Pediatrics Diaphragmatic Hernia Quiz

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Questions and Answers

What is the diagnosis for a baby born with respiratory distress and a scaphoid abdomen?

diaphragmatic hernia

What is the biggest concern with diaphragmatic hernia?

pulmonary hypoplasia

What is the best treatment for diaphragmatic hernia?

If diagnosed prenatally, delivery at a place with ECMO, let lungs mature for 3-4 days, then surgery

What is the diagnosis for a baby born with respiratory distress and excess drooling?

TE fistula Signup and view all the answers

What is the diagnostic test for TE fistula?

<ol> <li>Place feeding tube 2. Take x-ray 3. See it coil in thorax</li> </ol> Signup and view all the answers

What syndrome is TE fistula associated with?

VACTERL associated anomaly Signup and view all the answers

What does VACTER stand for?

V- vertebral anomaly, A- anal atresia, C- cardiac, TE- tracheoesophageal atresia, R- radial/renal Signup and view all the answers

What is the diagnosis for a 1-week-old baby who is cyanotic except when crying?

Choanal atresia Signup and view all the answers

What syndrome is associated with choanal atresia?

CHARGE Signup and view all the answers

What diagnosis is suggested by a 32-week premie with respiratory rate of 80, dyspnea, and nasal flaring?

Respiratory distress syndrome Signup and view all the answers

What would you check to measure prenatal RDS?

L/S myotomy Signup and view all the answers

What diagnosis can be suspected in a 2-week-old infant with bilious vomiting and polyhydramnios during pregnancy?

Intestinal atresia or annular pancreas Signup and view all the answers

Intestinal atresia is associated with what congenital disease?

Down syndrome (especially duodenal atresia) Signup and view all the answers

What is the diagnosis for a 1-week-old baby with bilious vomiting, who draws up his legs and has abdominal distension?

Malrotation and volvulus Signup and view all the answers

What is the pathophysiology of malrotation?

Intestine doesn't rotate 270 degrees counterclockwise around SMA Signup and view all the answers

What are the potential diagnoses for a 3-day-old with constipation and has not passed meconium?

<ol> <li>Meconium ileus - CF? 2. Hirschsprung's</li> </ol> Signup and view all the answers

What is the diagnosis for Hirschsprung's?

No poop in DRE, biopsy shows no ganglia Signup and view all the answers

What diagnosis is suggested for a 33-week premie who is now at day 5 and has bloody diarrhea?

Necrotizing enterocolitis Signup and view all the answers

What does an x-ray show in necrotizing enterocolitis?

Pneumatosis intestinalis (air in the GI wall because of bacteria) Signup and view all the answers

What is the treatment for necrotizing enterocolitis?

NPO, TPN + antibiotics, resection of bowel wall Signup and view all the answers

What are the risk factors for necrotizing enterocolitis?

<ol> <li>Premature gut 2. Introduction of feeds/formula</li> </ol> Signup and view all the answers

What is the diagnosis for a 2-month-old baby with colicky abdominal pain, currant jelly stool, and a RUQ 'sausage-shaped' mass?

Intussusception Signup and view all the answers

What is the diagnostic and treatment method for intussusception?

Both= Barium enema Signup and view all the answers

What is the treatment for cryptorchidism in newborns?

Surgery if not descended by 1 year Signup and view all the answers

Where are the testes located in cryptorchidism?

Inguinal canal Signup and view all the answers

What is the next best test for cryptorchidism?

Ultrasound if not palpable Signup and view all the answers

What is the diagnosis for a newborn with a urethral opening on the ventral surface?

Hypospadias Signup and view all the answers

What is a contraindication for circumcision in a newborn with hypospadias?

Circumcision (need foreskin to fix it) Signup and view all the answers

What is the diagnosis for a newborn with ambiguous genitalia, vomiting at one month, decreased Na+/increased K+/acidosis?

Congenital adrenal hyperplasia Signup and view all the answers

What is the most common cause of congenital adrenal hyperplasia?

21-hydroxylase deficiency Signup and view all the answers

What is the definitive test for congenital adrenal hyperplasia?

17-OH progesterone levels before and after ACTH bolus Signup and view all the answers

What is the treatment for congenital adrenal hyperplasia?

