Definition/General
Introduction:
Splenic torsion is a rare surgical emergency characterized by rotation of the spleen around its vascular pedicle, leading to vascular compromise and ischemia
It occurs due to abnormal splenic mobility from congenital or acquired laxity of supporting ligaments
The condition can be acute, chronic, or acute-on-chronic
Without prompt surgical intervention, it leads to splenic infarction and necrosis.
Origin:
Results from abnormal splenic mobility due to laxity or absence of supporting ligaments (splenorenal, splenophrenic, gastrosplenic, splenocolic)
Congenital causes include malrotation, absent ligaments
Acquired causes include trauma, pregnancy, splenomegaly
Wandering spleen predisposes to torsion due to excessive mobility.
Classification:
By onset: Acute torsion (<24 hours)
Chronic torsion (>24 hours)
Acute-on-chronic torsion
By degree: Partial torsion (<360°)
Complete torsion (≥360°)
By etiology: Congenital (absent/lax ligaments)
Acquired (trauma, pregnancy, splenomegaly)
By location: Ectopic spleen with torsion.
Epidemiology:
Very rare condition: <500 cases reported in literature
Bimodal distribution: Children (<10 years) and women of reproductive age
Gender: Female predominance (7:1 ratio) due to pregnancy-related cases
Pregnancy association: Hormonal relaxation of ligaments
Associated conditions: Wandering spleen, malrotation.
Clinical Features
Presentation:
Acute severe left upper quadrant pain (most common symptom)
Nausea and vomiting (80-90% of patients)
Abdominal distension and tenderness
Palpable mass in abnormal location (wandering spleen)
Signs of peritoneal irritation in advanced cases.
Symptoms:
Pain characteristics: Sudden onset, severe, colicky pain
May radiate to left shoulder
Gastrointestinal symptoms: Persistent nausea and vomiting
Decreased appetite
Systemic symptoms: Fever in complicated cases
Tachycardia and hypotension
Urinary symptoms: May mimic urologic pathology.
Risk Factors:
Congenital anomalies: Absent or lax splenic ligaments
Malrotation syndromes
Acquired conditions: Pregnancy (hormonal ligament relaxation)
Splenomegaly from any cause
Trauma history: Blunt abdominal trauma
Age factors: Pediatric age group, reproductive-age women.
Screening:
Clinical suspicion: Acute abdominal pain with wandering spleen
Imaging studies: CT scan with contrast showing "whirl sign"
Doppler ultrasound for vascular assessment
Laboratory studies: Elevated WBC, lactate in advanced cases
Emergency evaluation: High index of suspicion required.
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Gross Description
Appearance:
Variable appearance depending on duration and degree of torsion
Early torsion: Congested, dark red spleen
Complete torsion: Black, necrotic spleen with hemorrhagic infarction
Twisted vascular pedicle: Visible spiral configuration of vessels
Associated findings: Ascites, fibrinous exudate.
Characteristics:
Size changes: Initially enlarged due to congestion, later shrunken if chronic
Color changes: Dark red to purple (congestion) to black (necrosis)
Consistency: Firm due to congestion, soft if necrotic
Capsular changes: May show fibrinous deposits or rupture
Vascular pedicle: Twisted, thrombosed vessels.
Size Location:
Abnormal location: Spleen may be in pelvis, right abdomen, or midline
Size variation: Depends on degree and duration of vascular compromise
Mobile spleen: Excessive mobility on long vascular pedicle
Associated anomalies: May have other rotational anomalies.
Multifocality:
Uniform involvement: Entire spleen affected by vascular compromise
No multifocal pattern: Global ischemia rather than segmental
Associated findings: Adjacent organ involvement rare
Complications: Rupture, abscess formation, adhesions.
Microscopic Description
Histological Features:
Acute phase: Congestion, hemorrhage, early ischemic changes
Subacute phase: Coagulative necrosis, inflammatory infiltration
Chronic phase: Fibrosis, calcification, organized thrombi
Vascular changes: Thrombosis of splenic vessels, endothelial damage.
Cellular Characteristics:
Acute changes: Congested red pulp, dilated sinusoids
Loss of cellular details
Necrotic phase: Ghost cell architecture, pyknotic nuclei
Nuclear fragmentation and karyolysis
Inflammatory phase: Neutrophilic infiltration, later chronic inflammation
Repair phase: Fibroblastic proliferation, granulation tissue.
Architectural Patterns:
Early torsion: Preserved architecture with congestion
Established ischemia: Loss of normal splenic architecture
Complete necrosis: Coagulative necrosis throughout
Organizing phase: Granulation tissue and fibrosis
Chronic changes: Dense fibrosis, calcification.
