Pediatric Flashcards
Foregut, midgut, hindgut
Foregut – esophagus to 2nd portion of duo including ampulla. Gallbladder, liver, lungs, pancreas, bile duct – celiac artery
Midgut – 3rd portion of duo, first 2/3 of transverse colon. – SMA
Hindgut – last 1/3 of TV colon to anus – IMA and internal iliac
Umbilical blood supply
There are 2 umbilical arteries, one umbilical vein
Down’s syndrome associations
Down’s syndrome – Duodenal atresia, Hirshsprung’s, omphalocele, ASD -> ostium primum (endocardial cushion defect), annular pancreas
VACTERL associations
tracheoesophageal fistula, imperforate anus
Malrotation associations
Congenital diaphragmatic hernia, omphalocele, duodenal atresia
High associations with congenital anomlies
Duodenal atresia – Cardiac anomalies Need echo prior to surgery
Congenital diaphragmatic hernia – Malrotation, cardiac and neural tube defect Need echo before surgery
TEF – Cardiac abnormalities – get echo before surgery
Pediatric fluid resuscitation
Maintenance fluid = D5 0.9% NS with 10-20K
If Severe dehydration (mottled, lethargy, decreased cap refill) start with 20cc/kg of NS
The use of hypotonic or hypertonic crystalloid solutions for the purpose of emergent volume resuscitation is never recommended in pediatric patients – except pyloric stenosis
Oral rehydration therapy is the preferred treatment for children with mild to moderate hypovolemia or after severe dehydration and resuscitated
Infants with moderate or severe dehydration 2/2 to pyloric stenosis: D5 ½ (0.45) NS + 20K (1.5 to 2 times maintenance rate)
- If there is ANY renal dysfunction (rise in Cr or low UOP) DO NOT GIVE potassium until patient has UOP
- Alkalosis must be fixed prior to going to OR because it increases risk of post op apnea
Neonate and infant UOP should be 2-3 cc/kg/hr
Maintenance fluid for all pediatric patients = D5 ½ NS +/- potassium
- Avoid potassium in infants and toddlers until making urine
- All children need glucose in maintenance fluid
Congenital lobar hyperinflation (emphysema)
RESPIRATORY DISTRESS AND HYPOTESNION (Can look like a tension PTX) (SHIFT IN MEDIASTINUM)
CXR: shows hyperinflation of a lobe (hyper-lucent area); compression of other structures
Bronchus Cartilage fails to develop
Leads to air trapping with expiration
Vascular supply and lung tissues are normal lungs are just compressed by hyperinflation
LUL MC affected
Tx: Lobectomy
Pulmonary Sequestration
Pulmonary Sequestration
Sx: infections MC, resp distress, hemorrhage
Have a Continuous murmur
Dx: CT angio – shows lung mass with anomalous blood supply (key issue here)
MC LLL
Lung tissue is not connected to the airway, and has a SYSTEMIC ARTERIAL SUPPLY
- MC from thoracic aorta
- Can also be from abdominal aorta through inferior pulmonary ligament
Intra-lobar sequestration– remains in the native pleura viscera, pulmonary venous connection usual
Extra-lobar sequestration – enveloped by separate pleural lining, systemic venous drainage usual (MC Azygous)
Risk of CA
Tx: Ligate the systemic arterial supply FIRST (can bleed). Then perform segmentectomy vs lobectomy
Congenital cystic adenoid malformation (CPAM)
Sx: newborns respiratory distress, older children infections
Rapid decompensation can occur with ventilator needs emergent resection of cystic structure
Lobe of lung is replaced by a non-working cystic piece of abnormal lung tissue
Risk of malignancy
Communicates with airway bronchus unlike pulmonary sequestration
This is secondary to bronchiole tissue overgrowth!
Dx: in utero can measure CAM volume ration (CVR) = volume of lung/head circumference. If > 1.6 high risk for fetal hydrops and will need biweekly US and will need to give steroids to decrease size
Tx: Lobectomy
Choledochal cyst
Pediatric presentation → abdominal mass + obstructive jaundice
Adult presentation→ biliary or pancreatic symptoms AND symptomatic cholelithiasis
ASIANS common.
