Malformations of the musculoskeletal system in children. muscular torticollis

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Muscular Torticollis in Children

Definition

Congenital Muscular Torticollis (CMT) is a postural deformity of the neck characterized by unilateral contracture of the sternocleidomastoid (SCM) muscle, resulting in lateral head tilt toward the affected side and rotation of the chin toward the opposite side.

Etiology and Pathogenesis

The exact cause remains debated, but the leading theories include:
  • Intrauterine moulding — abnormal fetal positioning in the uterus leads to compartment syndrome-like ischemia of the SCM, followed by fibrosis (Bailey & Love, p. 651)
  • Birth trauma — difficult deliveries (breech presentation, forceps use) may cause SCM injury with subsequent hematoma and fibrous replacement
  • Venous occlusion theory — venous obstruction during labor causes edema and muscle necrosis
  • Intrauterine crowding — associated with other positional deformities (hip dysplasia, metatarsus adductus)
The fibrotic process within the SCM leads to a fibromuscular mass ("SCM tumor" or pseudotumor) palpable in the neonatal period, which gradually resolves or persists as a contracture.

Classification

TypeFeatures
Postural torticollisNo SCM mass or tightness; purely positional; most benign
Muscular torticollisTight SCM without discrete mass
SCM tumor/mass typePalpable fibrous mass within SCM; highest risk of persistent deformity

Clinical Presentation

Neonatal period:
  • Palpable firm, non-tender mass within the SCM (fibrous pseudotumor), typically appearing at 2–4 weeks of age
  • Head tilts to the ipsilateral side (toward the shortened muscle)
  • Chin rotates to the contralateral side
  • Restricted passive cervical rotation
Infants and older children (if untreated):
  • Plagiocephaly — flattening of the skull ipsilateral to the tilt
  • Facial asymmetry (hemihypoplasia) — the face on the affected side appears flattened and smaller; develops progressively after 3–6 months
  • Compensatory "C-curve" of the trunk and spine
  • Oblique pelvic tilt (global postural adaptation)
Infant with left-sided CMT showing head tilt, C-curve body posture, and pelvic obliquity
Left-sided CMT: characteristic lateral head tilt, compensatory trunk C-curve, and pelvic obliquity

Diagnosis

Clinical (primary):
  • Asymmetric head position, restricted cervical range of motion
  • Palpable SCM mass or tightness
  • Measure passive rotation and lateral flexion deficit
Ultrasound (first-line imaging):
  • Shows thickening and increased echogenicity of the SCM
  • Confirms fibrous mass, rules out vascular anomalies
  • Non-invasive, no radiation — ideal for infants
Radiography (cervical spine X-ray):
  • Excludes bony causes (atlanto-axial instability, Klippel-Feil syndrome, hemivertebrae)
MRI/CT: reserved for atypical cases or when cervical spine anomaly is suspected

Differential Diagnosis

ConditionKey Distinguishing Features
Osseous torticollis (Klippel-Feil, atlanto-axial instability)Bony abnormalities on X-ray/CT
Ocular torticollisHead tilt corrects with eyes closed; ophthalmologic exam abnormal
Neurogenic torticollisPosterior fossa tumor, syringomyelia — MRI required
Spasmodic torticollisOlder children; dystonic, intermittent movements
Cervical lymphadenitis / abscessTender, warm mass; fever; acute onset
Sandifer syndromeAssociated with gastroesophageal reflux; episodic

Management

Conservative (First-line, initiated early)

Physiotherapy / stretching exercises — mainstay of treatment:
  • Passive and active SCM stretching
  • Lateral cervical flexion and rotation exercises
  • Should begin within the first weeks of life
  • Success rate: ~90–95% if started before 3 months of age
Positioning and handling:
  • Visual and auditory stimuli placed on the contralateral side to encourage active correction
  • "Tummy time" to promote symmetric muscle use
Botulinum toxin (Botox) injection:
  • Used as an adjunct in refractory cases before surgery
  • Temporarily reduces SCM tension, augments physiotherapy

