Hand Out Peds Shikha

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PYLORIC STENOSIS

INTRODUCTION:-

Hypertrophic pyloric stenosis is one of the common surgical problems in infancy. It may be familial and usuallyfound in first born male child.Hypertro phic pyloric stenosis is a marked and progressive overgrowth or enlargement of circular musclefibers of pylorus causing partial or total obstruction of the stomach outlet due to narrowing of the lumen.

ETIOLOGY: -

The exact cause of hypertrophic pyloric stenosis is not yet fully understood. Previously it was thought to be acongenital problem. But it is now known that the condition is not a congenital problem but an acquired condition.

The etiological factors are considered as maternal stress in last trimester, elevated prostaglandin level, deficiencyof nitric acid and immature pyloric ganglion cells with abnormal muscle innervations. The local entericinnervations are involved and primarily argyrophilic nitrergic neurons are affected.

PATHOLOGY: - The circular musculature of the pylorus is thickened and increased in size as the shape and size of 'Olive'. The

pyloric muscles become elongated and enlarged causing narrowing of the lumen. Stomach becomes dilated. Gastricemptying becomes delayed.

CLINICAL MANIFESTATIONS: -

Clinical onset of the condition usually found in 3 to 12 weeks of age. Initially occasional regurgitation may befound. Gradually vomiting increases in frequency and intensity. The characteristic projectile non-bilious vomitingoccurs forcefully within 30 minutes of feeding. Vomitus contains milk and gastric juices but it may be blood stainedand coffee ground in color.

The child presents with other classical features like constant hunger, irritability, failure to thrive with loss of weight, constipation, decreased quantity of stools and urine output. The child is usually lethargic with shallowrespiration. Starvation diarrhea as greenish stools, jaundice and gastric hemorrhage occasionally may be

present. Dehydration due to lack of fluid intake is a common feature.The important clinical findings include epigastric fullness with visible peristalsis in the upper abdomen from left toright and a palpable olive shaped firm mass (2-3 cm) in epigastrium or right hypochondrium.

COMPLICATIONS: -

The common complications are fluid and electrolyte imbalance (especially alkalosis and dehydration),hematemesis, jaundice and tetany.

DIAGNOSIS

History of illness and clinical examination is diagnostic. Plain X-ray abdomen, barium meal X-ray, USG, help toconfirm the diagnosis. Blood examination for Hb%, serum electrolyte and urine examination may be done beforesurgery.


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MANAGEMENT: - There is no medical management of the condition. Surgical management is the choice. Ramstedt's pyloromyotomyis done at the age of 4 to 5 weeks after the initial conservative management of dehydration and dyselectolytemia. Incase of pylorospasm, gastric lavage with normal saline, atropine methyl nitrate and metoclopramide may be used.

NURSING MANAGEMENT: -Pre-operative nursing management should emphasis on maintenance of fluid and electrolyte balance along withnutritional intake by breastfeeding, if not contraindicated. Relief of parental anxiety and routine pre-operative careare important. Nasogastric aspiration may be needed to reduce vomiting. During feeding, baby should be placedin upright position on slightly right side. Small frequent feeding to be given. Gentle handling of the baby is essential.Measures to be taken for prevention of hospital acquired infection. Continuous monitoring of infants is veryimportant. Recording of vital signs, hydration, body weight, vomiting, stool, urine and complications should bedone frequent1% , involvement in baby care to be promoted, e. g. Hygienic care.

Post-operative nursing management should be done with basic post-anesthetic care and routine operated patent. Special attention to be provide_- feeding, usually after 8-12 hours of surgery, small feeds with expressed breast milk to be started in manner. Initially feeding can be started with 1 or of clear solution (5% glucose) givenevery 2 hours 8 hours, after the child comes out of anesthesia the baby does not start vomiting. After feeding L-1, be

placed with elevating the head for 45-60 mins. & on the right side of the baby.Routine post-operative care to be pro% i - warmth, feeding, wound care, medications, h\ ,.-_ - emotional support

to parents and health education with discharge advice for continuation of care at home & for follow-up. Baby isusually discharged on 3rd - operative day. Prognosis is good, if operated timely before, complications start.

Post-operative complication develop as infections, fluid-electrolyte persistent obstruction and duodenal perforation.

GASTRO-ESOPHAGEAL REFLUX (GER) DISEASE

INTRODUCTION: -

Gastroesophageal Reflex (GER) is malfunction of the distal end of the esophagus resulting spontaneous

effort less return of stomach contents into the esophagus.

It may be physiologic, below the age of one year, due to inadequate development of lower esophageal

pressure regulation. After the age of one year, the GER can be considered as pathological and

usually associated with appropriate transient relaxation of lower esophageal segment. It may occur

along with swallowing and pharyngeal contractions.

The cause of GER is mostly undetermined. The causes can be delayed neuromuscular development,

physiological immaturity, cerebral defects, and increased abdominal pressure &

obesity. The associated conditions which can cause GER are supine position, coughing and wheezing in case of

cystic fibrosis, bronchopulmonary dysplasia, asthma, indwelling orogastric or nasogastric tube, medications

like theophyllin and mechanical ventilations.


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CLINICAL MANIFESTATIONS

Clinical features of GER vary in infants and older children.

'In infants , the featur es are unexplained vomiting immediate after feeding, regurgitation, rumination,

refusal to eat and features of dehydration. The infants also presents irritabi lity, excess ive crying, sleep

disturbances, arch ing and stiffening. Respiratory symptoms like cough, sneeze, strider and pneumonia may

found. Loss of weight or failure to gain weight is usual feature.In older children features are non-cardiac chest -comfort, upper abdominal discomfort, chronic

cough, -2dor, nocturnal asthma, dysphagia, anemia, hemetemesis or melena.

Diagnosis

His tory of fee din g behav ior , pre sen ting sig ns and symptoms are essential for diagnosis.Barium swa llow, eso phag eal pH monito r ing, Technet ium scintigraphy, esophagealma nome try , endoscopy and esophageal histology are useful diagnostic evaluation procedure to confirm thedisease.

Management

Management to be planned to alleviate and relieve the mptoms and prev ent compl icatio ns. Medica lan d su rgical management may be required.Medical Management

1. Positioning It helps to reduce the amount of reflex.a. Infants younger than 6 months should be placed on right lateral position during sleep, head of the

crib should be raised at least 6 inches. The infant may also be held upright. b. Older children should be placed in head raised to 30- 45 angle position. Avoid recumbent position

after meal fo r at leas t 3 hours. Upright of semi-upright position during awaking is helpful.2. Feeding Special precautions to be taken during feeding of infants and older children.

a.Infants to be given thickened feed in small amount frequently followed by appropriate positioning, to prevent the reflex.

a. Older Children should be allowed nothing per mouth 2 hours before bed time. Low fat diet, spicyand acidic foods (onion, citrus products, apple juice, to ma to ), es op ha ge al ir ri ta nt s (c ho co la te ,

peppe rmi nt , pa ssi ve smo ke ) and ca rbonate d beverages should be avoided. Avoidance of obesity,tight or constricting clothing and NSAIDs at bedtime are also important measures. Chewing gum can beallowed to stimulate parotid secretions which increase esophageal clearance.

3. Medications Antacids H2 - receptor antagonists (cimetidine), prokinetic drugs (metoclopramide)and proton pump inhibitors (omeprazole) can be g iven alone or in combination.

