General Surgery Topic Review Flashcards
(197 cards)
1
Q
most common type of hernia in children?
A
inguinal hernia
2
Q
pathophysiology of inguinal hernias
A
a result of a patent processus vaginalis, lateral to the Hesselbach’s triangle (epigastric vessels, lateral border of rectus sheath, inguinal ligament)
3
Q
risk of incarceration of inguinal hernias
A
highest in infancy, rates of 30-40% in first year of life.
4
Q
risk factors for hernia development
A
LBW
Prematurity
Increased peritoneal fluid
Increased intraabdominal pressure
Ambiguous genitalia
Cystic fibrosis
Connective tissue disorders
5
Q
Classic clinical presentation of inguinal hernias
A
smooth firm mass emerges through external inguinal ring with increased intraabdominal pressure (i.e. coughing)
Silk glove sign - feeling of layers of hernia sac sliding over spermatic cord structures
6
Q
Diagnosis of inguinal hernia
A
clinical with history and physical exam, no imaging
7
Q
timing of repair inguinal hernia
A
All pediatric hernias should be repaired soon after diagnosis.
<1 yo: aim to repair within 1 month following surgical consult
>1 yo: aim to repeat within 3 months following surgical consultation
8
Q
appendicitis perforation rate at presentation
A
20-25% perforated at presentation
9
Q
clinical presentation of acute appendicitis
A
migratory pain: periumbilical to RLQ abdominal pain
fever
anorexia
peritonitis
+/- diarrhea
+/- constipation
nausea
10
Q
first-line investigations for appendicitis
A
CBC + diff
urinalysis
b-hcg
ultrasound abdomen
11
Q
U/s vs CT scan sensitivity and specificity and reasons to use pursue each - in diagnosis of appendicitis
A
Ultrasound—sensitivity 85%, specificity >90%
CT scan—sensitivity 94%, specificity 94%: Consider CT if nonvisualization of the appendix on ultrasound, or when the ultrasound findings are inconclusive.
12
Q
Name two scoring systems to assess for appendicitis and their criterion
A
PAS score - pediatric appendicitis score
Alvarado score
Similar clinical factors (migration of pain, anorexia, nausea, RLQ tenderness, rebound pain) and lab values (fever, leukocytosis, neutrophilia)
13
Q
Management of appendicitis
A
If early appendicitis AND/OR risk factors for perforation -> laparoscopic appendectomy within 24 h of dx. However, non-op management with systemic abx is reasonable for low risk patients.
14
Q
Clinical presentation of tubo-ovarian abscesses
A
severe pelvic and lower abdominal pain, high fever, chills, vaginal discharge (mucopurulent discharge), nausea, vomiting
if ruptures - acute abdomen and sepsis
15
Q
risk factors for tuboovarian abscesses
A
multiple sexual partners
age 15-25
history of PID*
16
Q
investigations in keeping with tubo-ovarian abscesses
A
leukocytosis (can do this with saline microscopy of vaginal fluid)
elevated ESR or CRP
pelvic ultrasound shows complex multilocular masses
17
Q
management of tubo-ovarian abscesses
A
surgical management (laparascopic or image guided percutaneous)
+
antibiotics (Ceftriaxone or metronidazole most commonly) x14d, usually inpatient first dose
oral step down is doxy + metronidazole
18
Q
Pathophysiology of intestinal malrotation
A
incomplete rotation of the intestine during fetal development and involves the intestinal non-rotation or incomplete rotation around the superior mesenteric artery
19
Q
most common type of malrotation
A
failure of cecum to move into RLQ bands of tissue - can obstruct the duodenum
20
Q
other anomalies associated with malrotation
A
Diaphragmatic hernia
Gastroschisis
Omphalocele
Heterotaxy syndrome (heart malformations, malrotation, biliary atresia, and either asplenia or polysplenia)
21
Q
clinical presentation of malrotation
A
- Vomiting- often bilious emesis in 1st week of life, can be non-bilious in infants and newborns.
- episodes of recurrent abdominal pain or vomiting that can mimic colic and suggest intermittent volvulus
- malabsorption or protein-losing enteropathy associated with bacterial overgrowth
- hemodynamic instability
22
Q
malrotation with volvulus pathophysiology?
A
when the small bowel twists around the superior mesenteric artery leading to vascular compromise of the bowel
23
Q
investigations of malrotation with volvulus and gold standard diagnosis
A
AXR: gasless abdomen, dilated intestine suggesting SBO or duodenal obstruction with double bubble sign or NORMAL
Upper GI series is gold standard for diagnosis (duodenum does not cross the midline)
24
Q
Management of malrotation with volvulus?
A
NG to LIS
Aggressive IV fluid resuscitation
IV antibiotics (amp, gentamicin or clindamycin)
Immediate transfer to surgical center
Surgical repair with Ladd’s procedure
25
What is Meckel's diverticulum?