Supplement what is missing when stressed: 1. Hydrocortisone 2. Fludrocortisone Signup and view all the answers

What complications do mothers with pre-existing type I diabetes face?

<ol> <li>IUGR 2. Congenital heart disease 3. NTD 4. Caudal regression syndrome 5. Small left colon syndrome</li> </ol> Signup and view all the answers

What newborn complications can result from mothers with gestational diabetes?

<ol> <li>LGA 2. Hypoglycemia 3. Hypocalcemia 4. Polycythemia 5. Jaundice 6. RDS</li> </ol> Signup and view all the answers

What are the complications of LGA?

Birth trauma (Clavicle, Erb's), TTN Signup and view all the answers

What are the complications associated with hypoglycemia?

Maternal hyperglycemia leads to fetal hyperinsulinemia, causing hypoglycemia after birth Signup and view all the answers

What are the complications of newborn hypoglycemia?

<ol> <li>Seizures</li> </ol> Signup and view all the answers

What is the treatment for newborn hypoglycemia?

Feed frequently if hyperbilirubinemia is present to avoid kernicterus Signup and view all the answers

Why does gestational diabetes cause respiratory distress syndrome (RDS)?

Insulin interferes with cortisol surge, decreasing lung maturation Signup and view all the answers

What is the diagnosis for a baby with a temperature of 100.4F?

Sepsis until proven otherwise Signup and view all the answers

What are the symptoms for sepsis in newborns regarding secondary sex characteristics?

To avoid osteoporosis Signup and view all the answers

What is Klinefelter's syndrome associated with in an 18-year-old male?

XXY Signup and view all the answers

What symptoms are associated with neurofibromatosis type 1?

Cafe au lait spots, large head, seizures, autosomal dominant inheritance Signup and view all the answers

What are the characteristics of Pierre Robin sequence?

Mandibular hypoplasia, glossoptosis, cleft soft palate, deletion in chromosome 17 Signup and view all the answers

What symptoms are associated with Prader-Willi syndrome?

Hypotonia, hypogonadism, hyperphagia, skin picking, aggression, deletion of paternal chromosome 15 Signup and view all the answers

What are the symptoms associated with Angelman syndrome?

Seizures, strabismus, sociable with episodic laughter, deletion of maternal chromosome 15 Signup and view all the answers

What are the characteristics of Williams syndrome?

Elfin appearance, friendly, increased empathy, verbal reasoning ability, deletion on chromosome 7 Signup and view all the answers

What are the symptoms associated with Cornelia de Lange syndrome?

IUGR, hypertonia, distinctive facies, limb malformation, self-injurious behavior, hyperactive Signup and view all the answers

What are the signs of fetal alcohol syndrome?

Microcephaly, smooth philtrum, thin upper lip, ADHD-like behavior Signup and view all the answers

What is the most common type of mental retardation in boys?

Fragile X syndrome Signup and view all the answers

What are the characteristics of Waardenburg syndrome?

Autosomal dominant, associated with advanced paternal age, short palpebral fissures, white forelock, deafness Signup and view all the answers

What is the diagnosis for a 2-year-old with multiple ear infections, diarrhea, and pneumonia with no tonsil exudate?

Burton agammaglobulinemia Signup and view all the answers

What is the inheritance pattern of Burton's agammaglobulinemia?

X-linked Signup and view all the answers

What lab findings are seen in Burton's agammaglobulinemia?

Absent B cells and low levels of all IgGs Signup and view all the answers

What are the diagnostic findings in a 17-year-old female with decreased levels of IgG, IgM, IgE, and IgA, with normal B cells?

Combined variable immune deficiency (CVID) Signup and view all the answers

Study Notes

Diaphragmatic Hernia

Characterized by respiratory distress and scaphoid abdomen in newborns.
Major concern is pulmonary hypoplasia, which limits lung development.
If diagnosed prenatally, management involves delivery at a center equipped for ECMO, allowing lung maturation before surgical intervention.

Tracheoesophageal Fistula (TE Fistula)

Symptoms include respiratory distress combined with excess drooling in newborns.
Diagnosis confirmed by placing a feeding tube and taking an X-ray to observe coiling in the thorax.
Associated with VACTERL anomalies, which include vertebral defects, anal atresia, cardiac issues, tracheoesophageal atresia, renal abnormalities, and limb defects.

