Exam 3 Neonatal Congenital Defects Flashcards
Failure of the nasal bone or membranous portion of nasopharynx to undergo regression during development
What is Choanal Atresia and Stenosis?
Key Sign of Choanal Atresia
Cyanosis at rest that resolves with crying or oral airway
5 conditions associated with laryngeal and tracheal obstruction
Webs Subglottic Stenosis Subglottic hemangioma Esophageal Atresia Tracheoesophageal Fistula
What up wit Webs?
Webs- can be laryngeal or tracheal, can produce incomplete fibrous membrane that can obstruct the airway.
Sx of Esophageal Atresia
Excessive Secretions
Regurg
Respiratory distress worsened with feeding
Recurrent Pneumonia
Most common form of Tracheoesophageal fistula
Esophagus ends in blind pouch with distal end connected to trachea just above the Corina.
Anatomic Defect of the diaphragm allowing intrusions of abdominal contents into the thoracic cavity
What is Congenital Diaphragmatic Hernia
Sx of Congenital Diaphragmatic Hernia
Dyspnea, Tachypnea, Cyanosis, Absence of breath sounds on affected side, and severe retractions.
Also- Barrel chest and scaphoid abd
CD Hernia Surgery is usually performed on day ____
4
Avoid these during CDH sugery
N20 (Increases GI tissue and impairs ventilation)
Hypoxia
Hypothermia
Acidosis (increases PVR)
Two types of emergency GI surgical conditions
1- those that are obstructive
2-those that compromise intestinal blood supply
2 types of obstructive GI lesions
Congenital and Aquired
Describe Congenital Obstructive GI lesions
Abnormal increase in maternal weight
polyhydramnios
fetal size > normal for gestational age
fetal abdominal distention
Describe Acquired congenital obstructive GI lesions
after birth have vomiting
abdominal distention
late passage of meconium
Hypertrophy of Muscularis Layer of Pyloris
Vomiting starts at 2-6 weeks of life
What is Hypertrophic Pyloric Stenosis
What is associated with Necrotizing Entercolitis
Birth Asphyxia Hypotension Recurrent Apnea Intestinal Ischemia Umbilical Vessel Cannulation Respiratory Distress Syndrome PDA Systemic Infections
Often have metabolic and hematologic abnormalities, hyperglycemia, thrombocytopenia, coagulopaty, and anemia.
Defects in abd wall occurring during gestation, usually at the umbilicus
Omphalocele and Gastrochisis
Omphacele
Herniated viscera emerge at umbilicus d/t failure of gut to move from yok sac into abdominal cavity.
Covered by membranous Sac.
Associated with other anomalies (gender, cardiac, GU and metabolic
Gastrochisis
Due to occulsion omphalomesenteric artery during gestation. Herniated viscera and intestines are periumbilical and usually on right side.
Intestines exposed to air- leads to inflammation, edema and dilated abnormal bowel
Failure of gut migration from yolk sac into abdomen
Located within umbilical cord
Associated with Beckwith-Weidemann Syndrome (marcroglossia, gigantism, hypoglycemia, hyperviscosity) CHD, Extrophy of blader
Omphalocele
Occusion of omphalomesenteric artery
Location- Periumbilical
Associated with exposed gut, inflammation, edema, dilation and foreshortened.
Gastroschisis
Anesthetic Implications with Omphalocele and Gastrochisis
Severe dehydration and massive fluid loss from exposed viscera
Gastrochisis lose more fluid
Potential for sepsis, hypothermia and postop vent.
May need Stage closures. (staging)
Due to abnormal migration or incomplete rotation of intestines from yolk sac back into abdomen
What is Malrotation and Midgut Volvulus?
Malrotation and Midgut Volvulus
If occurs DURING Development _____ may be formed
Atretic Segments
Malrotation and Midgut Volvulus
If occurs AFTER Development _____ may occur
bowel necrosis
Malrotation and Midgut Volvulus
Sx?
Bilious Emesis, Tender and Distended Abd, Abd pain
If Bloody Stools Present- OMINOUS SIGN
Neonatal Emergency- SURGERY ASAP
What is the most common CNS Defect?
Myelomeningocele
Myelomeningocele AKA
Spina Bifida
What is Myelomeningocele
Failure of neural tube to close leading to herniation of spinal cord and meninges through defect in spinal column
Meningocele contains only
meninges
Myelomeningocele contains ____ and ____
meningies and neural elements
Myelomeningocele is urgent surgery and will need to occur within _____
24 hours
Myelomeningocele usually occurs without other anomalies, but may have _______malformation, where hind brain is displaced downward into foramen magnum causing hydrocephalus
Arnold-Chiara malformation
May need VP shunt