Pediatric Surgery Flashcards
1
Q
How are hydroceles classified?
A
- *1. Communicating hydrocele**
- Processus vaginalis fails to close
- Fluid moves freely between the peritoneal cavity and the scrotal space through the patent processus
- Can lead to an indirect inguinal hernia
- Increases with size with standing and Vasalva
- Smaller in the morning, larger at night
- *2. Non-communicating hydrocele**
- Fluid trapped in the tunica vaginalis
- May be secondary to testicular pathology
2
Q
Hydroceles are usually PAINLESS, and are associated with prematurity and the male gender.
A
3
Q
What is the prevalence of hydroceles?
A
1-2% of live births
4
Q
What physical signs are specific for hydroceles?
A
- Transillumination
- Silk glove sign: palpating the hydrocele through the pubic tubercle feels like silk rubbing against silk
5
Q
What investigations are indicated for hydroceles?
A
Scrotal ultrasound to rule out other pathologies (especially testicular pathologies in older children)
6
Q
Most hydroceles resolve spontanesouly by the first year.
A
7
Q
What are the indications for surgical repair?
A
- Persistence for >2 years
- Pain
- Infection
- Fluctuation in size, suggesting a communication
- Cosmetic reasons
8
Q
Hydroceles are found in 5-10% of testicular tumours.
A
9
Q
Hydroceles are typically unilateral.
7-10% of hydroceles are bilateral.
A
10
Q
What is the main symptom for hydroceles not associated with epididymitis?
A
Painless scrotal swelling
11
Q
The processus vaginalis is not expected to close spontaneously in children over 18 months.
A
12
Q
Communicating hydroceles are usually surgically repaired at around 2 years of age to allow the processus vaginalis the chance and sufficient time to close by itself.
A
13
Q
Surgical repair is the preferred method for:
- Children over two years of age
- Those that reoccur after aspiration
- Removal of large or painful hydroceles in adults
A
14
Q
How does one choose between the transinguinal and the transscrotal approach for hydrocelectomy?
A
Usually an outpatient procedure under GA
- Transscrotal: uncomplicated hydrocele
>> Repair of patent processus
>> Removal of the hydrocele sac and fluid
>> Closure of scrotal incisio - Transinguinal: complications such as inguinal hernia
>> Repair of hernia + correction of hydrocele
15
Q
What are the complications of hydrocelectomy?
A
- General risks
- Anesthetic risks
- Bleeding from the surgical incision
- Infection
- Internal bleeding - Specific risks
- Injury to spermatic vessels –> infertility
16
Q
What is the prevalence of hypertrophic pyloric stenosis?
A
0.03-1% of live births
17
Q
At what age does hypertrophic pyloric stenosis present?
A
1-20 weeks
Usually at 6-8 weeks
18
Q
What is an important acquired risk factor for hypertrophic pyloric stenosis?
A
Hypertrophic pyloric stenosis is associated with early erythromycin exposure at <13 days old.
19
Q
What is the pathophysiology of hypertrophic pyloric stenosis?
A
ACQUIRED pyloric circular muscle hypertrophy resulting in gastric outlet obstruction
20
Q
How would children with hypertrophic pyloric stenosis present?
A
- *History**
1. Projectile non-bilious vomiting 30-60mins after feeding
2. Dehydration - *Physical Examination**
1. Palpable ~1-2cm “olive” mass above umbilicus
2. Visible left-to-right peristalsis after feeding - *Investigations**
1. Hypovolemia
2. Hypochloremic hypokalemic metabolic alkalosis
3. Paradoxical aciduria
21
Q
What investigations should be ordered in a child suspected to have hypertrophic pyloric stenosis?
A
- Electrolytes
>> Hypochloremia
>> Hypokalemia
>> Hydration status - Blood gas
>> Metabolic alkalosis
3. Ultrasound
>> Pyloric length >14mm
>> Pyloric muscle thickness >4mm
- Upper GI series
>> String sign
22
Q
What are the options for treating hypertrophic pyloric stenosis?
A
- Fluid resuscitation
- Correct electrolyte and acid/base disturbances
- Medical treatment (for poor surgical canditiates only)
- Atropine
- TPN and waiting - Surgical repair
- Open Ramstedt pyloromyotomy
- Transumbilical pyloromyotomy
- Laparoscopic pyloromyotomy
23
Q
The curative treatment and the procedure of choice for hypertrophic pyloric stenosis is the Ramstedt procedure.
A
- Open RUQ transverse incision
- Open circumbilical incision
- Laparoscopic
24
Q
How are congenital diaphragmatic hernias (CDH) classified?
A
- Posterolateral (Bochdalek)
- Left-sided: 85%
- Right-sided: 15%
- Bilateral: rare, often fatal - Anterior (Morgagni)
- Hiatus
25
What is the prevalence of congenital diaphragmatic hernias?
1 in 2000-5000 live births
26
How do CDH present?
- Early respiratory distress
- Cyanosis
- Bowel gas sounds in the chest
- Decreased air entry
- **Scaphoid abdomen**
\*\* Prenatal diagnosis\*\*
---------------------------------------------------------------------------
- Left-sided
\>\> Stomach
\>\> Small bowel
\>\> Large bowel
\>\> Solid organs: spleen, left hepatic lobe
- Right-sided
\>\> Liver
\>\> Large bowel
- **Pulmonary hypertension**
- **Pulmonary hypoplasia**
NB: \>10% are associated with other congenital abnormalities
27
What investigations should be performed in a patient with suspected congenital diaphragmatic hernia?
* *PRENATAL MRI/ULTRASOUND
- Polyhydramnios
- Location of fetal liver
- Estimation of fetal lung volumes**
## Footnote
* *_BLOOD WORK_**
- Arterial blood gas: keep pH \>7.2
**_IMAGING_**
- Chest X-ray
\>\> Bowel in the thorax
\>\> Loss of normal diaphragmatic contour
\>\> Mediastinal shift
- Echocardiogram
28
How is CDH treated?
- **IMMEDIATE POSTNATAL CARE**
\>\> Neonatologist in labour ward is usually required
\>\> Supplemental oxygen and ETT
\>\> ***NB: bag-mask ventilation should be AVOIDED***
- Decompression by orogastric/nasogastric aspiration
- Maintain ventilation and stabilization
\>\> HFOV/ECMO
\>\> NO/epoprostenol/sildenafil: pulmonary HTN
\>\> Minimize peak inspiratory pressure to \<25cmH2O
\>\> Maintain preductal oxygen saturation at \>85%
\>\> Ventilation rate: 40-60 breaths per minute
\>\> PEEP: 3-5
- Surgery only when stable
\>\> Open VS. thoracoscopy VS. laparoscopy
\>\> Reduction of herniated contents
\>\> Repair of diaphragmatic defect
~~ Primary repair
~~ Synethetic/muscular patch
29
**Excessive bag-mask ventilation should be avoided in patients with congenital diaphragmatic hernia as it can exacerbate GI distention which further impedes lung ventilation.**
30
What are the complications/commonly associated defects with CDH?
