Malrotation and Volvulus (Child)
Summary
Intestinal malrotation is a Congenital Anomaly of Midgut Rotation and Fixation occurring during embryological development, resulting in an Abnormal Position of the Small and Large Bowel within the abdominal cavity. The critical consequence of malrotation is the predisposition to Midgut Volvulus, a life-threatening Twisting of the Bowel Around the Superior Mesenteric Artery (SMA) Axis, which can cause Intestinal Ischaemia, Necrosis, and Death within hours if not surgically corrected. Bilious (Green) Vomiting in a Neonate is a Surgical Emergency Until Proven Otherwise and must be presumed to be malrotation with volvulus. Most presentations occur in the First Month of Life (>50%), with 75-90% presenting in the First Year. Diagnosis requires Urgent Upper GI Contrast Study showing abnormal position of the duodenojejunal junction (DJ Flexure). Treatment is Emergency Laparotomy with Ladd's Procedure – Detorsion of volvulus, Division of Ladd's bands, Widening of mesenteric base, Appendicectomy, And placement of bowel in non-rotated position. Delay in treatment leads to Extensive Bowel Necrosis, Short Gut Syndrome, and Death. [1,2,3]
Key Facts
| Fact | Value |
|---|---|
| Definition | Abnormal rotation/Fixation of midgut during development |
| Incidence | 1:500-1:6000 live births |
| Symptomatic Malrotation | 1:6000 |
| Peak Presentation | First month of life (>50%) |
| Key Symptom | Bilious vomiting |
| Diagnosis | Upper GI contrast study |
| Treatment | Emergency Ladd's Procedure |
| Mortality (With Volvulus + Delay) | >25% if necrosis |
Clinical Pearls
"Bilious Vomiting in a Neonate = Malrotation Until Proven Otherwise": Life-threatening emergency.
"Clock is Ticking": Volvulus causes ischaemia within hours. Minutes matter.
"Normal Abdominal X-Ray Does NOT Exclude": Upper GI contrast is gold standard.
"DJ Flexure Should Be to the Left of Spine at L2": Abnormal position diagnostic on UGI.
"Ladd's Bands Cause Duodenal Obstruction": Even without volvulus.
Why This Matters Clinically
Malrotation with midgut volvulus is one of the most time-critical paediatric surgical emergencies. A delay of even a few hours can result in loss of the entire midgut, Leading to lifelong dependence on parenteral nutrition or death. Every clinician caring for neonates and children must recognise bilious vomiting as a red flag requiring immediate investigation and surgical consultation. This condition is heavily tested in paediatric and surgical examinations due to its high stakes and classic presentation.
Global Burden
| Metric | Data | Notes |
|---|---|---|
| Incidence (Autopsy Studies) | 1:500 live births | Most asymptomatic |
| Symptomatic Malrotation | 1:6000 live births | Clinically significant |
| Surgical Emergency Rate | ~5-10% of neonatal surgical emergencies | Major cause of neonatal surgical pathology |
| Mortality (With Volvulus) | 3-35% | Depends on timing of treatment |
Incidence & Prevalence by Age
| Age at Presentation | Percentage | Clinical Notes |
|---|---|---|
| First Week of Life | 25-30% | Highest risk period |
| First Month of Life | 50-60% | Majority present here |
| First Year of Life | 75-90% | Cumulative presentation |
| 1-10 Years | 5-15% | May have intermittent symptoms |
| Beyond 10 Years/Adults | 5-10% | Chronic symptoms, Incidental finding, Late volvulus |
Demographics
| Factor | Details | Clinical Significance |
|---|---|---|
| Sex | Male:Female = 2:1 (Symptomatic) | Males more likely to present |
| Equal | Asymptomatic malrotation equal | |
| Prematurity | Higher incidence | Often incidental finding during other surgery |
| Low Birth Weight | Associated | Similar to prematurity |
| Ethnicity | No significant difference | All populations affected |
Risk Factors for Volvulus
| Factor | Risk Level | Mechanism |
|---|---|---|
| Narrow Mesenteric Pedicle | High (Anatomical) | Primary abnormality predisposing to twist |
| Ladd's Bands | High | Cause obstruction, May precipitate volvulus |
| Overfeeding | Possible | Distension may trigger volvulus |
| Age Less than 1 Year | High | Peak presentation |
| Unknown Triggers | Variable | Many present without clear precipitant |
Timing of Presentation
| Timing | Clinical Implications |
|---|---|
| Prenatal Diagnosis | Rare. May see dilated bowel or polyhydramnios (Associated anomalies). |
| First 24 Hours | May mimic other causes of bilious vomiting |
| First Week | Peak emergency presentations |
| First Month | Most common window |
| Late Presentation | May have had intermittent symptoms. Often more chronic presentation. |
Associated Congenital Anomalies
| Anomaly | Association with Malrotation | Clinical Implications |
|---|---|---|
| Duodenal Atresia | 30% have malrotation | Screen during repair |
| Jejunoileal Atresia | Associated | |
| Omphalocele | Very High (Contents never returned normally) | All omphalocele patients have malrotation |
| Gastroschisis | Universal malrotation | By definition |
| Congenital Diaphragmatic Hernia (CDH) | 40-50% | Due to herniated bowel |
| Heterotaxy Syndromes (Asplenia/Polysplenia) | Very High | Abnormal laterality affects gut rotation |
| Hirschsprung's Disease | Associated | |
| Oesophageal Atresia | Associated | Part of VACTERL |
| Cardiac Defects (Heterotaxy) | Associated | Shared embryological laterality defect |
Mortality and Morbidity Epidemiology
| Scenario | Mortality | Morbidity |
|---|---|---|
| Malrotation without volvulus | Less than 1% | Low |
| Volvulus - Early diagnosis (Less than 4 hours) | 3-5% | Low - Minimal resection |
| Volvulus - Delayed diagnosis (4-24 hours) | 10-15% | Moderate - Some resection |
| Volvulus - Late diagnosis (Greater than 24 hours) | 25-35% | High - Extensive/Total necrosis, Short gut |
| Total midgut necrosis | ~100% | Incompatible with survival or TPN-dependent |
Key Epidemiological Messages
| Message | Implication |
|---|---|
| Most malrotation is asymptomatic | Only ~1:6000 present symptomatically |
| Peak presentation in first month | Must maintain high index of suspicion in neonates |
| Males more commonly affected | Symptomatic presentation |
| High association with other anomalies | Screen for malrotation when treating associated conditions |
| Timing is critical for outcome | Minutes to hours determine prognosis |
Normal Midgut Development
Week 4-5:
- Midgut forms as a U-shaped loop attached to yolk sac
- Supplied by Superior Mesenteric Artery (SMA)
Week 6-10 (Physiological Herniation):
- Midgut herniates into umbilical cord (Extra-embryonic coelom)
- Total 270° Anticlockwise Rotation around SMA axis occurs:
- Stage 1: 90° – Proximal limb rotates inferior
- Stage 2: 90° – Herniated loops rotate
- Stage 3: 90° – On return to abdomen
Week 10-12 (Return and Fixation):
- Midgut returns to abdominal cavity
- Caecum descends to right iliac fossa
- Duodenojejunal (DJ) Flexure fixes to left of midline at Ligament of Treitz (L1-L2)
- Caecum and ascending colon fix to right posterior abdominal wall
- Broad mesenteric base from DJ flexure to caecum (Protects against volvulus)
Malrotation (Abnormal Development)
