PEDIATRIC SURGERY Flashcards
1
Q
Congenital diaphragmatic hernia - what causes it?
A
Failrue of normal closure of pleuroperitoneal canal
Posterolateral - Bochdalek
Anteromedial - Morgagni
2
Q
Majority of Bochdalek hernias are on?
A
Left side (80%) 10 % are bilateral
3
Q
When should you fix CDH?
A
When pulm vascular resistance decreases - usually days to weeks
4
Q
What pentalogy is associated with CDH (morgagni)
A
Pentalogy of cantrell: abdominal wall defect absent pericardium VSD sternal cleft (ectopia cordis)
5
Q
CDH associated with (4)
A
severe pulm HTN
cardiac and neural tube defects
malrotation
6
Q
How are both lungs impacted by CDH?
A
Hernia side is hypoplastic
Contralateral side has pulmonary HTN
7
Q
Tx for CDH
A
high frequency ventilation, inhaled NO, may need ECMO
Need to reduce bowel and repair defect with mesh maybe, look for visceral anomalies by running the bowel
8
Q
pectus carinatum vs excavatum - which one do you NOT need to fix?
A
carinatum (pigeon chest) - can repair for emotional distress
9
Q
Standard initial operation for biliary atresia
A
Kasai (roux en y hepatic portoenterostomy)
excision of entire extrahepatic biliary tree with transection of fibrous portal plate near hilum of the liver
bilioenteric continuity is then resstablished with a roux en Y limb
10
Q
Order of ligation when performing radical nephroureterectomy in Wilm’s tumor?
A
renal artery –> renal vein –> ureter
11
Q
Prognosis of Wilm’s tumor is based on what?
A
Tumor grade (ajnaplastic and sarcomatous variations have worse prognosis)
12
Q
Where do wilms turor metastasize to?
A
Bone and lung
13
Q
Abdominal CT of Wilms tumor shows
A
Replacement of renal parenchyma, NOT displacement
14
Q
Actinomycin and vincristine should be given to all wilms tumor unless?
A
Stage I and <500 g tumor
15
Q
Stage III wilm’s tumor
A
unilateral with regional LN involvement, tumor spillage or previous biopsy without resection
16
Q
Stage V wilm’s tumor
A
bilateral disease
17
Q
Wilm’s tumor with spillage or residual disease - what now
A
XRT
18
Q
What to do if Wilms tumor impinging on vital structures or IVC involvement
A
preoperative chemo
19
Q
What to do with bilateral Wilms tumor
A
conservative approach to save renal parenchyma; biopsy both kidneys followed by chemo and renal sparing partial nephrectomy
20
Q
What is WAGR
A
Wilms + aniridia + GU malformation _ mental retardation
21
Q
VSD that cause sx related to CHF can initially be treated with
A
Digoxin and diuretics
22
Q
How often do VSD close spontaneously
A
About 50%
23
Q
What is Eisenmenger’s syndrome
A
Long standing left to right shunt (usually due to VSD) causes pulmonary HTN and reversal into cyanotic right to left shunt
24
Q
Most common esophageal substitute in kids
A
Colon
25
Goal of ventilatory support in CDH
PaO2 >60 and PaCO2 < 60
26
Pyloric muscle thickness and length in pyloric stenosis
Thickness 3-4 mm
| Length 14-18 mm
27
IVF resuscitation (initial) for HPS
underlying metabolic alkalosis slowly corrected iwth normal saline
K is not given until intravasscular volume restored and normal UOP resumed
28
Reversed rectosigmoid junction
Hirschsprungs
29
MCC death in uncorrected Hirscsprungs
Enterocolitis
30
Waht stain can you use to diagnose hirschsprungs
Calretinin stain for nerve cells
31
Where should you do the rectal biopsy for hischsprungs
At least 2 cm ABOVE dentate
32
Duodenal atresia occurs due to?
Failure of recanalization of proximal small bowel
33
Adrenal mass + bony lesions
Neuroblastoma
34
MC extracranial solid tumor in children
Neuroblastoma
35
When diagnosis of neuroblastoma has been established, what is next step?