1. Pulmonary hypoplasia ---\> chronic lung disease
2. Failure to thrive
3. GERD
4. Recurrence
5. Associated congenital defects
- Hearing deficit
- MSK defects
31
What is the rule of 2s for Meckel's Diverticulum?
1. 2% of the population
2. 2:1 male to female ratio
3. 2% of those with Meckel's are symptomatic
4. Found within 2 feet from the ileocecal valve
5. 2 inches in length
6. 2 inches in diameter
7. 2 types of ectopic tissue: gastric and pancreatic
8. Often presents at 2 years of age
32
What are the possible presentations of vitelline duct remnants?
1. Meckel's diverticulum
2. Enterocyst/vitelline cyst
3. Vitelline fistula
**NB:** **Meckel's diverticulum is the most common remnant of the vitelline duct**
33
About 50% of all Meckel's diverticulae contain heterotropic/ectopic tissue: gastric or pancreatic.
34
What is the pathophysiology of Meckel's diverticulum?
Failure of the vitelline duct to regress at 5-7 weeks in utero
35
What are some associated anomalies with Meckel's diverticulum?
1. Omphalomesenteric fistula
2. Umbilical sinus
3. Umbilical cyst
4. Fibrous band
36
What are the common complications/presentations of Meckel's diverticulum?
1. PR fresh blood (ectopic gastric mucosal ulceration)
2. Abdominal sepsis (diverticulus +/- perforation)
3. Volvulus
37
What are some investigations commonly performed for symptomatic/suspected Meckel's?
1. Abdominal X-ray: perforation
2. **Meckel scan**
- Technetium Tc99m pertechnetate IV
- For ectopic gastric mucosae
- Sensitivity 85%
- Specificity 95%
38
Meckel's scan is less accurate (46%) in adults due to the reduced prevalence of ectopic gastric mucosa within the diverticulum.
39
How are Meckel diverticulae treated?
**Resection is curative**
## Footnote
- In symptomatic patients
- Simple diverticulectomy in uncomplicated cases
- Segmental small bowel resection
\>\> Acute diverticulitis
\>\> Wide-based diverticulum
\>\> Volvulus with necrotic bowel
\>\> Bleeding
- +/- incidental appendectomy
**NB: Incidental diverticulectomy during surgery for other abdominal pathology is NOT indicated --** lifelong morbidity associated with the presence of a Meckel's diverticulum is extremely low.
40
What is the most common congenital obstructive urethral lesion in male infants?
Posterior urethral valve
41
What is the pathophysiology of PUV?
Abnormal mucosal folds at the distal prostatic urethra causing varying degrees of obstruction
42
How do posterior urethral valves present?
Depends on the age of presentation
## Footnote
* *_Antenatal_**
- Bilateral hydronephrosis
- Bladder distension
- Oligohydramnios
* *_Neonatal (detected at birth)_**
- Palpable abdominal mass -- distended bladder
- Ascites -- transudation of retroperitoneal urine
- Respiratory distress -- pulmonary hypoplasia from oligohydramnios
- Weak urinary stream
* *_Neonatal (not detected at birth_**)
- Urosepsis
- Dehydration
- Electrolyte abnormalities
- Failure to thrive
* *_Toddlers_**
- UTI
- Voiding dysfunction
* *_School-aged boys_**
- Voiding dysfunction --\> urinary incontinence
43
What are the commmon complications of posterior urethral valve?
- Urinary retention
- Overflow incontinence
- -----------------------------------------------------------------------------------
- Urosepsis
- Urinary tract infections
- -----------------------------------------------------------------------------------
- Pulmonary hypoplasia leading to respiratory distress
- -----------------------------------------------------------------------------------
- **Secondary vesicoureteric reflux (VUR)**
- **Secondary renal dysplasia**
44
What investigations should be performed for a suspected case of posterior urethral valve?
Mostly on prenatal ultrasound
- Bilateral hydroneprhosis
- Thickened bladder
- "**Keyhole sign**": dilated posterior urethra
- Oligohydramnios
Postnatal -- **_VCUG/MCUG_**
- Dilated and elongated posterior urethra
- Trabeculated bladder
- Secondary VUR
45
How are PUVs treated?
- Immediate catheterization to relieve obstruction
- Cystoscopic PUV resection when stable
- Vesicostomy if cystoscopic resection no possible
46
What are the possible underlying causes for antenatal hydronephrosis?
_Kidney_
- Multicystic dysplastic kidney
_Renal pelvis_
- Ureteropelvic junction (UPJ) obstruction
_Ureter_
- Ureterocele
- Ectopic ureter
_Ureterovesical junction (UVJ)_
- UVJ obstruction
- VUR (vesicoureteric reflux)
_Urethra/Genitalia_
- PUV (posterior urethral valve) -- only in boys
_Systemic/All along the urinary tract_
- Duplication abnormalities
47
**Majority of antenatal hydronephroses resolve during pregnancy or within the first year of life.**
## Footnote
- - Detectable as early as first trimester
- - One of the most common ultrasound abnormalities in pregnancy
48
What are the possible causes of UPJ obstruction?
_Primary_
- Stricture
- Stenosis
- Adynamic ureteral segment
- Extrinsic compression
- Aberrant blood vessels
_Secondary/Underlying pathology_
- Tumour
- Stone
- Extrinsic causes
49
Up to 40% of UPJ obstructions are bilateral, which may be associated with worse prognosis.
50
What is the most common presentation of UPJ obstruction?
Asymptomatic finding on antenatal ultrasound
51
If symptomatic, how would UPJ obstruction present?
Depends on age of presentation
## Footnote
_Infants_
- Urinary tract infection
- Abdominal mass
_Children_
- Pain
- Vomiting
- Failure to thrive
_Adolescents/Adults_
- ***Dietl's crisis***: symptoms of retention etc. triggered by episodes of increased diuresis, such as following alcohol ingestion
52
How is UPJ obstruction treated?
Surgical correction by pyeloplasty
- Consider nephrectomy if \<15% differential renal function (as per DTPA/MAG3)
53
What is vesicoureteral reflux (VUR)?
Retrograde passage of urine from the bladder, through the UVJ (ureterovesicular junction) into the ureter
54
How is VUR classified?
**_Primary VUR_**
- From incompetent or incomplete closure of VUJ
- Possible causes
\>\> Lateral ureteral insertion
\>\> Short submucosal segment
**_Secondary VUR_**
- From abnormally high intravesical pressure resulting in the failure of VUJ closure
- Possible causes
\>\> Anatomical: posterior urethral valve (PUV)
\>\> Functional: neurogenic bladder dysfunction
55
What are the risk factors for VUR?
- White race (VS. black race)
- Female gender
- Age \<2 years
- Genetic predisposition
56
Incidence and clinical relevance is higher in children with _febrile UTIs_ and _prenatal hydronephrosis_.
57
What should be asked in a patient with suspected VUR/follow-up for VUR?