Surgical Management

If medical management fails even after 3 to 6 months course or in case of intractable respiratory disease,surgery is indicated.

1. Fundoplication (Nissen's operation) is most popular method. A gastric wrap procedure is done.Wrapping of the fundus around the lower esophageal sphincter (a 360 degree wrap) completely prevent


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reflex episodes.2. Antroplasty or pyloroplasty may also be performed in some cases. Gastrostomy may be needed

for feed ing purposes or temporarily to decompress the stomach.

Nursing Management

Special nursing interventions to be performed as follows: -

a. Preventing aspiration by positioning and appropriate feeding technique.

b. Maintaining fluid and electrolyte balance by I/V fluid therapy, (whenever needed), intake and outputrecording and estimation of electrolyte level.

c. Providing adequate nutritional intake.d. Continuous monitoring of vital signs, assessment of features of complications and necessary investigations.b. Reducing fear of eating and mod ifying feeding schedule.

I. Providing pre-operative and post-operative care as for abdominal surgery of children.9. Providing emotional support.h. Giving health education regarding, positioning, feeding, home-based care and follow-up.

COMPLICATIONS

The complications related to GER are associated with frequent and sustained reflux of acid gastric content. The possible complications are aspiration pneumonia, chronic esophagitis, failure to thrive, anemia, asthma, suddeninfant death syndrome (SIDS), esophageal stricture and hiatal hernia.

INTESTINAL OBSTRUCTION

INTRODUCTION

Intestinal obstruction is an interruption in the normal flow of intestinal contents through the intestine. The obstructionmay occur in small or large intestine. It may be complete or partial obstruction and may be due to mechanical or

paralytic cause. It may be found as congenital anomalies or as acquired conditions.

CAUSES OF INTESTINAL OBSTRUCTION

I) CONGENITAL INTESTINAL OBSTRUCTION

It is found as following conditions:

a) Intestinal a tresia Atresia of the intestine most commonly found in the duodenum. Other sites of obstructionare ileum, jejunum and colon. It can be found at multiple sites and may be complete or incomplete.

b) Malrotation of gut It is incomplete rotation of the gut during intrauterine life (12 th week), so that cecumcomes to lie below pylorus, root of mesentery becomes very narrow, ascending and transverse colon becomemobile and vulnerable to twisting in a clockwise direction. The rotation abnormalities are of two kinds (a) Ladd's

bands from the cecum pass to the posterior abdominal walls compressing the second part of duodenum and (b) theunfixed mesentery allow the small bowel to twist around it.


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c ) Meconium plug syndrome: - Obstruction of lower colon 1, thick plug of meconium, found in neonates.

d) Meconium ileus It occurs due to inspissated putty: meconium plugging the lumen of the terminal ileum a:cause obstruction. It occurs in lack of pancreatic enzyme and mucoviscidosis (cystic fibrosis).

e) Annular pancreas The head of the pancreas compress t, second part of the duodenum giving rise to anextrinfrom of obstruction.

f) Mickel's diverticulum It is an occasional sacculation appendage of the ileum, derived from an unobliterateyolk stalk. Diverticulum is circumscribed, pouch or sac occurring normally or created by herniation of the lint-mucus membrane through a tear in the muscular cu_- Mickel's diverticulum is the most common remnant ofvitello-intestinal duct and situated in the anterior, mesentric border of the ileum anywhere from 10cm to I_- from theiieo-cecal junction. It may remain asymptomatic, and may becomes symptomatic before 2 years of age. It can becomplicated with bleeding, intestinal obstruction, inflammation as derverticulitis and umbilical fistula.

g) Hirschsprung's Disease or congenital Megacolon. : - .

II) ACQUIRED INTESTINAL OBSTRUCTION The important causes of acquired intestinal obstructs, - are:

1. Intussuception It is telescoping of intestinal wall into itself. It is found as invagination or slipping of one partof intestine into another part just below it. In children, the most common site is ileocecal region.

1. Volvulus or twisted loop of intestine: - commonly occur in sigmoid colon.2. Tumor or hematoma as intrinsic or extrinsic to intestine3. Hernia and strangulation.

4. Stricture or stenosis of the intestine.5. Inflammatory diseases Ulcerative colitis, Crohn disease, appendicitis.

6. Foreign body (e.g. coin) or fecal impaction or polyp.7. Worm mass (commonly round worms) and amebiasis.8. Paralytic ileus due to toxic or traumatic disturbance of autonomic nervous system leading to ineffective

peristalsis by reduced motor activity.

PATHOLOGYObstruction leads to accumulation of gases and secretions e the blockage causing increase in intraluminal

pressure. Venous pressure of the affected area increases leading to circulatory stasis and edema. Bowelnecrosis gangrene may develop due to tissue anoxia and 7-.-oression of the arterial supply. Peritonitis developsdue to passage of bacteria and toxins across the intestinal membrane.

CLINICAL MANIFESTATIONS

The child with intestinal obstruction usually presents with abdominal colic (cramps), abdominal distension, biliousvomiting, and absence of flatus and no passage of stool. Fever, drowsiness, dehydration and toxicity are also foundin these children. There is increased bowel sound, which may reduce gradually. Minimal diffuse tenderness of abdomen may also present. The child may manifest with shock and respiratory distress.There may be rectal passage of bloody mucus (redcurrant jelly stool), sausage-shaped lump palpable in upper abdomen (in early stage) and cervix-like mass and blood in rectal examination. These features are diagnostic of intussusceptions.


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DIAGNOSIS: -History of illness and physical examination help in clinical diagnosis. The diagnosis is confirmed by X-rayabdomen and chest, barium enema, proctoscopy, sigmoidoscopy or USG. Blood examination shows reduction of

sodium, potassium and chloride level, elevated WBC count (in case of necrosis, strangulation and peritonitis) andelevated serum amylase level.

MANAGEMENT : -

i) Initial Management

Initial management is done with 1/V fluid therapy to correct fluid and electroyte imbalance, and nasogastricsuctioning to decompress the bowel. Analgesics and sedatives are administer to reduce pain and to providecomfort. Antibiotics to be given to treat and prevent infection. Causes and complications to be detected earlyand necessary management is planned to treat them promptly.

ii) Surgical Management

Surgical management is done to relieve the obstruction. Laparotomy followed by specific surgery to be done.a. Resection of bowel is done, for obstructing lesions or st ra ng ul at ed bo we l, al on g wi th en d- to -e ndanastomosis.b. In malrotation of gut, cutting of Ladd's band and lengthening of the roots of the mesentery is done.c. Enterotomy is performed for removal of foreign bodies in the intestine.d. Clo sed bowel procedure s may be done to reduce volvulus and intussusception or incarcerated hernia.

Conservative hydrostatic reduction is performed in case of intussusception. Hypertonic enema is given torelieve the obstruction due to round worms mass.

NURSING MANAGEMENT: -

Nursing assessment to be done to detect the nature and location of pain, presence or absence of abdomin al distension, flatus, vomiting, stools, constipation, bowel sound, etc. General condition of the child should be assessed thoroughly especially vital signs, intake, output and level of consciousness. The following nursing diagnoses require special attention. Pain related to intestinal obstruction and abdominal distension. Risk for fluid and electrolyte imbalance related to vomiting, poor intake of fluid and diarrhea. Ineffective breathing related to abdominal distension. Potential to shock related to toxicity. Fear and anxiety related to severity of illness. Ineffective coping related to life threatening symptoms. Knowledge deficit related to long-term care.