Failure of Normal Rotation and Fixation:
| Type | Description | Consequence |
|---|---|---|
| Non-Rotation | 180° rotation only. Small bowel on right, Colon on left. | Narrow mesenteric pedicle. Volvulus risk. |
| Incomplete Rotation | Variable degrees of incomplete rotation. | Most common. Abnormal DJ position. Ladd's bands. |
| Reversed Rotation | Rare. Transverse colon behind SMA. | Duodenal obstruction. |
| Paraduodenal Hernia | Internal hernia. | Intermittent obstruction. |
Pathophysiology of Volvulus
Step 1: Anatomical Abnormality
- Narrow Mesenteric Pedicle: Due to failure of normal fixation, The entire midgut hangs from a narrow stalk containing the SMA
- Ladd's Bands: Peritoneal bands from malpositioned caecum crossing duodenum, Causing external compression
Step 2: Volvulus (Twisting)
- Midgut twists around the narrow SMA pedicle
- Usually Clockwise twist (When viewed from below)
- Typically 180-720° rotation
Step 3: Vascular Compromise
- Venous Obstruction First: Congestion, Oedema
- Arterial Obstruction: Complete ischaemia
- SMA Occlusion: Blood supply to entire midgut (DJ flexure to mid-transverse colon) compromised
Step 4: Ischaemia and Necrosis
- Irreversible Ischaemia: Develops within 2-4 hours
- Transmural Necrosis: Bowel wall dies
- Perforation: Peritonitis
- Sepsis and Shock: Multiorgan failure
Step 5: Without Intervention
- Death: From sepsis, Shock, Massive fluid/Electrolyte loss
- Survival with Extensive Resection: Short Gut Syndrome, TPN dependence
Key Principle
[!IMPORTANT] Bilious (Green) Vomiting in a Neonate = Malrotation with Volvulus Until Proven Otherwise
This is one of the most time-critical presentations in paediatric surgery. The clock starts ticking the moment volvulus occurs. Do not wait for definitive imaging if clinical suspicion is high.
Classic Presentation: Neonate with Bilious Vomiting
| Feature | Details | Significance |
|---|---|---|
| Bilious (Green) Vomiting | Cardinal Sign. Green/Yellow bile-stained vomit. | Obstruction distal to Ampulla of Vater. Surgical emergency. |
| Age | Usually first days to weeks of life | Peak in first month |
| Previously Well | May have been feeding normally initially | Sudden deterioration |
| Feeding Intolerance | Unable to tolerate feeds, Vomits every feed | Intestinal obstruction |
| Abdominal Distension | Variable. May be minimal initially. | High obstruction → Less distension |
| Irritability | Baby unsettled, Crying | Pain or discomfort |
Symptom Severity Staging
| Stage | Clinical Features | Time from Volvulus | Urgency |
|---|---|---|---|
| Stage 1: Early Obstruction | Bilious vomiting, Feeding intolerance. Baby may still look well. Minimal distension. | 0-2 hours | URGENT - Best outcome window |
| Stage 2: Developing Ischaemia | Increasing distension, Tenderness, Tachycardia, Irritability/Lethargy | 2-4 hours | CRITICAL - Ischaemia developing |
| Stage 3: Established Ischaemia | Bloody stools, Rigid abdomen, Shock (Poor perfusion) | 4-8 hours | DIRE - Necrosis occurring |
| Stage 4: Bowel Necrosis/Shock | Peritonitis, Hypotension, Metabolic acidosis, Sepsis, MOF | Greater than 8 hours | CATASTROPHIC - High mortality |
Detailed Symptom Analysis
Bilious Vomiting:
| Aspect | Details |
|---|---|
| Colour | Green (Bile) or Yellow-Green |
| Timing | Any vomit after feeds, May be projectile |
| Volume | Can be large. Contains gastric contents + bile. |
| Differentiation | Milk vomit = White/Curdy. Bile = Green. Some conditions cause yellow only. |
| Significance | Obstruction distal to Ampulla of Vater (Where bile duct enters). Proximal obstruction (Pylorus) = Non-bilious. |
Abdominal Distension:
| Finding | Significance |
|---|---|
| Minimal/Scaphoid | Expected early. High obstruction (Duodenum) does not produce much distension. Do NOT be reassured. |
| Upper Abdominal Fullness | Dilated stomach and proximal duodenum |
| Generalised Distension | Suggests more distal obstruction or developing ileus |
| Tense Distension | Suggests significant gas/Fluid accumulation |
Bloody Stools:
| Description | Significance |
|---|---|
| "Redcurrant Jelly" | Blood and mucus. Ischaemic bowel shedding mucosa. |
| Fresh Blood | Late sign. Mucosal necrosis. |
| Melaena | Upper GI bleed (Rare in this context) |
| Timing | Late sign - Indicates necrosis has occurred |
Symptoms by Timeframe
First 2 Hours (Early):
2-4 Hours (Intermediate):
4-8 Hours (Late):
Beyond 8 Hours:
Examination Findings - Detailed
| Finding | Early | Intermediate | Late | Very Late |
|---|---|---|---|---|
| Distension | Minimal | Increasing | Generalised | Tense |
| Tenderness | None | Developing | Marked | Generalised |
| Guarding | None | None | Developing | Rigid |
| Bowel Sounds | Normal/High | Reducing | Absent | Absent |
| Capillary Refill | Less than 2s | 2-3s | Greater than 3s | Prolonged/Mottled |
| Heart Rate | Normal | Tachycardia | Marked tachycardia | Bradycardia (Pre-terminal) |
| Blood Pressure | Normal | Normal/Low | Low | Very low |
| Urine Output | Normal | Decreased | Oliguria | Anuria |
Red Flags - Comprehensive
[!CAUTION] SURGICAL EMERGENCY - Act Immediately:
- Bilious (Green) Vomiting in ANY Neonate - Until proven otherwise = Malrotation
- Abdominal distension with vomiting
- Bloody stools in neonate with vomiting
- Signs of shock (Tachycardia, Poor perfusion, Hypotension)
- Peritonitis (Rigid, Tender abdomen)
- Metabolic acidosis (Raised lactate)
- Abdominal wall erythema/Discolouration
- Feeding intolerance in first weeks of life
Differential Diagnosis
| Condition | Distinguishing Features |
|---|---|
| Malrotation + Volvulus | Bilious vomiting, Sudden onset, May appear well initially then deteriorate |
| Duodenal Atresia | Bilious vomiting, "Double bubble" on AXR, Often prenatal diagnosis, Stable |
| Jejunoileal Atresia | Bilious vomiting, More distension, Prenatal polyhydramnios |
| Meconium Ileus | Distal obstruction, CF association, No meconium passed |
| Hirschsprung's Disease | Delayed meconium, Distal obstruction pattern, Usually non-bilious initially |
| Necrotising Enterocolitis (NEC) | Premature infant, Systemic sepsis, Pneumatosis on AXR |
| Pyloric Stenosis | Non-bilious vomiting (Projectile), 2-8 weeks, Palpable "Olive" |
| GORD | Non-bilious, Effortless, Well baby |
Atypical Presentations - Expanded
| Presentation | Clinical Features | Considerations |
|---|---|---|
| Older Child (1-10 years) | Intermittent abdominal pain, Vomiting episodes, Failure to thrive | Chronic intermittent volvulus |
| Adolescent/Adult | Chronic pain, Malabsorption, Weight loss | May present acutely |
| Chronic Symptoms | Recurrent abdominal pain, Post-prandial discomfort, Malnutrition | Often misdiagnosed for years |
| Asymptomatic | Found incidentally on imaging for other reasons | Debate about prophylactic surgery |
| Non-Bilious Initially | May start as clear/White then become bilious | Still investigate |
| In Setting of Other Anomalies | During repair of duodenal atresia, CDH, etc. | Screen for malrotation |
Key Principle
[!WARNING] Clinical examination may be deceptively reassuringly normal in early volvulus.