Surgical (Second-line)

Indicated when:
  • Conservative treatment fails after 6–12 months
  • Significant residual deficit after 12–18 months of age
  • Progressive facial asymmetry (Bailey & Love, p. 651)
Procedure — SCM release (tenotomy/myotomy):
  • Distal release (sternal and/or clavicular head) — most common
  • Bipolar release (origin + insertion) — for severe contractures
  • Post-operative physiotherapy and neck orthosis essential
Outcomes: Best results when surgery is performed before 5 years of age. Facial asymmetry partially remodels after surgery in younger children but may be permanent if delayed.

Complications of Untreated Torticollis

  • Permanent facial asymmetry / hemihypoplasia
  • Plagiocephaly
  • Cervical scoliosis and compensatory thoracic scoliosis
  • Visual disturbances (from chronic head tilt)
  • Psychological impact (cosmetic deformity)

Prognosis

FactorOutcome
Early diagnosis + physiotherapy (<3 months)Excellent; ~90–95% resolution
SCM mass typePoorer conservative prognosis; more likely to need surgery
Treatment started after 1 yearHigher surgical rate
Surgery before age 5Good functional and cosmetic outcome
Surgery after age 5Functional improvement but facial asymmetry may persist

Key takeaway: CMT is the most common cause of neck deformity in infants. Early diagnosis and aggressive physiotherapy before 3 months of age yields excellent outcomes. The SCM mass type and delayed treatment are the main predictors of surgical need. Facial asymmetry is the most important long-term complication to prevent through timely intervention.
Bailey and Love's Short Practice of Surgery, 28th Ed., p. 651

Hernias: hernias of the anterior abdominal wall, inguinal hernias

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Hernias of the Anterior Abdominal Wall and Inguinal Hernias


General Definition

A hernia is the protrusion of a viscus (or part of it) through a defect or weakness in the wall of the cavity in which it is normally contained. Every hernia has three components:
  • Sac — peritoneal outpouching
  • Contents — bowel, omentum, bladder, ovary, etc.
  • Coverings — layers of abdominal wall draped over the sac

PART I — Hernias of the Anterior Abdominal Wall (Ventral Hernias)

The term ventral hernia refers to hernias occurring in the anterior abdominal wall, excluding inguinal and femoral hernias (though lumbar hernia is included despite its dorsolateral position). The European Hernia Society (EHS) classification (2009) divides them into:
  • Primary ventral hernias — occur spontaneously at anatomically weak points
  • Incisional hernias — occur at previous surgical wounds (Bailey & Love, 28th Ed., p. 1093)

1. Umbilical Hernia

In Infants

  • Due to failure of the umbilical ring to close after birth
  • Very common; majority close spontaneously by 3–4 years of age
  • Surgical repair indicated if persists beyond age 4–5 or if ring diameter > 1.5 cm

In Adults

  • Strictly termed paraumbilical hernia — hernia passes through a defect just above or below the umbilical ring (not through it)
  • More common in obese, multiparous women
  • Contents: omentum, transverse colon, small bowel
  • Risk of strangulation is relatively high — elective repair recommended even if asymptomatic
  • Repair: Mayo "vest-over-pants" repair or mesh repair for larger defects

2. Epigastric Hernia

  • Occurs through defects in the linea alba between xiphoid process and umbilicus
  • Usually small; contains extraperitoneal fat (pre-peritoneal fat plug), rarely a true sac
  • More common in young adult males
  • Often multiple; frequently symptomatic (pain disproportionate to size)
  • Treatment: surgical closure; mesh for larger defects