* *_Urinary symptoms/Voiding dysfunction_**
- Frequency
- Urgency
- Noctural/diurnal enuresis
- Noctural diuresis
* *_Bowel symptoms_**
- Constipation
- Encopresis (voluntary/involuntary fecal soiling in already toilet-trained children)
* *_Signs of infection_**
- UTI
- Pyelonephritis
- Urosepsis
* *_Signs of renal failure_**
- Uremia
- Hypertension
58
Initial evaluation of **_RENAL STATUS**_, _**BLOOD PRESSURE**_, and _**GROWTH PARAMETERS_** is warranted in any child with VUR confirmed due to the *high incidence of renal scarring*.
## Footnote
- Creatinine
- Urinarlysis and culture
- Renal ultrasound
- DMSA if at high risk
59
Primary VUR (due to incomplete closure or incompetence of the UVJ) resolves spontaneously in 60%.
60
How is VUR treated?
Depends on severity
## Footnote
- I-III: prevent infection or renal damage via medical treatment and monitoring
- IV-V: aggressive treatment
**_Medical treatment_**
- Long-term ABx prophylaxis at half the treatment dose for half the treatment time
\>\> TMP-SMX
\>\> Trimethoprim
\>\> Amoxicillin
\>\> Nitrofurantoin
* *_Surgical treatment_**
- Ureteral reimplantation +/- ureteroplasty
- Subureteral injection with bulking agents
61
What are the indications for surgical treatment in VUR?
1. Persistent high-grade VUR (Grades IV-V)
2. Breakthrough UTI
3. Failure of medical treatment
4. Renal scarring
\>\> Renal insufficiency
\>\> Hypertension
62
How is VUR graded?
I. Fills ureters
II. Fills ureter and pelvis
III. Fills ureter and pelvis with some dilation
IV. Fills ureter, pelvis and calyces with significant dilation
V. Fills ureter, pelvis and calyces with major dilation and tortuosity
63
What is hypospadias?
Urethral meatus opens on the ventral side (anterior side with the penis erected) of the penis, proximal to the normal location in the glans penis
## Footnote
- Complicated with
\>\> Voiding dysfunction
\>\> Difficulty in directing urinary stream
\>\> Infertility
64
What are the indications for urgent referral for hypospadias?
1. Micropenis
2. Bifid and prepenile scrotum
3. Asymmetrical gonads
4. Severe hypospadias
These features are associated with an increased likelihood for disorders of sexual differentiation (DSD).
65
How is hypospadias classified?
By the location of the urethral meatus
## Footnote
- **Glandular**
- **Coronal**
- Subcoronal
- Distal penile
- Midshaft
- Proximal penile
- Penoscrotal
- Scrotal
- Perineal
66
How prevalent is hypospadias?
Very common
1 in 300 male live births
67
What are some associated conditions with hypospadias?
- Ventral penile curvature (chordee)
- Disorders of sexual differentiation (DSD)
- Undescended testicles
- Inguinal hernia
68
How is hypospadias treated?
Early surgical correction
## Footnote
- Neonatal circumcision should be deferred since the foreskin may be utilized in the correction repair
- Optimal repair _before 2 years of age_
69
**Defer circumcision in patients with hypospadias.**
70
What is the epispadias-exstrophy complex?
A spectrum of defects depending on the time of the *_rupture of the cloanal membrane_* -- failure of closure of the cloacal membrane
## Footnote
- Epispadias
\>\> Mildest form
\>\> Urethra opens on the dorsal aspect of the penis
- Bladder exstrophy
\>\> Absence of a portion of the lower abdominal and anterior bladder walls
\>\> Exposure of the bladder lumen
- Cloanal exstrophy
\>\> Most severe
\>\> Exposed bladder and bladder with _imperforate anus_
\>\> Associated with spina bifida in \>50%
71
The epispadias-exstrophy complex is usually associated with high morbidity due to associated incontinence, infertility and reflux.
72
What is the treatment for epispadias-exstrophy complex?
Surgical correction at birth
## Footnote
Later corrections may be necessary for:
- Incontinence
- VUR
- Bladder capcacity
73
What is Wilm's tumour?
Nephroblastoma -- arises from abnormal proliferation of metanephric blastema (embryological precursor of the nephrons and proximal collecting system)
74
What is the average age of presentation of Wilm's tumour?
3 years old
75
Wilm's tumour is the most common primary malignant renal tumour of childhood.
76
5% of Wilm's tumours are bilateral.
77
Wilm's tumours take up 5% of all childhood cancers.
78
What is the most common presentation of Wilm's tumour?
Abdominal mass: large, firm and **UNILATERAL** (does not cross midline)
## Footnote
- *_Hypertension: 25%_*
- Flank tenderness
- Microscopic hematuria
- Nausea and vomiting
79
Always investigate the contralateral kidney in a case of Wilm's tumour.
80
How are Wilm's tumours managed?
* *_Unilateral disease_**
- Radical nephrectomy
- +/- Radiation
- +/- Chemotherapy
* *_Bilateral disease_**
- Neoadjuvant chemotherapy
- **NEPHRON-SPARING** surgery
*The difference in the management plan denotes the importance of investigating/examining the contralateral kidney in* *all cases of Wilm's tumours.*
81
What is the prognosis of Wilm's tumours?
5-year survival 80%
82
What is cryptorchidism?
Abnormal location of testes somewhere along the normal path of descent
## Footnote
* *_Locations according to incidence_** (from commonest to least common)
1. External inguinal ring
2. Inguinal canal
3. Abdominal
83
What is a retractile testis?
A "high" testies due to hyperactive cremasteric muscle, that can be milked down into the scrotum
84
Where are ectopic testes most commonly found?
Within the superficial pouch between the external oblique fascia and the Scarpa's fascia (**Denis Browne pouch**)
85
What is the difference between cryptorchidism and ectopic testes?
Cryptorchidism: testis misplaced somewhere along the normal path of descent
Ectopic testes: testis found OUTSIDE its normal path of descent
86
At what time of gestation does the descent of testes take place?
7th month of gestation
## Footnote
- 2nd month: testicles begin to form
- 4th month: testicles begin to take on its normal appearance
- 7th month: descent starts from the internal ring through the inguinal canal and extenal ring to the scrotum, surrounded in peritoneal covering
87
What are some differential diagnoses of cryptorchidism/ectopic testes?
- Atrophic testes
- Retractile testes
- Disorders of sexual differentiation (DSD) --- **BILATERAL** impalpable gonads
88
How common is cryptorchidism/ectopic testes?
2.7% of all full term newborns
| (0.7-0.8% at 1 year of age)
89
What is the treatment for cryptorchidism/ectopic testes?
Orchipexy
## Footnote
*Hormonal therapy has not been proven to be of benefit over surgical care.*
90
What does orchipexy do for those with cryptorchidism?
- NO EFFECT on malignant potential (still 1:1000 VS. 1:4000)
- Decreased risk for torsion
- Preserve spermatogenesis if OT before 1 year of age
91
What are the potential complications of cryptorchidism/ectopic testes?
