [5]. 337 --> 287-320 (BMI still 39-43)

ECMO Risk factors: cigarette smoking; HTN (or on HTN meds); LDL >160 or LDL 130-159 + 2 other RFs; HDL < 35; fasting blood glucose 110-125; FamHx of premature CHD; increased abdominal fat, hyperlipidemia, male, age < 40. Get to know the patient: level of motivation, energy intake (dietary recall), energy expenditure (physical activity); negative life events (social factors that might make wt loss difficult); psych problems (eating disorders) Patient-physician agreement Setting realistic and concrete goals: (0.4-0.5 kg/wk [5]) Educating patients (common problem, health risks, rationale for weight loss, chronic problem) 1 Diet eat what you like but reduce intake by about 500 to 1000 cal/day; can be accomplished simply by reducing portion size and fats and sugars 2 Exercise 30 minutes/d; 5-6x/wk; several brief walks during the day are equally beneficial or better than one single long bout of exercise [5] 3 More frequent follow up aids in behavioral therapy, also homework assignments, concrete goals, modifying cues that elicit unwanted eating, regularly recording weight and intake and output

ECMO Risk factors: cigarette smoking; HTN (or on HTN meds); LDL >160 or LDL 130-159 + 2 other RFs; HDL < 35; fasting blood glucose 110-125; FamHx of premature CHD; increased abdominal fat, hyperlipidemia, male, age < 40. Get to know the patient: level of motivation, energy intake (dietary recall), energy expenditure (physical activity); negative life events (social factors that might make wt loss difficult); psych problems (eating disorders) Patient-physician agreement Setting realistic and concrete goals: (0.4-0.5 kg/wk [5]) Educating patients (common problem, health risks, rationale for weight loss, chronic problem) 1 Diet eat what you like but reduce intake by about 500 to 1000 cal/day; can be accomplished simply by reducing portion size and fats and sugars 2 Exercise 30 minutes/d; 5-6x/wk; several brief walks during the day are equally beneficial or better than one single long bout of exercise [5] 3 More frequent follow up aids in behavioral therapy, also homework assignments, concrete goals, modifying cues that elicit unwanted eating, regularly recording weight and intake and output

Surgical Management Most important to recognize that obesity is a chronic condition so interventions are typically permanent (ie. Diet is a lifestyle change; OA people consider themselves addicts or recovering addicts for life; surgery will permanently reduce the size of your stomach, current recommendations suggest that obesity drugs may have to be taken indefinitely) Physician counseling (everyone should be counseled). Commercial programs (Jenny Craig, Weight watchers) Behavioral therapy/programs (OA, TOPS) Hospital-based programs (OPTIFAST, HMR) Pharmacological treatment (orlistat, sibutramine) Residential programs Bariatric surgery (Roux-en-Y, VBG) Relevant especially to primary care: simple physican counseling on diet, pharmacotherapy, referral to surgery.

Surgical Management Most important to recognize that obesity is a chronic condition so interventions are typically permanent (ie. Diet is a lifestyle change; OA people consider themselves addicts or recovering addicts for life; surgery will permanently reduce the size of your stomach, current recommendations suggest that obesity drugs may have to be taken indefinitely) Physician counseling (everyone should be counseled). Commercial programs (Jenny Craig, Weight watchers) Behavioral therapy/programs (OA, TOPS) Hospital-based programs (OPTIFAST, HMR) Pharmacological treatment (orlistat, sibutramine) Residential programs Bariatric surgery (Roux-en-Y, VBG) Relevant especially to primary care: simple physican counseling on diet, pharmacotherapy, referral to surgery.

Surgical Management Most important to recognize that obesity is a chronic condition so interventions are typically permanent (ie. Diet is a lifestyle change; OA people consider themselves addicts or recovering addicts for life; surgery will permanently reduce the size of your stomach, current recommendations suggest that obesity drugs may have to be taken indefinitely) Physician counseling (everyone should be counseled). Commercial programs (Jenny Craig, Weight watchers) Behavioral therapy/programs (OA, TOPS) Hospital-based programs (OPTIFAST, HMR) Pharmacological treatment (orlistat, sibutramine) Residential programs Bariatric surgery (Roux-en-Y, VBG) Relevant especially to primary care: simple physican counseling on diet, pharmacotherapy, referral to surgery.

Surgical Management

Developing Therapies Fetal surgery PLUG fetal surgery Growth factors

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