43 Pediatrics Flashcards
1
Q
foregut
A
lungs, esophagus, stomach, pancreas, liver, gallbladder, bile duct, duodenum proximal to ampulla
2
Q
midgut
A
duodenum distal to ampulla, small bowel, large bowel to distal 1/3 of transverse colon
3
Q
hindgut
A
distal 1/3 of t colon to anal canal
4
Q
rotation of gut during embryonic development
A
midgut rotation 270 degrees counterclockwise normally
5
Q
define low birth weight
A
<2500 g
6
Q
define premature
A
< 37 weeks
7
Q
immunity at birth
A
IgA from mom’s milk … IgG crosses placenta
8
Q
1 cause of childhood death
A
trauma
9
Q
trauma bolus in kids
A
20cc/kg x 2, then give blood 10cc/kg
10
Q
best indicator of shock
A
tachycardia …. neonat >150, <1yr >120, rest >100
11
Q
goal UOP in trauma
A
> 2-4 cc/kg/hr
12
Q
renal function in kids
A
kids <6mo only have 25% GFR cpacity of adults, poor concentrating ability
13
Q
mechanism of bone growth in kids
A
inc alk phos in kids relative to adults
14
Q
umbilical vessels
A
2 arteries, 1 vein
15
Q
mIVF amounts
A
4cc/kg/hr for 1st 10kg … 2 for 2nd 10kg … 1 for everything after that
16
Q
congenital cystic disease of the lung: list types
A
pulmonary sequestration, congenital lobar overinflation (emphysema), congenital cystic adenoid malformation, bronchiogenic cyst
17
Q
congenital cystic disease of the lung: pulmonary sequestration - describe
A
lung tissue has anomalous systemic arterial supply (via thoracic aorta or abdominal aorta through inferior pulmonary ligament), have either systemic venous or pulmonary vein drainage
18
Q
congenital cystic disease of the lung: pulmonary sequestration - locations
A
extra-lobar = more likely to have systemic venous drainage (azygous system) …. vs … intra-lobar = more likely to have pulmonary vein drainage
19
Q
congenital cystic disease of the lung: pulmonary sequestration - px
A
does NOT communicate with tracheobronchial tree … MC p/w infection, can also have resp compromise or abnormal CXR … tx w lobectomy
20
Q
congenital cystic disease of the lung: congenital lobar overinflation (emphysema) - pathophys
A
cartilage fails to develop in bronchus —> air trapping with expiration … vascular supply and other lobes are normal (except to the extent to which they are compressed by the hyperinflated lung) … can develop hemodynamic instability (same mech as tension PTX) or respiratory compromise
21
Q
congenital cystic disease of the lung: congenital lobar overinflation (emphysema) - MC lobe affected
A
LUL
22
Q
congenital cystic disease of the lung: congenital lobar overinflation (emphysema) - tx
A
lobectomy
23
Q
congenital cystic disease of the lung: congenital cystic adenoid malformation - pathophys
A
communicates with airway, alveolar structure is poorly developed, although lung tissue is present
24
Q
congenital cystic disease of the lung: congenital cystic adenoid malformation - sx
A
respiratory compromise or recurrent infection
25
congenital cystic disease of the lung: congenital cystic adenoid malformation - tx
lobectomy
26
congenital cystic disease of the lung: bronchiogenic cyst - describe
MC cysts of the mediastinum, usually posterior to the carina, extra-pulmonary cysts formed from bronchial tissue and cartilage wall
27
congenital cystic disease of the lung: bronchiogenic cyst - px
usually p/w mediastinal mass filled with milky liquid, can compress adjacent structures or become infected, have malignant potential, occasionally are intra-pulmonary
28
congenital cystic disease of the lung: bronchiogenic cyst - tx
resect cyst
29
mediastinal masses in children: MC
neurogenic tumors (neurofibroma, neuroganglioma, neurobalstoma) are the MC mediastinal tumors in kids, usually located posteriorly
30
mediastinal masses in children: px
respiratory sx, dysphagia - common to all mediastinal masses, regardless of location
31
mediastinal masses in children: list regions
anterior, middle, posterior
32
mediastinal masses in children: anterior masses
T cell lymphoma, teratoma, other germ cell tumors (MC anterior mediastinal mass in kids), thyroid CA
33
mediastinal masses in children: middle masses
T cell lymphoma, teratoma, cyst (cardiogenic or bronchiogenic)
34
mediastinal masses in children: posterior
T cell lymphoma, neuroblastoma, neurogenic tumor
35
mediastinal masses in children: thymoma
RARE in kids
36
choledochal cyst: casued by what?