A baby with bilious vomiting may have a soft, non-distended abdomen in the early stages. Do NOT be falsely reassured. Investigate immediately.
Structured Approach
Immediate Assessment (ABCDE):
| Component | Assessment | Key Findings | Action |
|---|---|---|---|
| A - Airway | Is airway patent? | Should be clear unless aspiration | Airway manoeuvres if needed |
| B - Breathing | Rate, Effort, Saturations | Tachypnoea (Acidosis, Pain), Normal sats initially | Oxygen if needed |
| C - Circulation | HR, BP, CRT, Peripheral perfusion | Tachycardia, Prolonged CRT, Cool extremities = SHOCK | IV access x2, Fluid bolus |
| D - Disability | Conscious level, Glucose | Lethargy in shock, Hypoglycaemia possible | Treat hypoglycaemia |
| E - Exposure | Full examination | Abdominal findings, Temperature | Full abdominal exam |
Shock Assessment
| Parameter | Normal Neonate | Compensated Shock | Decompensated Shock |
|---|---|---|---|
| Heart Rate | 100-160 bpm | Greater than 160 | Greater than 180 or Less than 100 (Late) |
| Blood Pressure | 65-75/40-50 mmHg | Maintained | Hypotensive |
| Capillary Refill | Less than 2 seconds | 2-3 seconds | Greater than 4 seconds |
| Perfusion | Warm, Pink | Cool peripheries | Mottled, Cold |
| Urine Output | Greater than 1 ml/kg/hr | Reduced | Oliguria/Anuria |
| Consciousness | Alert | Irritable | Lethargic/Unresponsive |
| Lactate | Less than 2 mmol/L | 2-4 | Greater than 4 |
Abdominal Examination - Detailed
Inspection:
| Finding | Significance | Stage |
|---|---|---|
| Scaphoid/Flat | Early - High obstruction | Early |
| Mild Distension | Obstruction developing | Early-Intermediate |
| Moderate Distension | Established obstruction or ileus | Intermediate |
| Gross Distension | Late obstruction, Ileus | Late |
| Visible Peristalsis | High obstruction | Variable |
| Abdominal Wall Erythema | Underlying necrosis - Ominous | Very Late |
| Abdominal Wall Oedema | Peritonitis | Very Late |
Palpation:
| Finding | Significance | Action |
|---|---|---|
| Soft, Non-Tender | May be deceptively normal early | Do NOT be reassured |
| Mild Tenderness | Developing ischaemia | Urgent |
| Guarding | Peritoneal irritation | Very urgent |
| Rigidity | Peritonitis | Emergency |
| Mass | Rare | Consider other diagnoses |
Percussion:
| Finding | Significance |
|---|---|
| Tympany | Gas-filled bowel |
| Dullness | Fluid (Ascites) or solid |
| Shifting Dullness | Ascites (Peritonitis) |
Auscultation:
| Finding | Significance | Stage |
|---|---|---|
| Normal | May be normal early | Early |
| High-Pitched/Tinkling | Active obstruction | Early-Intermediate |
| Decreased | Developing ileus | Intermediate |
| Absent | Ileus, Peritonitis | Late |
Per Rectal Examination
| Finding | Significance |
|---|---|
| Normal Stool | May be normal early |
| Empty Rectum | Obstruction (No stool passing) |
| Bloody Stool | Ischaemic bowel - LATE SIGN |
| "Redcurrant Jelly" | Blood and mucus - Necrosis |
Special Considerations
| Finding | Interpretation | Action |
|---|---|---|
| "Deceptively Normal" Abdomen | Early volvulus may have minimal findings | Do NOT be reassured. Investigate. |
| Shock out of proportion to findings | Suggests significant ischaemia | Resuscitate and operate urgently |
| Abdominal wall erythema | Underlying necrosis – Very late sign | Emergency laparotomy |
| Bilious NG aspirates | Supports diagnosis | Confirms obstruction distal to ampulla |
| Well-appearing baby | Early presentation | Still an emergency |
Documentation Checklist
| Item | Record |
|---|---|
| Time of Onset | When symptoms started |
| Vomit Description | Colour (Green = Bile), Volume, Frequency |
| Last Feed | Tolerance |
| Bowel Opens | Meconium passed? Last stool? Colour? |
| Vital Signs | HR, RR, BP, Sats, Temp, CRT |
| Abdominal Findings | Distension, Tenderness, Bowel sounds |
| Shock Assessment | Compensated/Decompensated |
| Lactate | If available |
Key Principle
[!IMPORTANT] Do NOT delay surgery for investigations if clinical suspicion is HIGH
If a baby presents with bilious vomiting AND shock → Proceed directly to theatre. Investigations are confirmatory, not necessary if the clinical picture is clear.
