Ludwig’s angina

This is a medical emergency that may progress to septic shock and threaten the airway…

Ludwig’s angina was named after the Stuttgart physician Karl Fredrich Wilhelm von Ludwig, who first describes the condition in 1836.

His description was based on the observation of 5 patients with “gangrenous  indurations of the connective tissues of the neck that advanced to involve the tissues that cover the small muscles between the larynx and the floor of the mouth”.

Ludwig’s angina, is a rapidly progressive cellulitis of the floor of the mouth, involves the submandibular, submaxillary, and sublingual spaces.

The infection typically occurs in those with poor dental hydiene or following dental procedures. The causative organisms are typical normal oral flora and infection is usually polymicrobial in nature.

Causative organisms include:

• Streptococcus pyogenes
• Staphylococcus aureus
• Prevotella melaninogenicus
• Fusobacterium spp.

Ludwig’s angina usually occurs in patients with no comorbidities, but some groups may be at higher risk of developing the condition. This includes patients with:

• Diabetes mellitus
• Chronic alcohol abuse
• intravenous drug abuse
• HIV/ AIDS
• Malnutrition
• Poor oral hygiene
• and smokers

Ludwig’s angina:

• mostly affects people between the ages of 20 and 60 years.
• has a male predominance.
• is uncommon in children, but can present with no obvious cause.
• Had mortality >50% before the advent of penicillin, and with today’s antibiotics, surgical interventions and high-level supportive care mortality is about 8%.

The clinical features of Ludwig’s angina can rapidly evolve.

Symptoms may include:

• Mouth and throat pain
• Fever and chills
• Difficulty in swallowing and drooling
• Trismus (limited mouth opening)
• Hot potato voice
• Stridor is a late-sign and signifies potentially life-threatening airway compromise

• Fever, tachycardia, and progression to septic shock
• Bull neck appearance
• Tripod position and respiratory distress
• Tongue appears displaced superiorly and anteriorly, and inability to protrude the tongue
• Tenderness over the neck and throat
• Submandibular “woody” induration, crepitus or tenderness

 Ludwig’s angina is predominately a clinical diagnosis.

Laboratory tests are of limited utility, but may include:

• FBC
• U&E
• LFT
• Lactate
• CRP

Imaging is performed to evaluate for:

• airway patency
• extent of soft-tissue swelling
• extension of infection
• presence of drainable abscesses and gas
• Underlying dental disease

CT is the imaging modality of choice. The timing and choice of imaging modality depends on the patient’s clinical stability, airway patency and their ability to lie flat — ensure the airway is secure first!

• Local skin thickening
• Increased attenuation of subcutaneous fat
• Muscle enlargement
• Loss of fat planes within the submandibular space
• soft tissue emphysema and focal fluid collections

Image used with thanks to: http://neuroradiologyonthenet.blogspot.com/

Your priorities are to:

• Secure the airway early.
• Prepare and be ready for a difficult airway — expect that the patient will require a surgical airway.
• Prevent the development of septic shock and multi-organ failure — give antibiotics early.

Airway compromise due to expanding oedema of the soft tissues of the neck is the leading cause of death.

http://www.youtube.com/watch?v=qn_7tNSsNfs

Assess for signs of impending or actual airway compromise:

• trismus with mouth opening limited to 2 cm or less(inter-incisal distance)
• inability ot flex the neck without airway obstruction
• Elevation or firmness of the tongue, or inability to protrude the tongue
• Dsyphagia
• Dyspnoea
• Stridor is a late sign

Approaching the airway:

• Assign roles and ensure that the most experienced airway doctor available is present — ask anesthetics and ENT to attend immediately.
• Have the difficult and surgical airway trolley at the bedside.
• Use the LEMON mnemonic to identify difficult laryngoscopy and intubation — patients with Ludwig’s angina should be considered difficult intubations by definition!

Specific management

Early administration of antibiotics:

• broad spectrum antibiotics within the first hour of recognition of infection/sepsis greatly reduces mortality.