reflux of pancreatic enzymes int the biliary system
37
choledochal cyst: inc risk for what?
cholangiocarcinoma, pancreatitis, cholangitis, obstructive jaundice
38
choledochal cyst: mgmt
need to resect
39
choledochal cyst: list types and frequencies
1 = 85%, 2 = 3%, 3 = 1%, 4 = 10%, 5 = 1%
40
choledochal cyst: rate, description, tx - type 1
85% .... fusiform dilation of entire CBD, mildly dilated common hepatic duct, normal intrahepatic ducts ... tx w resection, hepaticojejunostomy
41
choledochal cyst: rate, description, tx - type 2
3% .... true diverticulum that hangs off CBD ... resection off CBD, may be able to preserve CBD and avoid hepaticojejunostomy
42
choledochal cyst: rate, description, tx - type 3
1% .... dilation of distal intramural CBD, invovled sphincter of Oddi ... resection, choledochojejunostomy
43
choledochal cyst: rate, description, tx - type 4
10% ... multiple cysts, both intrahepatic and extrahepatic ... resection, may need liver lobectomy, possible TXP
44
choledochal cyst: rate, description, tx - type 5
1% ... caroli's disease - intrahepatic cysts, get hepatic fibrosis, may be assoc with congenital hepatic fibrosis and medullary sponge kidney ... resection, may need lobectomy, possible liver txp
45
lymphadenopathy: MC cause
acute suppurative adenitis assoc with URI or pharyngitis
46
lymphadenopathy: mgmt if fluctuant
FNA, culture and sensitivity, and abx ... may need I&D if fails to resolve
47
lymphadenopathy: chronic causes
cat scratch fever, atypical mycoplasma (often fluctuant)
48
lymphadenopathy: mgmt if a-sx
abx x10d ---> excisional bx if not improvement ... dx is lymphoma until proven otherwise
49
lymphadenopathy: cystic hygroma
aka lymphangioma, usually found in lateral cervical regions in neck, gets infected, is usually lateral to SCM muscle, tx w resection
50
diaphragmatic hernia and chest wall: overall survival rate
50%
51
diaphragmatic hernia and chest wall: px
80% on L side ... can have severe pulm HTN .... 80% have assoc anomalies (cardiac and neural tube defects mostly, malrotation)
52
diaphragmatic hernia and chest wall: dx
can be made with prenatal U/S
53
diaphragmatic hernia and chest wall: sx
respiratory distress
54
diaphragmatic hernia and chest wall: CXR
bowel in chest
55
diaphragmatic hernia and chest wall: tx
high frequency ventilation, inhaled nitric oxide, may need ECMO ... stabilize before OR .... need to reduce bowel and repair defect +/- mesh (abdominal approach) ... look for visceral anomalies (run the bowel)
56
Bochdalek's vs Morgagni's hernia
Bochdalek's = MC, located posteriorly .... Morgagni's = rare, located anteriorly
57
pectus excavatum vs carinatum
excavatum = sinks in, sternal osteotomy, need strut, performed if causing respiratory sx or emotional stree ... carinatum = pigeon chest, strut not necessary, repair for emotional stress
58
branchial cleft cysts: lead to what
cysts, sinuses, fistulas
59
branchial cleft cysts: types, MC
1st, 2nd, 3rd branchial cleft cysts ... 2nd = MC
60
1st branchial cleft cysts
angle of the mandible, may connect with external auditory canal, often assoc with facial nerve
61
2nd branchial cleft cysts
MC, on anterior border of mid-SCM muscle, goes through carotid bifurcation into tonsillar pillar
62
3rd branchial cleft cysts
lower neck, medial to or through the lower SCM
63
branchial cleft cysts: tx
resection
64
branchial cleft cysts: px
sinuses and fistulas usually in infants and young kids ... cysts usually appear at later age
65
thyroglossal duct cyst: pathophys, px, tx
from the descent of the thyroid gland from the foramen cecum, may be only thyroid tissue pt has, presents as a midline cervical mass, goes through the hyoid bone, tx w excision of cyst, tract, and hyoid bone (at least central portion)
66
hemangioma: px, tx