most common congenital anomaly of the GI tract, caused by the incomplete obliteration of the omphalomesenteric duct (connects the yolk sac to the gut in a developing embryo, provides nutrition until placenta is established) resulting in gastric mucosa in the bowel
26
Rule of 2's Meckel's Diverticulum
2% of population
2:1 F:M
2-6% symptomatic, complicated
50-75% symptomatic by age 2y
Within 2 feet of ileocecal valve
2 inches long
2 types of mucosa: gastric, pancreatic
27
Clinical presentation Meckel's Diverticulum
Painless rectal bleeding
Anemia
Well appearing
Hypovolemia
28
What is the diagnostic testing for Meckel's diverticulum
nuclear medicine scan - "Meckel's scan:
Tc scan detects gastric mucosa
The mucus-secreting cells of the ectopic gastric mucosa take up the radionuclide, permitting visualization
29
Management of meckel's diverticulum
resucitation pending degree of blood loss and hypovolemia
type and screen
Surgical resection
30
Umbilical hernia pathophysiology
Incomplete closure of peritoneal and fascial layers within umbilicus resulting in soft swelling covered by skin that protrudes during crying, coughing or straining and is easily reduced. It can contain omentum and portions of small intestine
31
Risk factors for umbilical hernia
African ethnicity
Prematurity and Low birthweight **
Ascites
Childhood obesity
Hypothyroidism
Mucopolysaccharidoses
Peritoneal dialysis
Trisomy 13,18,21
Syndromes (Beckwidth Wiedemann, EDS, Marfans)
32
Management of Umbilical hernia and reasons to consider surgery
Conservative - 90% close by age 2
Consider surgery if :
- Persistent facial defect past age 5yrs
- Large trunk like protrusion with fascial defect that has not closed or improved by age 2yrs
- Symptomatic (skin erosion over defect, becomes incarcerated or strangulated (rare), hernia rupture)
- Bowel injury or perforation
- Ascites
- Peritoneal dialysis
- Metabolic disorders (Hypothyroidism, Mucopolysaccharidoses)
- Genetic Syndromes (T13, T18, T21, BWS, EDS, Marfans)
33
name g-tube site complication and management - see photo
granuloma/granulation tissue
silver nitrate sticks q2-3 days
34
name g-tube site complication and management - see photo
fungal (candida) infection
satellite lesions spreading away from g-tube
nystatin, keep area dry
35
name g-tube site complication and management - see photo
Cellulitis/Bacterial infection (staph or b-hemolytic strep)
Spreading erythema from g-tube site, warmth, swelling, purulent drainage, foul odor, significant pain +/- systemic s/s
Systemic antibiotics (Keflex)
Change G-tube
36
pathophysiology of thyroglossal duct cyst
Abnormal descent of thyroid gland, goes through hyoid bone
37
presentation of thyroglossal duct cyst
midline neck mass - often moves with patient swallowing and tongue protrusion
rarely obstructive symptoms
often appears after URTI
38
branchial cleft cyst presentation
lateral neck mass in an infant dueto embryologic malformation
usually travels along the SCM
39
cystic hygroma presentation
lymphatic malformation
- Lateral neck mass in posterior triangle (behind SCM)
- Soft, cystic, loculated
- Recurrent infection
often seen in Turner Syndrome
40
Gastroschisis most common presentation
- abdominal wall defect to the RIGHT of the umbilicus
- usually less than 4cm in diameter
- 25% associated with atresias and volvulus
- abdominal contents not
41
risk factors for gastroschisis
advanced maternal age
IUGR
maternal serum alpha-fetoprotein is elevated in all pregnancies
42
initial management for gastroschisis (and omphalocele)
- NG/OG to LIS
- cover viscera with warm sterile saline-soaked gauze
- support bowel with baby on side to prevent strangulation of bowel
-fluid resuscitation
-prophylactic antibiotics (amp/gent)
- avoid hypothermia
- look for associated defects
- surgical reduction
43
management of rectal abscesses (surgical)
- surgical drainage - even with MRSA recommended
- send culture abscess to look for MRSA
- monitor for systemic and local symptoms or no improvement after 48h
44
age of children admission for IV antibiotics for rectal abscesses plus drainage
admit to hospital for IV antibiotics if <1 month old
45
antibiotic recommendations for children with rectal abscesses per age for WELL child
<1 month - IV vancomycin, consider oral clindamycin if well
1-3 months - TMP/SMX
>3 months - ONLY if child does not improve or grows organism other than staph aureus or systemically unwell
TMP-SMX and cephalexin
46
MRSA absesses common locations in kids
buttocks and lower limbs
47
48
What is the most common congenital anomaly of the GI tract?
Meckel diverticulum
49
Meckel diverticulum is caused by what?
Incomplete obliteration of the omphalomesenteric duct (which connects the yolk sac to the gut in a developing embryo and provides nutrition until the placenta is established, but usually separates from the intestine between the 5-7th week of gestation and involutes)
50
What is the rule of 2s for Meckel diverticulum
2% of population
2:1 F:M
50-75% present by age 2 years old
Within 2 feet of ileocecal valve
2 inches long
2 types of mucosa: diverticulum lined with gastric / pancreatic mucosa
51
What is the presentation of symptomatic Meckel diverticulum?
Painless, episodic LGIB
Can be massive with drop in Hgb
Stool is brick or current jelly coloured - frank blood
52
What is the test of choice to diagnose Meckel's diverticulum?
T-99 Meckel scan
Detects gastric mucosa
53
Treatment of Meckel's diverticulum?
Resuscitate / type and screen / PRBCs if massive bleed
Resection of the diverticulum
54
What is Hirschsprung disease?
Congenital aganglionic megacolon is a developmental disorder of the enteric nervous system characterized by the absence of ganglion cells in the submucosal and myenteric plexus --> aganglionic segment of colon fails to relax causes functional obstruction
55
Embryology defect relevant to Hirschsprung disease?
Defect in neural crest cell migration during fetal development between 5-12 weeks gestation
These cells typically migrate craniocaudally to populate the entire length of the GI tract by week 7
HD results from failure of these cells to complete their migration, leaving the distal end of the gut aganglionic and consequently functionally obstructed
The length of aganglionic bowel depends on the timing of the interruption; the earlier it happens the longer the affected segment
56
Congenital syndromes associated with Hirschsprung disease?
T21 - probably most important one to know
Others:
Joubert syndrome
Goldberg-Shprintzen syndrome
Smith-Lemli-Opitz syndrome
Cartilage-hair hypoplasia
Multiple endocrine neoplasm 2 syndrome
Neurofibromatosis
Neuroblastoma
congenital hypoventilation syndrome
Congenital anomalies: microcephaly, developmental delay, abnormal facies, autism, cleft palate, hydrocephalus, micrognathia
57
What is a significant complication of Hirschsprung disease that presents with bacterial translocation and impaired immune function?
