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I. Congenital diaphragmatic hernia

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For children born with a congenital diaphragmatic hernia (CDH), modalities such as nitric oxide (NO), high-frequency oxygenation (HFO), and extracorporeal membrane oxygenation (ECMO) have improved survival, resulting in a growing recognition of many long-term CDH-associated complications. Given the complexity of these children, comprehensive long-term multidisciplinary care is essential. Chapter 31 discusses the complex medical and developmental care of infants with a CDH. Below, surgical considerations of the CDH survivor are discussed.

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  1. Gastroesophageal reflux

    1. Gastroesophageal reflux disease (GERD) has a well-recognized association with CDH.

    2. Proposed mechanisms for GERD in babies with CDH include elevated intra-abdominal pressure from positioning the hernia contents into the abdominal cavity or abnormal anatomy of the esophagus or gastroesophageal junction with and incompetent lower esophageal sphincter (LES).

    3. The clinical presentation of GERD in these infants is similar to other children, including vomiting or regurgitation, discomfort associated with feeds, oral aversion, failure to thrive, and recurrent pneumonia.

    4. GERD appears to be more severe in children requiring a prosthetic patch repair of the CDH or who had ECMO support.

    5. Careful surveillance and treatment of GERD should be done for CDH survivors, including early consideration of antireflux surgery.

    6. Fundoplication (see Section VII)

    1. Antireflux surgical procedure in which the gastric fundus is wrapped around the lower portion of the esophagus and sutured in place, thereby functioning as a competent LES.

    2. May be performed as an open surgical procedure or laparoscopically. A gastrostomy tube is often placed at the same time due to the frequency of oral feeding difficulties in these infants, or need for venting or continuous feeds postop.

  2. Intestinal malrotation and obstruction

    1. Variable degrees of intestinal malrotation/nonrotation are seen in babies with CDH as a result of abnormal positioning and fixation of the intestines.

    2. Intestinal obstruction occurs in up to 20% of CDH patients, and the most commonly performed second surgical intervention in CDH is reexploration for small bowel obstruction.

    3. Obstructive symptoms can be vague, and include feeding intolerance, abdominal distention, vomiting (bilious or nonbilious), constipation, bloody stools, and abdominal pain.

    4. Suspicion of intestinal obstruction should prompt urgent surgical evaluation.

  3. Failure to thrive

    1. Failure to thrive (FTT) is seen in 20% to 50% of CDH survivors, despite attempts at optimization of oral caloric intake.

    2. The pathophysiology of FTT in CDH includes increased catabolic stress in the neonatal period, oral aversion, chronic lung disease, and GERD.

    3. Many CDH patients require a gastrostomy tube and high-caloric formulas.

    4. Gastrostomy (see Section VI)

    5. All CDH survivors should undergo regular nutritional assessments throughout early infancy. Early intervention by speech pathologists, consideration of supplemental feedings, and use of gastrostomy tube may avoid prolonged oral aversion, improve weight accretion, and foster development.

  4. CDH recurrence

    1. This risk of CDH recurrence is low following primary repair (ie, nonpatch closure), with most series reporting about a 5% risk. The risk of hernia recurrence is increased in babies requiring prosthetic patch closure, with up to a 40% risk of recurrence.

    2. Most recurrences present from months to several ...

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