1. Reduction in fertility
- Infertility 100% if bilateral and untreated
- Fertility 53% if bilateral and treated
- Fertility 90% if unilateral and treated
- Fertility 93% if normal
2. Increased malignancy risk
- Intraabdominal testes at higher risk than inguinal testes
- Surgical correction facilitates monitoring only
3. Increased risk for testicular torsion
- Risk reduced by surgical correction (orchipexy)
92
What are disorders of sexual differentiation (DSD)?
Abnormal genitalia for chromosomal sex due to:
1. Undermasculinization for males
2. Virilization of females
93
How are DSDs (disorders of sexual differentation) classified?
* *_1. 46XY DSD_**
- Defect in testicular synthesis of androgens or androgen resistance in target tissues
- Gonad palpable
- Genotypically male
* *_2. 46XX**_ _**DSD_**
- Usually due to CAH (21-hydroxylase deficiency is most common)
- Shunt in steroid synthesis pathway leads to excess androgens
- Can be complicated with life-threatening salt-wasting CAH
- Genotypically female
* *_3. Ovotesticular DSD_**
- Ovotestis: histology of a gonad that contains both ovarian follicles and testicular tubular elements
- Diagnosis of ovotesticular DSD is based solely on the presence of ovarian and testicular tissue in the gonad and NOT on the characteristics of the internal and external genitalia
* *_4. Mixed gonadal dysgenesis_**
- Usually 46XY or 45XO
- Partial testis determination to varying degrees/atrophic gonads
94
Palpable gonads signifies the patient is a chromosomal male.
95
What should be noted in the history a patient suspected to have DSD?
- Family history for consanguinity
- Maternal history -- medication/drug use during pregnancy (maternal hyperandrogenism in female DSDs)
96
What is important to note in a physical examination of a patient with suspected DSD?
**_Inspection_**
- Dysmorphic features
- Hyperpigmentation in genitalia
- Evidence of dehydration (CAH salt-wasting crisis)
- Hypertension
- Stretched phallus length in boys
- Position of urethral meatus
\>\> Hypospadias with associated defects such as cryptorchidism is suggestive of underlying DSD
* *_Palpation_**
- Palpable gonads signify that the patient is at least a chromosomal male
97
What investigations are indicated in a patient with suspected DSD?
* *_Specific blood tests_**
- Serum electrolytes -- salt-wasting CAH
- Sex karyotype
- Plasma 17-OH-progesterone (after 36 hours of life) -- 21-hydroxylase deficiency
- Plasma 11-deoxycortisol ----------------------------------------- 11-b-hydroxylase deficiency
- Adrenal steroid levels
- Serum testosterone and DHT (pre- and post-hCG stimulation - 2000IU/day x 4d)
* *_Imaging_**
- U/S adrenals
- U/S gonads
- U/S uterus and fallopian tubes in girls
- Endoscopy/genitography of urogenital sinus
98
In DSD, reconstruction of external genitalia should be performed at ages between 6-12 months.
99
How should DSDs be treated?
Depends on type and underlying cause
## Footnote
- Steroid supplementation as indicated (in CAH)
- Family consultation
\>\> Sex assignment
\>\> Long-term psychological guidance and support
- Reconstruction of external genitalia between 6-12 months
100
What is circumcision?
Removal of some or all of the foreskin from the penis
101
What are the medical indications for circumcision?
- Phimosis
- Recurrent paraphimosis
- Recurrent UTIs
- Balanitis xerotica obliterans and other chronic inflammatory conditions
NB: BXO = lichen sclerosus of the penis; common cause of pathological phimosis
102
What is the difference between phimosis and paraphimosis?
**_Phimosis_**
- The inability to retract the distal foreskin over the glans penis
- Physiological in newborn males
- Pathological:
\>\> Inability to retract foreskin that was previously retractable or after puberty
\>\> Usually secondary to distal scarring of the foreskin
* *_Paraphimosis_**
- Entrapment of a already retracted foreskin _behind the coronal sulcus_
- A disease of uncircumcised/partially circumcised males
- Can be a medical emergency --\> gangrene
103
What are the contraindications for circumcision?
- Unstable/sick infant
- Hypospadias
- Family history of bleeding
\>\> Relative contraindication
\>\> Warrants laboratory investigation prior to circumcision
104
What are the complications of circumcision?
* *_General_**
- Bleeding
- Infection
- Anesthetic risks
* *_Specific_**
- Penile entrapment
- Glans injury
- Penile sensation deficits
- Fistula
105
**The American Academy of Pediatrics in 2012 recommends that the preventative health benefits (prevention of UTI, penile cancer, transmission of certain STIs including HIV) outweigh the risks of the procedure.**
The AAP also claims that the procedure is well-tolerated with adequate pain management and sterility, with no effect on pernile sexual function, sensitivity or sexual satisfaction.
106
What is enuresis?
Involuntary urinary incontinence by day and/or night in a child **_\>5 years of age_**
107
How is enuresis classified?
* *_Primary Noctural Enuresis_**
- Wet ONLY at night DURING SLEEP
- In a child whose bladder contral has never been attained
- Due to maturational lag/developmental disorder
**_Secondary Enuresis_**
- In a child who has already had a sustained period (**_\>6 months_**) of bladder control
- Causes include:
\>\> Stress/anxiety
\>\> Organic diseases
~~ UTI/Pinworms
~~ DM/DI
~~ Neurogenic bladder dysfunction
~~ Cerebral palsy
~~ Seizures
~~ Sickle cell disease
**_Diurnal Enuresis_**
- Daytime wetting
- 60-80% also wet at night
- Causes
\>\> Psychosocial stressors leading to micturition deferral
\>\> Structural causes
~~ Ectopic ureter
~~ Neurogenic bladder
\>\> Other organic causes
~~ UTI
~~ Constipation
~~ CNS disorders
108
Treatment for primary nocturnal enuresis should NOT be considered until **_7 years of age_** due to the high rate of spontaneous cure.
109
20% of children with primary nocturnal enuresis resolve spontaneously each year.
110
How is primary nocturnal diuresis managed?
1. Time and reassurance
2. Behaviour modification
- Limiting fluids
- Voiding before sleep
- Bladder retention exercises
- Scheduled toileting (mehhh~)
3. Conditioning with "wet alarm" --- **70% success rate**
4. Medications (second-line)
- DDAVP: high relapse rate
- Imipramine: lethal at overdose with cholinergic S/Es, thus rarely used
111
What is encopresis?
Fecal incontinence in a child **_\>4 years of age_**, at least **once per month** for **3 months**
112
What are the possible causes of encopresis?
* *_Gastrointestinal causes_**
- Chronic (retentive) constipation
- Hirschsprung disease
- Anorectal malformations
* *_Systemic/Metabolic causes_**
- Hypothyroidism
- Hypercalcemia
* *_Neurological causes_**
- Spinal cord lesions
113
How is retentive encopresis/constipation treated?