Choanal Atresia

Presents in a one-week-old baby as cyanosis except when crying.
Linked to CHARGE syndrome, characterized by coloboma, heart defects, growth retardation, genitourinary anomalies, and ear abnormalities.

Respiratory Distress Syndrome (RDS)

Common in 32-week preterm infants, indicated by respiratory rate of 80, dyspnea, and nasal flaring.
Measurement of prenatal risk involves assessing the lecithin/sphingomyelin (L/S) ratio.

Intestinal Atresia

Occurs in a two-week-old infant with bilious vomiting and a history of polyhydramnios during pregnancy.
Often associated with Down syndrome, particularly in cases of duodenal atresia.

Malrotation and Volvulus

Presenting in a one-week-old infant with bilious vomiting, exhibiting abdominal distension and pulling of legs.
Condition arises when the intestine fails to complete a 270-degree counterclockwise rotation around the superior mesenteric artery.

Hirschsprung Disease

Diagnosed when a three-day-old infant has not passed meconium, with DRE revealing lack of stool.
Biopsy indicates absence of ganglia, which signifies a failure to develop neural cells in part of the bowel.

Necrotizing Enterocolitis (NEC)

Diagnosed in a 33-week preemie with bloody diarrhea.
X-ray findings show pneumatosis intestinalis, indicating air in the bowel wall due to bacterial infection.
Treatment involves discontinuation of feeding (NPO), total parenteral nutrition (TPN), and antibiotics, often necessitating bowel resection.

Intussusception

Identified in a two-month-old with colicky abdominal pain, "currant jelly" stools, and a right upper quadrant mass.
Barium enema serves as both diagnostic and therapeutic intervention.

Cryptorchidism

Occurs in newborn boys with non-palpable testicles, often residing in the inguinal canal.
Follow-up with ultrasound is advised if testicles remain non-palpable; surgical intervention is recommended if not descended by one year to prevent infertility or malignancy.

Hypospadias

Noted when the urethral opening is on the ventral surface of the penis.
Circumcision is contraindicated as foreskin may be needed for surgical correction.

Congenital Adrenal Hyperplasia (CAH)

Presents with ambiguous genitalia in newborns and subsequent symptoms such as vomiting and electrolyte imbalances.
Most commonly caused by 21-hydroxylase deficiency, confirmed through 17-OH progesterone levels.
Treatment includes glucocorticoid and mineralocorticoid supplementation.

Maternal Diabetes Complications

Type I diabetes in mothers increases risk for IUGR, congenital heart disease, neural tube defects, caudal regression syndrome, and small left colon syndrome.
Gestational diabetes can lead to newborn issues like LGA, hypoglycemia, polycythemia, jaundice, and respiratory distress syndrome.

Genetic Disorders

Klinefelter's Syndrome: XXY genotype, presenting with hypogonadism, gynecomastia, and risk of malignancy.
Neurofibromatosis type 1: Characterized by cafe-au-lait spots, seizures, large head, and autosomal dominant inheritance.
Pierre Robin Sequence: Features mandibular hypoplasia, glossoptosis, and cleft palate linked to a deletion on chromosome 17.
Prader-Willi and Angelman syndromes: Caused by deletions on chromosome 15, presenting with distinct behavioral and physical characteristics.
Williams syndrome: Associated with deletion on chromosome 7, characterized by elfin features and social friendliness.
Cornelia de Lange syndrome: Noted for IUGR, distinct facial features, and self-injurious behaviors.
Fetal Alcohol Syndrome: Causes microcephaly, smooth philtrum, thin upper lip, and is a common cause of mental retardation in children.

Immunodeficiency Disorders

Burton's agammaglobulinemia: X-linked condition leading to absent B cells and reduced immunoglobulin levels.
Combined Variable Immune Deficiency (CVID): Demonstrated by low levels of all immunoglobulin types with a normal B cell count.

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Test your knowledge on diaphragmatic hernia in pediatrics with these flashcards. Each question covers critical aspects such as diagnosis, concerns, and treatment options related to this condition. Perfect for medical students and pediatric healthcare professionals.