10-Surgical Disorders

Question 1

Choanal Atresia

Answer 1

A congenital condition in which the posterior portion of the nasal passage ends in a blind pouch, with complete obstruction of the passageway between the nose and the nasopharynx.
Unilateral or bilateral
Most common congenital nasal abnormality

Question 2

CHARGE syndroms

Answer 2

Coloboma
Heart abnormalities
Atresia Choanae
Retarded mental development
Genital hypoplasia
Ear deformities

Question 3

How do you definitively asses?

Answer 3

CT scan will show bony as well as soft tissue anatomy

Question 4

Management and treatment for Choanal Atresia

Answer 4

-Short-term: Oral airway, feeding nipple with a large opening, and intubation
-Primary repair: Creating choanal openings in one surgery
-Some patients require several procedures (dilation or nasal stents)

Question 5

Macroglossia

Answer 5

Enlarged tongue. Can be generalized, relative or focal macroglossia

Question 6

Generalized macroglossia

Answer 6

It’s associated with other congenital disorders:
-Beckwith-Wiedemann syndrome- growth disorder.
-Inborn errors of metabolism
-Hypothyroidism

Question 7

Relative macroglossia

Answer 7

-Small craniofacial structures
-Trisomy 21 (down syndrome)

Question 8

Focal Macroglossia

Answer 8

Tumors

Question 9

Symptoms of Macroglossia

Answer 9

-Stridor
-Speech impairment
-Feeding intolerance
-Drooling

Question 10

Macroglossia interventions

Answer 10

-Oral airway-Sedate
-Intubation
-Surgical interventions (Tongue reduction and Tracheostomy)

Question 11

Pierre Robin Sequence

Answer 11

Previously known as Pierre-Robin Syndrome
Consist of 3 different reasons:
–Micrognathia
–Glossoptosis
–Cleft palate

Question 12

Micrognathia

Answer 12

Small Mandible. Can push the tongue back and obstruct airway.

Question 13

Glossoptosis

Answer 13

Obstruction of the airway by the bulk of the tongue

Question 14

Cleft palate

Answer 14

Failure of parts of the hard palate to completely fuse together during the first 12 weeks’ gestation, leaving a connection between the oral and nasal cavities.

Question 15

Management of Pierre Robin Sequence

Answer 15

-Jaw thrust
-Prone positioning
-Nasopharyngeal airway
-Surgical management for severe cases
—–Tongue-lip adhesion
—–Mandibular advancement
—–Tracheostomy

Question 16

Tracheoesophageal Fistulas

Answer 16

Abnormal connection between trachea and esophagus. Two kinds of fistulas
-Esophageal atresia (EA)
-Tracheoesophageal Fistula (TEF)

Question 17

Esophageal Atresia (EA)

Answer 17

A congenital defect in which the esophagus ends in a blind-ended pouch rather than connecting normally to the stomach.
-Esophagus is disconnected

Question 18

Tracheoesophageal Fistula

Answer 18

A connection between the esophagus and the trachea

Question 19

Tracheomalacia

Answer 19

-Excessive tracheal collapse from anterior cartilaginous abnormalities, posterior coaptation, or extrinsic compression from aberrant vasculature
- Can resolve within 2 years of birth without intervention

Question 20

Tracheomalacia presentation

Answer 20

-Cough (Bark)
-Recurrent infection
-Poor clearance of secretions
-Stridor
-Respiratory distress
-Acute life-threatening events (ALTEs) or Brief Resolved Unexplained Events (BRUEs)

Question 21

How do you diagnose tracheomalacia?

Answer 21

Bronchoscopy must show >50% during expiration

Question 22

Treatment for Tracheomalacia

Answer 22

Treat symptoms
PEEP
Pharmacotherapy
Surgery (Aortoplexy and Posterior tracheopexy)

Question 23

Congenital Diaphragmatic Hernia (CDH)

Answer 23

Occurs when the segments of the diaphragm fail to fuse by the eighth week of gestation, and the abdominal contents herniate, or protrude through the wall, into the thoracic cavity.

Question 24

Which side hernia is more severe?

Answer 24

Left sided because stomach and intestines fit through hole. On the right side the liver blocks the hole
-Right side also closes before left side

Question 25

Lung hypoplasia occurs in CDH because…

Answer 25

diaphragm is developed before lungs. In CDH, when the lungs develop around week 20, the herniated intestines have settled into place in the thorax and are now competing with the developing lung for space

Question 26

What is lung hypoplasia (CDH)

Answer 26

-Organ or tissue underdevelopment
-This will also occur where the intestines have herniated on the affected side

Question 27

Clinical complications of CDH

Answer 27

-Pulmonary hypoplasia
-Patent ductus arteriosus- Due to low oxygen. (prostaglandin E doesn’t lower)
-Patent formen ovale
-Malrotation – heart turning.
-Scaphoid abdomen; a convex belly because the abdominal contents are in the thorax. (most obvious)
-Hypoxemia leading to hypoxia
-Oxygenation index > 20; concerns regarding patient’s ability to oxygenate despite high levels of support.
-Pulmonary hypertension
-Cardiac insufficiency

Question 28

Delivery room management CDH

Answer 28

-Respiratory distress.
-Immediate intubation prior to signs of hypoxia and hypercarbia.
-Bag-mask ventilation should be avoided to prevent air inadvertently being forced into the stomach and intestines, which would further compress the lung tissue and exacerbate respiratory distress.
-Nasogastric tube should be placed to remove any gastric air that may impede lung function.
-FIO2 1.0 should be used during ventilation.