Nursing interventions should emphasize on the following aspects:a. Providing rest and comfort.

b. Relieving pain by analgesics.


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c. Maintaining fluid and electrolyte balance by I/V fluid therapy and recording of intake and output.d. Providing adequate respiration by relieving abdominal distension through nasogastric tube aspiration.e. Reducing fear and anxiety by explanation reassurance and answering questions.f. Maintaining normal bowel elimination.

Providing basic pre-operative and post-operative care and administering prescribed medications.h. Promoting effective coping with hospitalized care.i. Giving information and instructions for home-based long-term care.

Diaphragmatic Hernia

What is a diaphragmatic hernia?

A diaphragmatic hernia is a birth defect, which is an abnormality that occurs before birth as a fetus is forming in themother's uterus. An opening is present in the diaphragm (the muscle that separates the chest cavity from the abdominalcavity). With this type of birth defect, some of the organs that are normally found in the abdomen move up into the chestcavity through this abnormal opening

DEFINITION:-

This relatively common birth defect results when the diaphragm does not completely close during fetal development.

The defect is thought to result from a failure of the pleural-peritoneum canals to completely close between the 8 th-10 t

weeks of gestation. The cause of this improper fusion of structures during fetal development is unknown. Some areassociated with a genetic defect, but most occur as an isolated event with no gene abnormality identified. Approximately40-50% of fetuses with CDH will present with other anomalies. The most common associated anomaly is a cardiac defect

The size of the defect can vary, ranging from very small to complete agenesis of the diaphragm and can occur as either aright or left-sided defect. The most common type is the Bochdaleks hernia, which is a posterolateral defect; however, thedefect can occur at multiple sites unilaterally or bilaterally. Anteromedial (Morgagnis foramen), hiatal, and diaphragmaticeventration (central weakening of the diaphragm) are other types of CDH. Bochdaleks hernias, the most common type,usually are left-sided but can occur on the right. Left-sided hernias occur five times more often than right sided. 90% of

posteriolateral hernias occur on the left side, 5% on the right and occasionally on both sides. One theory as to why thedefect is more common on the left side is that the liver obstructs herniation through a right-sided defect.

SurvivalRate :Approximately 60% of infants brought to term will survive; however this is an optimistic number given the fact that thecurrent prenatal termination rate is estimated to be 15%. With a left sided defect, prognosis is based on whether theherniation involves the intestine only, intestine, bowel, and stomach or intestine, bowel, stomach, spleen and liver. Themore abdominal contents involved result in a poorer prognosis. With a right-sided herniation, almost all will present with

a portion of the liver in the chest cavity. More than 50% of the liver in the chest is considered a poor sign. Some defectsoccur with the abdominal contents secluded in a sac. This is impossible to diagnose on ultrasound, but indicates a remnantof diaphragm was present. The resulting pathophysiology is less severe with this type of herniation.

Two theories exist in regards to this type of herniation;

1) that the defect occurred later in gestation or

2) that the diaphragmatic remnant holds back abdominal contents. The end result is that less lung is affected whichimproves prognosis.


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Diagnosis:Ultrasound imaging can show the following signs of CDH as early as 15 weeks:

Visualization of abdominal contents in chest cavity Mediastinal and cardiac shifts away from affected side Fetal Hydrops Polyhydramnios (due to obstruction of the fetal esophagus resulting in impaired swallowing of amniotic fluid.

This finding has been linked to a poor prognosis).

Low levels of maternal serum Alpha Fetoprotein have been associated with CDH however are also associated with other defects as well, therefore are not a definitive test but should lead to further investigation.

Ultrasound imaging remains unable to accurately predict the extent of pulmonary hypoplasia, which is a significant predictor of postnatal survival in this group of infants.

Radiograph in a male neonate shows that the nasogastric tube (arrow) deviates to the left of the thoracic vertebral bodiesas it passes through the inferior portion of the thorax

Associated Anomalies:Associated anomalies occur commonly (40%) in CDH. Most of the associated anomalies do not affect survival andinclude:

Atrioseptal Heart Defect Malrotation of the intestine Meckel Diverticulum Undescended Testes Unilateral Kidney

Other more serious anomalies include:

Serious Heart Defects (10%) Hypoplastic Left Heart Syndrome is the most common cardiac defect associated with CDH Chromosomal Anomalies (10-34%)

Turner Syndrome (Monosomy X)

Edward Syndrome (Trisomy 18)

Patau Syndrome (Trisomy 13)

Pathophysiology:The pathophysiology of CDH begins when the diaphragm does not close normally during the 8 th-10 th week of gestation.This defect results in an abnormal opening that allows the fetal abdominal contents to migrate into the chest cavity.Compression of the developing lung leads to pulmonary hypoplasia and pulmonary hypertension. Morbidity and mortality

are dependent on the size of the defect and the resulting herniation, which determines the degree of pulmonaryHypoplasia. Early herniation leads to changes in the architecture of the lung on the affected side. Changes include fewer airways, fewer alveoli, a smaller pulmonary artery, and a small pulmonary vascular bed. Gas exchange is impaired as aresult. The leading cause of inadequate oxygenation in the infant with CDH, however, is not the small lung area, butresults from Persistent Pulmonary Hypertension of the Newborn (PPHN). PPHN is the most common problem associatedwith CDH in the early postnatal period. Adequate oxygenation of the newborn is dependent on inflation of the lungs,closure of the fetal shunts, a decrease in pulmonary vascular resistance, and an increase in pulmonary blood flow. This isa result of the small pulmonary vascular bed, and an increased muscularization and thickening of the walls of the arteriesthat is found in infants with CDH. This fixed component can improve over time, but is slow to resolve. A reactivecomponent also is present due to the changing resistance of the pulmonary arterioles that is caused by loss of lung volume,


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hypoxia, infection, inflammation and idiopathic reasons. Additionally, because the bowel is located in an abnormallocation, the normal rotation of the bowel did not occur and the infant will also have non-rotation or malrotation of the

bowel, which can lead to digestive problems once feedings are established.

Prenatal Management:Postnatal surgery is effective in repairing the defect, however, the complications that are responsible for the mortality andmorbidity associated with CDH including pulmonary hypoplasia, respiratory failure and PPHN are not preventable with

postnatal surgery. research has focused on means to correct the defect in utero and/or prevent the resulting lung

hypoplasia caused by this space-occupying lesion. Fetal surgery has been attempted to prevent the complications of Congenital Diaphragmatic Hernia. The goal of prenatal surgery has been to increase lung development and prevent PPHN.

Presentation: dyspnea, cyanosis, dextrocardia, diminished chest movements, scaphoid abdomen, intestinal sounds in thechest.

Prenatal repair of CDH early in gestation:Fetal surgery was performed to repair the CDH. Direct repair of the defect was performed through a hysterotomy. Thefetus without liver involvement required a thoracotomy incision and a substernal incision. The surgery resulted in the

reduction of the viscera, closing of the diaphragmatic defect and enlarging the abdomen.The defect was repaired andcompensatory lung growth resulted; however when compared with fetuss receiving postnatal treatment only, the survivalrate was not improved. Treatment of infants with liver involvement was also attempted; however reduction of the liver

back into the abdomen resulted in obstruction of the umbilical venous blood flow and fetal death.