Hirschsprung related enterocolitis is a major cause of morbidity and mortality - results from aganglionosis and obstruction resulting in bacterial translocation, reduced IgA secretion/ impaired immune function, and WBC dysfunction which predisposes to infection
Risk factors:
- delayed diagnosis > 1 week
- Trisomy 21
- Remains a risk even after surgery
Presentation:
- presents with fever, abdominal pain, explosive diarrhea, poor perfusion, lethargy
Treatment:
- rectal irrigation, fluid resuscitation and systemic antibiotics
Major cause of morbidity and mortality
58
What are the 4 types of Hirschsprung segments that can be affected and what is the most common
Ultrashort segment (internal anal sphincter achalasia): < 3 cm distal rectum affected
Short Segment (80%): affects the large bowel distal to the splenic flexure - most common with 80% of cases
Long Segment: affects the large bowel beginning with or proximal to the splenic flexure
Total colonic aganglionosis (TCA) (5%): extends into the small bowel
59
Differential diagnosis for failure to pass meconium within first 48 hours of life?
CF (meconium ileus)
Hirshsprung disease
Imperforate anus
Congenital colonic atresia
Malrotation +/- volvulus
Spinal cord abnormalities
Congenital hypothyroidism
Need good history and physical exam
Investigations - abdo xray, contrast enema, sweat chloride, thyroid function, rectal biopsy etc
60
Gold standard investigation to diagnose Hirshsprung disease
Rectal suction biopsy (collected at 2-, 3- and 4- cm above the anal verge - ensures that biopsies are taken above the dentate line and avoid obtaining biopsies in the normal area of hypoganglionosis) is the gold standard for diagnosis
Will show:
- No ganglion cells
- Hypertrophic nerves (hematoxylin and eosin stain), nerve fiber trunk measurement > 40 um
- Increased acetylcholinesterase staining
61
Features of Hirschsprung's disease on contrast enema?
- Reversal of the rectosigmoid ratio (normally the diameter of the rectum is equal to the diameter of the sigmoid colon, in HD the ratio is < 1)
- presence of a transition zone
- saw-tooth or spiculated appearance to the rectal mucosa
62
Role of contrast enema and/or anorectal manometry in detecting Hirshsprung's?
Anorectal manometry --> can be used in children with severe constipation, if "rectoanal inhibitory reflex" is normal then this rules out Hirschsprungs and can avoid a rectal biopsy.
Nelson's recommends this over a contrast enema
Contrast enema --> also useful screening test when clinical suspicion for HS
AAP recommends contrast enema over manometry
Neither is considered "gold standard" but can be useful in screening for HS in patients with significant constipation
63
64
Treatment for Hirschsprung's disease?
Surgical management
Remove aganglionic segment and anastomose the proximal, normally ganglionated bowel to the anal canal
Short segment aganglionosis (most common) --> single stage transanal procedure
either "Swenson" or "Soave"
65
Complications of Hirschsprung's disease?
- Mechanical obstruction (strictures at the anastomosis post surgery)
- Internal anal sphincter dysfunction
- Colonic dysmotility (even when ganglion cells appear to be normal, motility may not be normal even after surgery)
- Functional constipation --> very common after history of anal surgery and repeated anorectal manipulation
Major complication:
- Hirschsprung associated Enterocolitis
- presents with fever, lethargy, abdominal pain, explosive diarrhea
- remains a risk even after surgery
66
Which patients are at highest risk for developing Hirschsprung's related enterocolitis?
Trisomy 21
Long segement aganglionosis
History of post-operative obstruction
Previous enterocolitis
67
Gastroschisis vs Omphalocele: name 5+ differences between the two
Gastroschisis
- patients frequently born preterm
- abdominal wall contents not covered by membrane
- defect almost always to the right of the umbilical cord
- contents are usually just bowel, occasionally bladder, ovaries, testes
- Intestinal function is usually harder to recover - may have prolonged ileus, may have short-gut if significant segments of bowel need to be removed
- associated defects most commonly intestinal atresia (up to 25%)
- defect usually small - 2-3cm
- no genetic or specific testing required after diagnosis
Omphalocele
- infants typically born at term
- abdominal wall contents covered by membrane / peritoneum
- defect is at base of umbilical cord, cord is included in defect
- contents are usually bowel +/- liver
- Intestinal function usually preserved, much less likely to have short-gut following surgery
- much more likely to be associated with genetic syndromes eg B-W, TOF, T21
- defect can be large, up to 15cm
- requires echocardiogram to look for cardiac abnormalities, consider genetic testing
68
What is the Pentalogy of Cantrell?
Ompahlocele
Anterior diaphragmatic hernia
sternal cleft
Ectopia cordis
Intracardiac anomalies (VSD, ASD, TOF)
Associated with T18, T13
69
Which syndromes have the highest association with omphalocele?
Beckwith-Wiedemann syndrome (omphalocele, macrosomia, hypoglycemia) occurs in 12%
Trisomy 13, 18 and 21
70
What complications can be associated with a giant omphalocele?
Pulmonary hypoplasia
Underdeveloped abdominal wall cavity
71
What intra-abdominal pressures indicate abdominal compartment syndrome (can be seen after surgery for omphalocele or gastroschisis)
Higher than 15-20 mmHg
72
What test is typically elevatd in maternal serum and amniotic fluid in patients with gastroschisis?
Alpha fetoprotein
73
Management of gastroschisis/ omphalocele?
Avoid clamping the sac when the umbilical cord is clamped
Insert NG/OG to low continuous suction
Cover viscera with warm sterile saline-soaked gauze and cellophane wrap or plastic bag
Support bowel, with baby on its side with the bowel supported by towels, to prevent angulation of bowel and its mesentery with consequent bowel ischemia
Fluid resuscitation with 20ml/kg NS
Prophylactic antibiotics (amp/gent)
Avoid hypothermia (use overhead warmer)
Look for associated defects, especially in omphalocele; consider genetics consult
Keep viscera moist and protected until surgical reduction with 1o abdominal closure or staged closure with silo
Goal of surgery is to replace bowel into the abdominal cavity avoiding increased intra-abdominal pressure.
Prompt recovery of bowel function as soon as possible.
74
What age group is most common for testicular torsion?
> 12 years
Very uncommon before age 10 but ALWAYS consider on differential as always possible
75
What is a bell clapper deformity?