appears at birth or shortly after, rapid growth during first 6-12 months of life, then begins to involute ... tx = observation, most resolve by age 7-8 ... if lesion has uncontrollable growth, impairs function (eyelid or ear canal) or is persistent after age 8 - can tx w oral steroids --> laser or resection if steroids are not successful
67
#1 solid abdominal malignancy in kids
neuroblastoma
68
CT of neuroblastoma vs nephroblastoma
displacement vs replacement
69
neuroblastoma: px
a-sx abd mass in kids (MC) ... can also have secretory diarrhea, raccoon eyes (orbital mets), HTN, and opsomyoclonus syndrome (unsteady gait)
70
neuroblastoma: location
MC is adrenal, can occur anywhere along sympathetic chain
71
neuroblastoma: age
MC in 1st 2 years of life
72
neuroblastoma: prognosis
best in kids <1yr
73
neuroblastoma: labs
inc catecholamines, VMA, HVA, metanephrines (HTN)
74
neuroblastoma: cell of origin
neural crest cells
75
neuroblastoma: vascular involvement
encases vasculature rather than invading
76
neuroblastoma: mets
rare, goes to lung and bone
77
neuroblastoma: abd xray findings
may show stippled calcifications in the tumor
78
neuroblastoma: worse prognosis
NSE, LDH, HVA, diploid tumors, N-myc amplification (>3 copies)
79
neuroblastoma: seen in pts with mets
NSE increase
80
neuroblastoma: tx
resection (adrenal gland and kidney), 40% cure rate ... intially unresectable tumors may be resectable after doxorubicin-based chemo
81
neuroblastoma: staging
1 = localized, complete excision .... 2 = incomplete excision but does not cross midline ... 3 = crosses midline +/- regional nodes .... 4 = distant mets (nodes or solid organ) ... 4s = localized tumor with distant mets
82
Wilms tumor: aka
nephroblastoma
83
Wilms tumor aka nephroblastoma: px
usually px as a=sx mass, can have hematuria or HTN, 10% b/l
84
Wilms tumor aka nephroblastoma: mean age at dx
3yo
85
Wilms tumor aka nephroblastoma: prognosis
based on tumor grade (anaplastic and sarcomatous variation have worse prognosis)
86
Wilms tumor aka nephroblastoma: mets
frequent, to bone and lung
87
Wilms tumor aka nephroblastoma: mgmt of pulm mets
resect if resectable
88
Wilms tumor aka nephroblastoma: abd CT findings
replacement of renal parenchyma and NOT displacement (differentiates from neuroblastoma)
89
Wilms tumor aka nephroblastoma: tx
nephrectomy (90% cure rate) ... if venous extension occurs in the renal vein, the tumor can be extracted from the vein ... need to examine the contralateral kidney and look for peritoneal implants ... avoid rupture of tumor with resection (this would increase stage) .... actinomycin and vincristine based chemo in all unless stage 1 and <500g tumor
90
Wilms tumor aka nephroblastoma: staging
1 = limited to kidney, complete excised ... 2 = beyond kidney but completely excised ... 3 = residual nonhematogenous tumor ... 4 = hematogenous mets ... 5 = b/l renal involvement
91
MC malignant tumor in kids
hepatoblastoma
92
hepatoblastoma: labs
inc AFP in 90%
93
hepatoblastoma: px
fractures, precocious puberty (from beta-hcg release)
94
hepatoblastoma: prognosis
better than HCC
95
hepatoblastoma: describe mass
can be pedunculated, vascular invasion is common
96
hepatoblastoma: tx
resection optimal, o/w doxorubicin and cisplatin based chemo --> may downstage tumors and make them resectable
97
hepatoblastoma: survival
primarily related to resectability
98
hepatoblastoma: best prognosis
fetal histology
99
#1 kids malignancy overall
leukemia (ALL)
100
#1 solid tumor class
CNS tumors
101
#1 general surgery tumor in kids ... in kids <2yo ... in kids >2yo
neuroblastoma .... neuroblastoma ... Wilms tumor
102
#1 cause of duodenal obstruction in newborns (<1week) vs after newborn period (>1week) and overall
duodenal atresia ... malrotation
103
#1 cause of colon obstruction
Hirschsprung's disease
104
#1 liver tumor in kids
hepatoblastoma ... 2/3 of liver tumors in kids are malignant
105
#1 lung tumor in kids
carcinoid
106
painful vs painless lower GI bleeding