Perinatal management for CDH includes:Prevention of preterm delivery, which is a potential secondary to polyhydramnios. Polyhydramnios is a uterine irritant.Prevention would involve early diagnosis and follow-up at a high-risk perinatal center. Bed rest may be advised alongwith the use of prenatal steroids to improve lung development. Magnesium Sulfate or Terbutaline decreases uterineirritability and is used to delay preterm labor. Planned delivery at a high-risk neonatal center. These infants requireintensive resuscitation and continued intensive care. Transport to a high risk neonatal center increases the risk to the infant

and increases the risk of developing retractable PPHN, therefore, proper identification of CDH in utero and planneddelivery at a high risk Perinatal center with a level III neonatal unit may increase the chance of survival.

Consideration of prenatal steroid administration:Maternal treatment with Betamethasone has been shown to accelerate surfactant production by the fetal lung reducing theincidence of neonatal respiratory distress in premature infants without CDH. A single course of antenatal glucocorticoidtherapy, 12 mg of Betamethasone IM followed by a second dose after 24 hours if baby is undelivered, given to the mother,has been shown to improve neonatal outcomes in infants less than 30 weeks gestation. Repeat treatment: 12 mg IM doseonce every 7 days if infant remains undelivered and infant is at substantial risk for respiratory distress resulting from

prematurity (generally less than 34 weeks gestation). Late prenatal corticosteroids are unlikely to provide significant benefit to fetuses with CDH because they most likely do not have decreased surfactant production.

Clinical Manifestations:The most severely affected infants will present with severe respiratory distress and evidence of Persistent PulmonaryHypertension of the Newborn (PPHN). These infants are at high risk of developing a pneumothorax as a result of thehypoplastic lung. Clinical presentation at the delivery will include the following:

Scaphoid (concave) abdomen (This finding is a result of the abdominal contents residing in the chest cavity). Respiratory distress Cyanosis Asymmetric chest movements (secondary to the hypoplastic lung). Absent breath sounds on the affected side (secondary to the hypoplastic lung).


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Shifted heart sounds Bowel sounds in the chest

Diagnosis is confirmed by chest x-ray revealing a mass with an air-filled bowel on the affected side.

Delivery Room Management:Immediate stabilization is crucial in the management of any infant; however the infant with CDH requires additionalstabilization that takes precedence. Immediate intubation is essential. Bag and mask ventilation must be avoided to

prevent dilation of the stomach and intestines, which could further compromise pulmonary function. A large size

oro-/nasograstric tube must also be placed immediately to minimize the effect of air in the intestines. The goal of initialstabilization is respiratory stability and prevention of bowel distention.

Treatment/Nursing Care:

Congenital Diaphragmatic Hernia has been described as a physiologic emergency rather than a surgical emergency. Thecritical concern is the avoidance of or improvement of PPHN. Removal of the abdominal contents from the chest cavity isnot emergent. Delayed surgical intervention is currently the standard of care for infants with CDH. In the past, thisdisorder was considered a surgical emergency, but it is now known that early surgical repair does not alter the outcome.

The standard of care is to stabilize the infant and manage the associated pulmonary dysfunction prior to surgicalintervention. Surgical intervention can be delayed from a few days to up to two weeks, depending on the infant. The goalof the medical management is to stabilize the pulmonary dysfunction and decrease the pulmonary hypertension. The goalof the respiratory management is to provide adequate ventilation and oxygenation but to avoid causing permanent lungdamage.

Treatment

Gentle ventilation:

Decreased inflation pressure or high frequency ventilation (HFOV)

Tolerance for hypercapnea and hypoxemia

Gastric decompression:

Oro/nasogastric tube to low continuous suction is used to decrease the amount of air entering the intestines andobstructing the lung.

Inhaled Nitric Oxide:

Nitric Oxide (NO) is a powerful vasodilator that is minimally toxic. Low doses of Nitrous oxide blended with oxygen inthe inspiratory limb of mechanical ventilators effectively reduces pulmonary vascular resistance and decreases extra

pulmonary right-to-left shunting at the ductus arteriosus and foreman ovale. It acts to dilate the pulmonary vascular bedand decrease the PPHN. When effective, it improves oxygenation quickly. Nitric oxide is administered via inhalation andinitially delivered at 20 parts per million (ppm). The goal is to decrease the dose to 2 ppm when tolerated. Late or

protracted pulmonary hypertension occurs in a subset of infants with CDH. Inhaled NO has been effectively delivered vianasal cannula to these infants no longer requiring mechanical ventilation. The dose usually administered via nasal cannulais 10 ppm which results in a nasopharyngeal concentration

Pressors:Pressors are used to increase systemic blood pressure to decrease the effect of PPHN. The goal of utilizing pressors is toincrease the systemic pressure to a level that will help to override the resistance from the pulmonary bed, to decrease the


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right-to-left shunting through the ductus arteriosus and foreman ovale and increase the pulmonary blood flow. The mostcommon pressors include:

Dopamine : Increases peripheral vascular resistance . 2mcg/kg/min to a max of 20mcg/kg/min . Continuous infusion

Dobutamine: Increases cardiac contractility . 2mcg/kg/min to a max of 20mcg/kg/min , Continuous infusion

Note: Both Dopamine and Dobutamine may be used concurrently to maximize the effects and increase the systemic

pressure. Surfactant Administration:

Recent studies indicate that a primary surfactant deficiency is unlikely in the infant with a CDH. Some infants with CDHare not receptive to exogenous surfactant .The usual dose of 4ml/kg may be too high based on the infants reduced tidalvolume. Current studies show no benefit to surfactant administration and some indicate a potential for worse outcomes inthe treated infants.

Arterial and central venous lines for monitoring and fluid infusion:

Umbilical arterial line are optimal for pressure monitoring .Umbilical venous lines with double lumen are optimal for fluid resuscitation and ongoing fluid maintenance.

Sedation:

Minimal handling (the goal is to decrease agitation to minimize effects of PPHN). Supportive positioning (Position infantwith affected side down to aid ventilation of the good lung. Also position infant in a nesting position to decreaseagitation).

Parent education:

Parents receiving the diagnosis of Congenital Diaphragmatic Hernia initially experience emotional distress, which has been shown to impair optimum learning. Description of Congenital Diaphragmatic Hernia . Explanation of what to expectin the delivery room . Tour of neonatal unit. Explanation of what to expect upon admission to NICU and first day of lifeincluding intubation, ventilation, antibiotics, umbilical venous/arterial lines, IV therapy, gastric decom pression andcardiac monitoring.

Discharge Education:

General Infant Care

Car seat Safety

Care for the infant with reflux (as appropriate)

Care for the infant on oxygen therapy (as appropriate)

Surgical Repair:Surgical repair for Congenital Diaphragmatic Hernia consists of reducing the herniated abdominal contents from the chestand closing the defect. If the defect is large, a prosthetic patch is needed to repair the diaphragm, which is a marker of more severe disease. These infants are at high risk for gastroesophageal reflux and future herniation as the patch does notgrow with the infant. In some cases, the abdominal cavity is too small to accommodate the abdominal contents. In thesecases, closure of the abdomen is not possible and a prosthetic patch is closed over the top of the abdominal contents


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leaving a ventral hernia, which will need to be repaired at a later time. Post-operative care is essentially the same as for any infant having surgery with the primary concern being ventilation and oxygenation. Preventing the reoccurrence of PPHN is a primary goal.