Current Therapeutic Guidelines – Antibiotics recommended for Ludwig’s angina in Australia:

• Metronidazole 500mg IV every 12 hours AND Benzylpenicillin 1.2g IV every 6 hours
• For patients with non-immediate hypersensitivity to penicillin:
  Cephazolin 1g IV every 8 hours.
• For patients with immediate hypersensitivity to penicillin:
  clindamycin 450 mg IV every 8 hours OR lincomycin 600 mg IV every 8 hours.

Steroids:

• Dexamethasone  8-12 mg IV initially then give in dose’s of 4-8mg every 6 hours for the first 48 hours.
• It’s postulated that dexamethasone provides initial chemical decompression by decreasing oedema and cellulitis, thus allowing improved penetration of antibiotics in the area.

Supportive care and monitoring

This patient requires 1:1 nursing care and monitoring:

• Patient will require continuous RR, ETCO2, ECG monitoring and invasive blood pressure monitoring.
• Fluid resuscitation to ensure adequate blood pressure and urine output.
• Indwelling catheter – ensure urine output of at lest 0.5-1mls/kg/hr.
• Stabilisation of blood sugar levels, and frequent monitoring.
• If airway secured consider lung protective ventilation strategies to prevent ARDS and ventilator associated pneumonia, including head up positioning.
• Consider other measures if patient boarded in ED for a while —- VTE and stress ulcer prophylaxis, regular pressure area care.

Remember FAST HUGS IN BED Please!

Disposition

• Early notification of ENT, anaesthetics and the operating theatres to facilitate definitive airway management.
• Early referral to the maxillofacial surgical team for surgical decompression of the sublingual, submental and submandibular spaces.
• Arrange for the patient to be admitted to ICU, for further supportive and post operative care.

http://www.youtube.com/watch?v=uvfsMhJtP7Y

The patient had a rocky course — he was initially taken from ED to the operating theater where an emergency tracheostomy was performed by the ENT team. He was then transferred to the CT scanner for imaging, and then back to the OT for surgical decompression of his submandibular and sublingual spaces. The patient arrived in ICU 8 hours after his initial presentation to the  ED with two drain’s in-situ and ongoing discharge of purulent fluid. On day 2 the patient developed ventilator-associated pneumonia and required mechanical ventilation for the next 6 days, during which time the oedema around his throat and the purulent discharge gradually decreased. ONce he was weaned from the ventilator he spent another 6 days on the ward prior to being discharged home with a tracheostomyy in-situ, and a plan for its reversal in the near future.

Anti-NMDA Receptor Encephalitis is the most likely diagnosis (see Q2).

Important differentials include:

• Infectious encephalidites (particularly HSV and HHV-6)
• Other autoimmune etiologies (e.g. limbic encephalitis due to autoantibodies against Hu, Ma2, CV2 and amphiphysin)
• Neuroleptic malignant syndrome
• Lethal catatonia
• Cerebral space-occupying lesions
• Metabolic disorders – hyper/hypothyroidism, Cushings syndrome, Addison’s disease
• Psychiatric disorders – schizophrenia, psychotic depression, pseudo-seizures
• Drugs, toxins or withdrawal (unlikely given the history)

Definitive diagnosis is achieved with positive NR1 and NR2 antibodies in CSF combined with a characteristic clinical picture (the antibodies have 100% sensitivity and specificity according to Wandinger et al 2011).

Relatively little is know about this disease, which was first described in 2007, with a subsequent case series published in 2008 in Lancet Neurology. Wandinger et al (2011) describe a typical clinical course:

“A non-specific flu-like prodrome (sub-febrile temperature, headache, fatigue) is always followed by a psychotic stage with bizarre behaviour, disorientation, confusion, paranoid thoughts, visual or auditory hallucinations and memory deficits. Because of these features a large proportion of patients end up in psychiatric therapy, and in many cases a drug-induced psychosis is initially diagnosed. In the following phase, decreased consciousness, hypoventilation, lethargy, seizures, autonomous instability and dyskinesias develop.”

Click to enlarge

To look for a pelvic tumour.

60% of patients with anti-NMDA receptor encephalitis have the presence of a tumour (most commonly teratoma). Early identification and removal of tumour is associated with better outcomes.

Antibodies against NR1-NR2 NMDA receptors are central to the pathogenesis of this condition.