painful = anorectal lesions (i.e. fissues, etc) ... painless = Meckel's
107
upper GI bleeding 0-1yo vs 1yo-adult
gastritis, esophagitis ... esophageal varcies, esophagitis
108
meckel's diverticulum: location
antimesenteric border of small bowel
109
meckel's diverticulum: embyrology
persistent vitelline duct
110
meckel's diverticulum: rule of 2s
2 feet from ileocecal valve, 2% of population, 2% symptomatic, 2 tissue types (pancreatic MC, gastric most liklely to be sympomatic), and 2 presentations (diverticulitis and bleeding)
111
meckel's diverticulum: #1 cause of
painless lower GI bleeding in kids
112
meckel's diverticulum: workup
can get Meckel's scan with pertechnetate if suspicious of diverticulum but having hard time locating
113
meckel's diverticulum: tx
resection with sx, suspicion of gastric mucosa, narrow neck ... diverticulitis involving base or if the base if >1/3 the size of the bowel, need to perform segmental resection
114
pyloric stenosis: typical pt
firstborn M, 3-12 weeks
115
pyloric stenosis: px
projectile vomiting ... get hypoCl, hypoK metabolic alkalosis ... feel olive mass in stomach
116
pyloric stenosis: U/S findings
pylorus >=4mm thick, >=14mm long
117
pyloric stenosis: fluid mgmt - boluses, what to avoid, infants
severe dehydration ---> resuscitate with NS boluses until making urine ---> switch to D5 NS with 10mEq K for maintenance .... AVOID fluid resus with K containing fluids in kids with severe dehydration b/c hyperK develops quickly, AVOID non-alt containing solutions in infants b/c hypoNa can quickly develop .... infants should always have a maintenance fluid with glucose b/c of their limited reserves for gluconeogenesis and vulnerability for hypoglycemia
118
pyloric stenosis: tx
pyloromyotomy = RUQ incision, proximal extent should be the circular muscles of the stomach
119
intussusception: typical age
3mo to 3yrs
120
intussusception: px
currant jelly stools (from vascular congestion, NOT an indication for resection), sausage mass, abdominal distention, RUQ pain, vomiting
121
intussusception: pathophys
invagination of one loop of intestine into another ... lead points in kids = enlarged peyer's patches (#1), lymphoma, Meckel's diverticulum
122
intussusception: recurrence rate
15% after reduction
123
intussusception: tx
reduce with air contrast enema, 80% successful and no surgery needed if reduced ... if fails, peritonitis present, or free air present, then go to OR
124
intussusception: enema characeristics
max P with air contrast enema is 120mmHg ... max column height with barium enema is 1meter (3ft) ... high perforation risk beyond
125
intussusception: surgical indications
peritonitis, free air, unable to reduce
126
intussusception: surgical technique
when reducing in OR, do NOT place traction on proximal limb of bowel - need to apply P to distal limb .... usually NO resection required unless associated with lead point (Meckel's)
127
intussusception: mgmt if px in adults
most likely malignant lead point (i.e. colon CA in cecum) ---> OR for resection
128
intestinal atresia: caused by what, sx, location, mgmt and tx
develop 2/2 intrauterine vascular accidents ... sx = bilious emesis, distention, most do NOT pass meconium ... MC in jejunum, can be multiple ... get rectal bx to r/o Hirschsprung's preop ... tx w resection
129
#1 cause of duodenal obstruction in newborns
duodenal atresia
130
duodenal atresia: location, px
usually distal to ampulla of vater ... p/w bilious emesis, feeding intolerance
131
duodenal atresia: associated with what
polyhydramnios in mom ... cardiac, renal, other GI anomalies ... 20% with Down's syndrome (check chromosomal studies)
132
duodenal atresia: xray
abd xr shows double-bubble sign
133
duodenal atresia: tx
resuscitation, duodenoduodenostomy or duodenojejunostomy
134
tracheoesophageal fistulas (TEF): MC type, 2nd MC type