Long-term Outcomes:Outcomes are quite variable depending on the extent of the defect and resulting pulmonary hypoplasia and PersistentPulmonary Hypertension of the Newborn. The typical course for these neonates is difficult requiring many interventions.

Neurodevelopmental delays, chronic lung disease, nutritional deficiencies, feeding problems, and Gastroesophageal reflux

are all potential long-term problems.

Abdominal Wall Defects: Omphalocele & GastroschisisAbdominal Wall Defects: Omp halocele & Gastroschisis

GUT DEVELOPMENTGUT DEVELOPMENT

Primitive gut - Divided into 3 regionsPrimitive gut - Divided into 3 regions

Foregut- Pharynx, esophagus and stomachForegut- Pharynx, esophagus and stomach Midgut- Small and large intestineMidgut- Small and large intestine Hindgut- Colon and rectumHindgut- Colon and rectum

Abdominal wall- somatic and splanchnic layers of the cephalic lateral and caudal folds . Failure in development of Abdominal wall- somatic and splanchnic layers of the cephalic lateral and caudal folds . Failure in development of one of these folds can result in anterior abdominal wall defectsone of these folds can result in anterior abdominal wall defects

EmbryologyEmbryol ogy

TheThe Midgut gives rise to:Midgut gives rise to:

Duodenum distal to the bile ductDuodenum distal to the bile duct ,, JejunumJejunum ,, IleumIleum ,, CecumCecum ,, AppendixAppendix ,, Ascending colonAscending colon ,, Hepatic flexure of Hepatic flexure of the colonthe colon ,, Proximal two-thirds of transverse colon.Proximal two-thirds of transverse colon.

Week 6Week 6 :-:-

Physiological Umbilical Herniation. As a result of rapid growth and expansion of the liver, the abdominal cavityPhysiological Umbilical Herniation. As a result of rapid growth and expansion of the liver, the abdominal cavity temporarily becomes too small to contain all the intestinal loops. The intestinal loops enter the extraembyronic cavitytemporarily becomes too small to contain all the intestinal loops. The intestinal loops enter the extraembyronic cavity within the umbilical cord during the sixth week of development. As herniation occurs, the loop undergoes a 90 degreewithin the umbilical cord during the sixth week of development. As herniation occurs, the loop undergoes a 90 degree counterclockwise rotation around the superior mesenteric artery.counterclockwise rotation around the superior mesenteric artery.

Week 10Week 10Return to Abdominal Cavity. During 10Return to Abdominal Cavity. During 10 thth week of development, herniated intestinal loops begin to return to the abdominalweek of development, herniated intestinal loops begin to return to the abdominalcavity. Undergoes additional 180 degree counterclockwise rotation about the superior mesenteric artery.Factorscavity. Undergoes additional 180 degree counterclockwise rotation about the superior mesenteric artery.Factors responsible for this return are not precisely known. It is thought that regression of the mesonephros (kidney), reducedresponsible for this return are not precisely known. It is thought that regression of the mesonephros (kidney), reduced growth of the liver, and expansion of the abdominal cavity all play roles.growth of the liver, and expansion of the abdominal cavity all play roles.

OMPHALOCELEOMPHALOCELEGreek-Greek- omphalosomphalos -navel,-navel, celecele - hernia . Absence abdominal wall fascia. Herniation abdominal contents . Eccentric- hernia . Absence abdominal wall fascia. Herniation abdominal contents . Eccentric displacement umbilical cord. Small underdeveloped abdominal cavity. Thin sac covering defectdisplacement umbilical cord. Small underdeveloped abdominal cavity. Thin sac covering defect

OmphaloceleOmp halocele

Herniation of abdominal viscera through an enlarged umbilical ring. Failure of the bowel to return to the body cavityHerniation of abdominal viscera through an enlarged umbilical ring. Failure of the bowel to return to the body cavity following physiological umbilical herniation. Defective mesodermal growth causes incomplete central fusion andfollowing physiological umbilical herniation. Defective mesodermal growth causes incomplete central fusion and

persistent herniation of the midgut. Extruded viscera may include persistent herniation of the midgut. Extruded viscera may include LIVER LIVER , small and large intestines, stomach, spleen, or , small and large intestines, stomach, spleen, or bladder. Covered by amnion and peritoneum (May rupture before or at time of delivery) bladder. Covered by amnion and peritoneum (May rupture before or at time of delivery)


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GastroschisisGastroschisis

Herniation of intestinal loops through the anterior abdominal wall. Defect lateral to the umbilicus (right>left). AbnormalHerniation of intestinal loops through the anterior abdominal wall. Defect lateral to the umbilicus (right>left). Abnormalinvolution of the right umbilical vein or vascular accident involving the omphalomesenteric artery causes localizedinvolution of the right umbilical vein or vascular accident involving the omphalomesenteric artery causes localizedabdominal wall weakness. No sac covers the extruded viscera. Having been bathed in the amniotic fluid and withabdominal wall weakness. No sac covers the extruded viscera. Having been bathed in the amniotic fluid and withcompression of the mesenteric blood supply at the abdominal defect, the bowel wall may be inflammed and edematous.compression of the mesenteric blood supply at the abdominal defect, the bowel wall may be inflammed and edematous.May appear to have a thick, shaggy membrane and loops may appear shortened and matted together.May appear to have a thick, shaggy membrane and loops may appear shortened and matted together.

Prenatal DiagnosisPrenatal Diagnosis

Elevated maternal serum alpha fetoproteinElevated maternal serum alpha fetoprotein UltrasoundUltrasound

EpidemiologyEp idemiology

Prevalence:Prevalence:

Omphalocele: 1/5,000 birthsOmphalocele: 1/5,000 births

Gastroschisis: 1/10,000 birthsGastroschisis: 1/10,000 births

Increasing in frequency, especially in young women.Increasing in frequency, especially in young women.

Mortality:Mortality:

Omphalocele: 25%. Related directly to presence of chromosomal and other abnormalitiesOmphalocele: 25%. Related directly to presence of chromosomal and other abnormalities

Gastroschisis:


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Primary Surgical Closure: Success dependent on size of the defect and size of the abdominal and thoracic cavities.Primary Surgical Closure: Success dependent on size of the defect and size of the abdominal and thoracic cavities. Staged Closure: done if the defect is large.Staged Closure: done if the defect is large.

