Gastro

• Ankyloglossia (Tongue-tie)


  Congenital anomaly that causes restricted tongue mobility and impaired tongue function

  
• Ankyloglossia


  • Frenotomy release can be performed in the newborn period
• Thyroglossal Cysts


  Irregular neck mass or lump that are congenital defects located in or around the midline of the neck, extending to the base of the tongue

  
• Thyroglossal cysts may occur as an autosomal dominant trait
• Infected Thyroglossal Cysts


  • Appear swollen and reddened, with drainage of mucus or pus from the anterior neck; requires antibiotic therapy
• Sistrunk Procedure


  Common option chosen for the surgery of a thyroglossal duct cyst
• Cleft Lip


  Most common orofacial cleft that may be unilateral or bilateral, caused by the transmission of multiple genes aided by teratogenic factors present during weeks 5 to 8 of intrauterine life

  
• Teratogenic factors for cleft lip


  • Viral infection, seizure medicines (phenytoin), maternal smoking or binge drinking, hyperthermia, stress, and maternal obesity
• Folic acid deficiency can cause cleft lip

  
• Cleft Lip Repair


  • Usually done when the baby is around 3 months old

  
• Cleft Palate


  Process does not close at approximately weeks 9 to 12 of intrauterine life, occurs more frequently in girls with an incidence rate of 1 out of every 1,000 births

  
• Cleft Palate Repair


  • Surgery called Palatoplasty (10-12 months old)

  
• Assessment of Cleft Lip and Palate


  • Cleft lip detected by a sonogram while an infant is in utero, cleft palate assessed for other congenital anomalies as well

  
• Therapeutic Management of Cleft Lip


  • Surgical repair at birth or between 2 and 12 weeks of age, rhinoplasty to straighten a deviated nasal septum may be necessary when the child reaches 4 to 6 years of age

  
• Pierre Robin Sequence (Syndrome)


  Congenital birth defects that occur during fetal development, a rare triad of micrognathia (small mandible), cleft palate, and glossoptosis (a tongue malpositioned downward) resulting in severe upper airway obstruction
• Cause of Pierre Robin Sequence is unknown
• Pierre Robin Sequence is associated with Stickler Syndrome
• Therapeutic Management of Pierre Robin Sequence

  • Monitor infants for possible airway obstruction, suctioning, maintaining prone positioning, severe cases may require tongue lip adhesion, mandibular distraction, or tracheostomy
• Esophageal Atresias and Tracheoesophageal Fistulas


  Incomplete formation of the esophageal lumen, resulting in the proximal (upper) esophagus forming a "blind pouch" that does not connect to any other structure, and an opening developing between the closed distal (lower) esophagus and the trachea
• Assessment of Esophageal Atresias and Tracheoesophageal Fistulas


  • Frothy saliva in the mouth and nose, excessive drooling, inability to swallow feeding, vomiting, abdominal distention, respiratory distress
• Therapeutic Management of Esophageal Atresias and Tracheoesophageal Fistulas


  • Emergency actions to prevent pneumonia, upright 60-degree angle positioning, nasogastric tube insertion, gastrostomy tube, IV fluids and parenteral nutrition, IV medications, surgery to close the fistula and anastomose the esophageal segments
• Umbilical Hernia


  Protrusion of a portion of the intestine through the umbilical ring, muscle, and fascia surrounding the umbilical cord
• Umbilical Hernia Management


  • If fascial ring is less than 2 cm, closure will usually occur spontaneously after the child begins to walk to around 2 years of age, so surgical repair is often not necessary for mild (small) cases, 3-4 years - closed spontaneously if not then surgery will be done
• Omphalocele


  Protrusion (herniation) of abdominal contents through the abdominal wall, involving organs like intestines, stomach and liver
• 67% of omphalocele cases are associated with other congenital disorders
• Gastroschisis


  Derived from the Greek word for "stomach cleft" or "fissure", where intestinal contents tends to herniate, increasing the potential for volvulus and obstruction, occurs due to failure of the abdominal wall to close, usually during the fourth week of development
• Assessment of Omphalocele and Gastroschisis


  • Sonogram, elevated maternal serum α-fetoprotein (MAFP) examination during pregnancy, cesarean birth may be performed if identified in utero
• Therapeutic Management of Omphalocele and Gastroschisis

  • TPN, surgery within 24 hours to replace the bowel before the blood supply becomes hampered, small gastroschisis - one-stage repair, large - use of a prosthetic patch repair or replace only a portion of the bowel at one time, omphalocele - external dressing producing mild pressure may be used over the intact membrane if unruptured
• Intussusception


  Invagination of one portion of the intestine into another, caused by idiopathic reasons or a "lead point" on the intestine like a polyp or bowel tumor
• Assessment of Intussusception


  • Severe pain and vomiting (Vomitus with bile), stool - a "red currant jelly" (blood + mucus), distended abdomen, if necrosis occurs - elevated temperature, increased WBC, rapid pulse, peritoneal irritation, episodes of crying every 15-20 minutes, confirmed by abdominal X-ray or ultrasound
• Therapeutic Management of Intussusception


  • Reduction by instillation of a water-soluble solution, barium enema, or air (pneumatic insufflation) into the bowel or surgery to reduce the invagination, observe for 24 hours for possible recurrence after reduction
• Inguinal Hernia


  Protrusion of a section of the bowel into the inguinal ring, occurs more commonly in boys
• Assessment of Inguinal Hernia

  • Appears as a lump in the groin, apparent only on crying, painless, diagnosed by history and physical appearance
• Therapeutic Management of Inguinal Hernia

  • Laparoscopy surgery, a surgical emergency, infants may have surgery before 1 year of age, keep suture line dry and free of urine or feces to prevent infection, assess circulation in the leg on the side of the surgical repair
• Hirschsprung Disease (Aganglionic Megacolon)


  Absence of ganglionic innervation to the muscle of a section of the bowel, usually the lower portion of the sigmoid colon just above the anus, resulting in chronic constipation or ribbonlike stools
• Hirschsprung Disease is caused by an abnormal gene on chromosome
• Assessment of Hirschsprung Disease

  • Become apparent by 6-12 months of age, appear thin and undernourished, history of not having a bowel movement more than once a week of ribbonlike or watery stools, rectal exam shows empty rectum, diagnosed by barium enema, ultrasound with contrast, or biopsy
• Therapeutic Management of Hirschsprung Disease

  • Repair involves dissection and removal of the affected section, with anastomosis of the intestine (pull-through operation), two-stage surgery with temporary colostomy first followed by bowel repair at 12-18 months, permanent colostomy if anus is deprived of nerve endings
• Inflammatory Bowel Disease: Ulcerative Colitis and Crohn's Disease


  Both involve the development of inflammation in the intestine, Ulcerative Colitis affects only the mucosal lining of the colon, Crohn's Disease can affect any part of the GI tract from the mouth to the anus
• Assessment of Ulcerative Colitis


  • Crampy abdominal pain, urgency, tenesmus, and frequent bloody stools, anemia and hypoalbuminemia due to losses in the stool