These antibodies cause a reversible reduction in post-synaptic NMDA receptor clusters without complement activation. These receptors, which are highly expressed in the forebrain, limbic system, and hypothalamus, are made up of two subunits: the NR1 subunit, which binds glycine, and the NR2 subunit, which binds glutamate.

Owing to the fact that patients frequently improve with immunotherapeutic treatment and tumour removal, an immune mediated mechanism is highly likely.

Current evidence suggests early removal of tumour (if present) and immunotherapy are the mainstays of treatment.

Immunotherapy includes consideration of corticosteroids, intravenous immunoglobulin and plasma exchange therapy in severe cases.

Of the 100 cases in the article by Dalmau et al, the following outcomes were observed over a mean follow up period of 17 months:

• 47% Full Recovery
• 28% Mild stable deficits
• 18% Severe deficits
• 7% Death

There was a trend for better outcomes in patients who had tumour identified and removed within 4 months of symptom onset.

Orf is a zoonotic infection occurring in humans which is characterised by erythematous weeping nodules found most commonly on the hands and feet.

It is caused by the orf virus which is of the Parapoxvirus genus of the family Poxviridae.

This virus causes a highly contagious disease amongst small ruminant animals (e.g. sheep and goats) and is endemic to most countries of the world. In veterinary medicine it also goes by the names contagious pustular dermatitis, contagious ecthyma, sheep pox and my personal favourite – scabby mouth.

It is transmitted by direct contact from infected animals and is usually described as an occupational disease occurring in farmers, shepherds and shearers and also in abattoir workers, butchers and veterinarians. It is not strictly an occupational disease however with some interesting case reports of the disease occurring after animal contact during the “feast of sacrifice”

Orf usually appears as a small papule occurring on the hand or finger about one week after animal contact.

There may be multiple lesions and it may occur elsewhere such as on the face or penis (depending on where the unhygienic individual has wiped their hands after touching the dirty beast). The lesion grows to form a pustule usually 2-3cm in diameter. Six clinical stages have been described; maculopapular, target, acute, regenerative, papillomatous and regressive. It may be associated with a low-grade fever, malaise and lymphadenopathy and is usually tender and susceptible to haemorrhage in the early stages.

The clinical manifestations and exposure risks have overlap with other zoonoses such as:

• milker’s nodules
• cowpox
• mycobacteruim marinum (fish tank granuloma), and
• cutaneous anthrax (one not to miss)

The differential also includes:

• herpetic paronychial abscess
•  pyogenic granuloma
• deep fungal infection
• keratoacanthoma, and
• malignant tumours such as basal cell carcinoma (BCC).

The diagnosis should be suspected primarily on clinical assessment through a history of occupation/animal exposure and from the duration and nature of the lesion.

Serological testing may detect an antibody response but cannot discriminate orf from other parapoxviruses. Electron microscopy of the crust or the fluid within the lesion may similarly confirm parapoxvirus but not identify orf virus specifically. Polymerase chain reaction (PCR) using tissue, crust or fluid will confirm orf virus as the causative agent.

Orf is usually a self-limiting disease that resolves typically within 6 weeks usually without a residual scar.

In the immunocompromised patient it may be an entirely different kettle of fish and develop into a progressive lesion resistant to surgical debridement and topical treatment.

As the lesion will resolve spontaneously, treatment is supportive and symptomatic with a simple dressings, a topical antiseptic agent to prevent secondary infection and splinting of the infected finger may also be helpful.

Topical treatment with the antiviral agent cidofovir or the immune response modifier imiquimod have been described in certain case reports. Surgical treatments such as curettage and electrodesiccation or shave excision have been used, as has cryotherapy with liquid nitrogen.

It is important to be aware of orf when confronted with an unusual lesion on the hand or finger and seek a history of animal exposure which may lead you to the diagnosis of orf.

This combined with appropriate laboratory testing can confirm the diagnosis and save the patient from unwarranted and over aggressive treatments such as amputation (!).

Definitions vary widely, but a useful definition of emergency thoracotomy is:

“a thoracotomy performed prehospital, in the emergency department or elsewhere that is an integral part of the initial resuscitation of a patient”

The indications and contraindications for emergency thoracotomy are controversial, and may vary between institutions.