Caused by reflux of pancreatic enzymes 2/2 to abnormal biliopancreatic junction, having a long common channel
Dx: RUQ US 1st. 2nd MRCP next to define anatomy (diagnostic test of choice)f. Need to look all bile ducts intra-extrahepatic
Type I - MC 85% Saccular/fusiform dilation of CBD tx: CBD resection, Roux en y hepaticojejunostomy, and cholecystectomy
Type II - diverticulum off CBD tx: resection off CBD, and close primarily. If communicating with biliary tree you will need Roux en y hepaticojejunostomy
Type III or choledochocele: Dilation of duodenal CBD or where pancreas joins CBD. tx: Transduodenal approach with marsupialization or cyst resection may need sphincteroplasty
Type IVa fusiform both intra and extra hepatic tx: If only affecting one lobe partial hepatectomy with hepaticoJ. Or liver transplant
Type IVb multiple extra hepatic cysts – Tx: same as type I: CBD resection, Roux en y hepaticojejunostomy, and cholecystectomy
Type V (Caroli’s disease) - intrahepatic cyst only, get hepatic fibrosis, associated with congenital hepatic fibrosis and medullary sponge kidney tx: Partial liver resection or transplant
Lymphadenopathy in peds
-usually acute suppurative adenitis following URI or pharyngitis.
- If fluctuant and TENDER - FNA, culture, and abx. Recheck in 2 weeks.
- Chronic causes- cat scratch fever, atypical mycoplasma
Asymptomatic adenopathy in peds CNBx this is lymphoma until proven otherwise
Congenital Diaphragmatic hernia
!!50% survival!!!
Most die from congenital defects (neural tube and cardiac defects)
Usually on Left
Associated with vitamin A deficiency and exposure to drugs
Both lungs are dysfunctional
Ipsilateral lung (with hernia) severe pulmonary hypoplasia
Contralateral lung – severe pulmonary HTN, and some hypoplasia
Pulmonary HTN is the most important physiological affect causing hypoxia
50% will have life-long chronic pulmonary disease
80% have, malrotation, cardiac and neural tube defects (Most of them die due to the congenital defects not CDH)
Most devastating ECMO complication intracerebral bleeding
Dx:
- Prenatal dx can be made with MRI or US
- CXR for newborns to confirm will show bowel is in chest
Tx = medical optimization first
- Urgent surgical intervention IS NOT INDICATED HERE
- high frequency ventilation, NO, may need ECMO.
- intra-abdominal approach – reduce bowel and repair with mesh!
- Need to look for visceral anomalies (malrotation)
Bochdalek hernia
- MC hernia, located posteriorly on LEFT – Usually do not contain a hernia sac
Morgagni
More common in adults. Located anteriorly on the RIGHT, usually does contain hernia SAC.
Pectus excavatum
chest in, needs strut (Nuss Procedure) bar across mediastinum.
Repair if causing respiratory/cardiac sx or emotional distress with sternal osteotomy
All patients need echo and EKG preop. Have high cardiac abnormalities
Pectus carinatum
Pigeon chest. Strut not needed. First line treatment is a brace. Repair for emotional stress only
Cystic hygroma (lymphangioma)
Lymphatic malformation
Benign - Lateral to SCM in posterior neck triangle
Can get infected and forms sinuses
Tx: excision
Branchial cleft cyst
Leads to cyst, sinuses, or fistulas
1st branchial cleft cyst – Arise between angle of mandible to external auditory canal; facial nerve affected
2nd branchial cleft cyst (MC TYPE) - On the lower anterior border of middle SCM, goes through carotid bifurcation and into tonsillar pillar
* MC present as a cyst
3rd – Arise anterior to SCM in low neck into the piriform sinus, inside the pharynx!!
Tx: for all is resection of cyst
Thyroglossal duct cyst
Between hyoid bone and thyroid isthmus
Midline cervical mass, moves up with swallowing
Formed from descent of thyroid from foramen cecum
Risk of CA and infection
May be only thyroid tissue patient has
Tx: Sistrunk procedure: lateral neck incision excises cyst tract, hyoid bone
Hemangioma
MC tumor of childhood
Appears at birth or shortly after
Rapid growth during first 12 months then begins to involute
Most Resolves by age 8: tx: Observations unless, uncontrolled growth, impairs function (eyelid/ear canal), Bleed or ulcers, or persistent after age 8 → oral steroids. Laser or resection if not successful
Congenital AVM
-treatment is embolization.
