Pediatrics

• author "me"
• tags "Absite"
• description ""
• fileName "Pediatrics"
• freezingBlueDBID -1.0
• Daily fluid requirement of infants/children weighing 10-20kg:
• 1000mL/day
• + 50mL/kg/day for every kg over 10kg

2-3.5g/kg/day

• Duodenal atresia - newborns, bilious
• Pyloric stenosis - 3-12 weeks, nonbilious
• Malrotation - first few weeks of life, bilious
• Midgut volvulus - first few weeks of life, bilious
• Intussusception - 3-18 mo

150mL/kg

100mL/kg

1500mL + 20mL/kg over 20kg

4-6 mg/kg/min

Start infusion at 0.5 g/kg/day and advance to 2.5-3 g/kg/day

2-5 mEq/kg

2-3 mEq/kg

A congenital pulmonary airway malformation (CPAM) that has a systemic arterial blood supply and a variable venous drainage

Arterial flow can originate anywhere along the descending aorta inlcuding subdiaphragmatic locations

A true sequestration has no tracheobronchial communication

Urinary loss of acid (H+) and potassium to retain sodium and water in order to preserve fluid volume

• Hypokalemia
• Hypochloremic metabolic alkalosis (contraction alkalosis)
• Paradoxical aciduria

• Meconium aspiration syndrome (most common)
• Respiratory distress syndrome
• Persistent pulmonary hypertension
• Sepsis
• Congenital diaphragmatic hernia

• Selection criteria:
• - failure of conventional therapy with A-a gradient >620 for 12h or 6h in a pt with extensive barotrauma requiring high inotropic support
• - oxygen index > 40%

• Gestational age < 34weeks
• Birth weight < 2kg
• Irreversible pulmonary disease
• Uncorrectable cyanotic congenital heart disease
• Intractable coagulopathy/hemorrhage
• Intracranial hemorrhage
• Hx of > 10-14 days of high pressure mechanical ventilation

• Hematocrit
• Platelet count
• Fibrinogen
• Activated clotting time values
• Daily cranial ultrasound

Trauma (MVA's, falls, bicycle accidents, child abuse)

• Painful distracting injury
• Significant head injury
• Unreliable exam

Needle cricothyroidotomy with percutaneous transtracheal ventilation

80 mL/kg

• PRBC's 10-20 mL/kg
• FFP 20 mL/kg
• Platelets 10-20 mL/kg

Doppler ultrasound

Horizontal lie of a testicle as a result of the failure of normal posterior anchoring of the gubernaculum, epididymis, and testis to the tunica vaginalis thereby predisposing the testis to torsion

Surgical repair within 6hrs of symptom onset and contralateral orchiopexy to prevent future torsion

Observation.

If hydrocele persists past 1yo peritoneal communication is likely and hydrocelectomy with ligation of the patent processus vaginalis is indicated.

Ovaries

In the front or back of the ear in the region of the mandible

Along anterior border of the sternocleidomastoid

Tract emrges superior to cranial nerve IX and crosses anterior to IX and XII. The cyst lies anterior to X and XII

Most common

At the sternal notch or supraclavicular region

Duplications of external auditory canal

Angle of the mandible and pass up throuh the parotid gland

Along anterior boarder of sternocleidomastoid deep to external carotid but superficial to the internal carotid, ending at he tonsillar fossa

Most common

Lower neck, enter pharynx at piriform sinus. Pass deep to internal carotid and glossopharyngeal nerve but superficial to vagus nerve

Rare

On the antimesenteric boarder of the ileum between 4-60 cm from the ileocecal valve

Incomplete closure of the omphalomesenteric (vitelline) duct

• Gastric mucosa (50%) - can lead to ulcer formation causing painless
• lower GI bleeding = most common presenting symptom
• Ectopic pancreatic tissue (5%)

Leukemia (40%)

• < 2yo = Neuroblastoma
• > 2yo = Wilms tumor

Neonatal hepatitis

• Severe cholestasis
• Bile duct proliferation
• Inflammatory cell infiltration

Progressive fibrosis of the extrahepatic and intrahepatic bile ducts leading to biliary cirrhosis, portal hypertension, and death by 2yo without surgical correction

Most common indication for pediatric liver transplant

• US of liver - extrahepatic bile ducts not seen
• US gallbladder - diminutive/absent
• HIDA scan
• Percutaneous liver biopsy