Inadequate fixation of the testis within the scrotum
Results from a reduntant tunica vaginalis
Allows mobility of the testis
76
Testis survival in torsion depends on what factors
Duration (4-6 hours is the time cut-off for irreversible loss of spermatogenesis)
Severity of torsion
77
Presentation of testicular torsion?
Acute constant pain (unless intermittent torsion)
Swelling of the scrotum
Testis is high riding
Cremasteric reflex is absent
Testis may be twisted (eg epididymis may be on anterior side)
May have N/V
78
Mangement of testicular torsion?
Immediate surgery with Urology / Gen Sx
Do not delay management for an ultrasound
If the testis is explored within 6h or torsion, up to 90% of gonads survive
79
What is the most common type of congenital malrotation?
Failure of the cecum to move to the RLQ
80
What is the mechanism / pathophysiology of Torsion of Testicular Appendage?
The appendix testis is a stalk-like structure that is a vestigial embryonic remnant of the mullerian ductal system that is attached to the upper pole of the testis.
Testicular appendage can undergo torsion --> progressive inflammation and swelling of the testis occurs --> gradual onset testicular pain and scrotal erythema
81
What does the intestine usually rotate around during fetal development (with normal rotation)?
the Superior Mesenteric Artery
82
What age group does Torsion of Testicular Appendage occur in?
Occurs in prepubertal males (age 7-10yrs). Rare in adolescents.
83
What anomalies can be associated with congenital malrotation?
Diaphragmatic hernia
Gastroschisis
Omphalocele
Heterotaxy syndrome (heart malformations, malrotation, biliary atresia, aplenia/polysplenia)
84
Presentation of Torsion of Testicular Appendage?
HINT:
- Symptoms ***
- Signs ***
Symptoms:
- Sudden onset PAINFUL, SWOLLEN testicle
Signs:
- NORMAL testicular lie
- Focal tenderness to upper pole of testicle
- 3-5mm tender indurated mass on upper pole
- BLUE DOT sign
- Cremasteric reflex present
Generally self resolving within 3-10days
85
Presentation of malrotation? Age groups, classic symptoms, physical exam
Age: 30% present by 1 month, 58% by 1 year, and 75% <5 years
Presents with vomiting, often bilious
Abdo pain, may have peritonitis
If GI bleeding --> late / ominous sign
86
What investigations should you pursue for concerns of Torsion of Testicular Appendage?
- US doppler of testicle (normal testicular bloodflow and some hyperemia of upper pole of testis)
87
What is volvulus
Volvulus: when the small bowel twists around the superior mesenteric artery leading to vascular compromise of the bowel (think about it in new small bowel obstruction with no hx of surgery)
Volvulus is a life-threatening complication of malrotation—resembles an acute abdomen or sepsis
88
What is the management for Torsion of Testicular Appendage
- Bedrest x24hr
- NSAIDs x5 days
+/- surgery (only indicated if the diagnosis is uncertain (aka concerns for testicular torsion) or prolonged not self-resolving symptoms
Generally self resolving within 3-10days
89
Gold standard for diagnosis of malrotation?
UGI series
- NORMAL should show complete C-loop of duodenum, DJ junction at the level of the pylorus, proximal jejunum to the left side of the left vertebral pedicle, no duodenal dilation
In malrotation, will show
- DJ junction FAILS to cross the midline (normally should be to the left of the left-sided vertebral body pedicle)
- DJ junction lies inferior to the duodenal bulb (normally should be at the level of the pylorus)
- D2 and D3 segments of the duodenum not located posteriorly in a retroperitoneal position
In volvulus, may see "corkscrew" filling pattern
90
Which surgical procedure is used in malrotation
"Ladd's procedure"
- lyse Ladd bands, widen mesentery, remove appendix
- is done laparoscopically
If voluvlus, often need open procedure
91
Diagnosis of hydrocele?
Clinical:
- fluid around/ adjacent to testis in the tunica vaginalis in the scrotum
- non painful, asymptomatic
- typically in infants (suspect testis tumour if you see this in an adolescent)
- positive transillumination
- either communicating or non-communicating
92
Differences in communicating vs non-communicating hydrocele and differences in management
Communicating:
- intermittent hydrocele because processus vaginalis is still open
- risk for indirect hernia
- should be referred for surgery right away
Non-communicating:
- fixed hydrocele
- processus vaginalis is obliterated
- typically resolves by 12-18 months
- if not resolved by 12-18 months, refer for surgery as might be a communicating hydrocele
93
Role of US in hydrocele
To confirm presence of scrotal testis if not palpable
To rule out inguinal hernia (especially if compression of the fluid-filled mass completely reduces the hydrocele, more likely an inguinal hernia associated with hydrocele)
To rule out testis tumor (if adolescent)
When associated with scrotal pain NYD
94
Management of hydrocele?
Most resolve by 12-18 months
If communicating, refer for surgery right away because risk of hernia
If non-communicating, wait for likely spontaneous resolution - refer for surgery if not resolved at 12-18 months
Other reasons to refer for surgery:
- hydrocele is large and tense
- hydrocele extends into abdominal cavity (eg abdominoscrotal hydrocele)
95
Name the anomaly:
Distal intestinal obstruction in a neonate, failure to pass meconium, excluded Hirschsprung's disease, mom has history of gestational diabetes mellitus
Small left colon syndrome
96
What workup is needed for VACTERL syndrome:
Vertebral:
- dedicated spine xrays including sacral views
- US for tethered cord
- consider MRI
Anorectal malformations:
- complete physical exam
- consider pelvic Xray
- abdo US to look for accompanying GU anomalies
Cardiac
- Echo
- ECG
TEF
- CXR with OG/NG in situ
Renal
- Renal US
- may need VCUG
Limb
- detailed physical exam
- xray of limb if suspected anomaly
97
What is the immediate management of suspected NEC:
Make baby NPO
OG/NG for decompression
Obtain central access
TPN
IV antibiotics --> cover for gram neg, gram pos, and anaerobic pathogens. Eg Amp, CTX, and Flagyl or Amp, Gent and Flagyl
Address electrolyte abnormalities and metabolic acidosis
50% require surgical management
98
What are common findings in VACTERL association?