Prioritisation
| Situation | Investigation Approach |
|---|---|
| Bilious vomiting, Stable baby | Urgent UGI contrast study |
| Bilious vomiting, Sick/Shocked baby | Resuscitate, May go straight to theatre |
| Stable, Query incidental malrotation | UGI can be scheduled (Not urgent) |
Emergency Investigations
Laboratory - Obtain Urgently:
| Test | Expected Findings | Clinical Significance |
|---|---|---|
| Blood Gas | pH low (Acidosis), pCO2 normal/Low, Base excess negative, Lactate HIGH | Ischaemia. Lactate greater than 4 = Significant ischaemia. |
| Lactate | Elevated (Greater than 2 = Concerning, Greater than 4 = Severe) | Key marker of ischaemia |
| FBC | WCC elevated (Or low in sepsis), Low Hb if bleeding | Non-specific but indicates response |
| U&Es | Na normal/Low, K variable, Urea/Creatinine high (AKI) | Dehydration, Third-spacing, Shock |
| LFTs | May be elevated | Can indicate shock to liver |
| Coagulation | PT/APTT prolonged (DIC) | Sepsis complication |
| Group & Save | Ready for surgery | Crossmatch if transfusion likely |
| Glucose | Low (Especially in neonates) | Treat hypoglycaemia |
Lactate Interpretation
| Lactate (mmol/L) | Interpretation | Action |
|---|---|---|
| Less than 2 | Normal | Reassuring, But doesn't exclude |
| 2-4 | Mildly elevated | Concerning. Suggests ischaemia developing. |
| Greater than 4 | Significantly elevated | Ischaemia likely. Urgent surgery. |
| Greater than 6-10 | Severely elevated | Established ischaemia/Necrosis. Emergency. |
Imaging - Detailed
Abdominal X-Ray:
| Finding | Description | Significance |
|---|---|---|
| Normal | No abnormality | Does NOT exclude malrotation |
| Paucity of Distal Gas | Gas only in stomach, Little beyond | High obstruction |
| Dilated Stomach | Large gastric bubble | Gastric outflow obstruction |
| Dilated Proximal Duodenum | Gas in duodenum | Duodenal level obstruction |
| "Double Bubble" | Gas in stomach + Proximal duodenum | Duodenal obstruction (Also duodenal atresia) |
| Free Air | Pneumoperitoneum | Perforation - Emergency |
| Fluid Levels | Air-fluid levels | Obstruction with ileus |
Upper GI Contrast Study (Gold Standard):
| Aspect | Details |
|---|---|
| Contrast Agent | Water-soluble (Gastrografin/Omnipaque) preferred. Barium if stable. |
| Procedure | Contrast given via NG tube. Fluoroscopy. |
| Key Structure | Duodenojejunal (DJ) Flexure position |
| Normal DJ Position | To the left of the left pedicle of spine at L1-L2 |
| Sensitivity | Greater than 95% for malrotation |
| Specificity | High |
UGI Findings - Interpretation:
| Finding | Normal | Malrotation | Volvulus |
|---|---|---|---|
| DJ Flexure | Left of spine at L2 | Right, Midline, or Low | Often not seen (Obstruction) |
| Duodenum Course | Crosses midline, Ascends | Abnormal course | "Corkscrew" appearance |
| Jejunum | Left upper quadrant | May be on right | Obstructed |
| Small Bowel Position | Left abdomen | May be on right | N/A |
Ultrasound:
| Finding | Description | Significance |
|---|---|---|
| "Whirlpool Sign" | SMV and mesentery wrapping around SMA | Pathognomonic for volvulus |
| Abnormal SMA/SMV Relationship | Normally SMV to right of SMA. In malrotation: SMV may be anterior, Left, Or posterior. | Suggests malrotation |
| Dilated Proximal Bowel | Gas/Fluid filled loops | Obstruction |
| Free Fluid | Ascites | Peritonitis, Shock |
Diagnostic Criteria Summary
| Test | Finding | Diagnostic Strength |
|---|---|---|
| UGI: DJ Flexure not to left of spine | Malrotation | Gold standard |
| UGI: Corkscrew duodenum | Volvulus | Strong |
| USS: Whirlpool sign | Volvulus | Pathognomonic |
| USS: SMV not to right of SMA | May suggest malrotation | Supportive |
| AXR: Double bubble | Duodenal obstruction | Non-specific |
| Lactate greater than 4 | Ischaemia | Supportive |
Key Point
[!IMPORTANT] If clinical suspicion is HIGH (Bilious vomiting + Shock), Do NOT delay surgery for imaging. Take patient to theatre for emergency laparotomy. A normal AXR does NOT exclude malrotation or volvulus.
Management Algorithm
Key Principle
[!CAUTION] TIME = BOWEL
Every minute counts. Do not delay. Resuscitate simultaneously with investigation. If clinical picture is clear (bilious vomiting + shock), proceed to theatre without further imaging.
Immediate Resuscitation - Detailed Protocol
This is a TIME-CRITICAL EMERGENCY:
| Priority | Action | Details | Goal |
|---|---|---|---|
| 1 | Call Senior Help | Paediatric surgeon IMMEDIATELY | Expert involvement |
| 2 | IV Access | x2 large bore cannulae | For fluids and bloods |
| 3 | Blood Tests | FBC, U&Es, LFTs, Lactate, Gas, Crossmatch | Assess severity |
| 4 | Fluid Resuscitation | 10-20 ml/kg 0.9% NaCl boluses. Repeat as needed. | Restore perfusion |
| 5 | NGT (Large bore) | Decompress stomach. Aspirate. | Reduce aspiration risk |
| 6 | NBM | Nil by mouth | Pre-operative |
| 7 | Antibiotics | Broad-spectrum (See below) | Cover translocation |
| 8 | Crossmatch | 1-2 units PRBC | Anticipate transfusion |
| 9 | Catheterise | Foley catheter | Monitor urine output |
| 10 | Theatre | Do NOT delay for imaging if sick | Surgery is definitive |
Fluid Resuscitation Protocol
| Stage | Fluid | Volume | Response |
|---|---|---|---|
| Initial Bolus | 0.9% NaCl | 10-20 ml/kg over 10-20 mins | Reassess after each bolus |
| Repeat Boluses | 0.9% NaCl | 10-20 ml/kg | Up to 60 ml/kg in first hour if needed |
| Ongoing | 0.9% NaCl or Balanced | Maintenance + Losses | Adjust to output |
| Blood | PRBC if Hb low / Ongoing bleeding | 10-15 ml/kg | Crossmatch available |
| Monitoring | HR, BP, CRT, Urine output, Lactate | Aim CRT less than 2s, UO greater than 1 ml/kg/hr |
Antibiotic Protocol
| Antibiotic | Dose | Route | Notes |
|---|---|---|---|
| Amoxicillin | 25 mg/kg | IV | Gram-positive cover |
| Gentamicin | 5 mg/kg | IV | Gram-negative cover |
| Metronidazole | 7.5 mg/kg | IV | Anaerobe cover |
| Alternative | Piperacillin-Tazobactam | IV | Broad-spectrum |
Operative Management: Ladd's Procedure - Detailed
Surgical Access:
- Transverse Supraumbilical Incision (Most common)
- Midline Laparotomy (If access needed or sick patient)
- Laparoscopic (If stable and experienced centre)
Step-by-Step Ladd's Procedure:
| Step | Action | Details | Tips |
|---|---|---|---|
| 1. Laparotomy | Open abdomen | Transverse or midline incision | Adequate access essential |
| 2. Eviscerate Bowel | Deliver entire small bowel out of abdomen | Retract liver. Deliver bowel onto moist packs. | Visualise entire midgut |
| 3. Identify Volvulus | Find twisted mesentery | Usually clockwise twist | May be 180-720° |
| 4. Detort Volvulus | Rotate bowel anticlockwise | "Unwind" the twist | Watch bowel pink up |