In general, the following are considered contraindications to performing an emergency thoracotomy:

• prehospital CPR performed for >15 minutes after penetrating chest injury without response
• prehospital CPR performed for >10 minutes after blunt chest injury without response
• the presence of coexistent injuries that are unsurvivable, e.g. severe head trauma
  (an exception maybe the patient who is a potential organ donor)
• asystole is the presenting rhythm, and there is no pericardial tamponade

Furthermore, it makes little sense to perform an emergency thoractomy in settings where there is no hope of providing definitive surgical interventions following the procedure.

The Moore et al (2011) study, which collected data from 18 US trauma centers, suggests that emergency thoracotomy is not as hopeless as once believed — hence blunt trauma alone is not listed as a contraindication. Also, compared to the recommendations of Hunt et al (2005) — as featured in EMCrit Podcast 36: Traumatic Arrest — longer CPR times are allowed (10 and 15 minutes, rather than 5 and 10 minutes for blunt and penetrating trauma respectively). Even with these increased time allowances there are still a few reported cases of patients with both penetrating or blunt chest trauma who have survived following even longer periods of CPR.

Survival rates directly correlate with the patient’s physiological status. This physiological status needs to be taken into account when considering the indications for an emergency thoracotomy.

According to Lorenz et al (1992) the patient’s physiological status can be classified as follows:

I — no signs of life (see Q4)

II — pulseless electrical activity

III — profound shock: SBP<60 mmHg; transient / no response to fluid resuscitation.

IV — mild shock: SBP 60-90 mmHg; stable response to fluid resuscitation.

According to Hunt et al (2005) ‘signs of life’ include:

• presence of a pulse or spontaneous movements
• GCS>3
• presence of pupillary reflexes, corneal reflexes or gag reflexes
• evidence of cardiac electrical activity on ECG, or contractile activity on bedside ultrasound
  (this information is rarely available in a prehospital setting)

The definition of what constitute ‘signs of life’ in this setting remains surprisingly controversial. As implied by the contraindications listed in Q2, emergency thoracotomy is essentially futile unless the patient has, or recently had, some signs of life.

Only if:

• the patient had definite signs of life at the scene, and
• none of the contraindications listed in Q2 are present.

Only if there is evidence of:

• intrathoracic hemorrhage
• severe extrathoracic hemorrhage
• pericardial tamponade
• systemic air embolism

Only if there is evidence of:

• intrathoracic hemorrhage
• severe extrathoracic hemorrhage
• pericardial tamponade
• systemic air embolism

The indications are the same as for scenario (b) above.

No

If possible, he should be urgently transferred to an operating theatre for an urgent thoracotomy instead.

Emergency thoractomy allows the following therapeutic interventions to be performed:

1. Release of pericardial tamponade —
   improves cardiac output and control of cardiac haemorrhage
2. Control of intrathoracic vascular or cardiac haemorrhage —
   facilitates  fluid resuscitation by ‘turning off the tap’
   improves cardiac output and myocardial perfusion
3. Control of massive air embolism or bronchopleural fistula —
   resolves myocardial ischaemia and hence  improves myocardial contractility as well as prevents neurological injury
4. Open cardiac massage —
   improves resuscitative cardiac output and coronary perfusion especially with limited ventricular filling pressures
5. Occlusion of the descending aorta (cross-clamping) —
   Redistribution of limited blood volume to myocardium and brain as well as limiting subdiaphragmatic losses.

Assess and manage the patient in a setting appropriately staffed and equipped for resuscitation using a coordinated team-based approach.

Perform the following key actions:

1. secure the airway by endotracheal intubation and commence ventilation and oxygenation.
2. seek and treat tension pneumothorax
   e.g. bedside ultrasound and bilateral finger thoracostomies.
3. seek and treat pericardial tamponade
   e.g. bedside ultrasound and emergency thoractomy.

If the patient has arrested and both tension pneumothorax and pericardial tamponade have been excluded, some experts would cease resuscitation at this point. Others would argue that there may be a role for emergency thoractomy if performed within 10 minutes of the arrest and the patient is actively resuscitated during this time.