Tissue biopsy to determine n-myc amplification status. If amplified will be placed in "High risk" categorywhich impacts ultimate treatment
36
AXR shows stippled tumor calcification
Neuroblastoma
37
Tumor markers for neuroblastoma
VMA and HVA
38
Stage III neuroblastoma
Tumros extend beyond midline, bilateral LN may be involved
39
Stage IV-S neuroblastoma
Would be stage I or II; remote disease confined to liver, subcutaneous tissues and bone marrow (but not cortex) in child < one year
40
Presentation of neuroblastoma
USually asymptomatic but can have HTN, diarrhea, raccoon eyes from orbital mets or unsteady gait
41
Most common location of neuroblastoma
Adrenal
42
CT scan for neuroblastoma will show
renal displacement (vs Wilms which replaces parenchyma)
43
High risk/worse prognosis neuroblastomas
```
NEuron-specific enolase
LDH
HVA
Diplooid tumor
N-myc amplification
```
44
Tx for unresectable neuroblastoma
NEoadjuvant doxorubicin
45
#1 tumor in child <2 yaears
neuroblastoma
46
How much intra abdominal esophagus is needed during lap nissen
2-3 cm
47
Bleeding Meckel diverticulum is best treated with
Diveticulectomy alone
48
When is segmental ileal resection indicated for Meckel diverticulum
Neoplasms
Ischemic or injured bowel
Diverticulum has a wide/broad base
49
At waht age should you refer pt for orchiopexy with undescended testicles
6 months
50
Tx of mesenteric or omental cyst
Simple excision q
51
Ladd's procedure - detorse volvulus in what direction
Counterclockwise
52
Which peritoneal attachments need lysed in Ladds procedure
abnormal peritoneal attachments (Ladd bands) between cecum and abdominal wall
53
What organ is removed during Ladd procedure
appendix
54
Where should the cecum and duodenum be placedi n Ladd procedure
Cecum in LLQ and duodenum in RUQ
55
What causes the respiratory distress inCDH
pulmonary hypoplasia
56
MC abnormalities of GU tract in WAGR
cryptorchidism and hypospadias in males and streak ovaries in females
57
Deletion of what chormosome is responsible for development of WAGR syndrome
short arm of chromosome 11
58
Neuroblastoma is assoc with which neurofibromatosis
Type 1
59
KArtagener syndrome
immotile cilia syndrome/primary ciliary dyskinesia --> recurrent sinusitis
60
Umbilical vessels
two arteries and one vein
61
Wher edoes oxygenated blood from the placenta go ?
into the SINGLE umbilical vein into the IVC
62
How does blood flow from right atrium to left atrium i nfetal circulation
Via foramen ovale
63
Less oxygenated blood returned to placenta via?
two umbilical arteries
64
Blood that is slightly less oxygenated flows from RA to RV and then into the PA. This blood is then largely shunted into ____ via the ductus arteriosus
Descending aorta
65
Describe a V shaped diverticulectomy
Segmenta lresection to include diverticulum as well as ulcer (usually opposite the diverticulum) by performing V shaped diverticulectomy. Then you perform transverse closure of ileum
66
Regardless of stage, all patients iwth hepatoblastoma should receive ..
chemotherapy
67
Tx for children with unresectable or metastatic hepatoblastomas
Biopsy to fonfirm diagnosis followed by chemo to reduce size of tumor and control mets
68
Incision for pyloromyotomy
Longitudinal serosal inciisson on anterosuperior aspect of pylorus begining 1-2 mm proximal t oduodenum and extending on the non hypertrophied antrum
69
What layer of gastric all should be visualized to ensure pyloromyotomy success
submucosa
70
MC presenting symptoms of ASD
dyspnea and SOB (CHF)
71
HEart murmur in ASD
systolic murmur, fixed split S2
72
MC TEF
TEF-EA which is TYpe C, proximal EA, distal TEF
73
Prenatal US shows small or absent gastric bubble plus polyhydraminos
TEF
74
VACTERL
```
Vertebral defects
Anorectal anomalies
Cardiac defects
Esophageal anomalies
REnal defects
Limb anomalies
```
75
CHARGE syndrome
```
Coloboma
Heart defects
ATresia choanae (choanal atresia)
Retardation
Genital hypoplasia
Ear deformities (deafness)
```
76
Which trisomy assoc with TEF
18
77
TEF pre op should have what two studies
```
Echo for cognenital heart defects and ascertain side of aortic arch
Renal ultrasound
ASSOC WITH VACTERL
check sacral US (vertebral anomalies)
rectal exam (imperforate anus)
```
78
Operative repair includes what 4 steps for EA-TEF
Right posterolateral thoracotomy, extrapleural approach
Division of azygous vein
Divison of fistula with primary anastomosis
Place chest tube or closed suction drain near anastomosis
79
If large TEF with severe respiratory distress what is treatment
Fogarty in fistula and emergent surgical divsiion of fistula plus gastrostomy tube
80
Repair of TEF
Repair via cervical approach after bronchoscopic stenting of fistula with asmall catheter
81
