10-Surgical Disorders Flashcards
Choanal Atresia
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
CHARGE syndroms
Coloboma
Heart abnormalities
Atresia Choanae
Retarded mental development
Genital hypoplasia
Ear deformities
How do you definitively asses?
CT scan will show bony as well as soft tissue anatomy
Management and treatment for Choanal Atresia
-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)
Macroglossia
Enlarged tongue. Can be generalized, relative or focal macroglossia
Generalized macroglossia
It’s associated with other congenital disorders:
-Beckwith-Wiedemann syndrome- growth disorder.
-Inborn errors of metabolism
-Hypothyroidism
Relative macroglossia
-Small craniofacial structures
-Trisomy 21 (down syndrome)
Focal Macroglossia
Tumors
Symptoms of Macroglossia
-Stridor
-Speech impairment
-Feeding intolerance
-Drooling
Macroglossia interventions
-Oral airway-Sedate
-Intubation
-Surgical interventions (Tongue reduction and Tracheostomy)
Pierre Robin Sequence
Previously known as Pierre-Robin Syndrome
Consist of 3 different reasons:
–Micrognathia
–Glossoptosis
–Cleft palate
Micrognathia
Small Mandible. Can push the tongue back and obstruct airway.
Glossoptosis
Obstruction of the airway by the bulk of the tongue
Cleft palate
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.
Management of Pierre Robin Sequence
-Jaw thrust
-Prone positioning
-Nasopharyngeal airway
-Surgical management for severe cases
—–Tongue-lip adhesion
—–Mandibular advancement
—–Tracheostomy
Tracheoesophageal Fistulas
Abnormal connection between trachea and esophagus. Two kinds of fistulas
-Esophageal atresia (EA)
-Tracheoesophageal Fistula (TEF)
Esophageal Atresia (EA)
A congenital defect in which the esophagus ends in a blind-ended pouch rather than connecting normally to the stomach.
-Esophagus is disconnected
Tracheoesophageal Fistula
A connection between the esophagus and the trachea
Tracheomalacia
-Excessive tracheal collapse from anterior cartilaginous abnormalities, posterior coaptation, or extrinsic compression from aberrant vasculature
- Can resolve within 2 years of birth without intervention
Tracheomalacia presentation
-Cough (Bark)
-Recurrent infection
-Poor clearance of secretions
-Stridor
-Respiratory distress
-Acute life-threatening events (ALTEs) or Brief Resolved Unexplained Events (BRUEs)
How do you diagnose tracheomalacia?
Bronchoscopy must show >50% during expiration
Treatment for Tracheomalacia
Treat symptoms
PEEP
Pharmacotherapy
Surgery (Aortoplexy and Posterior tracheopexy)
Congenital Diaphragmatic Hernia (CDH)
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.
Which side hernia is more severe?
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
Lung hypoplasia occurs in CDH because…
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
What is lung hypoplasia (CDH)
-Organ or tissue underdevelopment
-This will also occur where the intestines have herniated on the affected side
Clinical complications of CDH
-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
Delivery room management CDH
-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.
Preoperative Management of CDH
-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
Surgical Management CDH
-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.
Most commonly used surgical technique for CDH
Open laparotomy
Long-term outcomes of CDH
-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.
Omphalocele
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.
Common abnormalities of Omphalocele
-Cardiac
-Cranial
-Urogenital defects
To determine the size of the defect (omphalocel)
-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.
How early can Omphalocele be diagnosed?
In utero by gestational week 18 on fetal ultrasound.
Management and treatment of Omphalocele
-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.
Surgical closure of Omphalocele
-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.
Gastroschisis
-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.
Maternal risk factors of gastroschisis
-Cigarrette smoking
-Malnourishment
-Recreational drug use, specifically methamphetamines
-White or Hispanic mothers are at higher risk
-Maternal age < 20 years
When can gastroschisis be diagnosed?
Fetal ultrasound around week 20, depending on the size of the defect.
Why should delivery room be managed for gastroschisis
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.
Delivery room can cause further complications
-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)
Delivery room management of Gastroschisis
-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
Respiratory Management for Gastroschisis
-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.
Primary closure of gastroschisis
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.