-On lung: embolization.
-In bowel: endoscopy therapy
Neuroblastoma
#1 solid abdominal malignancy in < 2 years old
MC malignancy in infancy
<1 y/o have best prognosis. Prognosis is based on AGE and tumor biology
Overall survival <30%. 50% have mets at presentation
Symptoms:
* Asymptomatic abdominal mass
* Diarrhea
* Raccoon eyes (periorbital metastasis) proptosis, ecchymosis
* HTN
* opsomyoclonus syndrome (uncontrolled fast eye movement) (unsteady gait)
Mets to skin = blue skin lesions (blue berry muffin)
MC mets is adrenals but can occur anywhere on sympathetic chain
90% secrete catecholamines, Check UA: VMA, HVA, metanephrines
Derives from neural crest cells - Path = small round blue cells in ROSETTE pattern
Disease spectrum:
-ganglioneuroma (benign, still need to resect this)
-ganglioblastoma (malignant)
-neuroblastoma
One of the few malignancies in humans that can spontaneously regress
Worse prognosis:
- Age > 18 months, high grade, NSE (neuron specific enolase), LDH, > 3 copies of N-myc
Dx: CT scan: Calcifications compresses renal parenchyma rather than invades
MIBG – locates tumors
Metastasizes to bone, bone marrow, and liver (hepatomegaly)
Initially unresectable tumors can become resectable with doxorubicin-based chemo
NSE increased in all pt with mets
Tx: 5YS 40%
- Low risk (stage I and stage II): Just surgery, resect adrenal gland and kidney only
- Mod/high risk: neoadjuvant chemo (DECC) doxorubicin, etoposide, cisplatin and cyclophosphamide THEN RETINOIC ACID
- HIGH RISK: can down stage tumors; resection; Post op XRT
Resect even if stage IV
Stage I localized tumor completely excised
Stage II incomplete excision but does not cross midline
Stage III crosses midline +/- regional nodes
Stage IV distant mets (distant nodes or solid organ)
IV-S localized tumor with distant mets but age < 1
Wilm’s tumor (nephroblastoma)
10% are bilateral!
Hematuria, HTN and large abdominal mass in > 2 years old.
Most important prognostic factor is tumor grade
Much better prognosis than neuroblastoma
CT: replacement, of renal parenchyma. NO calcifications
Associated with WAGR: Wilms, aniridia, GU abnormality (cryptochordism and hypospadias), mental retardation. hemihypertrophy
Associated with Beckwith-Wiedemann (macroglossia, visceromegaly, hyper insulinemic hypoglycemia)
Tx:
- nephrectomy (90% cured)
- Dactinomycin and vincristine in all except If very low risk: stage I, < 500 g tumor, and favorable histology
- Pulm mets gets whole lung radiation
-If venous extension in renal vein, can still be extracted from the vein
-Need to Examine contralateral kidney and look for peritoneal implants
-Avoid rupture with resection can increase stage
-Need nephron sparing surgery for stage V (BL)
Stage I Limited to kidney, no capsule involvement Completely excised
Stage II Beyond kidney. Completely excised
Stage III residual non-hematogenous tumor. + nodes, or not completely excised
Stage IV hematogenous spread. Mets to any other organ
Stage V BL renal involvement
Time of repairs
-Cleft lip – (primary palate) –involves lip or alveolus or both. Failure of fusion of maxillary and medial nasal processes. Rule of 10, Repair at 10 weeks when Hgb is > 10, > 10 lbs weight
-Cleft palate - involves hard and soft palate. Hole in roof of mouth. Causes speech and swallowing issues. Repair at 1 year
-VSD: 80% close by 6 months. Surgical Tx: Repair when large (>2.5 shunt) at one year, and medium (2-2.5 shunt) at 5 years, Failure to thrive is MC reason for early repair
-ASD: Repair when symptomatic or asymptomatic at 5 years
-Tetralogy of Fallot: Tx: repair at 3-6 months with RV outflow tract obstruction removal. RVOFT enlargement, and VSD repair. VSD, RVH, overriding aorta, pulm stenosis
-PDA: Indomethacin only works for premature. Term babies need surgical repair with left thoracotomy immediately if symptomatic or @ 1-2 years old if asymptomatic
-Hypospadias - ventral opening tx: repair at 6 months with penile skin. Don’t circumcise
-Undescended Tests – Wait 6 months to repair
-Attempt to avoid splenectomy in children <5 years old. If < 10 years old and had splenectomy, needs daily augmentin for 6 months.
