Common Pediatric & Neonatal Surgical Emergencies

Tariq Altokhais

Overview

Neonatal	Pediatric
Malrotation/midgut volvulus	Malrotation/midgut volvulus
Pyloric stenosis	Intussusception
Hirschsprung’s disease	Hirschsprung’s disease
Esophageal atresia
Intestinal atresia/obstruction/NEC
Anorectal malformation (imperforate anus)	Acute appendicitis
Congenital Diaphragmatic Hernia	Acute scrotum

1. Acute Appendicitis

Acute appendicitis → Same presentation and management as in adult

Most common cause of acute surgical abdomen in children and adolescents
Peak incidence 10-12 years
Increased rate of perforation in children

2. Malrotation / Midgut Volvulus

If not treated, will be

Intestinal Rotation (Embryology)

Normal rotation of the intestine requires transformation from a simple, straight alimentary tube into the mature fixed and folded configuration normally present at birth.

Normal Midgut Rotation

Normal midgut rotation:
- 4-12 weeks of fetal life
- 270 counterclockwise movement of the midgut

Rotation – Midgut (Hindgut Derivatives)

Also note hindgut derivatives

C: caecum
A: appendix
V-I-D: vitellointestinal duct

Malrotation (Incomplete Rotation)

FIGURE 31-3 Incomplete rotation. Both the prearterial (lightly shaded) and postarterial (darkly shaded) segments have undergone partial, yet not complete, rotation. Ladd’s bands are seen attaching the cecum to the right posterior abdominal wall. The duodenum becomes compressed and possibly obstructed. Volvulus is a risk.

Duodenum and small bowel go down, and cecum is up, and there is a band → this band normally attaches cecum to abdominal wall so it is fixed
In malrotation, the band goes up, and when cecum expands, it pulls the bands which cause obstruction of duodenum

What Is the Problem with Malrotation?

Ladd’s band obstructing the duodenum >> bilious vomiting
Midgut volvulus (twisting) >> ischemia and bowel gangrene
If whole bowel got twisted multiple times and there is delayed diagnosis (of duodenum till transverse colon)
If not identified quickly >> patient will die due to septic shock
If the patient does not die >> entire bowel loss (short bowel syndrome) – not compatible with life

Presentation

75% present in the first month of life
15% in the first year

Cardinal symptom:

BILIOUS VOMITING → dark green (extremely important)

Other symptoms:

Abdominal pain
Scaphoid abdomen (initially)
Distended abdomen (late)
Rectal blood (not always)

The younger, the more common to get malrotation

30% present in the 1st week of life
+30% → 60% present in the 1st month of life
+30% → 90% present in the 1st year of life
10% present at any age

Physical Examination (P/E)

Lethargic
Dehydrated
Tachycardia, hypotension, +/- fever
Distended upper abdomen (stomach)
Distended abdomen (late)
Peritonitis (late)
Abdominal wall erythema (late)

Investigations

Abdominal X-ray: → non-specific
- Can be totally normal
- Dilated stomach
Upper GI contrast study (oral contrast):
- The most sensitive test to diagnose malrotation (gold standard)
- Takes time (should not be done if the patient has a picture of bowel ischemia)
- If not sure about the diagnosis → e.g. color of vomit is not dark or repeated vomiting led to bile reflux; do it if there is time and if the condition is stable enough

Upper GI Contrast Study – Duodenal Configurations

C Shafes: Duodenum

Normal Position of Duodenum

Nonrotation of Duodenum

Corkscrew Duodenum and Jejunum

Partial Duodenal Rotation with Jejunum in Right Upper Quadrant

Partial Duodenal Rotation with Duodenojejunal Junction over Right Pedicle

Redundant Duodenum – Malrotation to Right of Spine

Contrast Study – Normal vs Malrotation

Normal anatomy of the duodenum:
- Duodenal cap
- Duodenal loop
- C-shaped duodenum

Anything different than this is a malrotation

Abdominal X-ray Findings

Duodenum and jejunum on right side

Treatment

Intravenous fluid → rehydrate
NPO
NGT
Broad spectrum antibiotics
Immediate surgical consultation
Urgent Ladd’s procedure:
- Laparotomy:
  - ① Untwist the bowel (derolate)
  - Resect gangrenous intestines
  - ② Divide Ladd’s band → so duodenum will be free
  - ③ Place the bowel in non-rotation position (small intestine on the right, large intestine on the left of the abdomen)
  - Do appendectomy at the same time → because if later on gets appendicitis, will have different presentation