Om[halocele occurs due to
Incomplete closure of anterior abdominal wall at umbilicus
82
Persistent omphalomesenteric duct leads to
Meckel diverticulum
83
Deficiency in what vitamin is seen in CDH infants
Vitamin A
84
How to repair mucosal defect in pyloromyotomy
Single layer of absorbable sutures placed transversely at site of perforation with an omental patch. May perform second myootmy at 90-180 degrees if remiemains concern for continued obstructio nfo gastric outlet
85
Which is most effective imaging for biliary atresia
DISIDA (technetium labeled compound diisopropyl iminodiacetic acid)
86
If time allows, all jaundiced infants undergoing hepatobiliary scintigraphy should e pretreated with what before the study
phenobarbital (5 mg/kg/day) for 5 days
87
Most accurate prenatal diagnostic stool for CDH
MRI
88
MCC early death jejunoileal atresia
infection/sepsis
89
normal lateral sacral ratio
>0.7
90
best indicator of shock in peds patient
tachycardia
91
neonate tachycardia HR >
150 BPM
92
tachycardia in age 1-6
>120 BPM
93
tachycardia in age 6-12
>110 BPM
94
targets for crystalloid fluid resuscitation
20 mL/kg
95
targets for blood resuscitation
10 mL/kg (give after 2 fluid boluses in hypotensive)
96
UOP goal in neonate/infant
2-3 mL/kg/hr
97
UOP goal in toddlers and older
1 mL/kg/hr
98
Type A TEF
2nd MC
| only one without a TEF just esophageal atresia
99
Type C TEF on AXR
gas + distended stomach
100
Type A TEF on AXR
gasless abdomen
101
Important in diagnostic workup of TEF
check for VACTERL
102
top 3 etiologies for intussusception
```
#1 inflamed Peyers patches after viral illness
#2 lymphoma
#3 meckels diverticulum
```
103
etiology of malrotation
failure of normal 270 degree rotation during intestinal development
104
what is volvulus due to
Ladds bands (adhesions from right retroperitoneum)
105
#1 on differential in pediatric bilious emesis
malrotation with midgut volvulus
106
dx of malrotation
emergent upper GI (duodenum doesnt cross midline)
107
2 major associations with malrotation
CDH in 20%, omphalocele
108
#1 cause of duodenal obstruction in neonates
duodenal atresia
109
what will be seen on suction rectal biopsy in Hirschsprungs disease
absent galnglion cells in myenteric plexus (due to failure to progress caudad)
110
what do you need to remember to do prior to taking down ostomies after surgery for NEC
barium enema to rule out stenotic distal obstruction
111
etiology of gastroschisis
in utero rupture of umbilical vein
112
gastroschesis is assoc with
intestinal atresia (MC), malrotation
113
which (omphalocele vs gastroschisis) has worse prognosis
omphalocele
114
when to explore contralateral side in inguinal hernia repair
left sided
female
<1 year old
115
noncommunicating hydrocele usually resolve by
1 year
116
when to perform surgery for hydrocele
if not resolved by 1 year old or communicating
117
surgical tx of hydrocele
resect hydrocele ligate processus vaginalis
118
can you divide spermatic vessels during orchiopexy?
yes! due to vas deferns collateral
119
MC location neuroblastoma
adrenal
120
tx of neuroblastoma
resection, can try neoadjuvant doxorubicin if unresectable
121
tx of hepatoblastoma
resection, chemo if unresectable
122
MC indication for pediatric liver transplant
biliary atresia
123
liver biopsy showing periportal fibrosis, bile plug
biliary atresia
124
if <3 months of age with biliary atresia, tx is?
transplant
1/3 improve
1/3 progress to transplant
1/3 die
125
what is pulm sequestration
lung tissue that doesnt communicate with tracheobronchial tree with independent blood supply from aorta
126
extra lobar pulm sequestration - venous drainage is?
systemic
127
intra lobar pulm sequestration - venous drainage is
pulmonary
128
tx of pulm sequestration
ligate arterial supply --> lobectomy
129
what is the diff between congenital cystic adenoid malformation and pulm sequestration
CCAM communicates with tracheobronchial tree
130
congenital lobar emphysema is due to
failure of cartilage development
131
presentation of congenital lobar emphysema
similar to tension PTX BUT DONT PLACE A CHEST TUBE!
132
tx of congenital lobar emphysema
lobectomy
133
what is bronchogenic cyst
extrapulm cysts of bronchial tissue and cartilage, presents as mediastinal cystic mass
134
where do branchial cleft cysts present
mc 2nd branchial cleft cysts which are middle of anterior SCM
135
6 mo old with red lesions growing on face and scalp. Dx and tx?
hemangioma, will resolve by 8 years old otherwise steroids or laser
136
1 mo old with elevated AFP and beta HCG. Diagnosis?
sacrococcygeal teratoma - usually malignant after 2 months old
137
MC anterior mediastinal mass in kid
teratoma
138
MC overall mediastinal mass in kid
neurogenic tumor (posterior mediastinum)