Vertebral: sacral agenesis/dysgenesis, rib anomalies, butterfly vertebrae, vertebral fusions, tethered spinal cord, scoliosis
ARMs:
- imperforate anus
- anal atresia
- perineal fistulas
- accompanying GU anomalies: hypospadias, cryptorchidism, cloacal malformations etc
Cardiac:
- situs anomalies
- congenital heart defects
- vascular anomalies: right sided aortic arch, anomalous SVC
- arrhythmias
Tracheo-Esophageal
- TEF
- Esophageal atresia
Renal
- renal agenesis
- cystic and/or dysplastic kidneys
- renal fusions (eg horseshoe)
- ureteral anomalies
Limb
- radial anomalies
- thumb anomalies
- polydactyly
- digit or limb hypoplasia
Other findings:
- can have hydrocephalus
- can have other GI abnormalities
99
Relationship of pulmonary hypoplasia and CDH
Previously thought that lung hypoplasia was solely caused by the compression of the lung from the herniated abdominal contents which impaired lung growth
Now emerging evidence shows that pulmonary hypoplasia often preceeds the diaphragmatic defect
Pulmonary hypoplasia and malrotation of the intestine are considered part of the lesion, not associated anomalies
100
What is the embryology of the diaphragm and how does this relate to development of CDH?
Diaphragm is derived from 4 different sites:
Septum transversum (between primitive heart and liver)
Pleuroperitoneal folds
Body wall --> peripheral parts of diaphragm
Dorsal mesentery of esophagus
Diaphragm descends during development and becomes innervated on week 4 of gestation by the phrenic nerve
C 3, 4, 5
CDH
- failure of the pleuroperitoneal membrane to develop which causes abdominal contents to herniate into the pleural cavity
- most common on left side
101
Why do <5 year olds tend to present with more complicated rather than simple appendicitis?
More likely to have difficulty describing the location of their pain --> more likely to have delayed diagnosis
Also, appendix is less likely to be fixed to the mesentery < age 5 which means it has greater mobility which increases the liklihood of it rupturing
102
Name specific risk factors for thyroid cancer in children which warrant annual physical exam screening:
History of irradiation to head/neck region
Iodine deficiency
Genetic cancer predisposition (many, including MEN type 2)
Autoimmune thyroiditis
103
Best screening for patients with risk factors for thyroid malignancy?
Physical exam
No role for US screening for asymptomatic patients
104
Approach to evaluation of thyroid nodules?
Nodule of any size → US is recommended and TSH should be measured
Most children with thyroid nodules have normal thyroid function
if low TSH → raises suspicion for thyroid hormone overproduction by autonomous nodule or toxic adenoma → nuclear scintigraphy should be performed
Most autonomous nodules are benign but should still be removed
If any concern for malignancy on US → need FNA biopsy
105
Indications for FNA biopsy of thyroid?
FNA indicated for:
- solid or partially cystic nodules equal to or greater than 1cm on imaging
- nodules that are enlarging on repeat imaging
- nodules of any size with concerning features such as hypoechogenicity, irregular margins, increased intranodular blood flow, microcalcifications, and/or surrounding cervical lymphadenopathy
- nodules of any size in patients with risk factors for thyroid malignancy (see above)
106
Next steps of management based on results of FNA biopsy of thyroid?
Inconclusive / nondiagnostic = repeat FNA
Benign nodules → monitor with repeat thyroid US in 6-12 months, subsequent US every 1-2 years if stable. Repeat FNA if nodules develop concerning features
Intermediate results → directed by experienced team, FNA vs surgical resection
Suspicious for malignancy → referral to Endo, Oncology, multi-D team
107
List 3 types of benign thyroid nodules and 3 malignant
Benign
- Benign adenomatous or colloid nodules
- Follicular adenoma
- Hyperfunctioning adenoma
Malignant:
- Papillary thyroid cancer (most common)
- Follicular thyroid cancer
- Medullary thyroid cancer with ret mutations
108
Name the syndrome: Autosomal dominant syndrome characterized by multiple hamartomatous polyps in the gastrointestinal tract, mucocutaneous pigmentation (gray/brown spots on the lips, palms of the hands, buccal mucosa, soles of the feet), and an increased risk of gastrointestinal and nongastrointestinal cancer.
Peutz-Jeghers syndrome
109
Most likely source of GI bleed?
An otherwise healthy 8 year old girl has had painless blood per rectum, coating her stool, for the past several weeks. Stooling habits are otherwise unchanged, she has not lost weight, she is not anemic.
Juvenile polyp — most common cause of painless rectal bleeding in children
- most likely because it coats the stool in this stem, not dripping on the stool after defecation
110
Most likely source of GI bleed?
A 3 year old boy is being potty trained. He has a painful stool, associated with a streak of bright red blood on his stool, and dripping into the toilet bowl.
Anal fissure
- painful - caused by passage of hard stools, tearing of the anal mucosa
- small amount of bright red blood on the stool or toilet paper, not mixed throughout the stool
111
Most likely source of GI bleed?
A 36 hour old term newborn boy is passing dark bloody stools. He is otherwise feeding well, and examines well.
Swallowed maternal blood from delivery or cracked nipples during breastfeeding
Presents within the first couple of days of life
Blood may appear dark or tarry (mimicking melena) or reddish in stool
Baby is otherwise well: feeding, vital signs, and physical exam all normal
112
Name the source of bleeding:
A 4 year old boy has had several episodes of large volume bright red blood per rectum, not associated with stools, nor pain, over the last 24 hours. He is pale and tachycardic.
Meckels diverticulum
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Name the source of bleeding:
A 3 week old, ex 29weeks, baby boy was previously doing well, tolerating enteral feeds. Last night, he had some brady spells, increased gastric residuals, abdomen became somewhat distended, and this morning he has had bloody stools x 2.
NEC
114
Name the source of bleeding:
After uncomplicated newborn admission, a 5 day old term baby girl is presented to ER with 24 hours of bilious vomiting, and then blood per rectum.