-Umbilical hernia; Delay until 5 years old unless incarcerated or patient has a VP SHUNT
-Hemangioma in children - Usually resolved by age 8: tx: Observations unless, uncontrolled growth, impairs function (eyelid/ear canal), it persistent after age 8 → oral steroids
Hepatoblastoma
MC liver CA in kids
Elevated AFP 90%, and BHCG (fractures and precocious puberty)
Better prognosis than HCC
Can be pedunculated, vascular invasion common
Survival is related to resectability
Strongly associated with beckwith-weidman
Tx: Resection followed by platinum based chemo, neoadjuvant doxorubicin based chemo can downstage and help with resection. Liver transplant
Fetal histology has the best prognosis
1 child malignancy
leukemia ALL
MC tumor in newborn
sacrococcygeal teratoma
1 in child < 2 years
neuroblastoma
1 in child > 2 years
Wilms tumor
MC malignant intestinal tumor
SB lymphoma
1 cause of duodenal obstruction in < 1 week old; after 1 week and overall MC in pediatric
1 cause of duodenal obstruction in < 1 week old = duodenal atresia
after 1 week and overall MC in pediatric = malrotation
1 cause of colon obstruction
Hirschsprung’s disease
1 liver tumor in children
hepatoblastoma
1 lung tumor
carcinoid
Painful lower GIB
1 benign anorectal lesions (fissures)
Painless LGIB
1 is Meckel’s
UGIB 0-1 y.o
gastritis/esophagitis
UGIB > 1 y.o and adult
esophageal varices, esophagitis
Double bubble sign
malrotation, duodenal atresia, or annular pancreas
Pyloric stenosis
RF: 1st born white male, family history, erythromycin
US best for Dx: >4 mm thick or pylorus channel > 14 mm length
Resuscitate with bolus NS (20cc/kg) and then D5 ½ (0.45) NS (1.5 to 2 times maintenance rate) Only add potassium if making urine
- If there is ANY renal dysfunction (rise in Cr or low UOP) DO NOT GIVE potassium until patient has UOP
- Alkalosis must be fixed prior to going to OR because it increases risk of post op apnea
Pyloromyotomy – incision from vein of mayo (junction of pylorus and duodenum) to proximal extent 1-2 mm into antrum of stomach. Submucosa and mucosa are NOT entered
Intussusception
Intussusception
3 months to 3 years
Currant jelly stools (from vascular congestions, not an indication for surgery)
Sausage shaped mass
Often have an antecedent viral infection (enlarged peyers patches)
MC ileum into right colon in children
15% recur after reduction
Pathological lead point– Meckel’s (MCC of intussusception due to pathological lead point), polyp, tumor, foreign body, rotavirus vaccine, Henlock shlonien purpura, etc.
Non-pathological – Peyer’s patch (MCC of intussusception in children OVERALL and non-pathological lead point), viral, shigella, yersinia
Tx:
* For children:
o MC caused by non-pathological lead point (idiopathic) = caused by Peyer’s Patches
o Dx US
o Pathological lead point 10%
o Non-pathological 90%
o Air contrast enema (DO AIR ENEMA first because less risk of perf) #2 choice is Barium enema→ 80% reduce no surgery required
o Max pressure 120 mmHg
o Max column high with enema 1 meter
o Go to OR if exceeds these values after 1-hour, high perforation risk
o Can do repeat enema if recurs
o Do not place traction on proximal limb, need to apply pressure to distal limb
o Usually do not require resection in kids unless it has a pathological lead point (Meckel’s) which all require resection
In adults:
o Dx: CT
o Most adults have MALIGNANT (MC cecal CA) pathological lead point (90%) and require resection
o MC presentation is obstruction
o All must go to OR for resection
o Must perform Lymphadenectomy during resection!