type C = 80% = proximal esophageal atresia (blind pouch) and distal TE fistula ... type A = 5% = esophageal atresia and no fistula
135
tracheoesophageal fistulas (TEF): type C - describe, sx, imaging
MC type (80%), proximal esophageal atresia (blind pouch) and distal TE fistula ... sx = newborn spits up feed, excessive drooling, respiratory sx with feeding, cannot place NGT in stomach ... abd xray = distended, gas-filled stomach
136
tracheoesophageal fistulas (TEF): type A - describe, sx, imaging
2nd MC type (5%), esophageal atresia and no fistula ... sx similar to type C = newborn spits up, XS drooling, resp sx w feeds, can't place NGT in stomach ... abd xray = gasless abdomen
137
tracheoesophageal fistulas (TEF): tx
R extrapleural thoracotomy for most, perform primary repair, placed G tube, azygous vein often needs to be divided
138
tracheoesophageal fistulas (TEF): mgmt of infants that are premature, <2500g, or sick
replogle tube, treat resp sx, place G tube, delay repair
A Replogle tube is a medical device used in the treatment of babies with oesophageal atresia. It is a double-lumen tube which is inserted through the baby's nostril into the blind-ending oesophageal pouch and used to drain saliva
139
tracheoesophageal fistulas (TEF): complications of repair
GERD, leak, empyema, stricture, fistula
140
tracheoesophageal fistulas (TEF): survival related to what
birth weight, assoc anomalies
141
describe VACTERL syndrome
Vertebral, Anorectal (imperforate anus), Cardiac, TE fistula, Radius/renal, Limb anomalies
142
malrotation: px
sudden onset bilious vomiting (Ladd's bands cause duod obstruction, coming out from the R retroperitoneum)
143
malrotation: pathophys
assoc with compromise of SMA --> infarct of the intestine .... failure of normal COUNTERclockwise rotation (270 degrees) ... Ladd's bands cause duo obstruction, coming out from the R retroperitoneum
144
malrotation: age at presentation
75% in the first 1 month, 90% present by 1 year old
145
malrotation: dx
UGI - duo does NOT cross midline, duo-jej junction displaced to the R
146
malrotation: tx
resect Ladd's bands, counterclockwise rotation (may require multiple turns), place cecum in LLQ (cecopexy), place duo in RUQ, and appendectomy
147
meconium ileus: leads to what
distal ileal obstruction, abd distention, bilious vomiting, distended loops of bowel
148
meconium ileus: assoc with what
CF - occurs in 10% of children with CF
149
meconium ileus: workup
need sweat chloride test or PCR for Cl channel defect (b/c of frequency of occurrence with CF)
150
meconium ileus: imaging
abd xr: dilated loops of small bowel without air-fluid levels - b/c meconium is too thick to separate from the bowel wall, can have ground glass or soapsuds appearance
151
meconium ileus: complications
can cause perforation, leading to meconium pseudocyst or free perforation ---> requires laparotomy
152
meconium ileus: tx
gastrografin enema (effective in 80%), can also make the dx and potentially treat the pt ... can also use N-acetylcysteine enema ... if surgery required, manueal decompression and create a vent for N-acetylcysteine antegrade enemas
153
necrotizing enterocolitis (NEC): px
bloody stools after 1st feeding in premature neonate
154
necrotizing enterocolitis (NEC): risk factors
prematurity, hypoxia, sepsis
155
necrotizing enterocolitis (NEC): sx
lethargy, resp decompensation, abd distention, vomiting, blood per rectum
156
necrotizing enterocolitis (NEC): imaging
abd xr may show pneumatosis intestinalis, free air, or portal vein air ... need serial lateral decubitus films to look for perforation
157
necrotizing enterocolitis (NEC): initial tx
resuscitation, NPO, abx, TPN, orogastric tube
158
necrotizing enterocolitis (NEC): surgical indications
free air, peritonitis, clinical deterioration
159
necrotizing enterocolitis (NEC): surgical approach
resect dead bowel and bring up ostomies ... need Ba contrast enema before taking down ostomy to r/o distal obstruction from stenosis