**Complete clean-out of bowel with PEG3350 (osmotic laxative) orally is the most effective** **treatment**
## Footnote
- *_Complete clean-out_* with enemas and suppositories (invasive)
\>\> Biscodyl (stimulates colonic movement + contact laxative)
\>\> Picosalax (contact laxative)
- *_Maintenance_* of regular bowel movements
\>\> Adequate _fluid_ intake
\>\> Adequate _dietary_ fibre
\>\> Stool softeners with PEG3350 and/or mineral oil
\>\> Appropriate toilet training
- Assessment and guidance of *_psychosocial stressors_*
- *_Behavioural modification and reassurance_*
114
What are the possible complications of encopresis?
- Recurrence
- Toxic megacolon
- Bowel perforation
115
Toxic megacolon secondary to retentive encopresis will require \>3-12 months to treat.
116
**Bilious vomiting in infants is a life-threatening emergency secondary to midgut volvulus until proven otherwise.**
117
What is malrotation?
Failure of the gut to normally rotate around the superior mesenteric artery (SMA)
118
What is the typical age of presentation for malrotation of the gut?
1/3: 1 week of life
3/4: 1 month of life
90%: 1 year of life
119
What is the clinical significance of differentiating non-rotation and malrotation?
Non-rotation is not a risk factor for volvulus and duodenal obstruction by Ladd's bands. Malrotation is for both.
## Footnote
- In nonrotation, the small bowel stays on the right and large bowel stays on the left with the duodenojejunal flexure at the RUQ and cecum at the LLQ. Therefore, the mesenteric base is not narrow.
- In malrotation, the small bowel can still be on the right (or inferior or on the left), while the large bowel can still be on the left (or can be on the right). However, the cecum almost definitely stays in the upper abdomen -- either RUQ or LUQ. The duodenojejunal flexure is located in the RUQ. Therefore, the mesenteric base is narrow and thus predisposes the gut to volvulus.
120
**Bilious emesis is THE cardinal sign for malrotation, especially if the abdomen is non-distended**. If the abdomen is distended, consider further investigations to rule out volvulus (volvulus can, of course, happen with malrotation).
Bilious emesis in malrotation is caused by _DUODENAL OBSTRUCTION_ due to the presence of Ladd's bands from the cecum obstructing the duodenum.
Volvulus in malrotation is caused by the narrow mesenteric base, and usually presents with abdominal pain and distension, and eventually bloody stools as necrosis occur.
It should be noted, though, that severe duodenal obstruction can also cause abdominal pain and distension.
121
What investigations should be performed in a patient with suspected malrotation with bilious emesis?
**_Blood tests_**
- CBC
- RFT/electrolytes
\>\> Dehydration from vomiting/3rd space fluid loss
\>\> Electrolyte disturbances from 3rd space loss and vomiting
- Amylase for perforated viscera
**_Imaging_**
- AXR
\>\> Dilated bowels
\>\> Double-bubble sign in duodenal obstruction
- Contrast studies
\>\> Duodenojejunal segment right of midline and not fixed posteriorly over spine
\>\> Cecum and appendix in left abdomen
\>\> Corkscrew sign for volvulus
- Ultrasound
\>\> To rule out intussusception (bull's eye sign)
\>\> Whirlpool sign in volvulus
\>\> Abnormal SMA/SMV relationship
122
How is malrotation treated?
- Fluid resuscitation and stabilization (10-20mL/kg fluid bolus in children)
- IV antibiotics
- **EMERGENT LAPAROTOMY**: diagnostic and therapeutic
\>\> U/S to rule out intussusception, which can be treated by pneumatic reduction
\>\> **LADD PROCEDURE**
~~ Counterclockwise reduction of midgut volvulus if present
~~ Division of Ladd's bands
~~ Broadening of the mesentery (like opening a book)
~~ Incidental appendectomy
123
Why is an appendectomy performed routinely in the Ladd procedure?
In malrotation, the appendix (with the cecum) is situated in either the RUQ or LUQ. Therefore, any future presentation of appendicitis will be atypical and thus hard to diagnose. The incidental appendectomy will prevent misdiagnosis of any future appendicitis and the potential complications that will follow.
124
What is the prognosis of malrotation?
Mortality is related to the length of bowel loss
- 10% necrosis: 100% survival
- 75% necrosis: 35% survival
125
What is the difference between gastrochisis and omphalocele?
* *_Gastrochisis_**
- Defect of the abdominal wall with free extrusion of intestines into amniotic cavity
- Associated with younger maternal age and IUGR
- Not associated with genetic syndromes
- 10% associated with intestinal atresia
- Associated with short bowel syndrome from antenatal volvulus
* *_Omphalocele_**
- Defect of the abdominal wall with extrusion of _sac-covered_ viscera with the amnion, Wharton's jelly and peritoneum
- Associated with increased maternal age and prematurity/young GA
- Associated with genetic syndrome sin 30-70%
- Associated with pulmonary hypoplasia
126
Where is gastrochisis usually located at?
Defect usually lateral to the cord (typically right to the cord)
Note that in contrast, the omphalocele sac *includes* the cord
127
Both omphalocele and gastrochisis are associated with elevated MS-AFP.
128
How is gastrochisis treated?
- NG decompression
- IV fluids/fluid resuscitation (10-20mL/kg fluid bolus in children)
- IV antibiotics
- Keep exposed viscera warm, moist and protected until surgery is possible
- Surgical reduction
\>\> Primary abdominal closure
\>\> Staged closure with silo
\>\> May be complicated with bowel dysmotility requiring pharmacotherapy
129
How is omphalocele treated?
- NG decompression
- IV fluids (10-20mL/kg in children)
- IV antibiotics
- Surgical closure
\>\> Small defect (\<2cm): primary closure
\>\> Medium (2-4cm) and large (\>4cm) defect
~~ Silver sulfadiazine to promote epithelilization
~~ Compression to allow for gradual reduction
~~ Followed by surgical repair with or without mesh
130
How common are umbilical hernias?
Incidence is 2-14% and increases with prematurity
131
What is the definition of clinically significant umbilical hernia?
Incomplete closure of peritoneal and fascial layers within the umbilicus by **_5 years of age_**
132
What is the most common presentation of umbilical hernias?
Asymptomatic
133
The majority of umbilical hernias spontaneously resolves by 5 years of age.
134
Incarceration of umbilical hernias before the age of 5 years is very rare.
135
Most umbilical fascial defects **_\>1.5cm_** in infancy will not close spontaneously.
136
It is important to differentiate umbilical hernias from less common abdominal wall hernias that don't spontaneously resolve (e.g. epigastric hernias).
137
What are the indications for surgical repair in umbilical hernias?
Lack of spontaneous closure by the age of 5
138
How common is intestinal atresia?
Incidence 2-14%
139
What is the pathophysiology of duodenal, jejunoileal and colonic atresia respectively?