Intraoperative cholangiogram demonstrating lack of opacification of the intrahepatic biliary tree

Surgical treatment of biliary atresia = Resection of the gallbladder, extrahepatic bile ducts, and fibrotic portal plate followed by Roux-en-Y portojejunostomy to restore biliary flow

Long-term successful outcome low if performed aftee 90 days of life

• Cholangitis (most common)
• Biliary cirrhosis
• Hepatic failure
• Portal hypertension

Rectal biopsy demonstrating submucosal hypertrophied nerve endings, abscent ganglion cells in the Meissner and Aeurbach plexuses, and acetylcholinesterase staining

Midgut volvulus = abrupt cutoff from failure of contrast material to pass beyond the distal duodenum or corkscrew pattern of partial obstruction

Malrotation = aberrant course of the duodenum and duodenal-jejunal junction

• Clockwise
• Counterclockwise

Present in malrotation, they are peritoneal bands between the ascending colon and the posterior abdominal wall in the rigt upper quadrant that must be divided so the duodenum can be mobilized and the small bowel can be positioned in the right side of the abdomen with the colon in the left side

True esophageal atresia without tracheoesophageal fistula (incidence = 6%)

Distal esophageal atresia with a proximal tracheoesophageal fistula (incidence = 2%)

Proximal esophageal atresia with distal tracheoesophageal fistula

Most common (incidence = 85%)

Proximal and distal tracheoesophageal fistula (incidence = 1%)

Intact esophagus with a single tracheoesophageal fistula (incidence = 2%)

• Defect > 2cm
• History of incarceration
• Large skin proboscis
• Presence of ventriculoperitoneal shunt

Duodenal atresia

S/S: bilious vomiting within first 24hrs of life without abdominal distention

In utero vascular accident

S/S: bilious vomiting with abdominal distention

• 10% of pts with jejunoileal atresia have cystic fibrosis
• Most common & significant morbidity a/w jejunoileal atresia = short gut syndrome

• Vertebral anomalies
• Imperforate Anus
• Cardiac defects
• Tracheoesophageal fistula
• Radial & Renal malformation
• Limb defects

Primary repair with division of the fistula, closure of its tracheal end, and end-to-end anastomosis of the esophageal segments

Gastrostomy and sump drainage of the blind proximal pouch

If respiratory problems, may benefit from primary fistula ligation with delayed esophageal repair

• Esophageal strictures
• Anastomotic leak
• Recurrent fistula
• Gastroesophageal reflux

• Perinatal stress
• Maternal cocaine use
• Prematurity
• Enteral feeding

Intestinal mucosa

Spectrum of severity ranges from isolated mucosal injury to transmural bowel necrosis

Terminal ileum and right colon

But disease can be segmental or affect entire GI tract

• Initial: nasogastric decompression, bowel rest, broad-spectrum antibiotics, optimal fluid management, parental nutrition
• Close monitoring with physical exam, serial radiographs, & biochemical assessment for signs of deterioration

• Operative: bowel resection with proximal enterostomy
• Indicated to treat acute complications including:
• - intestinal perforation
• - persistent necrosis evidenced by progressive clinical deterioration
• - persistent bleeding
• - obstruction

Operative intervention does not prevent progression of disease

Pathognomonic radiographic finding of NEC thst indicates invasion of the bowel wall by gas-forming organisms

Not an absolute indication for surgery

Capillary refill. Should be < 1sec

Heart rate.

Ability to increase cardiac output depends on increase in heart rate, not stroke volume

Low immunoglobulin and C3b levels

1 in 2000-5000 live births

Posterolateral location

Known as Bochdalek hernia.

Anteromedial (retrosternal) defect in the diaphragm leading to herniation

Prenatal ultrasound.