160
necrotizing enterocolitis (NEC): mortality rate
10%
161
congenital vascular malformation: surgical indications, tx
surgery for hemorrhage, ischemia, CHF, nonbleeding ulcers, functional impairment, limb-length discrepancy ... tx w embolization (may be sufficient on its own) and/or resection
162
imperforate anus: MC patients
M
163
imperforate anus: workup
check for associated VACTERL anomalies (vertebral, cardiac, TE fistula, Radius/Renal, Limb)
164
imperforate anus: types
high (above levators) ... low (below levators)
165
imperforate anus: high type - px, tx
high = above levators ... meconium in urine or vagina (fistula to bladder/vagina/prostatic urethra) ... tx w colostomy, later anal reconstruction with posterior sagittal anoplasty (pull anus down into sphincter mechanism) ... need postop anal dilatation to void structure, these pts are prone to constipation
166
imperforate anus: low type - px, tx
low = below levators ... meconium to perineal skin ... perform posterior sagittal anoplasty (pull anus down into sphincter mechanism), NO colostomy needed ... need postop anal dilatation to void structure, these pts are prone to constipation
167
imperforate anus: px, tx - high vs low
high = above levators, low = below ... high = mec in urine or vagina (fistula to bladder/vag/prostatic urethra), low = mec to perineal skin ... BOTH = tx w posterior sagittal anoplasty (pull anus into sphincter mechanism), but high = colostomy first and later recon, low = no colostomy ... BOTH = need postop anal dilatation to void structure, these pts are prone to constipation
168
gastrochisis vs omphalocele
gastro = intrauterine rupture of umbilical vein, does NOT have peritoneal sac, 10% assoc congenital anomalies, to R of midline ... omphalo = failure of emb development, WITH peritoneal sac, 50% with assoc abnL, midline defect ... malrotation may occur with both ... omphalo has worse prognosis 2/2 assoc abnormalities
169
gastroschisis: pathophys
intrauterine rupture of umbilical vein, does NOT have a peritoneal sac
170
gastroschisis: describe appearance
to R of midline, NO peritoneal sac, stiff bowel from exposure to amniotic fluid
171
gastroschisis: assoc congential anomalies
only about 10%, malrotation
172
gastroschisis: tx
initially place saline-soaked gauze and resuscitate pt (can lose a lot of fluid from the exposed bowel), TPN, NPO ... repair when pt is stable ... at operation may try to place bowel in abdomen, +/- vicryl mesh silo - primary closure at a later date if use silo
173
Cantrell pentalogy
(1) cardiac defect, (2) pericardium defects (usually at disphragmatic pericardium), (3) sternal cleft or absence of lower sternum, (4) diaphragmatic septum transversum absence, (5) omphalocele
174
omphalocele: pathophys
failure of embryoneal development, HAS peritoneal sac with cord attached
175
omphalocele: describe appearance
midline defect, WITH peritoneal sac with cord attached, ac can contain structures other than bowel (i.e. liver, spleen, etc)
176
omphalocele: assoc congenital abnormalities
50%, midline defect
177
omphalocele: tx
initially place saline-soaked gauze and resuscitation pt, can lose a lot of fluid from exposed bowel, TPN, NPO ... repair with pt is stable ... at operration, try to placed bowel back in abdomen, may need Vicryl mesh silo, primary closure at a later date if mesh used
178
omphalocele: prognosis
worse than gastroschisis 2/2 congenital anomalies
179
#1 cause of colonic obstruction in infants
Hirschsprung's disease
180
Hirschsprung's disease: gender
MC in M
181
Hirschsprung's disease: MC sign
(#1 cause of colonic obstruction in infants), MC sign = infants fail to pass meconium in 1st 24 hours ... can also p/w chronic constipation in ages 2-3
182
Hirschsprung's disease: px
infant fails to pass meconium in 1st 24hours, chronic constipation in ages 2-3 .... distention, occasionally colitis ... explosive release of watery stool with anorectal exam