Closed chest cardiac compressions and standard resuscitation drugs such as adrenaline are ineffective in the resuscitation of arrested trauma patients.

Despite this, CPR is routinely performed in such patients, especially in the prehospital setting. At best, CPR can be viewed as a temporising measure until emergency thoracotomy can be performed. It is far more important to give these patients blood products — not drugs — during resuscitation, while attempting to control the source of hemorrhage.

These presentations are potentially challenging for a number of reasons:

• patient factors —
  embarrassment, unreliable history, psychiatric illness
• possible sexual assault
• body packers require special consideration due to forensic issues and potential toxicity of package rupture
• potential harm to patient and staff if the object is dangerous, e.g. sharp objects
• procedural sedation and local/ regional anesthesia is usually required for attempted removal in the ED
• life threats are rare but possible, e.g. perforation

Patients need to be treated with the utmost sensitivity, no matter how strange and seemingly comical the situation is. Patients may have coexistent mental illness, may have been subjected to terrible assaults or may just have had an unfortunate mishap!

Our role is to help… not judge.

History

• type of foreign body
• delay since foreign body placement
• factors leading to the presence of the foreign body
  

1. sexual —
   autoerotic (most common type of rectal foreign body presentation)
   assault
2. non-sexual —
   body packing or stuffing of drugs
   psychiatric illness (present in about a third of cases)
   intellectual disability
   ingestion, e.g. toothpicks, bones, plastic objects
   iatrogenic, e.g. retained rectal thermometer

• previous extrication attempts
• previous foreign body presentations and treatments employed
• complications —
  pain, impaction, bowel obstruction, perforation, urinary retention, gastrointestinal hemorrhage, package rupture in body packers
• presence of other injuries if assault/ non-accidental injury suspected
• past medical history —
  comorbidities, previous surgery, medications, allergies
• social history

Examination

• assess for life threats —
  use an ABC approach and assess vital signs
  rule out perforation as a priority (suspect if  hypotension, tachycardia, peritonism and/or fever)
  look for evidence of gastrointestinal hemorrhage, package rupture in a body packer and life-threatening associated injuries if suspected assault
• abdominal examination —
  look for palpable mass, urinary retention, obstruction and perforation/ peritonism
  consider other causes of abdominal pain and perform genital examination
  
• rectal examination —
  assess for a palpable foreign body, for hemorrhage and for anal tone
  beware of potentially hazardous foreign bodies (e.g. sharp object)
• assess for associated injuries (head-to-toe exam)
• assess for toxidromes, e.g. heroin or cocaine toxicity in a body packer
• mental status examination

Laboratory tests are usually of limited utility in this setting

Bedside

• blood glucose and VBG —
  e.g. suspected obstruction or perforation, vomiting
• urine bHCG if female
• anoscopy, e.g. with small vaginal speculum

Laboratory

• FBC, UEC, LFTs, lipase —
  consider the differential diagnosis of abdominal pain
• Group and Hold —
  if significant hemorrhage

Imaging

• AXR and erect CXR —
  identify type and location of foreign body; stable patients with suspected complications such as obstruction or perforation
• CT abdomen —
  if body packing or foreign body is suspected but not visuallised on XR; stable patients with suspected complications such as obstruction or perforation

A circular radio-opaque object is present in the rectum. Complications cannot be excluded based on this image alone.

Photo by MHorama (click image for source)

Immediate management

• manage ABCs; seek and treat life threats

Specific management

• consider removal of the foreign body in the ED if it is:

1. not a dangerous object
   (e.g. light bulbs have high risk of breakage)
2. palpable on PR exam
3. distal to the sigmoid on AXR
   (foreign bodies proximal to the sigmoid tend to abut the sacrum preventing removal)

• provide adequate analgesia and sedation —
  e.g. titrated morphine and midazolam
  Ideally ensure the patient remains awake enough to help ‘push’
• provide local/ regional anesthesia —
  Perform a perianal block, e.g. circumferential subcutaneous infiltration around the anus using lignocaine with adrenaline, followed by a deeper intersphincteric block.
  A pudendal nerve block can also be performed.