* *_Duodenal atresia_**
- Congenital problem
- Failure of bowel to recanalize after endodermal epithelial perforation at week 8-10
* *_Jejunoileal atresia_**
- Acquired problem
- A result of vascular disruption --\> ischemic necrosis --\> resorption of necrotic tissue --\> blind proximal and distal ends
* *_Colonic atresia_**
- Acquired problem
- Similar to jejunoileal atresia
140
What are the presenting features of duodenal atresia?
- Antenatal polyhydramnios
- Gastric distension
- Bilious vomiting
- Feeding difficulties
---------------------------------------------------------------------------------------------------
- 25-30% associated with Down syndrome
- Other anomalies
\>\> Tracheoesophageal fisulta (TEF)
\>\> Cardiac abnormalities
\>\> Renal abnormalities
\>\> Vertebral abnormalities
141
**25-30% of children with duodenal atresia has Down syndrome.**
142
What is the common age of presentation for duodenal, jejunoileal and colonic atresia respectively?
* *_Duodenal atresia_**
- A disease of newborns
- Onset of vomiting within _hours of birth_
* *_Jejunoileal atresia_**
- Within _2 days_ of birth
- Associated with cystic fibrosis
* *_Colonic atresia_**
- Within _3 days_ of birth
143
Jejunoileal atresia is associated with cystic fibrosis.
144
What physical signs should be looked out for in a child presenting with suspected intestinal atresia?
**SIGNS OF VOLUME DEPLETION** -- for every child presenting with vomiting and potential of bowel obstruction
## Footnote
- Thorough abdominal examination
- Include examination of perineum and anus
- Jaundice
- Other congenital abnormalities/dysmorphic facies
- Presence of respiratory distress
145
What investigations are indicated in a child with suspected intestinal atresia?
* *_Blood tests_**
- CBC
- Electrolytes/RFT
- LFT if jaundice present
- Clotting, T&S and X-match for surgery
* *_Imaging_**
- AXR for dilated bowel loops or double-bubble sign in duodenal atresia
- Contrast enema +/- UGI series with small bowel follow through (SBFT\_
146
How is intestinal atresia treated?
- NPO
- "Drip and suck"
\>\> IV fluid resuscitation -- 10-20mL/kg fluid bolus for children
\>\> NG tube decompression
- IV broad spectrum antibiotics
- TPN as necessary
-------------------------------------------------------------------------------------------
**_Surgery_**
- Duodenal atresia
\>\> Duodenoduodenostomy
\>\> Duodenojejunostomy
- Jejunoileal atresia
\>\> Primary anastomosis
\>\> End stoma (short bowel)
\>\> Defer surgery for bowel lengthening procedures (short bowel)
~~ LILT procedure (longitudinal intestinal lengthening and tapering)
~~ STEP procedure (serial transverse enteroplasty)
:: Bowel dilated and partially transected at certain points
:: Creates a zigzag pattern
:: Lengthening of surface area available for digestion
147
What is short bowel syndrome?
A condition in which a patient cannot absorb adequate nutrients because a portion of the small intestine is damaged or absent
Usually requires patient to be on parenteral nutrition (long term TPN can be associated with liver failure)
* *_Common causes_**
- NEC
- Gastrochisis
- Crohn's disease
148
What is Hirschsprung's disease?
Defect in migration of _neurocrest cells_in the intestine resulting in an aganglionic bowel that fails to peristalse, and in an internal sphincter that fails to relax
## Footnote
- Intestinal achalasia
- Sphincter achalasia
- Results in both functional and mechanical bowel obstruction
149
**Hirschsprung's disease always starts in the rectum**, with variable involvement proximally.
150
Hirschsprung's disease is related to RET mutation.
151
How common is Hirschsprung's disease?
1 in 5000 births
## Footnote
- M:F 4:1
- Approaches 1:1 when the whole colon is involved
152
How does Hirschsprung's disease present?
**Failure to pass meconium within 48 hours**
## Footnote
- Abdominal distension
- Constipation
- Bilious vomiting
- Enterocolities with gut flora overgrowth \>\>\> sepsis
- Failure to thrive
153
**Failure to pass meconium spontaneously within 48 hours of life is the classic presenting symptom of Hirschsprung's disease.**
Normal children will pass meconium within 24 hours in 95%, and the remaining 5% will pass meconium within 48 hours.
154
What investigations are indicated for Hirschsprung's disease?
**Rectal biopsy showing aganglionosis and neural hypertrophy is the GOLD STANDARD** **of diagnosing Hirschsprung's disease.**
## Footnote
* *_Blood tests_**
- CBC
- RFT: dehydration and electrolyte abnormalities for vomiting and obstruction
- Clotting, T&S and X-match for surgery
**_Imaging_**
- AXR: bowel loop dilation and free gas
- Contrast enema
\>\> Narrow rectum
\>\> Transition zone
**_Functional tests_**
- Anal manometry
\>\> Unreliable in infants
\>\> Absence of rectoanal inhibitory reflex
155
What is the treatment for Hirschsprung's disease?
**"Pull-through procedure"**
Surgical resection of aganglionic intestinal segment and anastomosis of the remaining intestine to the anus
- In newborn period
- Staged if extensive aganglionosis
- Complications
\>\> Fecal incontinence
\>\> Constipation
\>\> Post-operative enterocolitis
156
As a rule, forceful manual reduction is recommended in all cases of incarcerated hernia.
This is unless:
1. The clinician suspects the possibility of inguinal hernia strangulation
2. The patient has signs of systemic toxicity (leukocytosis, severe tachycardia, abdominal distension, bilious vomiting and discoloration of entrapped viscera).
***Manual reduction is successful in 90% and poses minimal risk to the entrapped structure.***
157
Successful reduction of an incarcerated inguinal hernia results in:
1. Immediate patient comfort
2. Relief of obstruction
3. Prevention of strangulation
158
Unsuccessful manual reduction in an incarcerated inguinal hernia indicates for immediate surgery.
159
How common are pediatric inguinal hernias?
5% of all term newborns
## Footnote
- Twice as common in preemies (and more likely to be bilateral)
- M:F ratio 4:1
- Increases with prematurity and low birth weight
160
On which side does the pediatric inguinal hernia usually occur?
60% right
30% left
10% bilateral
Indirect hernias are more common on the right because of the delayed descent of the right testicle.
161
**All infant hernias are _INDIRECT_ due to a patent processus vaginalis.**
162
**1/5th inguinal hernias will become incarcerated if the patient is \<1 year old.**
163
**Incarceration is more common in girls.**
164
How do pediatric inguinal hernias usually present?
**Painless intermittent mass in the groin with/without extension into groin**
## Footnote
- Usually during crying/straining
- Resolves at night during sleep
- Note that a scrotal mss without an inguinal mass indicates a hydrocele
- Signs of incarceration should be noted
\>\> Tenderness
\>\> Firm mass (VS. soft)
\>\> Vomiting
\>\> Accompanying skin changes: erythematous, cyanosis
165
How should a child with suspected inguinal hernia be examined?