Intestinal atresia
Develop from intrauterine vascular accident
Commonly in the jejunum can be multiple
Sx: newborn with bilious vomiting
Dx: ABD xray
Will see a microcolon because colon not used
Tx: resection with primary anastomosis
RF: polyhydramnios in prenatal US
Get rectal bx to r/o Hirschsprung’s before surgery
Duodenal atresia
1 cause of duodenal obstruction in newborns < 1 week
MC type of intestinal atresia
MC type: distal to ampulla of vater (80%) causes bilious emesis
Etiology ischemic insult vs genetics
RF: polyhydramnios in mother, prematurity, Down’s syndrome, biliary atresia
20% have Down’s syndrome and annular pancreas
- Also associated with cardiac, renal and GI anomalies (malrotation)
- Must do workup for cardiac anomalies prior to surgery Must get an echo prior to surgery
Dx: X-ray will show classic double bubble sign (air in stomach and distal duodenum)
- Once you see this, you MUST order UGI to rule out malrotation
NOTE: The finding of double bubble sign with distal air in bowel can ALSO indicated duodenal stenosis, duodenal web or annular pancreas
Tx: duodenoduodenostomy or duodenojejunostomy
Persistent obstruction after surgery = duodenal webs resection of webs via longitudinal duodenostomy at the time of initial surgery if found
Duodenal Webs
2/2 to incomplete recanalization of the duodenum leaves a structure inside of the duodenum
This appearance = Windosock = pathognomonic when seen on UGI
Can presents in pediatric and adults
Dx: UGI will see retention of barium at site of web, mimics the appearance of duodenal diverticula
Tx: longitudinal Duodenotomy and excision of web
Malrotation
Associated with congenital diaphragmatic hernia, duodenal atresia
Can cause midgut volvulus or duodenal obstruction
Sudden onset of bilious vomiting
Usually present < 1 year, except newborns < 1 week = duodenal atresia
Ladd’s bands (bands between cecum and abdominal wall) cause duodenal obstruction
Embryonically supposed to rotate 270 degrees counter clockwise
Volvulus associated with compromise of SMA because axis of midgut is around SMA, leading to infarction
Volvulus occurs clockwise
75% by first month, 90% by 1 year
Any child < 2 years old with bilious vomiting needs UGI to r/o malrotation
Dx: UGI shows dilated duodenum and stomach. Duodenum does not cross midline, and duodenum and jejunum displaced to the right
Barium enema: cecum displaced to left or midline
Tx:
* Rotate counterclockwise
* Resect Ladd’s bands
* Mobilize duodenum
* Place cecum in LLQ (cecopexy) place small bowel in RUQ
* Appendectomy
Persistent obstruction after surgery = duodenal webs resection of webs via longitudinal duodenostomy
Meconium ileus
Causes distal ileal obstruction
Sx: Failure to pass meconium, Bilious vomiting
MC reason for neonate to require ECMO is meconium aspiration
Sweat chloride test or PCR for Cl channel defect (CTFR)
Thick meconium develops because of deficits in pancreatic enzymes
Colon is decompressed = compare to Hirschsprung’s disease
X-ray will show dilated SB WITHOUT air-fluid levels because meconium is too thick to separate from bowel wall.
* Can have ground glass or soap suds appearance
Can cause perforation leading to meconium pseudocyst (calcification on abdominal film) or free perforation Enema now Contraindicated, straight to laparotomy
Tx:
- FIRST RESUSCITATE and start abx and place NGT FIRST
- gastrograffin/N-acetylcysteine ENEMA (80% effective) under FLOUROSCOPY will dx and treat.
