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## Altered Nutrition and Metabolism ### Hypertrophic Pyloric Stenosis Hypertrophic pyloric stenosis (PS) is obstruction of the pyloric sphincter between the gastric pylorus and small intestine, caused by hypertrophy and hyperplasia of the circular muscle of the pylorus so the enlarged tissue obstr...

## Altered Nutrition and Metabolism ### Hypertrophic Pyloric Stenosis Hypertrophic pyloric stenosis (PS) is obstruction of the pyloric sphincter between the gastric pylorus and small intestine, caused by hypertrophy and hyperplasia of the circular muscle of the pylorus so the enlarged tissue obstructs the sphincter. PS is more common in boys than girls and has a genetic predisposition. Diagnosis is based on ultrasound. Decreased sodium and potassium levels may not be evident with dehydration. Onset of symptoms is usually >3 weeks. Symptoms include: * Projectile vomiting (1-4 feet) usually shortly after eating but may be delayed for a few hours. Emesis may be blood-tinged but non-bilious. * Child hungry and eats readily, but shows weight loss and signs of dehydration. * Upper Abdominal distension with palpable mass in epigastrium (to right of umbilicus). * Visible left to right peristaltic waves. * Decreased sodium and potassium levels (may not be evident with dehydration). Treatment includes: * Intravenous fluids to restore hydration and electrolyte balance. * Surgical pyloromyotomy: longitudinal incisions through the circular muscle fibers down to the submucosa to release the restriction and allow the muscle to expand. ### Orofacial Clefts Cleft lip (CL), cleft lip with cleft palate (CLP), and cleft palate (CP) are orofacial clefts, which can be a part of a more complex syndrome or isolated anomalies: * **CL** occurs when the medial nasal and maxillary processes fail to merge during the fifth week of embryological development. The cleft is generally at the junction of the central and lateral parts of the upper lip and may be on the left or right side. If the cleft extends into the maxilla, it may be accompanied by a cleft palate and be deemed a CLP. Surgical repair is usually at 10 weeks. * **CP** occurs when the palatal shelves of the maxillary bone fail to fuse. Fusion usually begins at the eighth week and continues until the twelfth week of embryological development. A spectrum of CP exists. Bifid uvula is the mildest form. Clefting of the soft palate is more severe. A complete CP (most severe form) involves clefting of the uvula, soft palate, and hard palate. Surgical repair usually begins at about 4 months. ### Feeding Infant With Cleft Lip Or Cleft Palate Infants with severe cleft lip and palate may require enteral feedings, but many can be fed with a bottle or breastfed, using care to position the child with the head and trunk elevated and feeding slowly. An infant with an isolated cleft palate has difficulty creating negative pressure within the oral cavity, so breastfeeding is more of a challenge than bottle-feeding. To overcome the pressure problems, the infant must learn to squeeze the milk out of the nipple by compressing it between the remaining palate and the tongue. The mother should be taught to seal the cleft with her breast by positioning the infant with the cleft uppermost. The mother may also use her finger to seal the cleft while breastfeeding. For infants with a bilateral cleft, breastfeeding is usually impossible. The mother can pump her breast milk into a bottle, so the infant still benefits from her antibodies. The goals are to have appropriate weight gain in the infant, minimize aspiration, and reduce the incidence of ear infections.