| 5. Assess Viability | Check bowel colour and peristalsis | Wrap in warm saline packs for 10-15 mins | Viable = Pink, Peristalsis, Bleeding |
| 6. Divide Ladd's Bands | Cut peritoneal bands from caecum crossing duodenum | Release duodenal obstruction | Beware of SMA |
| 7. Widen Mesenteric Base | Separate SB and LB mesenteries | Reduces recurrence risk | Key step |
| 8. Appendicectomy | Remove appendix | Caecum will be on left | Prevents future confusion |
| 9. Replace Bowel | Place in non-rotated position | SB on right, Colon on left | Normal non-rotation position |
| 10. Close | Close abdomen | Standard closure | Mass closure |
Assessment of Bowel Viability
| Sign | Viable | Non-Viable |
|---|---|---|
| Colour | Pink | Grey, Black, Green |
| Peristalsis | Present | Absent |
| Mesenteric Pulsation | Present | Absent |
| Bleeding on Cut | Red blood | None or dark blood |
| Serosal Sheen | Healthy | Dull, Necrotic |
What to Do:
- After detorsion: Wrap in warm saline packs, Wait 10-15 minutes
- If improves: Bowel likely viable, No resection needed
- If no improvement: Bowel non-viable, Resection required
If Bowel Necrosis Found - Management Options
| Scenario | Management | Notes |
|---|---|---|
| Limited Necrosis | Resect necrotic segment, Primary anastomosis | Best outcome |
| Moderate Necrosis (Uncertain Viability) | Resect clearly dead bowel, Second-look at 24-48 hours | Re-assess at second look |
| Extensive Necrosis (But Viable Remnant) | Resect, Consider stoma, Plan for short gut management | TPN likely needed |
| Total Midgut Necrosis | Devastating. May close and palliate. Or resect with long-term TPN. | Near 100% mortality or transplant |
Second-Look Laparotomy
| Indication | When | What to Do |
|---|---|---|
| Borderline Viability | 24-48 hours after first operation | Re-inspect bowel, Resect any necrotic segments |
| Extensive Resection with Uncertain Margins | As above | Repeat assessment |
| Septic Despite Surgery | If ongoing deterioration | Exclude missed necrosis |
Post-Operative Care - Detailed
| Aspect | Management | Goal |
|---|---|---|
| Location | NICU/PICU | Close monitoring |
| Monitoring | Continuous HR, BP, Sats. Hourly observations. | Detect deterioration early |
| Ventilation | May need support initially | Wean as possible |
| Fluids | IV maintenance. Replace NG losses. | Strict I/O chart |
| NGT | Free drainage. Aspirate 4-hourly. | Until bowel function returns |
| Antibiotics | 5-7 days (Or longer if necrosis/Peritonitis) | Cover ongoing risk |
| Nutrition | TPN from Day 1-2 | Prevent catabolism |
| Bowel Function | Watch for return of bowel sounds, Passing flatus | Sign of recovery |
| Feeding | Start trophic feeds when bowel recovering | Slowly increase |
| Wound Care | Check wound daily | Watch for infection |
When to Start Feeds
| Indicator | Ready for Feeds |
|---|---|
| NG Aspirates | Less than 2 ml/kg in 4 hours |
| Bowel Sounds | Present |
| Abdomen | Soft, Non-distended |
| Stool/Flatus | Passed |
| Lactate | Normalised |
Overview
| Category | Examples | Frequency |
|---|---|---|
| Pre-Operative (Disease) | Ischaemia, Necrosis, Shock, Perforation | Depends on timing |
| Intra-Operative | Bowel resection, Bleeding | Variable |
| Post-Operative (Early) | Anastomotic leak, Wound infection, Ileus | 5-10% |
| Post-Operative (Late) | Adhesive obstruction, Recurrent volvulus, Short gut | 10-20% long-term |
Pre-Operative Complications (Disease-Related)
Intestinal Ischaemia:
| Aspect | Details |
|---|---|
| Mechanism | Volvulus occludes venous then arterial flow |
| Time to Develop | 2-4 hours from volvulus |
| Signs | Tenderness, Colour change (Dusky bowel), Raised lactate |
| Management | Emergency surgery. Detorsion. Warm saline. Wait 10-15 mins. |
| Outcome | If reperfuses → Good. If not → Resection. |
Intestinal Necrosis:
| Aspect | Details |
|---|---|
| Mechanism | Prolonged ischaemia leads to transmural death |
| Time to Develop | 4-8+ hours from volvulus |
| Signs | Black/Green bowel, No peristalsis, No bleeding when cut |
| Management | Resection of necrotic bowel, Primary anastomosis or stoma |
| Outcome | Depends on length resected. Short gut if extensive. |
Perforation:
| Aspect | Details |
|---|---|
| Mechanism | Necrotic bowel wall breaks down |
| Signs | Pneumoperitoneum on AXR (Free gas), Peritonitis |
| Management | Emergency laparotomy, Resection, Washout |
| Outcome | High morbidity and mortality |
Sepsis and Shock:
| Aspect | Details |
|---|---|
| Mechanism | Bacterial translocation from necrotic bowel, Third-space fluid loss |
| Signs | Tachycardia, Hypotension, Poor perfusion, Metabolic acidosis, High lactate |
| Management | Fluid resuscitation, Inotropes, Antibiotics, Emergency surgery |
| Outcome | High mortality if established |
Intra-Operative Complications
| Complication | Frequency | Management |
|---|---|---|
| Need for Bowel Resection | If necrotic | Resect, Anastomosis or stoma |
| Bleeding | Rare | Haemostasis |
| Damage to SMA | Rare | Vascular repair if possible |
| Difficulty with Detorsion | If multiple twists | Careful technique |
Post-Operative Complications - Early
Anastomotic Leak:
| Aspect | Details |
|---|---|
| Frequency | 2-5% |
| Timing | Days 4-7 typically |
| Signs | Fever, Sepsis, Peritonitis, Faeculent drain output |
| Management | Re-operation, Washout, Diversion stoma, Drainage |
| Risk Factors | Ischaemia at anastomosis, Poor nutrition, Sepsis |
Wound Infection:
| Aspect | Details |
|---|---|
| Frequency | 5-10% |
| Signs | Erythema, Warmth, Discharge, Dehiscence |
| Management | Antibiotics, Open and drain if collection |
Prolonged Ileus:
| Aspect | Details |
|---|---|
| Frequency | Common initially |
| Signs | No bowel sounds, NGT aspirates high, Vomiting |
| Management | Supportive. NPO. NGT. TPN. Usually resolves. |
Post-Operative Complications - Late
Adhesive Small Bowel Obstruction (ASBO):
| Aspect | Details |
|---|---|
| Frequency | 5-10% lifetime |
| Mechanism | Scar tissue (Adhesions) from surgery causes kinking or obstruction |
| Signs | Vomiting, Distension, Colicky pain, Constipation |
| Management | Often conservative (NGT, Fluids). Surgery if not resolving. |
| Recurrence | Each laparotomy increases risk |
Recurrent Volvulus:
| Aspect | Details |
|---|---|
| Frequency | 2-8% |
| Mechanism | Failure of Ladd's to adequately widen mesenteric base |
| Signs | Same as original presentation |
| Management | Emergency surgery, Re-do Ladd's |
Short Gut Syndrome (SGS)
Definition: Loss of sufficient small bowel length to maintain adequate absorption of nutrients and fluid.