183
Hirschsprung's disease: dx
rectal bx = absence of ganglion cells in myenteric plexus
184
Hirschsprung's disease: pathophys
failure of neural crest cells (ganglion cells) to progress in caudad direction (which is why you see the absence of ganglion cells in myenteric plexus on rectal bx)
185
Hirschsprung's disease: surgery
resect colon proximally to the point where ganglion cells appear ... tx = may need to bring up initial colostomy, then eventually connect the colon to the anus (Soave or Duhamel procedures)
186
Hirschsprung's colitis: describe, px, tx
may be rapidly progressive ...abd distention, foul-smelling diarrhea, +/- lethargy and signs of sepsis .... tx = rectal irrigation and try to empty colon, may need emergency colectomy
187
umbilical hernia: describe, MC pts, tx
failure of closure of linea alba, most close by age 3, rare incarceration ... inc in Af American and premature infants ... tx = surgery if not closed by age 5, incarceration, pt w VP shunt
188
inguinal hernia: pathophys
persistent processus vaginalis
The vaginal process (or processus vaginalis) is an embryonic developmental outpouching of the parietal peritoneum. It is present from around the 12th week of gestation, and commences as a peritoneal outpouching.
In males, it precedes the testes in their descent down within the gubernaculum, and closes. This closure (also called fusion) occurs at any point from a few weeks before birth, to a few weeks after birth. The remaining portion around the testes becomes the tunica vaginalis. If it does not close in females, it forms the canal of Nuck.
189
inguinal hernia: rate in infants, MC gender
3% of infants, M > F
190
inguinal hernia: laterality
60% on R, 30% on L, 10% b/l
191
inguinal hernia vs hydrocele
extension of hernia into the internal ring
192
inguinal hernia: tx
non reducible ---> emergent operation ... o/w elective repair with high ligation ... consider exploring the contralateral side IF L sided, F, chidl <1yo .... need operation within 24 hours of reduction
193
hydrocele: describe, tx
most disappear by 1yo, noncommunicating will resolve, shoudl transilluminate ... tx = surgery at 1 year if not resolved or if through to be communicating (waxing and waning size), resect hydrocele and ligate processus vaginalis
194
cyst duplication: MC site, tx
MC in ileum, often on mesenteric border, tx = resect cyst
195
MC cause of neonatal jaundice requiring surgery
biliary atresia
196
biliary atresia: px
progressive jaundice persisting >2weeks after birth is suggestive
197
biliary atresia: pathophys
can involve the extrahepatic, intrahepatic, or both biliary trees
198
biliary atresia: dx
liver bx --> periportal fibrosis, bile plugging, eventual cirrhosis
199
biliary atresia: imaging
U/S and cholangiography can reveal atretic biliary tree
200
biliary atresia: progression
get cholangitis, continued cirrhosis, eventual liver failure
201
biliary atresia: surgical tx
Kasai procedure (hepaticoportojejunostomy, resect atretic extrahepatic bile duct segment) ... BEFORE 3mo old, o/w get irreversible liver damage
202
biliary atresia: surgical outcomes
1/3 get better, 1/3 need liver txp, 1/3 die
203
teratoma: labs
inc AFP and b-HCG
204
teratoma: MC site
neonates = sacrococcygeal ... adolescents = ovarian
205
teratoma: tx
excision
206
sacrococcygeal teratomas: malignancy, marker, tx
90% benign at birth (almost all have exophytic component), but HIGH malignant potential ... 2 months is an important transition point: <2mo usually benign, >2mo usually malignant ... AFP = good marker ... tx = coccygectomy and long-term follow up
207
undescended testicles: treatment age
wait until 2yo to treat
208
undescended testicles: inc risk for what
testicular cancer ... inc cancer risk stay the same even after the testicles are brought into the scrotum ... get seminomas