Midgut malrotation with volvulus
115
Name the source of bleeding:
A 4 year old boy has had multiple short episodes of severe abdominal pain over the past 48 hours. Initially, he was well between the episodes, but he is getting increasingly lethargic. He passed a jelly-like red stool.
Intussusception
116
Name the source of bleeding:
A 15 year old girl has intermittently had loose stools over the past 6 months, sometimes with dark blood. She has secondary amenorrhea. She is anemic.
IBD
117
What are the different types of TEF and which is the most common?
Type A: EA without TEF
Type B: EA with proximal TEF
Type C: EA with distal TEF (most common, 85%)
Type D: EA with proximal AND distal TEF
Type E: H-type TEF with no EA (most likely to get missed at birth if small defect)
118
Clinical presentation of TEF?
Excessive secretions, drooling, choking, coughing, inability to feed
119
Diagnosis of TEF?
EA can be diagnosed by attempt to pass a catheter into the stomach
OG/NG cannot pass below 10-15 cm
Confirm with AP CXR showing catheter curled in upper esophageal pouch
120
Xray findings of TEF?
OG/NG curled in upper esophageal pouch (assuming there is EA which is present in most types of TEF)
Distal TEF: CXR will show gas-filled GI tract
Proximal TEF: may have aspiration pneumonia,
121
Role of contrast UGI in diagosing TEF?
Consider if CXR (with OG/NG in situ) not conclusive but do NOT USE barium --> must use water soluble contrast as risk of aspirating into lungs if proximal TEF and could cause pneumonitis
122
Most common congenital anomalies with TEF?
VACTERL or CHARGE
Congenital heart disease is the most common associated anomaly, followed by GU abnormalities
T21, 18, 13
123
Risk factors for TEF
Advanced maternal age
European ethnicity
Obesity
Low socioeconomic status
Smoking
124
Anatomy of esophagus?
The muscularis propria of the upper 1/3 of the esophagus is predominantly striated
- Affected by cricopharyngeal dysfunction, cerebral palsy
The lower 2/3 of the esophagus is smooth muscle
- Affected by clinical conditions involving smooth muscle (achalasia, reflux esophagitis)
125
Management of TEF?
NPO, maintenance IVF
Keep head up at 45 degrees and pass Replogle NG tube (10 Fr) via nose or mouth to keep upper pouch empty with continuous low suction
Consider IV antibiotics if aspiration pneumonia is present
Avoid intubation if possible because it can worsen abdomen distention
Surgical ligation of the TEF and primary end-to-end anastomosis of the esophagus via right-sided thoracotomy is standard approach
126
Post-op complications of TEF
Infections
Chylothorax
Vocal cord dysfunction
Tracheomalacia
Feeding & growth concerns
Reflux
Esophageal stricture
Recurrent TEF - up to 10% of cases
Anastomotic disruption (leak)
Tracheomalacia
127
Differential diagnosis for direct / conjugated hyperbilirubinemia in an infant?
Hepatocellular
- Neonatal hepatitis (CMV, HSV, rubella, idiopathic)
- Metabolic disorders (Galactosemia, Tyrosinemia, Alpha 1 antitrypsin, Niemann Pick disease, Gaucher disease)
- Hemochromatosis
Obstructive / Cholestatic
- Biliary atresia
- Choledochal cyst
- CF
- Alagille syndrome
Infectious causes
- TORCH
- sepsis
- UTIs
Genetic
- Progressive familial intrahepatic cholestasis
- Dubin-Johnson
- Rotor syndrome
Iatrogenic/ Other
- TPN associated
- Drugs/toxins (AEDs in mom)
- Congenital hypothyroidism
- Panhyopituitarism
128
What is the most common indication for liver transplant in children
Biliary atresia
129
How does biliary atresia present (including bloodwork)
Progressive jaundice that may appear from birth up to 8 weeks of age
Acholic stools
Dark urine
May have hepato/splenomegaly
Conjugated hyperbilirubinemia
Mild to moderate increase in serum aminotransferases, with disproportionately increased GGT/ ALP
130
Liver biopsy findings suggestive of biliary atresia:
Inflammation, portal tract fibrosis, cholestasis, bile duct proliferation
131
Findings on US suggestive of biliary atresia:
Absence of common bile duct
Enlarged hepatic hilar lymph node
Absent or shrunken gallbladder
“Triangular cord sign”: a triangular echogenic density seen just above the porta hepatis
Cirrhotic liver
132
Gold standard diagnosis of BA?
Intraoperative cholangiogram in the OR
133
What time frame is ideal for the Kasai procedure?
Ideally before 8 weeks (60 days) of age = better outcomes
134
How is the Kasai procedure (for BA) done?
In the Kasai, the bile ducts are excised and a limb of jejunum is anastomosed to the liver. The distal duodenum is anastomosed to the jejunal limb to create a Roux-en-Y configuration
135
What is the most common type of choledochal cyst?
Type 1 most common --> Fusiform dilation of the CBD
136
Classic triad of choledochal cyst?
Jaundice
Palpable RUQ mass
Abdominal pain
Seen in < 50%
137
Epididymitis etiology in an adolescent
sexually transmitted infection
138
Epididymitis etiology in an adolescent
E. coli
139
Management of Epididymitis
symptomatic management with analgesia, can consider antibiotics is bacterial etiology suspected
140
Diagnostic investigation for epididymitis
Doppler US - increased perfusion to the scrotum seen with inflammatory changes
141
Indications for a g-tube (6)
1. Neurologically impaired
2. Poor oral intake and weight gain despite optimizing calories
3. Recurrent aspiration or prolonged feeding times
4. GERD, if it leads to insufficient oral intake
5. Dysmotility despite medical treatment
6. Expected prolonged need for NG feeds (more than 3-6 months)
142
Short term risks of G-tube feeding (6)
1. Peritonitis
2. Bleeding
3. Infection
4. Anesthesia related problem
5. Abdominal organ puncture
6. Perioperative death
143
Longer term risks of G-tube feeding
1. Malfunction (blockage, dislodgement, breakage)
2. Issue with stoma (infection, bleeding, irritation)
3. Worsening GERD
144
What is diaphragm eventration?