Plain CXR demonstrating intrathoracic location of gastric air bubbles, nasogastric tube, or intestines

Wait 24-72 hours for cardiorespiratory stabilization followed by surgical repair. (Emergent repair does not improve outcomes)

Survival rate 70-90%

Pulmonary hypoplasia & pulmonary HTN (due to pulmonary arteriolar vasoconstriction)

High pulmonary vascular resistance produces right --> left shunting via the patent ductus arteriosus

Goals= correction of hypoxia, metabolic acidosis, & hypothermia to relieve pulmonary vasoconstriction

• Endotracheal intubation with high-frequency oscillatory ventilation or inhaled nitric oxide (pulm vascular dilator) to avoid barotrauma
• - high pressure ventilation may cause PTX requiring chest tube
• Orogastric tube placement
• IV fluids
• ECMO in infants who remain critically ill despite conventional support

• Congenital Pulmonary Airway Malformation (CPAM)
• -Pulmonary sequestrations (intralobar or extralobar) - associated with congenital cardiac defects
• -Congenital cystic adenomatoid malformation (CCAM)
• Congenital lobar emphysema

Overdistention of a histologically normal lung because of air trapping secondary to abnormal cartlaginous support of the feeding bronchus

• Omphalocele - umbilical cord arises from the layers of peritoneum and amnion covering the abdominal wall contents
• 50% of infants born with omphalocele have other malformations including cardiac and chromosomal

Gastroschisis - a complete abdominal wall defect located to the right of the umbilical ring

Initial: nasogastric decompression, IV fluids, broad-spectrum antibiotics, protection of abdominal wall contents

• Omphalocele - cover sac with non-occlusive dressing
• Gastroschisis - place silo bag over exposed intestines

Later surgical correction

Urine vanillylmandelic acid (VMA) level - elevated in neuroblastoma

CT/MRI - neuroblastoma = displacement of intact kidney, Wilms = tumor of renal origin

• Denys-Drash syndrome
• WAGR syndrome (wilms, aniridia, GU abnormalities, mental retardation)
• Beckwith-Wiedemann syndrome

Neuroblastoma

• #1 - leukemia
• #2 -

Ileocolic intussusception

• Infection (most common)
• Hemorrhage
• Resporatory distress
• Malignant degeneration (rare)

Congenital lymphangiomatous malformation that commonly occurs in the posterior neck, axilla, groin, or mediastinum

Meconium ileus

• Gastrografin enema
• The detergent and hyperosmolar effects may loosen the thick meconium and relieve the obstruction

Surgery if no response to gastrografin enema or if peritonitis/perforation present

Barium or air enema for diagnosis & possible reduction (successful in 80%) - do not attempt if perforation/peritonitis

Operative manual reduction with appendectomy

Surgical ressection with primary ileocolic anastomosis (primary tx if nonviable bowel is present)

• Perforation/peritonitis
• Nonviable bowel
• Multiple recurrences
• Suspected lead point - intestinal polyp, Meckel diverticulum, tumor (lymphoma)

5-10% regardless of radiographical or operative reduction

• Duodenal atresia
• Normal finding (air takes 6-12hrs to reach distal colon)
• Malrotation - air present in distal intestines
• Midgut volvulus - air present in distal intestines

Duodenoduodenostomy

Also pass foley catheter through distal part of duodenum to assess for intraluminal windsock diaphragm or partial webs

12-24hr esophageal pH study is the most sensitive & specific

Tumors arising from more than one of the three embryonic germ layers containing tissue that is foreign to the anatomic site in which they are found

Sacrococcygeal

Gonads

• Hemihypertrophy
• Very low birth weight
• Familial adenomatous polyposis
• Beckwith-Wiedemann syndrome

• Perinatally acquired Hep B & C
• Mutations in c-met
• Tyrosinemia
• Biliary cirrhosis
• Alpha-1-antitrypsin deficiency

70%

25%

Hepatoblastoma

• Hepatoblastoma
• - under 3yo
• - unifocal
• - alpha-fetoprotein level parallels disease activity

• HCC
• - children and adults
• - multicentric and invasive
• - alpha-fetoprotein level parallels disease activity

• Thyroglossal duct cyst
• Dermoid cyst
• Lymphadenopathy
• Ectopic thyroid
• Thymic cyst
• Ranula

Hyoid bone

Ultrasound and thyroid function tests to confirm thyroid tissue in normal anatomic location so patient is not rendered hypothyroid post-operatively

Sistrunk procedure = removal of cyst tract along with central hyoid bone

< 5%

• Urine output (1-2mL/kg/day)
• Osmolarity (infant can only concentrate urine to max 700 mOsm/kg vs. 1200 in adult)

120 calories/kg to achieve ideal growth of 25-35 g/kg/day weight gain