Rhinolith (Nasal Calculus)

Definition

• K.J. Lee's Essential Otolaryngology: "calcareous concretions around intranasal foreign bodies within the nasal cavity, usually in the anterior nasal cavity"
• Scott-Brown's Otorhinolaryngology, Head & Neck Surgery, Vol. 2: "Rhinoliths consist of salts of calcium, magnesium phosphate and carbonate"

Pathogenesis

• Exogenous - an inhaled or inserted foreign body (most common - beads, seeds, food material, tooth fragments). Foreign body introduction is most common in children aged 2-4 years.
• Endogenous - dried blood clot, inspissated mucus, desquamated epithelium, a deviated nasal bone fragment, or a displaced tooth root

Clinical Features

• Unilateral nasal obstruction (most common)
• Unilateral purulent, foul-smelling nasal discharge (characteristic - the combination of unilateral purulence + malodor is the classic presentation)
• Oral malodor (halitosis)
• Facial pain / pressure
• Epistaxis (less common)
• Long-standing cases can cause hypoplasia of the inferior turbinate

Differential Diagnosis

• Nasal/sinus foreign body
• Mucocele
• Encephalocele
• Osteoma
• Benign and malignant neoplasms (inverted papilloma, SCC)
• Nasal polyps
• Chronic rhinosinusitis
• Granulomatous disease (Wegener's/GPA, sarcoidosis, TB)

Diagnosis

Management

• For most cases: endoscopic removal (functional endoscopic nasal surgery) under local, sedation, or general anesthesia
• For very large or anteriorly-placed stones: piecemeal fragmentation and extraction
• For anterior, small rhinoliths: simple forceps extraction may suffice in an outpatient setting
• The mother's kiss technique (used for simple foreign bodies in children) is generally not applicable for calcified rhinoliths
• Post-removal: nasal irrigation and antibiotics if secondary sinusitis is present
• The removed specimen should be sent for pathological examination to confirm nature and identify any nidus

Key Points to Remember

• Classic presentation: unilateral foul-smelling nasal discharge + obstruction
• Radio-opaque on plain X-ray; CT is the best imaging modality
• Can be present for years before diagnosis - high index of suspicion needed
• Always consider in children with a history of nasal foreign body insertion
• Differential from sinonasal tumors (including malignancy) is important - CT and histopathology clarify the diagnosis
• Excellent prognosis after removal

Tympanosclerosis

Definition

Otoscopic Appearance

Epidemiology / Incidence

• Incidence in children with OME: ~10% (aged 4-15 years, average 4-year follow-up)
• In chronic otitis media generally: 9-43% across different series (~25% overall estimate)
• Strong association with tympanostomy (grommet) tube insertion: 59% of grommet ears developed tympanosclerosis vs. 13% of ears treated with myringotomy alone (Tos & Stangerup)
• Meta-analysis of 134 studies: 32% post-intubation tympanosclerosis vs. 10% in controls

Pathogenesis

1. Repeated middle ear inflammation triggers damage to the subepithelial connective tissue
2. Collagen undergoes hyaline degeneration (acellular hyalinization of the lamina propria)
3. Mineral salts (calcium phosphate, calcium carbonate) deposit in the degenerated tissue
4. Osteoneogenesis can also occur within these lesions
5. Microscopically: deposition occurs in concentric onion-ring layers, building up to several millimeters thick

• Direct collagen degeneration → calcified plaques
• Fibrous connective tissue degeneration → calcified plaques

• Chlamydia pneumoniae has been identified as a possible infectious trigger
• Genetic predisposition - animal models (LP/J mouse; Enpp1asj mutant mice) support a genetic component
• Molecular pathways implicated in recent research: TNF-α, IL-6 (inflammatory cytokines), oxidative stress enzymes (CAT, iNOS), Wnt signaling, TGF-β1, osteopontin (bone remodeling)