Ladd’s Procedure

Delayed Diagnosis / Management

Bowel gangrene
Short bowel syndrome → stay on TPN until they die
Septic shock
Multi-organ failure
Death
(Bowel transplantation is difficult to find donor)
Prevention is key in these cases

3. Intussusception

In case of delayed presentation, child will present with ischemia, require resection and anastomosis

Part of the intestine telescopes into the distal bowel by peristaltic activity
Bowel is compressed > venous obstruction > bowel wall edema > arterial insufficiency > ischemia & bowel wall necrosis

Causes

3m-3y: idiopathic – 95% (Peyer’s Patch)
>3y: pathologic lead point – 4% Meckel diverticulum
- Polyp
- Neoplasm
- Inverted appendix
- Celiac disease, CF, HSP
- Feeding tubes (GJ tube)
Something leading bowel to go inside & any mass
1-2 weeks or even month before episode of intussusception

Inflammation goes down to LN of the same lymphatic tissue of glodomen (Peyer’s patch)
Swollen, so pulls the bowel, leading to intussusception

Presentation

Typical age: 3 months-3 yrs
Episodic colicky abdominal pain:
- Every 1-2 hours
- Every part of the child is flexed during the attack
Child is well between the attacks
Bilious vomiting
Red currant jelly stool
Sausage-shaped abdominal mass in RUQ (65% of cases)
Empty RLQ

When bowel goes inside, part of bowel gets congested (venous congestion) so mucosa will slough off and go in stool; stool is mixed with mucosa.

Investigations

① AXR: RUQ mass, dilated bowel, air/fluid levels → not specific

② US: gold standard, target sign
- Los Asueda kidney shape

Management

NPO
IVF
Reduction enema: under fluoroscopic guidance (done by a radiologist)
- Successful in >75% of cases → if failed, do surgical reduction (depending on presentation and duration)
- By fluid, contrast, or by air, under controlled pressure
- Insert enema by Foley’s catheter or any catheter and see progression under x-ray fluoroscopy

Surgical reduction: if enema reduction is not successful or not available
- → Laparoscopy

Reduction (Enema Technique)

Should continue contrast until completely reduced

Water-soluble contrast: → gastrografin ↓ barium

Air contrast

4. Hypertrophic Pyloric Stenosis

Gastric outlet obstruction due to hypertrophied pyloric muscle

The pylorus opens to let food out of the stomach into the small intestine

With pyloric stenosis, thickened muscle around the pylorus prevents food from leaving the stomach

Classic Triad

Term baby
2-8 weeks of life → unknown if congenital or acquired
Projectile, non-bilious vomiting (not as emergency as malrotation)
- May be progressive, that’s why it doesn’t present right after birth
- → Color is white due to milk

Olive sign – can be felt by palpation

Barejo’s 2013

Projectile Vomiting

Clinical Features & Laboratory Findings

Hungry & dehydrated baby
Palpable pyloric muscle (Olive sign)
Gastric peristalsis can sometimes be seen

Electrolytes + blood gas:

Hypochloremia
Hyponatremia
Hypokalemia
Metabolic alkalosis

Imaging

① X-ray → will not show anything
② US: gold standard
- Thick and elongated pyloric muscle
- Diagnosis depends on measurement of the thickness

Thickened pylorus

Management

NPO
IVF
Correct electrolytes and pH
Surgical separation of pyloric muscles (pyloromyotomy) – no excision

Pyloromyotomy

The pylorus opens to let food out of the stomach into the small intestine
With pyloric stenosis, thickened muscle around the pylorus prevents food from leaving the stomach

Spread pyloric muscles to see → mucosa

5. Esophageal Atresia (Congenital; with or without Tracheoesophageal Fistula)

Interruption in the continuity of the esophagus with or without fistula
Caused by error in separation of esophagus & trachea (4th week gestation)
1:4500 live birth
Male > female
50-60% associated anomalies
- VACTERL: Vertebral, Anal, Cardiac, TracheoEsophageal, Renal, Limb

Types of Esophageal Atresia

Proximal pure esophageal atresia (no fistula): 5-10%
Type 2/A
Type 3a/B
Type 3b/C: most common type (>85%); distal is altered to trachea (tracheoesophageal fistula)
- No gas = no fistula
Type 4/E

Presentation

Prenatal:

Polyhydramnios
- → Fetus doesn’t swallow the amniotic fluid, so it accumulates

Postnatal:

Drooling
Vomiting
Respiratory distress
Resistance upon insertion of NGT (coiled in the chest)
Choking and aspiration (milk goes to trachea)

Part of routine neonatal exam: should rule out choanal atresia or obstruction by inserting NGT → do it before giving 1st milk; if there is resistance → x-ray

→ Coiled NGT ↓ esophageal atresia until proven otherwise

Management

Urgent surgery → separate esophagus from trachea and connect the esophagus
Right thoracotomy
Division of fistula
Repair esophagus

6. Congenital Diaphragmatic Hernia

Easy to diagnose and manage

7. Imperforate Anus – Ano-Rectal Malformation (ARM)

Various forms of anorectal anomaly
Normal anus:
- Normal size
- Location at the center of the muscle sphincter
1:4000
Male > female
65% has associated anomalies (VACTERL)
- Vertebral, anorectal, Cardiac, Tracheoesophageal, renal, limbs
- If you have one of these anomalies, you should look for the remaining other anomalies
→ Should look for other anomalies; types differ in male and female

Presentation

Abdominal distension and vomiting (rectum is closed)
But shouldn’t wait for this – in neonatal exam, should look for anus to make sure it’s in normal location and size

Types of Anorectal Malformation

Sex	Type	Notes
Female	Perineal fistula
Female	Vestibular fistula
Female	Persistent cloaca	<3 cm common channel / >3 cm common channel
Female	Imperforate anus without fistula
Female	Rectal atresia
Male	Perineal fistula
Male	Rectourethral fistula	Bulbar / Prostatic
Male	Rectovesical fistula
Male	Imperforate anus without fistula
Male	Rectal atresia

Vestibular Fistula (or Rectovaginal Fistula)

No complete obstruction like other parts
Do anoplasty at age 6 weeks - 2 months (without colostomy)
Rectum should open here, but here it opens just beside the vagina and the rectum shares a hole with the vagina (one hole for both) → seen by physical examination
Called vestibular anus because anus opens in vestibule

Seen by physical examination

FIGURE 35-7 ■ (A) Schematic drawing of a rectovestibular fistula. (B) Female neonate with a rectovestibular fistula is in the prone position. The rectal fistula (arrow) is located in the posterior aspect of the vestibule.

Perineal Fistula

Close to normal site → do anoplasty
Anus should open here, in the perineum

FIGURE 35-2 ■ This male infant has a rectoperineal fistula with a subepithelial tract filled with either mucus or meconium that extends into the scrotal raphe.

Rectourethral Fistula (Male)

Most common in boys
Presentation: stool in urine
There is a single wall between rectum and urethra, and doing anoplasty immediately after birth can injure the urethra
Opens in urethra, and there is no anus → far from normal site
→ Do colostomy and after 3-6 months do anoplasty

Surgical Options

Primary Anoplasty

If rectum is close to skin

Low type → better prognosis
X-ray to decide which surgery to do: child in prone position, elevate pelvis, and do lateral X-ray while putting marker at site of anus, and see the gas of the rectum

Colostomy with Delayed Anoplasty

If rectum is far from skin

High type → usually have incontinence
They don’t have enough fibers and muscles to contract – as part of the disease, not part of the surgery

8. Hirschsprung’s Disease

→ Functional disease, not anatomical

Absence of ganglion cells in the myenteric & submucosal plexus of the intestine
- → Absence of parasympathetic → overacting of sympathetic → bowel contracting all the time
Arrest in ganglion cells migration from proximal to distal part of GI tract
Transition zone (TZ): area between normal & aganglionated bowel
Types:
- 80% - rectosigmoid TZ
- 10% - proximal colon TZ
- 5% - total colonic HD
- Rare - near total intestinal HD

Presentation

Neonatal:

Delayed passage of meconium >24hr
Bilious vomiting → picture of obstruction
Abdominal distension
Feeding intolerance

Delayed:

Chronic refractory constipation
Abdominal distension
Failure to thrive

When doing PR exam (with cotton tip or temp probe): gush of stool and gas due to stimulation of the area

Diagnosis

Rectal manometry: absent recto-anal inhibitory reflex
Contrast study (enema)

Then, to confirm:

Rectal biopsy:
- Gold standard
- → Bedside biopsy (suction biopsy) → no general anesthesia, it’s not painful
- → Look for ganglion cells; if no ganglion cells → reach diagnosis (histopathological diagnosis)
- Look for ganglion cells in the myenteric & submucosal plexus
- (This is the only disease where it’s asked for absence of cells)