**Examine the patient both standing up and supine.**
**Physical examination is the gold standard for diagnosing a hernia.**
* *_Inspection_**
- Scrotal/inguinal swelling
- Skin changes that may suggest incarceration
- Signs of systemic toxicity
**_Palpation_**
- Bimanual testicular examination
\>\> To rule out undescended/retractile testes
\>\> Bag of worms -- possible varicocele
\>\> "Silk sign" palpable thickening of cord
- Is the mass reducible?
- Is there a cough impulse if the mass is reducible?
- Can you get above the mass?
- Fluctuancy suggests hydrocele
- Assess the superficial/external inguinal ring
166
How should pediatric inguinal hernias be treated?
**Manual reduction should always be tried if the patient has no signs of systemic toxicity.**
## Footnote
1. Manual reduction
- Relieves acute symptoms
- Successful in 90%
- Minimal risk to entrapped structures
2. Herniorraphy/Herniotomy
- Definite treatment
- Reduction of hernia contents + high ligation of sac
- Open VS. laparoscopic
- Laparoscopic needle-assisted inguinal hernia repair
NB: Classically, if an incarcerated hernia is manually reduced successfully, an open herniotomy is postponed to 24-72 hours later as an elective procedure to allow the resolvement of the edema and inflammation of the entrapped bowel --- this poses significant difficulty in an emergent operation. However, with the advent of laparoscopic operations, the manipulation of inguinal structures intraperitoneally bypasses the edematous bowel in the emergency setting, and thus allows for emergent operation to be done laparoscopically.
Laparoscopy also allows for simultaneous examination of the contralateral side for patent processus vaginalis.
However, laparoscopy is associated with the risk of bowel perforation/damage (open does not have this danger), and a higher risk for recurrence (2-3% VS. \<1% in open hernia repair).
167
What is the most common cause of bowel obstruction between the ages of 6-36 months?
Intussusception
168
What is intussusception?
Telescoping of bowel into itself causing an obstruction and vascular compromise
169
What are some underlying causes of intussusception?
- Enlarged Peyer's patches due to GI viral infections
- GI polyps
- Meckel's diverticulum
Usually idiopathic
170
Where is the commonest site of intussusception?
Ileocecal valve
171
How do patients with intussusception present?
**Acute onset of episodic "colicky" abdominal pain**
\>\> The child draws legs up to the abdomen and kicks his/her legs in the air during episodes of pain, and appears calm and relieved between episodes.
## Footnote
- Nonbilious --\> bilious vomiting
- Abdominal mass
- *_Current-jelly stool_* suggests mucosal necrosis
- Lethargy
172
What physical signs should be looked for in a patient suspected to have intussusception?
- **_Sausage-shaped_** upper abdominal mass (**_RUQ_**)
- Emptiness in the RLQ (**_Dance sign_**)
- Distented abdomen
- Abdominal tenderness and other peritoneal signs
-----------------------------------------------------------------------------------
***Patients usually have no classic signs and symptoms***
Localized peritonitis suggests transmural ischemia
Onset often preceded by an URTI
173
**Any child with bilious vomiting is assumed to have a condition that MUST BE TREATED _SURGICALLY_ until proven otherwise.**
174
What investigations are indicated in a child suspected to have intussusception?
* *_Blood tests_**
- CBC: leukocytosis if gangrene present
- RFT: vomiting and dehydration
- Clotting, T&S and X-match for possible surgery
**_Imaging_**
- AXR
\>\> Dilated small bowels
\>\> Paucity of gas in RUQ and RLQ
\>\> If distension is generalized with colonic air-fluid levels, it is more suggestive of acute GE
- Ultrasonography
\>\> Target sign
\>\> Pseudokidney sign
- CT scan
\>\> Ying-yang sign
175
The ying-yang sign on CT in a patient with intussusception
176
How is intussusception managed?
Depends on the patient's condition
## Footnote
**_- Patient stable: nonoperative treatment_**
\>\> Pneumatic reduction: air-contrast enema/air
\>\> Hydrostatic reduction: barium/water-soluble contrast
\>\> Under fluroscopic/ultrasonographic guidance
\>\> A patient who becomes asymptomatic after reduction can be safely observed
\>\> Traditionally, an attempt is considered successful only until the reducing agent was observed refluxing back into the terminal ileum
**_- Peritonitis present: surgery_**
\>\> Strict indications
:: Peritonitis
:: Signs of perforation on AXR
:: Nonoperative reduction fails
\>\> Usually by right paraumbilical incision
\>\> Manual reduction should be tried
\>\> If irreducible, end-to-end anastomosis
\>\> Search for lead points, esp. in \>2-3 years old
177
Patients aged 5 months to 3 years who have intussusception rarely have a lead point -- idiopathic.
They are usually RESPONSIVE to NONOPERATIVE/pneumatic reduction.
178
In children with successful nonoperative reduction, allow **_2 weeks_** for resolution fo edema.
Follow-up with a **_contrast small bowel follow through_** (SBFT) to rule out pathologic lead points.
179
What is the commonest type of tracheoesophageal fistula?
Type C: fistula with proximal blind-end esophagus (86%)
Type A: 8%
Type E: 4%
Type D: 1.4%
Type B: \<1%
180
50% of TE fistulae have associated anomalies (VACTERL).
181
What is the VACTERL association?
Non-random co-occurance of birth defects
## Footnote
- **V**ertebral anomalies (hemivertebra/butterfly vertebra)
- **A**nal atresia
- **C**ardiac defects (VSD/ASD/TOF)
- **T**racheo**E**sophageal fistula
- **R**enal and **R**adial abnormalities
- **L**imb defects (hypoplastic thumb, polydactyly)
182
How do TE fistulae present?
Depends on type
## Footnote
- Antenatal polyhydramnios
- Non-bilious vomiting
- Coughing and cyanosis during feeding
- Respiratory distress
- Recurrent pneumonia
- ***Frothy bubbles of mucus in mouth and nose despite continuous suctioning***
183
What is the most common/significant X-ray finding for TE fistulae?
Curling of NG tube in the pouch
## Footnote
Other findings:
- Gas in the stomach
- Aspiration pneumonia
184
How are TE fistulae treated?
**Early repair by surgical ligation**
## Footnote
- In healthy infants without pulmonary complication, primary repair is performed within **first few days of life**
- Delay surgical repair in patients with:
\>\> Low birth weight
\>\> Pneumonia
\>\> Other congenital abnormalities
_Medical treatment before surgery_
- Upper pouch suction (to prevent aspiration)
- Elevate head of bed
- Gastrostomy (to minimize reflux)
- Jejunostomy feeding tube/Parenteral nutrition
- Cuffed endotracheal intubation placed distal to fistula site to prevent reflux of gastric contents into lungs
185
For those with TE fistulae, most infants are recommended to undergo primary care, but a staged repair several *_weeks_* following birth is recommended for preemies and those with severe respiratory distress syndrome (RDS).
Tracheostomy is required only if planning a staged repair.