3. Incisional Hernia

  • Develops through a previous surgical incision in the abdominal wall
  • Risk factors:
    • Wound infection, dehiscence
    • Obesity, malnutrition, steroid use
    • Poor surgical technique (excessive tension, inadequate closure)
    • Midline incisions carry highest risk
EHS Classification — based on:
  • Location (midline vs. lateral)
  • Width of defect (W1 <4 cm, W2 4–10 cm, W3 >10 cm)
  • Recurrent vs. primary
Treatment:
  • Small defects: primary suture repair
  • Defects ≥ 4 cm: mesh repair (open or laparoscopic) — lower recurrence rate
  • Component separation technique for massive defects

4. Spigelian Hernia

  • Occurs through the spigelian fascia (aponeurosis of the transversus abdominis) at the lateral border of the rectus sheath, typically just below the arcuate line
  • Interparietal — sac lies between muscle layers (difficult to detect clinically)
  • Often missed on examination; diagnosed by ultrasound or CT
  • High risk of strangulation
  • Treatment: surgical repair (laparoscopic preferred)

5. Lumbar Hernia

  • Through the superior lumbar triangle (Grynfeltt) or inferior lumbar triangle (Petit)
  • Rare; often contains retroperitoneal fat
  • Treatment: mesh repair

6. Obturator Hernia

  • Through the obturator canal in the obturator foramen
  • Predominantly elderly, thin women
  • Classic sign: Howship-Romberg sign — pain along inner thigh (obturator nerve compression)
  • Frequently presents as small bowel obstruction; diagnosed on CT
  • High morbidity due to delayed diagnosis

PART II — Inguinal Hernias

Anatomy of the Inguinal Canal

The inguinal canal is an oblique passage (~4 cm long) in the lower anterior abdominal wall:
StructureDetails
Deep (internal) ringDefect in transversalis fascia; lateral to inferior epigastric vessels
Superficial (external) ringDefect in external oblique aponeurosis; above pubic tubercle
Anterior wallExternal oblique aponeurosis (+ internal oblique laterally)
Posterior wallTransversalis fascia (+ conjoint tendon medially)
RoofArched fibers of internal oblique and transversus abdominis
FloorInguinal ligament (Poupart) + lacunar ligament medially
Contents of the canal:
  • In males: spermatic cord (vas deferens, testicular artery, pampiniform plexus, cremasteric muscle, processus vaginalis remnant) + ilioinguinal nerve
  • In females: round ligament of the uterus + ilioinguinal nerve
The inferior epigastric artery (branch of external iliac) runs on the posterior abdominal wall just medial to the deep ring — this is the key landmark distinguishing indirect from direct hernias.

Classification of Inguinal Hernias

(Bailey & Love, 28th Ed., p. 1086)
FeatureIndirect (Lateral/Oblique)Direct (Medial)
PathThrough deep ring → inguinal canal → superficial ringDirectly through posterior wall (Hesselbach's triangle)
Relation to inferior epigastric vesselsLateral to vesselsMedial to vessels
SacEnters spermatic cord (covered by all cord coverings)No cord involvement
OriginCongenital (patent processus vaginalis) or acquiredAcquired (weakness of transversalis fascia)
AgeAll ages; most common in young malesMiddle-aged to elderly males
NeckNarrow — high strangulation riskWide — low strangulation risk
Descent into scrotumYes (can become scrotal)Rarely
Reduction on pressureLateral pressure controlsNot controlled laterally
Hesselbach's triangle (site of direct hernia):
  • Medially: lateral border of rectus abdominis
  • Laterally: inferior epigastric vessels
  • Inferiorly: inguinal ligament

Epidemiology

  • Most common hernia overall (~75% of all hernias)
  • Lifetime risk: 27% in males, 3% in females
  • Right side > left side (due to later descent of right testis)
  • Indirect > direct (ratio ~2:1)
  • Bilateral in ~20% of cases