• attempt foreign body removal using a stepwise approach and set a 10-20 minute limit on the duration of  attempted removal in the ED —
  Position the patient, e.g. lithotomy position; alternatives include: lateral decubitus, prone, or knee-chest position
  First attempt removal by pushing on the abdomen to propel the foreign body distally while pulling on the object with the fingers of your other hand inside the patient’s rectum. Forceps or a clamp may also be used.
  If unsuccessful, consider passing a foley catheter past the foreign body to help break the suction around the object (air can be insufflated proximal to the foreign body via the foley). Then inflate the balloon and withdraw to help dislodge the object.

• if still unsuccessful, obtain a surgical consult and consider the following options:

1. patient repositioning
2. sigmoidoscopy
3. vacuum devices
4. per rectal removal under general anesthesia
5. use of obstetric forceps
6. endoscopy-assisted removal
7. laparoscopy-assisted removal
8. laparotomy

• seek and treat complications —
  e.g. perforation, obstruction, hemorrhage, urinary retention, anal tone/ sphincter dysfunction, toxicity from package rupture in body packers, and medication adverse reactions (e.g. allergy, effects of procedural sedation)
• aftercare —
  consider performing anoscopy/ sigmoidoscopy following removal of a rectal foreign body to check for evidence of trauma

Supportive care and monitoring

• may include —
  IV hydration, analgesia, sedation, IDC, NGT if obstruction
• psychosocial support
• consider observation for a few hours post-removal and a repeat abdominal XR to check for evidence of perforation

Disposition

• see Q7 below

In the case of this billiard ball, passing a foley catheter beyond the object to release suction may be necessary. Gripping the ball may also be difficult. Vacuum devices or more invasive measures may be required.

Regarding the perianal block —
Rob Orman of ERCAST fame is the man I turn to when confronted with an anus problem, given his considerable experience with these issues … He describes the perianal block as an ‘auricular block of anus’ and finds it is usually successful. Infiltrate subcutaneously, pointing away from the rectum, by making 4 linear infiltrations in the shape of a box around the anus. Easy, eh. But, don’t try it at home kids…

Rectal regrets —
Check out this fantastic short ‘procedurette video’ by Whit Fisher which shows how to create a suction device out of a neonatal suction bulb and how to break suction by passing a foley catheter:

http://www.youtube.com/watch?v=t8GGQWMu004

Disposition depends on the nature of the foreign body, the success of the removal attempts, the presence of complications, and the patient’s mental state.

Disposition

• Successful removal in the ED —
  consider need for review by mental health team, sexual assault team and the social work team.
  provide written and verbal patient advice
  contact GP (phone/ fax/ letter) and arrange follow up, ideally the next day
  ensure careful documentation (especially if forensic issues)

• Body packers —
  consult toxicology and general surgery
  admit for consideration of whole bowel irrigation +/- endoscopic/ surgical removal

• Foreign body unable to be removed in the ED —
  admit under general surgery
  keep NBM pending possible transfer to the operating theatre

Cannabinoid Hyperemesis Syndrome

Cannabinoid hyperemesis syndrome is a rare condition associated with long-term or excessive  cannabis use that is characterised by recurrent episode of cyclical vomiting associated with episodes of abdominal pain. Patients are often noted to be compulsive showerers as it provides transient symptomatic relief.

Evidence is scarce but the following theories are postulated:

• Susceptible patients develop a hypersensitivity to cannabis following several years of exposure.
• Cannabis has a long half-life of weeks or months in the body. Regular use is accumulative and this gives rise to toxicity in the hypersensitive patient.
• It has been shown that cannabis delays gastric emptying and in the toxic patient this may lead to gastric stasis and hence hyperemesis.
• The patient may compulsively bathe because of the presence of the cannabinoid receptors in the limbic system of the brain.  The toxicity may disrupt the thermoregulatory systems of the hypothalmus and this disruption might settle with hot bathing or showering.