The defect is first placed into a sac (silo) made of silastic, mersilene or other synthetic material. The sac is sutured to theThe defect is first placed into a sac (silo) made of silastic, mersilene or other synthetic material. The sac is sutured to thefascia or the full thickness of the abdominal wall around the defect & tied with tape at the top to form a pouch. The areafascia or the full thickness of the abdominal wall around the defect & tied with tape at the top to form a pouch. The areawhere the sac is in contact with skin should be protected from infection by antiseptic solution. The top of the sac iswhere the sac is in contact with skin should be protected from infection by antiseptic solution. The top of the sac issupported from the top of the incubator so that it does not rest on the defect. The sac is gradually reduced in size by tyingsupported from the top of the incubator so that it does not rest on the defect. The sac is gradually reduced in size by tyingnew tapes at the lower level closure to the abdominal wall. As a result the abdominal wall is gradually stretched as thenew tapes at the lower level closure to the abdominal wall. As a result the abdominal wall is gradually stretched as thecontents of the omphalocele are slowly & gently pushed into the abdominal cavity, without excess pressure placed on thecontents of the omphalocele are slowly & gently pushed into the abdominal cavity, without excess pressure placed on theinferior vena cava or the diaphragm. The length of the time for procedure will depend on the size of omphalocele. In caseinferior vena cava or the diaphragm. The length of the time for procedure will depend on the size of omphalocele. In caseof very large omphalocele, some surgons postpone operation & paint the area with 2% merthiolate applied 2-3 timesof very large omphalocele, some surgons postpone operation & paint the area with 2% merthiolate applied 2-3 timesdaily. This produces an eschar that eventually converts the omphalocele into a large ventral hernia.daily. This produces an eschar that eventually converts the omphalocele into a large ventral hernia.

Nursing careNursing care

The infant will need to spend some time after the surgery in theThe infant will need to spend some time after the surgery in the intensive care unitintensive care unit . Because infants are unable to. Because infants are unable to properly regulate their temperature, they are placed in special beds that are kept warm. Care must be taken that no properly regulate their temperature, they are placed in special beds that are kept warm. Care must be taken that no pressure on or torsion of the sac occurs and that the sac must be covered with sterile saline dressings. These dressings pressure on or torsion of the sac occurs and that the sac must be covered with sterile saline dressings. These dressingsshould consist of moist gauze covered with dry sterile towel to keep the area warm & moist & to prevent contamination.should consist of moist gauze covered with dry sterile towel to keep the area warm & moist & to prevent contamination.

Nasogastric suctioning is began to prevent distension of stomach & intestine caused by swallowed air. Postoperatively the Nasogastric suctioning is began to prevent distension of stomach & intestine caused by swallowed air. Postoperatively theinfant must be fed by peripheral hyper alimentation & then by gastrostomy.Increasing amount of glucose & then milk areinfant must be fed by peripheral hyper alimentation & then by gastrostomy.Increasing amount of glucose & then milk aregiven. Observe the area for the signs of infection. Maintain clear airway. Change infants position slightly to preventgiven. Observe the area for the signs of infection. Maintain clear airway. Change infants position slightly to preventatelectasis.atelectasis.

ANORECTAL MALFORMATIONS

What are Anorectal Malformations?Anorectal malformations are defects that occur during the fifth to seventh weeks of fetal development. Withthese defects, the anus (opening at the end of the large intestine through which stool passes) and the rectum(area of the large intestine just above the anus) do not develop properly.

Anorectal Malformations IncidenceAnorectal Malformations affect 1 in 5,000 babies and is slightly more common in males. The exact cause of anorectal malformations is unknown. In some cases, environmental factors or drug exposure during pregnancymay play a role, but this is still unclear. During a bowel movement, stool passes from the large intestine to therectum and then to the anus. Nerves in the anal canal help us sense the need for a bowel movement and alsostimulate muscle activity. Muscles in this area help control when we have a bowel movement.

Synonyms

Imperforate anus, Anorectal malformations, Anorectal anomaly

TYPES

HIGH TYPE {>1.5 cm b/w anal dimple & blnd end of colon} LOW TYPE {,1.5 cm distance}

TYPES


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1. Stenosis of anus: - stricture may be at the anus at the level of 1-4 cm above the anus.

2. Anal membrane atresia : - in which the persistent anal membrane produces the obstruction behind whichmeconium can be seen.

3. Anal agenesis: - in which there is imperforate anus, possibly seen as dimple with a rectal pouch ending blindly some distance above the anus and the fistulus tracts between the rectum and another area.

4. Rectal atresia: - there is normal anus & anal pouch with the rectal pouch ending blindly in the hollow of thesacrum.

Embryology:-

In the 8 th week of embryonic life the membrane that separates the entodermal hindgut from the ectodermal analdimple perforates & a continuous canal is formed. If this membrane separating the rectum from the anus is notabsorbed & the union does not take place an anorectal anomaly takes place.

With an anorectal malformation, any of the following abnormalities can occur: The anal passage may be narrow or misplaced in front of where it should be located A membrane may be present over the anal opening The rectum may not connect to the anus The rectum may connect to part of the urinary tract or the reproductive system through a passage called

a fistula, and an anal opening is not present

Are other disorders associated with anorectal malformations?

Approximately 50% of babies with anorectal malformations have other coexisting abnormalities. Thesecommonly include:

Spinal abnormalities, such as hemivertebra, absent vertebra and tethered spinal cord Kidney and urinary tract malformations, such as horseshoe kidney and duplication of parts of the urinary

tract Congenital heart defects Tracheal and esophageal defects and disorders Limb (particularly forearm) defects Down syndrome , Hirschsprung's disease and duodenal atresia can also occur with an anorectal

malformation.

How are anorectal malformations diagnosed?When a baby is born, the physician performs a thorough physical examination that includes seeing if the anus isopen and in the proper position. A number of diagnostic tests may also be done to further evaluate a problemand to determine whether other abnormalities are present.

Abdominal X-rays - These provide a general overview of the anatomical location of the malformation in across-table lateral view, and may help determine if it's high or low in the anorectal area. They also let us know if there are abnormalities of the spine and sacrum, a triangular-shaped bone just below the lumbar vertebrae.


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Abdominal ultrasound and spinal ultrasound -

These are used to examine the urinary tract and spinal column.

They also provide evidence of a tethered spinal cord, an anatomical abnormality where the end of the spinalcord is abnormally anchored. A tethered spinal cord may cause neurological difficulties, such as incontinenceand leg weakness as the child grows.

Echocardiogram -- This test is performed to determine if there are heart defects.

Magnetic resonance imaging / MRI -- In selected cases, this diagnostic study is necessary to make a definitediagnosis of tethered cord or other spinal abnormalities. It is also used to help define the anatomy of pelvicmuscles and structures

Clinical Findings

Findings are associated with a high malformation

A flat perineum, as evidenced by the lack of a midline gluteal fold

Absence of an anal dimple, indicates that the patient has poor muscles in the perineum.

Perineal signs found in patients with low malformations include

the presence of meconium at the perineum, a bucket-handle malformation Anal membrane (through which meconium is visible).

TREATMENT

Treatment will depend on the type of anorectal malformation, the presence and type of associated abnormalities,and the child's overall health.

Rectoperineal Malformation:-Infants with a rectoperineal malformation require an operation called an anoplasty, which involves moving theanus to an appropriate place within the muscles that control continence called the anal sphincter.

Colostomy for Infants with Anorectal Malformations without a Fistula:- Newborn boys and girls diagnosedwith anorectal malformations without a fistula will require one or more operations to correct the malformation.An operation to create a colostomy is generally initially performed. With a colostomy, the large intestine isdivided into two sections, and the ends of intestine are brought through small openings in the abdominal wall.The upper section allows stool to pass through the opening, called a stoma, and into a collection bag. Intestinalmucus exits through the opening of the lower section of intestine.By performing this surgery, digestion will not

be impaired and growth can occur before the next required operation. By diverting the stool, the risk of infection is minimized when the later reconstructive operation is performed.