Clinical Presentation

Symptoms:
  • Groin lump, typically appearing on standing/straining and reducing on lying down
  • Dragging, aching discomfort
  • In scrotal hernias: visible and palpable scrotal swelling
Signs:
  • Visible/palpable impulse on coughing
  • Invagination test: finger in superficial ring — indirect hernia impacts fingertip, direct hernia impacts pulp
  • Reducibility assessment
  • Irreducibility = hernia cannot be reduced (may still be viable)
  • Obstruction = bowel obstruction without vascular compromise
  • Strangulation = vascular compromise → surgical emergency

Imaging

Usually a clinical diagnosis. Imaging is used in:
  • Uncertain or occult hernias: ultrasound (first line)
  • Complex cases, recurrence, or pre-operative planning: CT scan
  • Identification of bilateral occult hernias: MRI (sports hernia evaluation)
Intraoperative view showing two direct hernia sacs (S1 lateral, S2 medial to IEA) and an indirect sac, with anomalous superficial course of the inferior epigastric artery
Intraoperative anatomy: S1 and S2 = two direct hernia sacs on either side of the inferior epigastric artery (IEA); indirect sac visible laterally. Note anomalous superficial course of IEA along the posterior canal wall.

Differential Diagnosis of Groin Swelling

ConditionKey Feature
Femoral herniaBelow and lateral to pubic tubercle; more common in women
LymphadenopathyFirm, non-reducible nodes; look for source of infection/malignancy
HydroceleTransilluminates; cannot get above it; testicular
Varicocele"Bag of worms" consistency; left side predominant
Undescended testisAbsent testis in scrotum
Lipoma of cordSoft, reducible, no impulse on cough
Femoral artery aneurysmPulsatile; non-reducible
Psoas abscessFluctuant; systemic signs; comes from above inguinal ligament

Management

Conservative

  • Watchful waiting is acceptable for asymptomatic or minimally symptomatic direct inguinal hernias (low strangulation risk)
  • Truss: rarely used; not curative; may mask symptoms

Surgical Repair

Indications: symptomatic hernia, irreducibility, strangulation/obstruction, all indirect hernias (high strangulation risk), all hernias in children

Open Repair

TechniquePrinciple
Lichtenstein tension-free mesh repairGold standard for open repair; polypropylene mesh placed in inguinal canal
Shouldice repairMulti-layer sutured repair of posterior wall; best non-mesh results
Bassini repairHistorical; sutured repair; higher recurrence
Herniotomy (in children)Simple sac ligation without posterior wall repair

Laparoscopic / Minimally Invasive Repair

(Bailey & Love, p. 1086)
TechniqueApproach
TAPP (Trans-Abdominal Pre-Peritoneal)Laparoscopic; peritoneum incised, mesh placed, peritoneum closed
TEP (Totally ExtraPeritoneal)Laparoscopic; mesh placed entirely in preperitoneal space; no peritoneal breach
RARP (Robot-Assisted)Emerging; especially for bilateral/complex cases
Advantages of laparoscopic repair: less post-op pain, faster recovery, better for bilateral hernias and recurrent hernias after open repair.

Complications

Intraoperative: injury to vas deferens, spermatic vessels, ilioinguinal/genitofemoral nerves, bladder, bowel
Post-operative:
ComplicationNotes
Seroma / hematomaMost common early complication
Wound infectionHigher with mesh → mesh infection rare but serious
Urinary retentionEspecially in elderly males
Chronic groin painInguinodynia; neuropathic; most feared long-term complication
Testicular atrophyInjury to testicular artery
Recurrence~1–5% with mesh repair; higher with pure tissue repair

Special Situations

  • Strangulated hernia: Emergency repair after resuscitation; bowel resection if non-viable; mesh use controversial in contaminated field
  • Inguinal hernia in children: Always indirect (patent processus vaginalis); treated by herniotomy (sac ligation only); high incidence of contralateral patent processus — many surgeons explore both sides
  • Inguinal hernia in women: Rare; always exclude femoral hernia; round ligament may be in sac; repair principles same

Bailey and Love's Short Practice of Surgery, 28th Ed., pp. 1086, 1093
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