Question 29

Preoperative Management of CDH

Answer 29

-Hemodynamic stabilization and resp. support
-Conventional ventilation
-High-frequency ventilation
-Surfactant: due to primary surfactant deficiency.
-Pulmonary vasodilators for treatment of PPHN (iNO, Sildenafil, Prostacyclin).
-Inotropes: agents that improve cardiac contractility (normal saline, lactated ringers, dopamine, and dobutamine).
-Analgesia and paralysis
-ECMO

Question 30

Surgical Management CDH

Answer 30

-Radiographic clearing of lung fields.
-Resolution of right-to-left shunting across PDA, as a sign of improved PPHN.
-Right ventricular pressures < 2/3 systemic pressures, with good right ventricular function of echocardiography.
-Systemic blood pressure > 60 mm Hg consistently over previous 12 hours.
-Stable urination of > 1 ml/kg/hr over previous 12 hours.
-VT > 4 ml with PIP < 25 cm H2O and PEEP < 5 cm H2O.
-Optimal values of ABG were reached with FIO2 < 40% for at least the previous 24 hours.

Question 31

Most commonly used surgical technique for CDH

Answer 31

Open laparotomy

Question 32

Long-term outcomes of CDH

Answer 32

-Survival rates for CDH vary widely.
-Overall survival rates range from 60% to 68%.
-Patients treated with ECMO survive at lower rates, ranging from 46% to 67%.
-More than half of CDH survivors have gastrointestinal dysfunction, gastroesophageal reflux disease, oral aversion, poor oral feeding skills, neurocognitive delay, delays in reaching developmental milestones, behavioral disorders, and hearing loss.

Question 33

Omphalocele

Answer 33

A midline defect with the umbilical cord rising from the middle. The small intestines, liver, and stomach are frequently the herniated organs that are encapsulated by the peritoneal sac.

Question 34

Common abnormalities of Omphalocele

Answer 34

-Cardiac
-Cranial
-Urogenital defects

Question 35

To determine the size of the defect (omphalocel)

Answer 35

-The circumference of the omphalocele and the abdomen are taken and compared. Called the OC/AC ratio
-A higher ratio is associated with herniation of the liver and increased risk of respiratory insufficiency at birth.

Question 36

How early can Omphalocele be diagnosed?

Answer 36

In utero by gestational week 18 on fetal ultrasound.

Question 37

Management and treatment of Omphalocele

Answer 37

-Initial management is similar to patients presenting with gastroschisis.
-Nasogastric tube placed to suction for decompression of the GI tract.
-Care must be taken to protect the defect from contamination should the sac rupture.
-Surgical closure is based on the size of the defect.

Question 38

Surgical closure of Omphalocele

Answer 38

-Primary closure for small defects.
-Spring-loaded silo: allows for gradual reduction of visceral contents.
-Tissue expanders placed under the skin and filled with saline to stretch the abdominal skin and increase space in the abdominal cavity.
-Repair may be delayed for months or years due to epithelialization (growth of skin). Patients will be discharged home with topical medications such as mercurochrome, povidone-iodine, silver sulfadiazine, and neomycin-bacitracin ointments.

Question 39

Gastroschisis

Answer 39

-An abdominal wall defect characterized by protrusion of the intestines through the abdominal wall not covered by amnion.
-The defect develops between weeks 3 and 8 of gestation.

Question 40

Maternal risk factors of gastroschisis

Answer 40

-Cigarrette smoking
-Malnourishment
-Recreational drug use, specifically methamphetamines
-White or Hispanic mothers are at higher risk
-Maternal age < 20 years

Question 41

When can gastroschisis be diagnosed?

Answer 41

Fetal ultrasound around week 20, depending on the size of the defect.

Question 42

Why should delivery room be managed for gastroschisis

Answer 42

Intestines located outside the abdominal wall are sensitive to various insults. Bowel damage occurs in utero because of exposure to amniotic fluid and constriction at the abdominal wall.

Question 43

Delivery room can cause further complications

Answer 43

-Volvulus atresia (closure of part of the intestine)
-Bowel (Twisting of the bowel on itself, obstruction)
-Intestinal perforation (Formation of a hole in the large intestine; this portion of bowel will need resection)
-Bowel necrosis (Death of a bowel tissue)

Question 44

Delivery room management of Gastroschisis

Answer 44

-Newborn should be placed in a sterile drawstring bag, known as a bowel bag.
—–Protect from contamination or infection
—–Prevent fluid loss.
-Nasogastric tube
-Temperature regulation

Question 45

Respiratory Management for Gastroschisis

Answer 45

-Primary closures: Performed at the bedside in the NICU under sedation and paralysis. Patients will be intubated and placed on mechanical ventilation.
-Mechanical Ventilation: Provide support during the surgical resection. Overcome newly decreased thoracic compliance.

Question 46

Primary closure of gastroschisis

Answer 46

Intestines and other abdominal contents to elevate the diaphragm, anatomically increasing thoracic cavity space.
———Can cause increased work of breathing.
———Elevated PIPs on mechanical ventilator.
———Decreased delivered tidal volume in pressure ventilation.
———Patients may require high-frequency ventilation.