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Nursing staff and other health care professionals who work with the patient's surgeon will help parents learnhow to take care of the colostomy, and they will assist them in making the transition from the hospital to home.Local and national support groups may also be very helpful during this time. The next operation creates aconnection between the rectum and the newly created anal opening. This procedure is usually performed from a

posterior midline approach. In some cases where the rectum ends within the abdomen (high lesions), minimallyinvasive (laparoscopic) surgery or traditional open surgery can be used to bring the rectum down to the analopening. The colostomy remains in place for six to eight weeks after this procedure so the area can heal without

being infected by stool and so the patient can undergo a dilation protocol and the anus can reach the sizeappropriate for age. Even though the rectum and anus are now connected, stool will leave the body through thecolostomy until it is closed with surgery.

Anal Dilatation After Surgery to Repair Anorectal Malformations :-A few weeks after surgery, parents are taught to perform anal dilatations to ensure the anal opening is largeenough to allow normal passage of stool. The colostomy is closed in another operation at least six to eightweeks later. Several days after surgery, the child will begin passing stools through the rectum. Shortly after surgery, stools may be frequent and loose, and diaper rash and skin irritation can also be a problem. Within afew weeks after surgery, however, stools become less frequent and firmer. Anal dilatations should continue for several weeks or months. Some infants may become constipated. To avoid this, we encourage following a high-fiber diet. Laxatives may be required prior to the age of potty training. In cases of severe constipation, a bowelmanagement program may be developed according to the particular needs of the child. The program mayinclude child and parental education in the use of laxatives, stool softeners, enemas, bowel training techniquesand biofeedback. Toilet training should be started at the usual age, generally when the child is around 3 yearsold. Children who have had anorectal malformations generally gain bowel control more slowly, and dependingon the type of malformation and its surgical repair, some children may not be able to gain good bowel control

What is the long-term outlook for children with anorectal malformations?

Children who have had an anorectal malformation that involves a rectoperineal fistula are usually able to gaingood control over their bowel movements after surgical repair. However, those with more complex variations of anorectal malformation may need to participate in a bowel management program to help them achieve controlover their bowel movements and prevent constipation. Nurses and other health care professionals who work with the child's physicians can outline a program tailored to the child's individualy.

Associated malformations:-genitourinary :- Absent, dysplastic, or horseshoe kidneys , Vesicoureteral reflux , Hydronephrosis , Hypospadias ,Bifid scrotum

COLOSTOMYDefinitive repair Anoplasty:

posterior sagittal ARP

LAPARATOMY

PSARP

NURSING MANAGEMENT:-


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Gastric suction is done after diagnosis Withold oral feedings & give parentral hydration before surgery. i/v feedings & nasogastric suction to relieve abdominal distention. Give breast milk after surgery. Avoid tension on perineal sutures. Placed either on side or supine with legs suspended at 90 degree from the trunk. Colostomy care, skin care.

Tracheo esophageal fistula

A tracheoesophageal fistula (TEF)(known as TOF in the UK) is an abnormal connection ( fistula ) between theesophagus and the trachea . TEF is a common congenital abnormality, but when occurring late in life is usuallythe sequela of surgical procedures such as a laryngectomy .

Causes

Congenital TEF can arise due to failed fusion of the tracheoesophageal ridges during the third week of embryological development. [1]

A fistula, from the Latin meaning a pipe, is an abnormal connection running either between two tubes or between a tube and a surface. In tracheo-esophageal fistula it runs between the trachea and the esophagus. Thisconnection may or may not have a central cavity; if it does, then food within the esophagus may pass into thetrachea (and on to the lungs) or alternatively, air in the trachea may cross into the esophagus.

Embryology:-

Successive stages in the development of the tracheoesophageal septum during embryologic development

A) The laryngotracheal diverticulum forms as a ventral outpouching from the caudal part of the primitive pharynx.

(B) Longitudinal tracheoesophageal folds begin to fuse toward the midline to eventually form thetracheoesophageal septum.

(C) The tracheoesophageal septum has completely formed.

(D) If the tracheoesophageal septum deviates posteriorly, esophageal atresia with a tracheoesophageal fistuladevelops

Upper part of esophagus is developed from retropharengeal segment & the lower part from the pregastricsegment of the first part of primitive gut. At 4 weeks of gestation the larengeotracheal groove is formed. Twolongitudnal furrows develop & separate the respiratory primodium from esophagus. Deviation or altered cellgrowth of the septum results in the formation of fistula between esophagus & trachea. Esophageal atresia is acongenital abnormality in which the mid-portion of the esophagus is absent.

Incidence is between 1 in 3,570 and 1 in 4,500.

Associations
http://en.wikipedia.org/wiki/Fistulahttp://en.wikipedia.org/wiki/Esophagushttp://en.wikipedia.org/wiki/Vertebrate_tracheahttp://en.wikipedia.org/wiki/Congenitalhttp://en.wikipedia.org/wiki/Laryngectomyhttp://en.wikipedia.org/wiki/Tracheoesophageal_ridgeshttp://en.wikipedia.org/wiki/Tracheoesophageal_fistula#cite_note-pmid10068713-0http://en.wikipedia.org/wiki/Fistulahttp://en.wikipedia.org/wiki/Esophagushttp://en.wikipedia.org/wiki/Vertebrate_tracheahttp://en.wikipedia.org/wiki/Congenitalhttp://en.wikipedia.org/wiki/Laryngectomyhttp://en.wikipedia.org/wiki/Tracheoesophageal_ridgeshttp://en.wikipedia.org/wiki/Tracheoesophageal_fistula#cite_note-pmid10068713-0


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Babies with TEF or esophageal atresia are unable to feed properly. Once diagnosed, prompt surgery is requiredto allow the baby to take in food. Few TEF children have problems after surgery, however a number developfeeding difficulties and chest problems. Some TEF babies are also born with other abnormalities, mostcommonly those described in VACTERL association - a group of anomalies which often occur together,including heart, kidney and limb deformities. 6% of babies with TEF also have a laryngeal cleft

Classification

Fistulae between the trachea and esophagus in the newborn can be of diverse morphology and anatomicallocation however, various pediatric surgical publications have attempted a classification system based on the

below specified types.

Not all types include both esophageal agenesis and tracheoesophageal fistula, but the most common types do.

Gross Description EA TEF- Esophageal agenesis. Very rare, and not included in the classification by Gross. Yes NoType A Proximal and distal esophageal buda normal esophagus with a missing mid-segment. Yes NoType B Proximal esophageal termination on the lower trachea with distal esophageal bud. Yes Yes

Type CProximal esophageal atresia (esophagus continuous with the mouth ending in a blindloop superior to the sternal angle ) with a distal esophagus arising from the lower tracheaor carina . (Most common , up to 90% of cases.)

Yes Yes

Type D Proximal esophageal termination on the lower trachea or carina with distal esophagusarising from the carina. Yes Yes

Type E (or Type H) -

A variant of type D: if the two segments of esophagus communicate, this is sometimestermed an H-type fistula due to its resemblance to the letter H. TEF without EA. No Yes

Clinical presentation

Tracheoesophageal fistula is suggested in a newborn by copious salivation associated with choking , coughing ,vomiting, and cyanosis coincident with the onset of feeding. A fistula may also be a the cause of

polyhydramnios while in utero.

