109 Lec_27

• • Ankyloglossia also known as "tongue tied". The abnormal restriction of tongue by abnormally tight frenulum.
  • The only management is surgery
• Cleft Lip is a failure of the maxillary & median nasal processes to fuse that range from small notch in the upper lip to total separation of lip & facial structure up into floor of the nose, with even upper teeth and gingiva absent.
• Cleft Lip: This condition can be unilateral or bilateral.
• Cleft Palate is a palate opening that usually on the midline & involves anterior hard palate, posterior soft palate, bath.
• Cleft Palate: The palate process closes at 9th-12th WOIL
• Cleft Palate Management:

  • In uterus: fetal surgery. 
  • 2-10 weeks:  surgical repair
  • Nasal mold apparatus to shape better nostril.
  • Revision of original repair if needed
• Cleft lip and cleft palate are birth defects that occur when a baby's lip or mouth do not form properly during pregnancy. Together, these birth defects commonly are called “orofacial clefts”.
• • The Pierre Robin syndrome (also called Pierre Robin Sequence) is a triad of micrognathia (small mandible), cleft palate, and glossoptosis (a tongue malpositioned downward)
• • ATRESIA: orifice/passage in body that is closed or absent
• FISTULA: abnormal/ surgically made passage between hollow or tubular organ & body surface, between 2 hollow or tubular organs
• ESOPHAGEAL ATRESIA: obstruction of esophagus
• ESOPHAGEAL ATRESIA WITH FISTULA: connected distally to the trachea is the most common kind af esophageal malformation (classified as Gross type C)
• Tracheoesophageal Atresia and Fistula: This condition must be ruled out in infants born with hydramnios. Every hydramnios patient should examine the infant for other congenital anomalies as (Vertebral Defects, Anal Atresia, Cardiac Defects, Tracheoesophageal Fistula, Renal Anomalies, Limb Abnormalities) VACTERL Syndrome.
• Tracheoesophageal Atresia and Fistula: During first feeding WOF infant cough that can progress into cyanosis and DOB 
• VACTERL SYNDROME

  Vertebral Defects

  Anal Atresia

  Cardiac Defects

  Tracheoesophageal Fistula

  Renal Anomalies

  Limb Abnormalities
• Omphalocele

  • Abdominal contents protrude through the wall of abdomen at the junction of UC and abdomen. Intestines are usually herniated but sometimes it includes stomach and liver.
  • This usually converted & contained by thin amnion & chorion layer with UC protruding from exposed sac. Defect < 4cm usually there is hernia of umbilical cord while > 10cm true omphalocele exist.
• Gastroschisis 

  • is a birth defect where a hole in the abdominal (belly) wall beside the belly button allows the baby's intestines to extend outside of the baby's body. The hole can be small or large and sometimes other organs, such as the stomach and liver, can be found outside of the baby's body as well.
• INTESTINAL OBSTRUCTION

  • For this condition, anticipate the patient had hydramnios throughout pregnancy.
  • The findings for newborn < 30mL gastric contents can be aspirated, distended abdomen and tender to palpate.
  • When the infant vomits, it is not sour smelling and the appearance may be green or black.
  • The pain can be manifested by hard, forceful, distressful crying, and legs pulling up against the abdomen.
  • Tachypnea as a diaphragm is pushed up.
• IMPERFORATE ANUS

  • Anal stricture is narrowing the end of tube that takes stool out of the body
  • The other term is “anal stenosis”
  • This condition may be detected by prenatal sonogram and inspection at birth.
  • Sometimes, membrane is filled with meconium and protrudes from anus
  • Upon assessment, wink reflex is absent, no stool will be passed, distended abdomen.
• Clinical Manifestations

  Newborns with imperforate anus are usually identified upon the first physical examination.

  • Absence of stool. There is no passage of stool within a day or two of birth.
  • Passing of stools in other openings. The infant may pass stools through another opening like the urethra in boys or vagina in girls.
  • Swollen belly. The newborn could not pass out stools, resulting in a swollen belly.
  • Absence of anal opening. The opening of the anus is missing or not in its usual place; in girls, this may be near the vagina.
• • A diaphragmatic hernia is a protrusion of an abdominal organ (usually the stomach or intestine) through a defect in the diaphragm into the chest cavity. This usually occurs on the left side, causing cardiac displacement to the right side of the chest and collapse of the left lung.
• An umbilical hernia is a protrusion of a portion of the intestine through the umbilical ring, muscle, and fascia surrounding the umbilical cord
• Umbilical Hernia creates a bulging protrusion under the skin at the umbilicus.
• Umbilical Hernia is rarely noticeable at birth while the card is still present but becomes increasingly noticeable at health care visits during the first year.
• Hydrocephalus is an excess of CSF in the ventricles of the subarachnoid space
• • Signs of Increased Intracranial Pressure
  • temperature +
  • pulse -
  • respiration -
  • blood pressure +
  • vomitting
  • unconsciousness
• SPINA BIFIDA is a latin word which "divided spigen"
• Spina Bifida: This happens when the fusion of posterior laminae of vertebrae fails.
• Spina bifida occulta is a benign disorder that does not need immediate intervention.
• MENINGOCELE is when the meninges herniate through unformed vertebrae.
• Meningocele: Protruding mass appears and usually have same size of an orange at the center of back.
• MYELOMENINGOCELE is the spinal cord & meninges protruding the vertebrae same with meningocele.
• Myelomeningocele:

  • There is an absence of motor & sensory function at the end of spinal cards -> lower motor neuron damage.
• An encephalocele is a cranial meningocele or myelomeningocele.
• Encephalocele:

  • The disorder occurs most often in the occipital area of the skull but may occur as a nasal or nasopharyngeal disorder.
• ANENCEPHALY, the absence of cerebral hemispheres. The mother may experience difficulty in labor, most of this condition present as breech.
• MICROCEPHALY is a Slow growth of brain that falls more than 3x below normal on growth charts.
• Hip Dysplasia:

  • The femur head don't lie deep enough within acetabulum & slips out in movement.
• Acetabular dysplasia - mildest form; femoral head remains in acetabulum.
• Subluxation - most common form; femoral head partially displaced.