- Can repeat above Q12 hours for several days
- If surgery required manually decompress (milk meconium out) through an enterotomy and create a tube enterostomy for N- acetylcysteine antegrade enemas
- Can cause late strictures from ischemia
Tracheoesophageal fistula
RF: Male, diabetic mother, Low birth weight infant < 2500 (big one)
Prenatal US shows polyhydramnios
Sx: spits up feed, excessive drooling, respiratory sx with feeding
Can get X-ray below to confirm dx
Key presentation Can’t pass NGT
VACTERL, vertebral anomalies, anorectal (MC imperforate anus), cardiac defects (MC VSD), tracheoeosophageal fistula, renal/urinary, limb abnormalities NEED TO R/O THESE
70% have associated anomalies MC IS CARDIAC
MC type is Type C (85%) with proximal blind pouch and distal TE fistula
- Gas in stomach on ABD x-ray
2nd MC Type A (5%) with proximal blind pouch but no fistula. Sx same as above. X-ray = Gasless abdomen
TYPE E – (AKA H type)– TE fistula with no esophageal atresia. MC type to present later in infancy aspiration, recurrent PNA
Tx:
- 1st step is to place Replogle tube
- Always keep patient upright
- Avoid intubation or positive pressure ventilation because it increases risk of ventilation through fistula resp failure
- If need to intubate, patient needs G tube
- Right extrapleural posterolateral thoracotomy for most tie off and divide fistula, resect atretic segment
- Mobilize PROXIMAL esophagus, not distal primary anastomosis
- PLACE G TUBE
- NEEDS division of azygous vein (TE fistula underneath it)
- H type right cervicotomy
- If premature, too sick (Pneumonia, cardiac anomaly), or weight < 2500 g then just place replogle tube and G tube and fix later
- MC complication after repair = GERD
Most important prognostic factors = birth weight and associated anomalies MC is cardiac
Necrotizing enterocolitis
Thought to be caused by an episode of intestinal hypoperfusion then reperfusion mucosal injury bacterial translocation
Sx: bloody stools after FIRST feeding in a premature neonate = pathognomonic
RF: premature, hypoxia, sepsis
Abd Xray shows: pneumotosis, free air, portal venous air
- Need a lateral decub x ray to check for free air
Initial Tx: Abx, IVF, NPO, TPN, orogastric tube, X 2 weeks
Indications for operation:
- Free air or peritonitis
- Persistent metabolic acidosis
- Persistent thrombocytopenia <100 despite transfusion
- Erythema on abdominal wall = indicates dead bowel
- Positive paracentesis
- Pneumotosis by itself is NOT a indication for an operation
Resect all dead bowel bring up ostomies
If extremely ill and can’t tolerate laparotomy Place drains only
Complications = stricture
Need barium contrast enema AND UGI with SBFT before taking down ostomies, and before starting feeds to r/o stricture
Imperforate anus
- rectum fails to descend through the external sphincter complex
Rectums ends as a blind pouch, usually with fistulous tact to genitourinary system (males – urethra, bladder; scrotum. Females - vagina) or perianal skin
Associated with VACTERL
DO NOT PRESENT WITH BOWEL OBSTRUCTION
Males usually have high lesions
Females usually low
High lesions Males (60%) usually have high lesionsrectum ends above levator ani - Males MC with meconium in urine (MC rectum to prostatic urethra)
- Females MC rectum to upper vagina (may have cloacal deformity)
- If there is no visible meconium in perianal area in 24 hours presume it is high lesion sigmoid colostomy
- Treatment Sigmoid COLOSTOMY soon after birth, not an emergency.
- Later will need a posterior sagittal anoplasty in 3-6 months (places rectum into external sphincter complex)
Low lesions – Females usually have low lesions rectum ends below levator ani Meconium to perianal skin, scrotum, or lower vagina - Treatment posterior sagittal anoplasty at birth no colostomy
Usually require post op anal dilation to avoid stricture.
ALL PATIENTS ARE PRONE TO CONSTIPATION
Portal vein thrombosis in pediatrics
- MCC is umbilical vein infection
- Portal HTN in children MCC is portal vein thrombosis MCC of hematemesis in children
- Esophageal varices, splenomegaly WITHOUT cirrhosis is highly indicative of portal vein thrombosis
- Most children have a history of umbilical vein catheterization, neonatal omphalitis (umbilical sepsis) or neonatal intra-abdominal sepsis
- Dx: Duplex US
- Tx: Anticoagulation
Gastroschisis
No sac
Caused by: Intrauterine rupture of umbilical vein
Bowel is thickened/stiff due to irritation from amniotic fluid
Low congenital anomalies (10%, less common than omphalocele)
Except malrotation (all patients with gastroschisis have malrotation) and intestinal atresia (MC associated GI abnormality)
To the right of midline
Tx: Initially place saline-soaked gauzes, resuscitate. Abx, NPO, NGT, TPN
When patient is stable then you can operate place bowel back in abdomen and try to close primarily. If can’t get primary repair
Silastic mesh silo to abdominal wall