DIAGNOSIS OF ESOPHAGEAL ATRESIAAntenatal Diagnosis (maternal polyhydramnios, a small stomach, a distended upper esophageal pouch, or abnormal swallowing). Diagnostic suspicion is increased when abnormalities known to be associated withesophageal atresia are identified.

Clinical DiagnosisPrematurity, Any excessively drooling (copious, fine, white, frothy bubbles of mucus in the mouth and,sometimes, the nose).(A) Diagnosis of esophageal atresia is confirmed when a 10-gauge (French) catheter cannot be passed beyond10 cm from the gums.(B) A smaller-caliber tube is not used because it may curl up in the upper esophageal segment, giving a falseimpression of esophageal continuityA plain radiograph will confirm the tube has not reached the stomach ,Absence of gas in the abdomen suggests that the patient has either atresia without a fistula or atresia with a

proximal fistula only
http://en.wikipedia.org/wiki/VACTERLhttp://en.wikipedia.org/wiki/Laryngeal_clefthttp://en.wikipedia.org/wiki/Esophageal_atresiahttp://en.wikipedia.org/wiki/Sternal_anglehttp://en.wikipedia.org/wiki/Sternal_anglehttp://en.wikipedia.org/wiki/Carina_of_tracheahttp://en.wikipedia.org/wiki/Carina_of_tracheahttp://en.wikipedia.org/wiki/Salivationhttp://en.wikipedia.org/wiki/Chokinghttp://en.wikipedia.org/wiki/Coughinghttp://en.wikipedia.org/wiki/Cyanosishttp://en.wikipedia.org/wiki/Polyhydramnioshttp://en.wikipedia.org/wiki/VACTERLhttp://en.wikipedia.org/wiki/Laryngeal_clefthttp://en.wikipedia.org/wiki/Esophageal_atresiahttp://en.wikipedia.org/wiki/Sternal_anglehttp://en.wikipedia.org/wiki/Carina_of_tracheahttp://en.wikipedia.org/wiki/Salivationhttp://en.wikipedia.org/wiki/Chokinghttp://en.wikipedia.org/wiki/Coughinghttp://en.wikipedia.org/wiki/Cyanosishttp://en.wikipedia.org/wiki/Polyhydramnios


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CONTRAST STUDIESshould be performed by an experienced pediatric radiologist, or after transfer to the tertiary institution, and withthe use of a small amount (0.5 to 1 mL) of water-soluble contrast. Care must be taken to avoid aspiration.

Operative Repair of Esophageal AtresiaSurgical repair is delayed (1-2days) in infants with low birth weight, pneumonia or other major anomalies.

MANAGEMENTMeasures should be taken to reduce the risk of aspiration(continuous suctioning of the upper pouch, the infant'shead should be elevated).In infants with respiratory failure, endotracheal intubation should be performed.Transfer to a major tertiary pediatric institution is best not delayed .Esophageal pouch should be sucked every 5 minutes or continously with gentle pressure of about 50 cm of water.PREOPERATIVEPREPARATION :-minimize pulmonary complicationNPOhead-up position

sump tube (repogle) on low continuous suctionabdominal x-ray, renal ultrasound

echocardiogram : mandatoryiv access arterial line

LABORATORY STUDIESCBC, Electrolytes, Glucose, Calcium, ABGs, 24-48 hr medical stabilizationAntibiotics: ampicillin and gentamicinEnsure availability of blood in the OR Optimize volume status and metabolic state

Intubation preferably in the operating room under controlled situation

INTRAOPERATIVE MANAGEMENTMain Concernoxygenation and ventilationsecuring the airwayMonitors invasive : arterial line

Surgical repair ligation of fistula

check air leak in suture lineesophageal repair identify the pouch

placement of feeding tubechest tube placement and closure of thoracic cavity

Postoperative Management

Early extubation desirablecaution: disruption of surgical repair with reintubation


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Postop Pain ManagementIV narcoticsepidural infusion: 0.1% bupivacaine +fentanyl 0.5 mcg/ml at 01.-0.2 ml/kg/hr

SURGERY:-Most infants are recommended to undergo primary care; however, a staged repair several weeks following birth

is recommended for infants who are premature and have severe respiratory distress syndrome.The presence of other severe comorbidities, such as aspiration pneumonia, congenital cardiac disease, or other life-threatening conditions, should also delay the primary repair.The repair is performed via right thoracotomyin the left lateral decubitus position, and the head of table is elevated to avoid gastric reflux. A posterolateralthoracotomy incision is made through the fourth intercostal space, and a retropleural exposure is obtained.During the dissection, the azygos vein is divided and the vagus nerve is identified. If a fistula lies between theesophageal pouch and trachea, it is divided and closed. The esophageal anastomosis is performed in 1-2 layersand is covered with mediastinal pleura. A nasogastric feeding tube is placed through the esophagus into thestomach prior to the chest closure, and a chest tube is placed in the retropleural space.

Postoperatively, the infant is ventilated as needed, nasogastric or gastrostomy feedings are resumed, and acontrast swallow radiographic examination is performed on the seventh postoperative day. If no leak is detected,

oral feedings are resumed. Approximately 3 weeks later, the esophagus is dilated up to a 24F size in order to prevent future esophageal stenosis.The most common complications of surgery are pneumonia and atelectasisleading to respiratory failure in postoperative period. A leak at the anastomotic site and pneumothorax are other complications. Most patients who develop an anastomotic leak also develop strictures, which may be dilatedlater. Rarely, a recurrent TEF may develop. The management of recurrent TEFs usually requires repeat surgicalrepair. Some patients develop periodic apneic spells that are likely secondary to gastro esophageal reflux andassociated laryngospasm. Repair of esophageal atresia and tracheoesophageal fistula has traditionally been

performed via thoracotomy. Recent attempts at thoracoscopic repair have shown that such repair is feasible buttechnically challenging.However, more data are needed for further evaluation of this approach, particularly in long-gap defects thatrequire more extensive dissection and difficult anastomosis.

NURSING MANAGEMENTNURSING DIAGNOSIS:- (PREOPERATIVE)risk for aspiratioon related to esophageal abnormality.risk for fluid volume deficit related to inadeqquate intake.

parental anxiety related to congenital anomelies of the neonate.

POSTOPERATIVE DIAGNOSISInfective airway clearance related to surgical interventionsAltered body nutrition lass than body requirement related to inadequate oral intake.Pain related to surgical intervention.

Risk for infection related to hospital procedures.Risk for injury related to complex surgery.Impaired tissue integrity related to post-operative drainage.Risk for altered parenting related to prolonged illness.Knowledge deficit related to home based long term care.

NURSING INTERVENTIONPRE-OPERATIVE:-Prevent aspiration by positioning, suctioning, NPO, thus reducing chance of respiratory infections.Preventing dehydration by I/V fluids, I/O recording, monitoring vital signs.


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Preventing infection by infection control measures.Reducing parental anxiety by emotional support.

POST- OPERATIVEMaintain clear airway.Provide adequate feeding by I/V fluid or gastrostomy feeding.Reducing pain by analgesics & comfort measures.Maintain chest tube drainage with necessary precautions.Prevent infection by aseptic techniques& administering antibiotics.Stimulate parent- child bondage by parental participation in the care of the infant.Improving knowledge by health education, encoureging questions & explaining the answers.

Hand Out Peds Shikha

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