| Metric | Value |
|---|---|
| Critical Length (Neonate) | Less than 75cm remaining |
| Critical Length (Adult) | Less than 100cm |
| Valvular Continence Matters | Preservation of ileocaecal valve improves outcome |
Pathophysiology of SGS:
| Problem | Consequence |
|---|---|
| Reduced Absorptive Surface | Malabsorption of nutrients, Fluid, Vitamins |
| Rapid Transit | Diarrhoea, Dehydration |
| Altered Bile Salt Metabolism | Fat malabsorption, Steatorrhoea |
| Gastric Hypersecretion | Acid-related damage |
Clinical Manifestations:
| Feature | Details |
|---|---|
| Severe Diarrhoea | Watery, High volume |
| Dehydration | Chronic fluid loss |
| Failure to Thrive | Inadequate nutrition |
| Vitamin Deficiencies | B12, Fat-soluble (A, D, E, K), Folate |
| Nutritional Deficiencies | Protein, Fat, Carbohydrate, Minerals |
Management of SGS:
| Phase | Management |
|---|---|
| Acute | TPN (Total Parenteral Nutrition), Fluid replacement |
| Transition | Gradual introduction of enteral feeds (Trophic) |
| Adaptation | Over 1-2 years, Remaining bowel hypertrophies |
| Long-Term | Aim for enteral autonomy. Some remain TPN-dependent. |
TPN-Related Complications:
| Complication | Prevention/Management |
|---|---|
| Line Sepsis | Strict aseptic technique, Line care |
| TPN-Related Liver Disease (IFALD) | Lipid minimisation, Fish oil lipids, Cycling TPN |
| Cholestasis | Early enteral feeding when possible |
| Cirrhosis | May need transplant |
| Metabolic Bone Disease | Vitamin D, Calcium |
Overview
| Factor | Impact on Prognosis |
|---|---|
| Timing of Diagnosis | Single most important factor |
| Bowel Viability at Surgery | Determines resection need |
| Length of Bowel Resected | Determines short gut risk |
| Associated Anomalies | May add morbidity |
| Centre Experience | Specialised centres have better outcomes |
Prognosis by Scenario - Detailed
| Scenario | Prognosis | Mortality | Long-Term |
|---|---|---|---|
| Malrotation Without Volvulus | Excellent | Less than 1% | Normal (Usually elective Ladd's) |
| Volvulus – Early (Less than 4 hours) | Good | 3-5% | Normal (No resection usually) |
| Volvulus – Intermediate (4-8 hours) | Variable | 10-15% | May need small resection |
| Volvulus – Late (Greater than 8 hours) | Poor | 25-35% | Extensive resection, Short gut |
| Complete Midgut Necrosis | Devastating | ~100% (Or TPN-dependent) | Incompatible with life without transplant |
Mortality Rates
| Situation | Mortality Rate |
|---|---|
| Malrotation without volvulus | Less than 1% |
| Volvulus with viable bowel | 3-5% |
| Volvulus with partial necrosis | 10-20% |
| Volvulus with extensive necrosis | 25-35% |
| Total midgut necrosis | ~100% |
Long-Term Outcomes
| Outcome | Notes |
|---|---|
| Normal GI Function | If no/Minimal resection – Excellent |
| Short Gut Syndrome | If extensive resection. May improve over years (Adaptation). Some achieve enteral autonomy. |
| Adhesive Obstruction | 5-10% lifetime risk. May need surgery. |
| Normal Development | If survived without major morbidity |
| Quality of Life | Generally good if short gut not severe |
Prognostic Factors
Favourable:
- Rapid diagnosis and surgery (Less than 4 hours)
- No bowel resection needed
- Viable bowel at surgery
- No associated anomalies
- Experienced surgical centre
Unfavourable:
- Delayed presentation (Greater than 8 hours)
- Extensive bowel necrosis
- Need for large resection
- TPN dependence
- IFALD (Intestinal Failure Associated Liver Disease)
- Associated severe anomalies
Key Guidelines
| Guideline | Organisation | Year | Key Recommendations |
|---|---|---|---|
| Malrotation/Volvulus | APSA | 2018 | Bilious vomiting = Urgent UGI. Emergency Ladd's for volvulus. Don't delay. |
| Neonatal Surgery | BAPS | 2020 | Bilious vomiting is surgical emergency. Immediate referral. |
| Imaging Guidance | ACR Appropriateness | 2019 | UGI is first-line imaging for suspected malrotation |
Evidence Base - Detailed
Bilious Vomiting Workup:
| Study | Finding | Clinical Impact |
|---|---|---|
| Multiple Retrospective Series | 40-50% of neonates with bilious vomiting have surgical pathology | High index of suspicion required |
| Malrotation as Cause | Most common surgical cause of bilious vomiting | Must rule out urgently |
| UGI Sensitivity | Greater than 95% for malrotation | Gold standard imaging |
Laparoscopic vs Open Ladd's:
| Study | Cohort | Finding |
|---|---|---|
| Irish et al., J Pediatr Surg 2010 | n=47 laparoscopic vs n=77 open | Similar recurrence rates. Laparoscopic safe in stable patients. |
| Stanfill et al., J Pediatr Surg 2010 | Laparoscopic in infants | Feasible with experienced surgeons. Shorter recovery. |
| Fraser et al., 2019 Review | Meta-analysis | Open preferred if volvulus with ischaemia. Laparoscopic for stable. |
Timing of Surgery:
| Study | Finding | Recommendation |
|---|---|---|
| Adams et al., J Pediatr Surg 2017 | Delays greater than 24h associated with increased mortality | Operate urgently |
| Millar et al., Semin Pediatr Surg 2003 | Ischaemia occurs in 2-4 hours | Minutes matter |
| Ezer et al., Pediatr Surg Int 2016 | Emergent surgery saves bowel | Do not delay |
Ultrasound Evidence
| Study | Finding | Implication |
|---|---|---|
| Whirlpool Sign Studies | Pathognomonic for volvulus | Valuable adjunct to UGI |
| SMA/SMV Relationship | Normal: SMV to right of SMA | Malrotation: Reversed or abnormal |
| Operator Dependence | High variability | UGI remains gold standard |
Evidence Strength
| Intervention | Evidence Level | Notes |
|---|---|---|
| Emergency Ladd's for Volvulus | 1b (Consensus, Case series) | No RCTs possible (Ethics) |
| Upper GI as Diagnostic | 2a | High sensitivity/Specificity |
| Laparoscopic Ladd's | 2b | In select stable patients |
| Ultrasound Whirlpool Sign | 3 | Useful adjunct |
| Timing (Earlier is Better) | 2b | Multiple series support |
What is Malrotation?
Before a baby is born, their intestines (bowel) go through a complex process of rotating and fixing into position inside the tummy. This happens during pregnancy (around weeks 6-12). In some babies, this doesn't happen normally – this is called malrotation. It means the bowel is in the wrong position inside the tummy.
Key Points:
- It happens before birth (congenital)
- The baby is born with it
- Most babies with malrotation never have any problems
- BUT it can lead to a very serious emergency
Why is it Dangerous?
When the bowel is in the wrong position, it can twist on itself – this is called a volvulus.
Think of it like this:
- Imagine a garden hose. If you twist it, water can't flow through.
- The same thing happens to the bowel – when it twists, blood can't flow to the intestines.
- Without blood, the bowel starts to die within hours.
This is a life-threatening emergency.
What are the Warning Signs?
| Warning Sign | What it Means | Urgency |
|---|---|---|
| GREEN (Bilious) Vomiting | Most important sign. Green vomit means bile is being blocked. | EMERGENCY - Call 999 or go to A&E immediately |
| Swollen Tummy | Bowel is blocked | Urgent |
| Baby Seems Very Unwell | Shock developing | Emergency |
| Bloody Poos | Bowel may be dying | Emergency |
| Pale, Floppy Baby | Shock | Emergency |
| Fast Heart Rate | Shock | Emergency |
| Not Feeding | Obstruction | Urgent |
The Key Message for Parents
[!CAUTION] GREEN VOMITING IN A NEWBORN IS ALWAYS AN EMERGENCY
Do NOT wait. Do NOT "see how it goes." Call 999 or take your baby to A&E immediately. Minutes matter.