Contains viscera outside of body. Progressively tightened over days-weeks this stretches abdominal cavity primary closure at a later date
MC complication = sepsis
Mortality 10%
Omphalocele
Has sac with cord attached
Caused by: Failure of embryonal development
Majority (60%) have congenital anomalies MC is cardiac
MC GI finding = malrotation
Sac can contain structures other than bowel (Spleen, liver)
Midline defect
Herniation of organs more common
Associated with Down’s syndrome
Need to check for chromosomal abnormalities
Centrell pentalogy
- Cardiac defect
- Pericardium defect
- Sternal cleft or absence
- Diaphragmatic septum transversum absence Diaphragmatic hernia
- Omphalocele
Tx: Same as above
Much worse prognosis when compared to gastroschisis because of the associated congenital anomalies
Hirschsprung’s
MCC of colonic obstruction in infants
More common in males 4:1
MC symptom; Failure to pass meconium 1st 24 hours
Can also present in 2-3 y.o. as chronic constipation
Explosive release of watery stool with rectal exam
Associated with Down’s Sydrome
Dx: rectal biopsy (Suction biopsy is best test) shows absence of ganglions in Auerbach myenteric plexus
Failure of neural crest cell migration
Disease starts in rectum
75% just rectum affected, in 5% all of colon affected
Tx:
- Resect colon and rectum until you see ganglion
- Re-anastomose with Pull-through procedure Soave or Duhamel procedure
- Duhamel has less risk of anastomotic stricture and less pelvic dissection
- Duhamel – posterior wall of rectum anastomosed to anterior wall of colon
Hirschsprung’s colitis – may be rapidly progressive; abd pain and foul smelling diarrhea, SEPSIS, FEVER
- Can present as shock and sepsis
- Tx: 1st step is Rectal irrigation and IV abx
- If patient fails above and is still sick loop colostomy
Inguinal hernia
In children < 18 almost all are indirect hernia from persistent processus vaginalis
MC males, MC on right, more common in premature infants
RF prematurity
10% bilateral if present at birth
Lump that goes into the internal ring = hernia – differentiates from hydrocele
Always need to be surgically repaired in children
Incarceration risk: infants > children > adults
Incarceration (highest risk age is < 1 year). Treatment
- ALL need to be repaired regardless of age, question is, the timing laparoscopic repair without mesh
- First try to manually reduce
- Incarcerated and UNABLE to reduce/strangulated Emergency operation if not able to reduce
- If incarcerated and able to reduce admit, repair within 24-48 hours (this allows for tissue edema to subside)
- Inguinal hernia in premature infant Repair before discharge
- Repair - Needs high ligation of SAC, NO MESH, do this for up to age 16
- Explore contralateral side if left sided, female or child < 1 year old
Cystic duplication
MC in ileum on mesenteric border. Tx: Resect
Biliary atresia
MCC of neonatal jaundice requiring surgery
MC indication for liver TXP in children
Jaundice > 2 weeks after birth is suggestive of atresia (refractory to phototherapy)
- Acholic stools, palpable liver
Labs: elevated direct bili, GGT and LFT
Dx:
- 1st start with US will not visualize a gallbladder
- HIDA (used to screen) if negative, rules out atresia – liver lights up by does not go into intestine = biliary atresia
- Give phenobarbital as pretreatment prior to HIDA so liver excretes conjugated bili
- Best test to confirm: Operative cholangiography. ALL patients need this to investigate biliary atresia
- liver biopsy (does not confirm) Don’t need this. Periportal fibrosis, bile plugging, → eventual cirrhosis
Tx:
If extra-hepatic ducts affected only Kasai procedure (Roux-en-y hepaticoportojejunostomy)
- Resect atretic extra hepatic bile duct segment to porta hepatis. Then anastomose jejunum to porta hepatis
- Need to perform before 3 months otherwise irreversible liver damage and need transplant
- Majority will still need Liver TXP or die from cirrhosis
If intra hepatic ducts involved (ductal hypoplasia) Liver transplant
Teratoma pediatric (dermoid cyst)
Teratoma = non-seminoma = germ cell tumor
Neonates have embryonal type, occur along midline. MC sacrococcygeal = MC location overall
Adolescents have germ cell type Ovaries are MC, testicles
At risk for malignant degeneration
Tx: excision
Elevated AFP or BHCG suggests malignant transformation
Sacrococcygeal teratomas
- MC in females presents as mass at birth
- Can be found on prenatal U/S If hydrops seen, this has a very poor prognosis
- 90% are benign at birth
- almost all are exophytic but can be internal (presacral) and found later in life
- Great potential for malignant degeneration (adenocarcinoma)
- 2-month mark is a huge transition
- < 2 month usually benign
- > 2 month usually malignant
- Need CT to assess extent
- Tx: Coccygectomy and long-term f/u
- AFP is a good marker for recurrence if malignant
Undescended testes
If BL undescended get chromosomal studies
If you cannot feel testes in inguinal canal you need surgical exploration next. Imaging not very good.