Grading Criteria:
Acute reversible: Congestion without necrosis
Acute irreversible: Early necrotic changes
Subacute: Established necrosis with inflammation
Chronic: Fibrosis and calcification predominant
Complicated: Secondary infection or rupture.
Immunohistochemistry
Positive Markers:
Vascular markers: CD31, CD34 for assessing vascular integrity
Smooth muscle markers: SMA in vessel walls and organizing areas
Inflammatory markers: CD68 in macrophages, myeloperoxidase in neutrophils
Proliferation markers: Ki-67 in repair areas.
Negative Markers:
Normal splenic markers: Loss in necrotic areas
Viable tissue markers: Absent in infarcted regions
Infectious markers: To exclude secondary infection
Tumor markers: To exclude underlying neoplasm.
Diagnostic Utility:
Vascular assessment: Degree of vascular compromise
Viability assessment: Extent of necrosis vs viable tissue
Inflammatory evaluation: Secondary inflammatory response
Repair assessment: Healing and organization.
Molecular Subtypes:
Acute ischemic: Vascular markers with preserved architecture
Necrotic phase: Loss of normal markers
Organizing phase: Repair and angiogenesis markers
Chronic fibrotic: Fibrosis and remodeling markers.
Molecular/Genetic
Genetic Mutations:
Connective tissue genes: Collagen gene variants may affect ligament strength
Angiogenesis genes: VEGF variants in repair response
Inflammatory genes: Cytokine gene polymorphisms affecting response
Developmental genes: Associated with congenital anomalies.
Molecular Markers:
Ischemia markers: HIF-1α in hypoxic tissue
Inflammatory cytokines: IL-1, TNF-α, IL-6 in acute phase
Angiogenic factors: VEGF, PDGF in repair
Tissue damage markers: Elevated LDH, cellular enzymes.
Prognostic Significance:
Time to intervention: Critical factor in salvage potential
Degree of torsion: Complete vs partial affects outcome
Patient age: Children may have better recovery potential
Associated conditions: Pregnancy complicates management
Complications: Secondary infection worsens prognosis.
Therapeutic Targets:
Emergency surgery: Detorsion and splenopexy vs splenectomy
Conservative detorsion: If spleen viable after untwisting
Splenectomy: If spleen non-viable
Preventive measures: Splenopexy for wandering spleen.
Differential Diagnosis
Similar Entities:
Splenic infarction: Ischemic necrosis without torsion
Acute abdomen: Appendicitis, ovarian torsion, renal colic
Splenic abscess: Infected collection vs ischemic necrosis
Splenic trauma: History of injury vs spontaneous torsion
Splenic tumor: Mass lesion vs ischemic spleen.
Distinguishing Features:
Torsion vs infarction: Twisted pedicle vs patent vessels
Abnormal location vs normal position
Torsion vs other acute abdomen: Location-specific pain patterns
Imaging findings
Clinical correlation: Wandering spleen history
Surgical findings: Twisted vascular pedicle diagnostic.
Diagnostic Challenges:
Intermittent torsion: May have episodes of pain with spontaneous resolution
Chronic torsion: May present with less acute symptoms
Pregnancy cases: Symptoms may be attributed to pregnancy
Pediatric cases: Communication limitations in history taking.
Rare Variants:
Intermittent torsion: Recurrent episodes with spontaneous detorsion
Torsion with splenomegaly: Enlarged spleen predisposes to torsion
Post-traumatic torsion: Following injury to supporting ligaments
Ectopic splenic torsion: Accessory spleen torsion
Pregnancy-related torsion: Due to hormonal ligament relaxation.
Sample Pathology Report
Template Format
Sample Pathology Report
Complete Report: This is an example of how the final pathology report should be structured for this condition.
Specimen Information
Splenectomy specimen with clinical history of [acute abdominal pain] and [wandering spleen/abnormal location]
Gross Description
[Congested/necrotic] spleen with twisted vascular pedicle and [ischemic changes]
Torsion Assessment
[Complete/partial] torsion with [degree of rotation] and [vascular compromise]
Microscopic Findings
[Acute/subacute/chronic] ischemic changes with [extent of necrosis] and [inflammatory response]
Viability Assessment
[Viable/non-viable] splenic tissue with [percentage] involvement
Diagnosis
Splenic torsion with [acute/chronic] ischemic changes
Complications
[Present/absent]: [secondary infection/rupture/abscess formation]
Clinical Correlation
[Splenectomy appropriate/splenopexy could have been considered] based on viability assessment