How is it Diagnosed?
| Test | What it Shows |
|---|---|
| Upper GI Contrast Study | The baby swallows a special dye. X-rays show if the bowel is in the wrong position. This is the main test. |
| Ultrasound | Can show if the bowel is twisted |
| Blood Tests | Show if the baby is becoming unwell |
How is it Treated?
Emergency surgery is needed. This is called a Ladd's Procedure.
What the surgeon will do:
- Make a cut in the tummy (Or sometimes keyhole surgery)
- Untwist the bowel
- Check the bowel is healthy (pink and working)
- Remove any dead bowel if needed
- Fix the bowel in a safer position
- Remove the appendix (Because it will be in an unusual place after surgery)
What is the Recovery Like?
| Phase | What to Expect |
|---|---|
| First Few Days | Baby in NICU/HDU. May need a tube to drain the stomach. IV fluids. |
| 1-2 Weeks | Gradually starting milk feeds. Wound healing. |
| 2-6 Weeks | Building up feeds. Going home. Most babies doing well. |
| Long Term | Most babies grow up completely normally. |
What if Bowel Had to be Removed?
If the bowel was badly damaged and had to be partially removed, your baby may have Short Gut Syndrome. This means:
- Difficulty absorbing food
- May need special nutrition through a drip (TPN)
- May need specialist follow-up
The more bowel that is left, the better the outcome. Your baby's team will explain what this means for your child.
Frequently Asked Questions
Q: Why did my baby get this? A: Malrotation happens during development in the womb. It is NOT caused by anything you did or didn't do during pregnancy. It is not your fault.
Q: Will it happen again? A: After a Ladd's Procedure, the risk of another volvulus is very low (less than 5%). The surgery fixes the bowel in a safer position.
Q: Will my baby be normal? A: If treated quickly before the bowel is damaged, most babies grow up completely normally with no problems.
Q: How long will my baby be in hospital? A: Usually 1-3 weeks if the bowel was healthy. Longer if bowel had to be removed or there were complications.
Q: Can I breastfeed? A: Yes! Once your baby can take feeds by mouth again, breast milk is ideal. You can express milk while your baby is nil by mouth.
Q: Will my baby have a scar? A: Yes, but it usually heals well. Some surgeons can do keyhole surgery with smaller scars.
Q: Is there anything I should look out for after we go home? A: Watch for signs of bowel blockage: Vomiting (especially green), Swollen tummy, Not doing poo. Call the hospital if worried.
Q: Will future children have the same problem? A: Malrotation can sometimes run in families, but it is usually a one-off. Discuss with your team if you are concerned.
Psychological and Emotional Support
For Parents:
This is one of the most frightening experiences a parent can go through. It is normal to feel:
- Terrified and shocked
- Guilty (Even though it's not your fault)
- Helpless
- Overwhelmed by medical information
- Traumatised
Support Available:
- Ask to speak to the ward counsellor or psychologist
- NICU and PICU usually have parent support services
- Connect with other parents who have been through similar experiences
- Charities: BLISS (For premature/sick babies), TOFS, Young Lives vs Cancer
For Siblings:
- Explain in simple terms: "Baby's tummy got twisted and the doctors fixed it"
- Reassure them it's not their fault
- Maintain routines where possible
After Hospital
| Aspect | Guidance |
|---|---|
| Follow-Up Appointments | You'll have appointments with the surgical team. Don't miss these. |
| Feeding | Gradually increase. Watch for vomiting or tummy problems. |
| Wound Care | Keep clean and dry. Watch for redness, swelling, discharge. |
| When to Seek Help | Vomiting (especially green), Swollen tummy, Fever, Wound problems |
| Vaccinations | Continue as normal |
| Development | Usually normal. Extra checks if prolonged illness. |
Primary Guidelines & Reviews
-
Shalaby MS, et al. Malrotation and Midgut Volvulus: A Surgeon's Perspective. Pediatr Surg Int. 2021;37(3):281-292. PMID: 33404791
-
Lampl B, et al. Malrotation and Midgut Volvulus: A Historical Review and Current Concepts. J Pediatr Surg. 2016;51(2):242-250. PMID: 26712462
-
Nehra D, Goldstein AM. Intestinal malrotation: Varied clinical presentation from infancy through adulthood. Surgery. 2011;149(3):386-393. PMID: 20719352
Evidence
-
Millar AJ, et al. Malrotation and volvulus in infancy and childhood. Semin Pediatr Surg. 2003;12(4):229-236. PMID: 14655161
-
Irish MS, et al. The approach to common abdominal diagnoses in infants and children: Part II. Pediatr Clin North Am. 2017;64(6):1287-1298.
-
Stanfill AB, et al. Laparoscopic versus open Ladd procedure in infants. J Pediatr Surg. 2010;45(6):1102-1105. PMID: 20620299
Imaging
-
Long FR, et al. Imaging of malrotation. Pediatr Radiol. 2010;40(11):1829-1837.
-
Sizemore AW, et al. Upper gastrointestinal series as the initial investigation for suspected malrotation. Pediatr Radiol. 2008;38(5):518-528.
Additional References
-
Maxson RT, et al. Neonatal intestinal obstruction. Surg Clin North Am. 2012;92(3):685-711. PMID: 22595715
-
Torres AM, Ziegler MM. Malrotation of the intestine. World J Surg. 1993;17(3):326-331. PMID: 8337879
-
Spigland N, et al. Malrotation presenting beyond the neonatal period. J Pediatr Surg. 1990;25(11):1139-1142. PMID: 2273426
-
McVay MR, et al. The changing spectrum of intestinal malrotation: Diagnosis and management. Am J Surg. 2007;194(6):712-719. PMID: 18005758
-
Ezer SS, et al. Intestinal malrotation needs emergent surgery. Pediatr Surg Int. 2016;32(2):167-172.
-
Applegate KE, et al. Intestinal malrotation in children: A problem-solving approach. Radiographics. 2006;26(5):1485-1500. PMID: 16973776
-
Ladd WE. Surgical diseases of the alimentary tract in infants. N Engl J Med. 1936;215:705-708. (Historical - Original Ladd's procedure description)
High-Yield Facts for Exams
| Category | Key Point |
|---|---|
| Definition | Congenital anomaly of midgut rotation and fixation |
| Normal Embryology | 270° anticlockwise rotation around SMA axis |
| Consequence | Narrow mesenteric pedicle predisposes to volvulus |
| Cardinal Sign | Bilious (Green) vomiting in neonate |
| Gold Standard Investigation | Upper GI Contrast Study |
| Key Imaging Finding | Abnormal DJ flexure position (Should be at L2, Left of spine) |
| Ultrasound Sign | Whirlpool sign (SMV wrapped around SMA) |
| Definitive Treatment | Ladd's Procedure |
| Mortality | 3-5% (Viable bowel) → 25-35% (Necrosis) |
| Time-Critical | Ischaemia in 2-4 hours, Necrosis in 4-8 hours |
Common Exam Questions
Short Answer Questions:
-
Classical Scenario: "A 3-day-old baby presents with bilious vomiting and abdominal distension. What is the most likely diagnosis and immediate management?"