MC location inguinal canal. Can be anywhere in the retroperitoneum, ectopic or vanished
Inguinal hernias are common
MC get seminoma. 5X risk if unilateral, 10 X if BL
CA risk stays the same even if testicles brought to scrotum
Infertility risk decreases but STILL NOT NORMAL if cryptorchidism is corrected
There is a risk of torsion in undescended testes
Wait until 6 months to repair
Tx: orchiopexy through inguinal incision AND high ligations of processus vaginalis
- If unable to get testes down due to length Ligation spermatic vessels (testicular vessel). Artery of vas will collaterize
- Want orchiopexy done by TWO YEARS OLD
Adult cryptorchidism –> resect testicle, almost all are non-functional
Prune belly syndrome
Hypoplasia of abdominal wall muscles, GU abnormalities, pulmonary hypoplasia
Tracheomalacia
Elliptical tracheal rings instead of C shaped
RF - TE fistula, or esophageal atresia
Wheezes, can have dying spells (usually with feeding)
Usually gets better after 1-2 years
Dx: Lateral CXR FIRST shows narrowing of trachea. THEN must do awake bronch shows A-P area of trachea collapsing
Usually does not require surgery
Indications for surgery - Dying spell (MC), failure to wean from vent, recurrent infections
Surgery – aortopexy. Aorta sutured to back of sternum opens up trachea
Laryngomalacia
Most common cause of airway obstruction in infants
Intermittent Respiratory distress and stridor exacerbated in the supine position
Caused by immature epiglottis cartilage with intermittent collapse of the airway
Most outgrow this by 12 months
Surgical tracheostomy reserved for very small # of patients
Choanal atresia
Obstruction of nasal passage by bone or mucous membrane
Usually, unilateral
Sx: intermittent respiratory distress, poor suckling
Tx: surgical excision of obstruction
Laryngopapillomatosis
MC tumor of pediatric larynx
Usually, involutes after puberty
Tx: endoscopic removal or laser but usually comes back
Associated with HPV in mother during passage through birth canal
Cerebral palsy
Many develop GERD
Patent urachus
connection between umbilicus and bladder (wet umbilicus). Tx: resection
Patent omphalomesenteric duct (vitelline duct)
Connects ileum to umbilicus. Drains succus. (meckel’s forms from this) Tx: resect duct
Persistent omphalomesenteric vessels (vitelline vessels)
Artery passes from umbilicus to aorta
- Vein passes from umbilicus to portal vein
- MC presents with torsion of bowel, possible strangulation.
- Occasionally attaches to meckel’s diverticulum
- Tx: De-torse bowel +/- resection. Ligate persistent vessels
Omphalitis
infection of umbilical stump after birth. Leads to portal vein thrombosis (varices) without cirrhosis. Tx: Heparin and abx
Pediatric airway
ETT size in pediatric – age/4 +4
Uncuffed ETT if under 1 year
Use cuffed ETT for >1 year
Don’t do sniffing position in infants, obstructs airway
Never do a cricothyroidotomy if <12 years old do NEEDLE cricrothyroidotomy instead
Percutaneous transtracheal jet insufflation – good temporizing measure, can clear airway too
Pediatric shock
Trauma bolus 20cc/kg X 2 then 10cc/kg blood
Best indicatory of shock in children: tachycardia
Even better is HR/SBP
Toddler = 1 - 3 years
Wilms tumor radical nephroureterectomy order/steps
-Ligation of renal artery before renal vein to prevent tumor engorgement (prevents unhindered blood flow to kidney/tumor without outflow which could lead to tumor rupture)
-Palpate renal vein before ligating it to rule out renal vein thrombus
Resect ureter close to the bladder
Biopsy lymph nodes at renal hilum, vena cava, and aorta