209
undescended testicles: mgmt if b/l
get chromosomal studies
210
undescended testicles: imaging needed
if you cannot feel the testes in the inguinal canal, then get MRI to confirm their presence
211
undescended testicles: tx
orchiopexy through inguinal incision ... if unable to get testicles down --> close and wait 6mo before trying again ... if they will not come down then perform division of spermatic vessels
212
tracheomalacia: describe
elliptical, fragmented tracheal rings instead of C shape
213
tracheomalacia: px
wheezing, usually improves after 1-2 years
214
tracheomalacia: surgical indications
dying spell, failure to wean from vent, recurrent infections
215
tracheomalacia: surgical approach
aortopexy (aorta suture to the back of the sternum, open up trachea)
216
MC cause of airway obstruction in infants
laryngomalacia
217
laryngomalacia: pathophys
immature epiglottis cartilage with intermittent collapse of the epiglottis airway
218
laryngomalacia: sx
intermittent respiratory distress and stridor exacerbation in the supine position
219
laryngomalacia: natural history
most children outgrow this by 12months
220
laryngomalacia: surgical mgmt
surgical tracheostomy for very few patients
221
choanal atresia: describe
obstruction of choanal opening (nasal passage) by either bone or mucus membrane, usually unilateral
222
choanal atresia: sx
intermittent resp distress, poor suckling
223
choanal atresia: tx
surgical correction
224
MC tumor of the pediatric larynx
laryngeal papillomatosis
225
laryngeal papillomatosis: natural hx
frequently involutes after puberty
226
laryngeal papillomatosis: tx
can tx w endoscopic removal or laser, but frequently recurs
227
laryngeal papillomatosis: caused by what
thought to be 2/2 HPV in the mom during passage through the birth canal
228
GI issue assoc with cerebral palsy
GERD
229
dx studies and likely dx with following hx and PE: bilious emesis, abd distention
plain abd film ... dx = intestinal atresia or stenosis
230
dx studies and likely dx with following hx and PE: failure to pass meconium, bilious emesis, acholic meconium, gastric distention, trisomy 21
contrast enema, plain abd film, upper GI contrast study ... dx = duodenal atresia or stenosis
231
dx studies and likely dx with following hx and PE: failure to pass meconium, bilious emesis (late), absent anus or visible fistula, abd distention
plain chest and abd films, U/S of kidneys, sacrum, rectum ... dx = imperforate anus
232
dx studies and likely dx with following hx and PE: high-risk premature infant, bilious emesis, abd distention, hematochezia, guaiac positive stool
plain abd film ... dx = necrotizing enterocolitis
233
dx studies and likely dx with following hx and PE: CF (10%), bilious emesis, acholic meconium, abd distention
plain abd film, contrast enema ... dx = meconium ileus
234
dx studies and likely dx with following hx and PE: bilious emesis, term healthy infant, no abd distention
plain abd film ... dx = malrotation
235
dx studies and likely dx with following hx and PE: delayed passage of meconium, bilious emesis, abd distention, trisomy 21
plain abd film, contrast enema ... dx = Hirschsprung's disease
236
GI anomalies assoc with trisomy 21
Hirschsprung's, duodenal atresia
237
ddx of bilious emesis in infant
intestinal atresia (also w abd distention) ... duodenal atresia (also w failure to pass or acholic mec, gastric distention, trisomy 21) ... imperforate anus (late bilious emesis, also w failure to pass mec) ... NEC (in high risk, premature infant w abd distention and hematochezia) ... mec ileus (assoc w CF) ... malrotation (no abd distention, healthy term infant) ... Hirschprung's (with delayed mec, abd distention, trisomy 21)
238
ddx of failure to pass meconium in infant
duodenal atresia or stenosis, imperforate anus, meconium ileus (10% of CF patients), Hirschsprung's (delayed passage, >24hr)