Clinical Features

Distribution and Severity

Hearing Loss

• Myringosclerosis alone - rarely causes significant hearing loss and usually requires no intervention
• Middle ear tympanosclerosis - can immobilize the malleus, incus, and/or stapes, causing moderate-to-severe conductive hearing loss
• Mixed hearing loss (conductive + sensorineural) can occur in long-standing middle ear disease
• Suspect ossicular fixation from tympanosclerosis when: history of chronic infections + CHL + tympanosclerosis visible on TM

Key Clinical Clue

Investigations

Management

Myringosclerosis (TM only)

• Observation - most cases require no treatment
• Hearing aid if mild CHL is present and surgery is declined or high-risk

Middle Ear Tympanosclerosis (Ossicular Fixation)

1. Wide atticotomy when disease is restricted to the attic
2. Tympanosclerotic plaques are progressively removed from lateral, anterior, superior, and medial surfaces of malleus/incus
3. Preserve the intact ossicular chain whenever possible
4. Laser (otologic CO2 or KTP) is preferred to reduce mechanical trauma to the ossicles - thin shell of plaque is vaporized rather than dissected

• Remove plaques without opening the vestibule; dissect posterior-to-anterior using the stapedius tendon for stability
• Stapedotomy - air-bone gap <20 dB achieved in ~70% in one series
• Stapedectomy - reserved for total stapes fixation; do not combine with TM repair (risk of labyrinthitis and profound SNHL)
• Attic disease addressed first, then stapes, in combined cases

• Option 1: Remove the bony fixation directly, preserve intact chain; Silastic sheet interposed between malleus head and attic wall
• Option 2: Remove incus and malleus head, interpose autograft/allograft between manubrium and stapes (TORP/PORP)

• Excellent hearing results (ABG <20 dB) in ~79% with ossicular reconstruction in two-stage procedures (Smyth)
• Recurrence of tympanosclerosis post-surgery is reported but not universal
• Risk of cochlear damage is higher than in other middle ear surgery due to extensive dissection required

Key Points Summary

• Tympanosclerosis = hyaline degeneration + calcification of TM and/or middle ear, a sequela of otitis media or trauma
• Grommet insertion doubles the risk compared to myringotomy alone
• TM plaques alone rarely affect hearing - middle ear involvement causes CHL via ossicular fixation
• Stapes and malleus/incus in the attic are equally commonly affected
• Surgery is technically demanding; laser use reduces mechanical cochlear trauma
• Hearing aids are a valid alternative when surgery is high-risk or declined

Tracheoesophageal Fistula (TEF)

Definition

Embryology (Congenital TEF)

Classification (Gross Classification)

Associated Anomalies - VACTERL

• V - Vertebral anomalies (assessed by plain X-ray)
• A - Anal atresia (anorectal defects, ~8%)
• C - Cardiac defects (~38% - the most important predictor of survival; assess with echocardiography)
• TE - Tracheoesophageal fistula
• R - Renal anomalies (~15%)
• L - Limb anomalies / radial limb hypoplasia (~19% skeletal defects)

Clinical Presentation

Type C (EA + Distal TEF) - the typical neonate

• Excessive oral secretions / drooling from birth - the nursing staff often make the diagnosis at first feeding attempt
• Choking, coughing, and cyanosis at first feed
• Abdominal distension - air passes through the fistula into the stomach with each breath or cry
• Chemical pneumonitis - regurgitated gastric juice enters the trachea via the distal fistula
• Unable to pass NG tube into stomach - tube coils in the proximal blind pouch
• On CXR: gas-filled abdomen (confirms a distal TEF is present)

Type A (Isolated EA, no fistula)

• Regurgitation + scaphoid (gasless) abdomen - no pathway for air to enter bowel
• Saliva pools in the pouch

H-type TEF (Type E)