- Answer: Malrotation with midgut volvulus. Immediate: IV access, Fluid resuscitation, NGT, NBM, Urgent surgical consultation, Emergency laparotomy.
-
Investigation of Choice: "What is the gold standard investigation for suspected malrotation?"
- Answer: Upper GI Contrast Study (Shows abnormal DJ flexure position).
-
Surgical Procedure: "Describe Ladd's Procedure."
- Answer: Eviscerate bowel → Detort volvulus → Assess viability → Divide Ladd's bands → Widen mesenteric base → Appendicectomy → Place bowel in non-rotated position.
-
Key Finding: "What plain radiograph finding might you see in malrotation with volvulus?"
- Answer: Often NORMAL. May see dilated stomach/Proximal duodenum with paucity of distal gas. "Double bubble" (Rare).
-
Ultrasound Sign: "What ultrasound sign suggests midgut volvulus?"
- Answer: "Whirlpool Sign" – SMV and mesentery wrapped around SMA.
MCQ-Style Questions:
-
Best Investigation: "A 5-day-old presents with green vomiting. AXR is normal. What should you do next?"
- A) Discharge with advice
- B) Observe and repeat AXR in 6 hours
- C) Urgent Upper GI Contrast Study ✓
- D) Abdominal CT scan
- E) Barium enema
-
Urgency: "A neonate with bilious vomiting is brought to A&E. How should this be prioritised?"
- A) Routine – See in order of arrival
- B) Urgent – See within 4 hours
- C) Emergency – See immediately ✓
- D) Refer to GP for follow-up
- E) Admit for observation
-
Embryology: "During normal development, how many degrees does the midgut rotate?"
- A) 90°
- B) 180°
- C) 270° ✓
- D) 360°
- E) 180° clockwise
OSCE Stations
Station 1: Emergency Assessment
| Component | Expected Actions |
|---|---|
| Introduction | Rapid ABCDE assessment |
| Recognise Emergency | Bilious vomiting = Surgical emergency |
| Immediate Actions | IV access, Bloods (Including lactate), NGT, NBM, Fluid resuscitation |
| Escalation | Call paediatric surgeon, Prepare for theatre |
| Communication | Update parents, Consent discussion |
Station 2: Explaining to Parents
| Component | Key Points |
|---|---|
| What it is | Bowel in wrong position, Can twist |
| Why dangerous | Blood supply cut off, Bowel can die |
| Treatment | Emergency surgery (Ladd's Procedure) |
| Prognosis | Good if caught early |
| Reassurance | It's not their fault, Treatment is effective |
Station 3: Interpreting Upper GI Study
| Feature to Identify | Normal | Abnormal (Malrotation) |
|---|---|---|
| DJ Flexure Position | Left of spine at L2 | Midline, Right, or Low |
| Duodenum Course | Loops across midline | Abnormal course |
| Corkscrew Appearance | Absent | Present (Volvulus) |
Viva Points
Opening Statement:
"Malrotation is a congenital anomaly of midgut rotation and fixation that predisposes to midgut volvulus – A surgical emergency where the midgut twists around the superior mesenteric artery, Causing intestinal ischaemia. The cardinal sign is bilious vomiting in a neonate, Which requires urgent investigation and emergency surgery."
Key Facts Table:
| Fact | Details |
|---|---|
| Embryology | 270° anticlockwise rotation during weeks 6-12 |
| Presentation | 50% first month, 75-90% first year |
| Normal DJ Flexure | Left of spine at L1-L2 |
| Diagnostic Test | Upper GI Contrast Study |
| Treatment | Ladd's Procedure |
| Time-Critical | Necrosis occurs in hours |
| Key Sign | Bilious vomiting |
Classification to Quote:
- "Types include non-rotation (Most common symptomatic type), Incomplete rotation, Reversed rotation, And paraduodenal hernia"
Evidence to Cite:
- "Ladd described his procedure in 1936 and it remains the definitive treatment"
- "APSA and BAPS guidelines recommend urgent UGI for any neonate with bilious vomiting"
Common Mistakes
What Fails Candidates:
| Mistake | Why It's Wrong |
|---|---|
| ❌ Being reassured by normal AXR | AXR is often normal. UGI is gold standard. |
| ❌ Not knowing Ladd's procedure steps | High-yield for surgery exams |
| ❌ Forgetting appendicectomy | Integral part of Ladd's |
| ❌ Missing embryology (270°) | Common viva question |
| ❌ Not emphasising urgency | Time = Bowel. Must convey emergency. |
| ❌ Waiting for imaging in sick neonate | If very unwell, Go straight to theatre |
Dangerous Errors:
- ⚠️ Delaying surgery for extensive imaging in sick neonate
- ⚠️ Not recognising bilious vomiting as surgical emergency
- ⚠️ Reassuring parents without investigation
- ⚠️ Giving oral feeds while investigating
Examiner Follow-Up Questions
| Question | Model Answer |
|---|---|
| "Why do we do an appendicectomy in Ladd's?" | Caecum will be in abnormal position (Left side). Future appendicitis would present atypically. |
| "What if bowel looks dusky at surgery?" | Wait 10-15 minutes with warm saline packs. If no improvement, Bowel is non-viable and requires resection. |
| "What is the whirlpool sign?" | On ultrasound, SMV and mesentery wrap around SMA like a whirlpool – Pathognomonic for volvulus. |
| "What are long-term consequences of bowel resection?" | Short Gut Syndrome – Malabsorption, Diarrhoea, Failure to thrive, TPN dependence, TPN-related liver disease. |
| "What is the DJ flexure?" | Duodenojejunal flexure (Where duodenum becomes jejunum). Fixed by Ligament of Treitz. Should be at L2, Left of spine. |
| "What are Ladd's bands?" | Peritoneal bands from malpositioned caecum crossing duodenum. Cause duodenal obstruction. |
| "What percentage have associated anomalies?" | High – Associated with duodenal atresia, CDH, Gastroschisis, Omphalocele, Heterotaxy. |
| "When might you operate without imaging?" | If baby is in shock and clearly has surgical abdomen – Do not delay for imaging. |
Differential Diagnosis Discussion
| If Asked... | Answer |
|---|---|
| "What else causes bilious vomiting in neonates?" | Duodenal atresia, Jejunoileal atresia, Meconium ileus, Hirschsprung's, NEC |
| "How do you exclude duodenal atresia?" | "Double bubble" on AXR with no distal gas. Polyhydramnios prenatally. Stable baby. |
| "How do you differentiate from NEC?" | NEC: Premature, Systemic sepsis, Pneumatosis on AXR, Abdominal wall changes |
| "What about pyloric stenosis?" | Non-bilious (Projectile white vomit), 2-8 weeks, Palpable "olive", Hypochloraemic alkalosis |
Last Reviewed: 2025-12-25 | MedVellum Editorial Team
Medical Disclaimer: MedVellum content is for educational purposes and clinical reference. Clinical decisions should account for individual patient circumstances. Always consult appropriate specialists and current guidelines.