abnormal elevation of the diaphragm due to weakness or paralysis of the muscle that can lead to respiratory distress and compromised lung function
145
Syndromes associated with unilateral diaphragmatic eventration
Beckwith-Wiedemann Syndrome and Trisomy, 13, 14, 15, 18
146
Syndromes associated with bilateral diaphragmatic eventration
toxoplasmosis, CMV, arthrogryposis, Werdnig Hoffman disease
147
Cause of acquired diaphragmatic eventration
phrenic nerve injury from birth trauma, chest surgery or rare tumors of the chest
148
Diagnostic testing for diaphragmatic eventration
Chest US or Fluoroscopy
149
Definitive management for diaphragmatic eventration
Surgical Repair however most eventrations are asymptomatic and do not require repair.
150
Indications for surgical repair diaphragmatic eventration (3)
1. Continued need for mechanical ventilation
2. recurrent infections
3. failure to thrive
151
What is a Diaphragmatic hernia
communication between the abdominal and thoracic cavities with or without abdominal contents in the thorax
152
Type of diaphragmatic hernia
1. Esophageal hiatus (hiatal)
2. Paraesophageal (adjacent to the hiatus)
3. Retrosternal (Morgagni) 2-6%
4. Posterolateral (Bochdalek) portion of the diaphragm - usually what’s referred to by CDH
153
What side is it more common to have a diaphragmatic hernia
85% of the time defect in on the left side
154
Anomalies associated with diaphragmatic hernia (3)
1. Esophhageal Atresia
2. Omphalocele
3. Neural Tube Defects
155
Syndromes associated with diaphragmatic hernia
Trisomy 21, 13, & 18; Pallister-Killian and Turner syndrom
156
Diagnostic test for diaphragmatic hernia
chest x-ray
157
Management of diaphragmatic hernia
A: intubation and sedations (avoid bagging)
B: target pre ductal saturations > 85%, low PIP, permissive hypercapnia, iNO for pulmonary vasodilator to reduce ductal shunting and pulmonary pressure
C: can consider ECMO
158
Midline neck mass that appears after URTI?
Thyroglossal duct cyst
Presents in children or adolescents (or young adults)
Remnant of the thyroglossal duct tract, related to abnormal descent of the thyroid gland
Usually within 2cm of the midline (can be lateral but less common) at the level of the thyrohyoid membrane
Presents as mobile, soft, usually painless neck mass that appears to be cystic. Usually move up with swallowing or protrusion of the tongue. Easily seen with the neck in extension.
Overall, presence of a mass and infection are the two main presenting symptoms of TGDC
Dx
- US
- Confirm presence or absence of normal thyroid tissues outside of the TGDC
- Often MRI is indicated
- Largely benign, 1% develop malignancy
159
Differential for congenital neck mass?
Thyroglossal duct cyst (most common - up to 70%)
Branchial cleft anomalies (2nd most common)
Vascular anomalies
Benign neoplastic masses
Cervical teratomas
Dermoid cysts
Thymic cysts
Lymphatic malformations
Goiter
160
Distinguish between thyroglossal duct cyst and dermoid/ epidermoid cysts?
Both are similar in quality and location, characteristically well circumscribed, nontender and midline
Dermoid/Epidermoid cysts do NOT elevate with tongue protrusion or swallowing, as occurs with TGDCs
161
What should be considered if patient presents with recurrent otorrhea WITHOUT evidence of middle ear disease or inflammation
Branchial cleft cyst
162
Presentation of branchial cleft cyst
Typically lateral
Anterior to SCM
Unilateral, palpable soft mass
Some can present with throat soreness, dysphagia, abscesses or an enlarging mass in the setting of infection
Can have a fistula present, may have chronic drainage from an opening along the anterior border of the SCM with recurrent infections
Related to incomplete obliteration of clefts or pouches
Cysts --> more common in older children
Fistulas --> more common in infants
163
Name two types of lymphatic malformations
Cystic hygromas
Lymphangiomas
164
Presentation of lymphatic malformation
Most patients are asymptomatic
Lesions may present as painless, soft, and compressible without skin discoloration unless there is an underlying hemorrhage
There may be skin dimples from dermal involvement
The oral cavity and airway are often involved --> may have symptoms related to mass effect
165
Most common cause of a GIANT neck mass at birth
Teratoma
Contains all 3 embryonic layers: ectoderm, mesoderm and endoderm
166
What is the most common type of anorectal malformation in a girl? in a boy?
Girl = rectovestibular fistula
- The rectum opens into the vestibule (between the vagina and urethra)
Boy = rectourethral fistula
167
What x-ray finding is associated with duodenal atresia?
Double bubble
168
What associated anomalies or syndromes are specifically associated with duodenal atresia
1/3 of children with duodenal atresia have T21
1/3 also have congenital heart disease
1/3 have malrotation
1/3 have annular pancreas
Also associated with VACTERL
50% have chromosomal abnormalities
169
Presentation of duodenal atresia?
Usually presents within 24 hours of birth
Usually baby is somewhat preterm
Bilious vomiting
Abdominal fullness
170
Best diagnostic test for duodenal atresia?
Can be diagnosed with Xray (double bubble with no air distally)
No other further imaging needed for operative purposes
However if ANY possibility of malrotation (eg dilated bowel loops) need UGI to rule out associated malrotation
171
Imaging for jejunal atresia?
Since Abdo Xray for JA will show multiple dilated loops of bowel, typically will do UGI
Unlike DA where xray is usually conclusive and don't necessarily need further imaging
172
Difference in embryology between Jejunal atresia and Duodenal atresia?
DA = Malformation of the developing intestinal tract
JA = more likely to be the consequence of developmental disruption (eg vascular event in utero leads causes the intestine to heal which leads to complete obstruction)
173
A child with a cold gets cancelled for surgery. How long until they can be rescheduled?