• Diagnosis often delayed to childhood or even adulthood
• Presents with recurrent aspiration pneumonia, chronic cough, bronchiectasis
• Symptoms relate to aspiration (not obstruction) since the esophagus is patent
• Diagnosed by esophagography ± bronchoscopy; methylene blue ingestion with bronchoscopic visualization of blue-stained fistula is a useful diagnostic trick for small, elusive H-type fistulas

Prenatal Diagnosis

• Polyhydramnios (impaired fetal swallowing - fetus cannot swallow amniotic fluid into the bowel)
• Absent stomach bubble on ultrasound (no fluid reaching the stomach)
• Fetal MRI (as shown above) can clearly demonstrate the proximal blind pouch

Postnatal Diagnosis

Initial (Pre-operative) Management

1. Position neonate with head elevated ≥30° to minimize aspiration
2. Sump catheter in upper pouch on continuous suction - prevents aspiration of pooled saliva
3. IV antibiotics + warmed electrolyte solution
4. Avoid right upper limb IV if possible - it may interfere with surgical positioning
5. Definitive repair is not a surgical emergency in the stable infant - repair can be planned 1-2 days after birth allowing thorough investigation

Special case: Premature neonate on ventilator

• Position endotracheal tube distal to the fistula
• Emergency gastrostomy to decompress the stomach
• Early surgical fistula ligation

Surgical Repair (Congenital EA-TEF)

• Fistula identified, ligated, and divided
• Esophageal ends mobilized
• End-to-end esophageal anastomosis performed (primary anastomosis)
  • Short-gap (< 3 vertebral bodies): direct primary anastomosis
  • Long-gap EA: options include:
    • Bougienage (Foker procedure) or circular myotomy to lengthen upper pouch
    • Magnetic compression anastomosis (magnamosis)
    • Esophageal replacement using stomach (gastric pull-up), colon interposition, or jejunal graft if approximation impossible

Long-term Complications (Post-repair)

Acquired TEF

Causes

• Post-intubation (most common acquired cause) - ETT cuff pressure causes pressure necrosis of the posterior membranous trachea and anterior esophageal wall, forming a "party wall" fistula
• Malignancy - esophageal or lung cancer eroding between structures (poor prognosis: median survival ~10 weeks)
• Trauma - blunt or penetrating neck/chest trauma
• Iatrogenic - post-tracheotomy, post-esophageal surgery
• Infection - tuberculosis, actinomycosis (rare)

Surgical Repair (Benign Acquired TEF - Grillo Principles)

1. Preoperative: Barium swallow to define anatomy; treat pulmonary sepsis; optimize nutrition; wean ventilator if possible
2. Approach:
   • Proximal TEF: anterior collar incision (may extend to sternotomy)
   • Distal TEF: right posterolateral thoracotomy (4th/5th interspace)
3. Technique:
   • Segmental tracheal resection often required (especially for post-intubation TEF with circumferential cuff damage)
   • Esophagus closed in two layers over an NG tube (inner layer - mucosa; outer layer - musculature) with interrupted non-absorbable sutures
   • Trachea reanastomosed with interrupted absorbable sutures
   • Pedicled muscle flap (infrahyoid strap muscle / sternohyoid) interposed between tracheal and esophageal suture lines - this is mandatory to prevent recurrence
   • "Guardian suture" (chin-to-sternum) maintains 30° neck flexion post-op to reduce anastomotic tension
4. Outcomes (benign TEF): 95% successful closure; 83% resume oral feeding; 72% tracheostomy-free

Malignant TEF - Palliation

• Surgery rarely appropriate (poor condition, poor prognosis)
• Self-expanding covered metal/plastic stents across the fistula (esophageal ± tracheal) - 80-100% success for sealing fistula
• Tracheal stent placed first (under bronchoscopy) before esophageal stent to prevent airway compression
• Stenting contraindicated at or above cricopharyngeus (pharyngeal foreign body sensation; unable to swallow)
• Peripheral bronchoesophageal fistulas: esophageal stent ± tracheobronchial stent

Summary Table