186
Infants with TE fistuale and severe RDS may require the use of a Fogarty balloon catheter to obliterate the TEF while awaiting surgery.
187
What are the possible complications of an untreated TE fistula?
- Pneumonia
- Sepsis
- Reactive airway disease
- Failure to thrive
188
What are the possible long-term post-operative complications in patients with TE fistulae?
- Recurrence
- Tracheal stenosis
- Esophageal strictures and stenosis
- GERD
- Poor esophageal motility
\>\> Esophagitis
\>\> Barrett's esophagus
\>\> Hiatal hernea
189
What is biliary atresia?
A **progressive**, idiopathic/multifactorial, fibro-obliterative disease of the **extrahepatic** biliary tree presenting with biliary obstruction exclusively in the neonatal period
190
**Biliary atresia is the most common cause of neonatal jaundice for which surgery is indicated, and the most common indication for liver transplant in children.**
191
How common is biliary atresia?
1 in 10,000-15,000 live births
192
How can we classify biliary atresia?
* *_Isolated/Postnatal biliary atresia_**
- 65-90% of cases
* *_Fetal/Embryonic biliary atresia_**
- 10-35% of cases
- Associated with situs invertus, polyspenia or asplenia
- With or without other congenital anomalies
------------------------------------------------------------------------------------
**Type I**: obliteration fo the common bile duct with patent proximal ducts
**Type II**: atresia of the common hepatic duct with cystic structures found in the porta hepatis
**Type III**: MOST COMMON; atresia of left and right hepatic ducts to the level of the porta hepatitis -- not to be confused with intrahepatic biliary hypoplasia
193
How does biliary atresia present?
Most infants are full term and perfectly healthy up to point of presentation
## Footnote
- Jaundice
\>\> First symptom/sign
\>\> Occurs anytime from birth up to 8 weeks of life
\>\> Acholic stools and tea-coloured urine
- Firm and enlarged liver
- Splenomegaly
194
What investigations are indicated in patients with prolonged obstructive neonatal jaundice, suspicious of biliary atresia?
**_Blood tests/Urine_**
- CBC, d/c: leukocytosis may indicate infectious cause
- RFT: pre-op baseline
- LFT
\>\> Total and direct bilirubin
:: Direct bilirubin elevated to \>20% of total bilirubin
:: Indicates conjugated hyperbilirubinemia
\>\> ALT
\>\> ALP
\>\> GGT
- Clotting profile
\>\> Coagulopathy likely due to vitamin K deficiency
\>\> Also for pre-op baseline
- Assays to rule out metabolic diseases
\>\> Serum AAT
\>\> Urinalylsis x reducing agents etc.
\>\> TORCH infection screening
\>\> Hepatitis serology
**_Imaging_**
**- Abdominal ultrasound**
\>\> To rule out choledochal cysts
\>\> Signs suggestive of biliary atresia
:: "Triangular cord sign"
~~ Triangular echogenic density
~~ Just above the porta hepatis
~~ Fibrotic tissue replacing the hepatic duct
:: Gallbladder -- abnormal size and shape
:: Absence of common bile duct
**- Hepatobiliary scintigraphy**
\>\> IV injection of mebrofenin
\>\> Need for phenobarbital controversial
:: 5mg/kg/day for 5 days
:: Induces hepatic enzymes
:: Increases biliary secretion of radiotracers
:: Prevents false-positive -- patent biliary system but poor excretion
\>\> To assess for patency of biliary system
\>\> Dynamic images in first hour
\>\> Delayed images at 4 hours and 24 hours
**- Cholangiogram**
\>\> Intraoperative cholangiogram: gold standard
\>\> Patency should be investigated both proximally into the liver and distally into the bowel
\>\> If cholangiogram positive, proceed with Kasai HPE
* *_Histology: liver biopsy_**
- For all infants with suspected biliary atreia
- For two reasons
1. Identify histologic changes consistent with obstruction to warrant surgical exploration
2. Differentiate BA from other causes of intrahepatic cholestasis that would not require surgery
- Any evidence of obstruction mandates imaging and a definitive cholangiogram
195
What is the Kasai procedure?
HPE: hepatoportoenterostomy
## Footnote
- To restore bile flow from liver to proximal small bowel
- Excision of biliary remnant and portal fibrous plate
- Roux-en-Y loop directly anastomosed to the hilum of the liver
196
Revision of a non-functioning HPE is generally not recommended -- if it didn't work the first time, it won't work the second time!
197
Many patients, despite a successful Kasai procedure, will have slowly progressive liver disease. Thus, **the vast majority of individuals with BA will eventually require liver transplantation.**
198
The function of HPE is to alleviate the need for liver transplant in the selected few, and delays transplant for most patients -- pre-emptive transplant is avoided due to the advantages of transplanting older and larger patients.
199
What are the specific complications of the Kasai procedure?
**_- Unsuccessful anastomosis_**
\>\> Fail to achieve adequate bile drainage
\>\> Occurs in up to 1/3rd of patients
**_- Ascending cholangitis (40-90%)_**
\>\> Most have at least one episode \<2 years
\>\> Due to abnormal anatomy and bacterial stasis on the roux limb
\>\> Prevention: prophylactic ABx in 1st year of life
\>\> Recurrent cholangitis may predict the need for liver transplant with progression to cirrhosis
**_- Portal hypertension_** (part of the natural course)
**_- Fat-soluble vitamin deficiencies_**
200
What are the indications for liver transplant in a child with biliary atresia?
- Primary failure of Kasai HPE
- Refractory growth failure
- Complications of portal hypertension
- Progressive liver dysfunction
\>\> Intractable pruritus
\>\> Refractory coagulopathy
201
Neonatal hepatitis and biliary atresia account for 70-80%, and alpha-1 antitrypsin deficiency accounts for 5-15% of cases of conjugated hyperbilirubinemia in **term** infants.
TPN and sepsis is the major cause of cholestasis in **preterm** infants.
202
Infants with cholangitis often only presents with high fever with no other symptoms. Therefore, in a febrile child with a history of biliary atresia +/- Kasai procedure, **treat as cholangitis until proven otherwise to prevent progressive cirrhosis and thus liver failure.**
203
What are choledochal cysts?
Congenital cystic/fusiform dilatation of the bile duct
204
How are biliary/choledochal cysts classified?
**Todani's classification**
## Footnote
I: dilation of hepatic duct and CBD (most common)
II: CBD diverticulum
III: Intraduodenal CBD dilation
IV: intrahepatic and extrahepatic biliary ductal dilation
V: intrahepatic biliary ductal dilation
205
What investigations are warranted in a child with suspected choledochal/biliary cyst?
- Blood test x work-up of conjugated hyperbilirubinemia
- Imaging
\>\> USG
\>\> **_MRCP_**
\>\> ERCP
\>\> Intraoperative cholangiogram
206
How are choledochal/biliary cysts treated?
- Complete excision of the cyst + GB + intrahepatic BDs
- Roux-en-Y hepaticojejunostomy if \>= Todani Type 4