3-6 wks after resolution of symptoms
URTI increases risk of laryngospasm!
174
Presentation of varicocele?
painless (or dull ache) of testicle
"bag of worms" on palpation (from abnormal dilation of scrotum venous plexus)
more prominent when patient STANDING (and enlarges with Valsalva)
usually LEFT testicle
adolescent and older (not diagnosed until puberty after there is increased blood flow)
175
what investigations are required for varicocele?
semen analysis for sexually mature adolescents (ie Tanner stage V)
176
treatment of varicocele?
Varicocelectomy surgery to maximize fertility
Indications (any of):
- if one testicle (usually left) much smaller than other, as this indicates impaired spermatogenesis in left testicle
- testicular pain
- semen analysis shows oligospermia
- large, visible (grade 3) varicocele
FYI this is the only surgery that can improve fertility in males (the small testes will catch up after surgery!)
177
what is a spermatocele?
cystic lesion containing sperm, attached to upper pole of testis
painless
incidental finding on physical exams
indication to remove is significant pain or enlarging
178
what is intussusception?
telescoping of the bowel causing obstruction and vascular compromise
179
presentation of intussusception?
3 mo to 3 yrs typically
Males >Females (3:1)
sudden onset, colicky intermittent pain
inconsolable and draws legs up during episode (tired/lethargic between episodes)
vomiting (non-bilious -> bilious)
sausage-shaped abdo mass
currant jelly stool (<30%)
180
pathophysiology of intussusception?
75% idiopathic:
predisposing factors are URTI (adenovirus), diarrheal illness (enterovirus)
8% lead point:
*Meckel's diverticulum*
polyp
duplication cyst
HSP
malignancy
(others)
181
investigation and diagnosis of intussusception?
Ultrasound (gold standard test - "target sign")
once US suggests intussusception, diagnosis confirmed with air enema (therapeutic and diagnostic)
182
management of intussusception?
small bowel-small bowel intuss = reduce spontaneously, monitor
ileum-colon = can lead to intestinal infarction, perforation, peritonitis, needs treatment
tx = air/saline/contrast enema
10% recurrence rate, can repeat enema (no limit)
Surgery if contraindication to air enema
183
Contraindications to using air enema in intussusception?
Peritonitis
Persistent hypotension, shock
Free air/ pneumoperitoneum
184
What is pyloric stenosis?
thickening of the pyloric channel muscle causing a complete or near-complete gastric outlet obstruction
185
Presentation of pyloric stenosis?
~6wks old (2 wks to 3 mo)
M:F is 6:1
Non-bilious, projectile emesis 30-60 mins post feeds
Lose H+ and Cl- in vomit →hypOchloremic hypOkalemic metabolic ALKALOSIS
186
Diagnosis of pyloric stenosis?
Ultrasound; pylorus length > 14mm, thickness > 3mm
(memory aid is the number pi 3.14)
187
Treatment of pyloric stenosis?
Fix alkalosis and dehydration, need ++ Cl- and K+, then surgery
Pre-Op: 20ml/kg bolus, then 1.5 x maintenance (D5 0.45NS or NS+20-40 KCL)
Pyloromyotomy
- risk of perforation (vomiting, fever, sepsis) or incomplete procedure
188
What are the main reasons for treating cryptorchidism?
- * Risk of Infertility *
- * Risk of Testicular Malignancy *
- * Risk of Testicular Torsion *
- Allow for testicular exams to monitor for testicular neoplasm in future
- Psychological benefit of normal anatomy
189
What is the differential for a nonpalpable testicle?
- Retractile testicle
- Undescended testicle
(intra-abdominal, inguinal, ectopic (perineal, femoral, pre-pubic)
- Testicular atrophy / agenesis
Note: always consider unifying diagnosis if other features present (ex: DSD, CAH, PMDS, syndromic association)
190
How do you diagnose cryptorchidism?
(HINT: Do you need any investigations?)
Cryptorchidism is a CLINICAL DIAGNOSIS
- Ultrasound not useful for position (does not differentiate between retractile and undescended)
191
If cryptorchidism persists past *** months then referral to general surgery for a orchidopexy is required and should be performed between *** - *** months of life.
If cryptorchidism persists past 6 months then referral to general surgery for a orchidopexy is required and should be performed between 6 - 18 months of life (ideally <12mo).
192
If you diagnosed CRYPTORCHIDISM (unilateral but especially bilateral) AND other GUS ABNORMALITIES (ex: hyposapdius, inguinal hernia, ipsilateral scrotal hypoplasia ect), what underlying diagnosis would you suspect?
DSD (Disorders of Sexual Development)
193
If you diagnose BILATERAL CRYPTORCHIDISM (nonpalpable in canal) with normal male phallus, what condition must you always consider in this infant?
(HINT: especially if there is features of adrenal insufficiency)
CAH - Congenital Adrenal Hyperplasia
Females with classic CAH will often present early with features of Adrenal Insufficiency and virilization of external genitalia but will have bilateral cryptorchidism.
194
Cryptorchidism:
You locate a 7mo old infant's testicle in the inguinal canal, it is persistently found here and is not retractile. What type of surgery would you perform?
BILATERAL SURGICAL ORCHIOPEXY
- You would complete surgery through scrotum or inguinal canal.
- Typically complete bilateral orchipexy even if the other testicle is palpable in the scrotum.
- DO NOT require exploratory laparoscopy
195
Cryptorchidism:
You complete a thorough genital exam on a 7mo old infant and are unable to locate the testicle in the canal or ectopically. What type of surgery would you perform?
1. EXAM UNDER ANESTHESIA
2. If testicle still not found then DIAGNOSTIC LAPAROSCOPY +/- INGUINAL / SCROTAL EXPLORATION in order to locate the testicle or confirm testicular agenesis
3. If testicle located then complete BILATERAL ORCHIOPEXY.
196
If cryptorchidism is diagnosed after puberty what management is typically recommended?
EXPLORATORY LAPAROSCOPY + ORCHIECTOMY (as intra-abdominal/inguinal testicle has higher rate of cancer)
197