Features of cannabinoid hyperemesis syndrome include:

• A history of several years of cannabis abuse prior to the onset of  hyperemesis in susceptible individuals.
• The hyperemesis will follow a cyclical pattern every few weeks or months, often for many years, against a background of regular cannabis abuse.
• Cessation of cannabis leads to cessation of the hyperemesis in the presence of a negative urine drug screen for cannabinoids.
• A return to cannabis use will see a return of the hyperemesis many weeks or months later.
• The patient will compulsively bathe i.e. will take multiple hot showers or baths during the acute phase of the illness in an attempt to quell the hyperemesis.

Yes that’s true.

• Cannabinoids do have an active compound (delta-nine-tetrahydrocannabinol) that has been shown to act on the CB1 receptors in the brain to suppress emesis.
• However the majority of research is from animal trials and only limited human data is available to support this theory.

Cyclical vomiting is characterised as a phenomena of intermittent episodes of nausea and vomiting, separated in time by symptoms free periods.

• Hyperemesis gravidarum (always check beta-HCG in women of child-bearing age)
• Metabolic disorders (Addison’s disease, porphyria)
• Paediatric cyclical vomiting
• Migraine variants
• Drug withdrawal syndrome
• Bulimia and anorexia nervosa

These patients are often extensively worked up on previous presentations to the emergency department. Be sure to determine what previous investigations they have had and the results obtained.

Investigations may include:

• Bedside —
  BSL, VBG for acid-base status, lactate and electrolytes, urinalysis including bHCG
• Laboratory —
  FBC, U&E, LFT, lipase
• Consider a drug screen —
  may assist in the diagnosis of patients that deny cannabis use but clinical suspicion remains. Cannabinoids can be detected up to six weeks post-cessation of chronic use.

Management involves supportive care, symptom relief and behavioural modification.

Initial measures

• Attend to life threats:  airway,breathing and circulation and check glucose
• Commence cannabis withdrawal chart (if available)
• Consider intravenous hydration if dehydrated.
• Correct any electrolyte imbalances (especially potassium and magnesium)
• Administer antiemetics:
  e.g. Metoclopromide 10-20mg IV, ondansetron 4-8mg IV, or prochlorperazine 12.5mg
• Consider an antispasmodic: buscopan 10-20mg IV

If nausea and vomiting persists consider:

• Antipsychotics and low dose benzodiazepines —
  these have antiemetic effects and help relieve agitation:
  Droperidal 1-2.5mg IV
  Midazalam 0.5-1mg IV boluses titrated to effect

Long-term management

• Abstinence is the definitive treatment. Cessation of canabinoid use will lead to resolution of all symptoms and recommencement will lead to a relapse of the canabinoid hyperemesis syndrome.
• Follow-up by drug and alcohol counselling service.

Retrieval triage and planning:

• This is an urgent priority (1-3hrs response time). The patient is currently stable but is in a facility without surgical services or a blood bank with no obstetric trained staff.
• Ideally a two person retrieval team should be sent. At least one of them should be competent in acute obstetric emergency care including managing obstetric haemorrhage
• It is difficult to define best practice but, in general, taking blood products to a known obstetric haemorrhage case, even if the patient is currently no longer bleeding is prudent retrieval practice. This is particuarly important with remote locations where transport times may be prolonged. Prehospital ultrasound assessment is a growing field with no best practice established yet. Obstetric retrieval cases are ideal candidates for prehospital USS assessment as majorly relevant findings can be determined rapidly ( number of foetuses, presenting part, placental location)
• Ideally prior to setting off on the retrieval, basic antenatal information including obstetric examination findings would be handy!

Antenatal history:

• G7P5, 1 termination
• Poor antenatal care this pregnancy
• Had one USS but results lost
• EDC from USS was recorded and 34 weeks currently by that
• All previous pregnancies delivered vaginally
• Rh positive

Current obstetric exam by midwife:

• BP 120/80, PR 80, RR12
• Not in distress, no contractions
• FHR 130
• Cervical exam not done
• Foetal fibronectin not done

There are a number of worrying features of the history.

• A multiparous mother is paradoxically at more risk of obstetric complications such as post partum haemorrhage.
• She has had almost no routine antenatal care with the only obstetric USS result being lost. We do not know the placental location with certainty and in the setting of a small PV bleeding in third trimester this could spell disaster such as a praevia or worse.

Whilst the obstetric exam findings are reassuring for now the remoteness of the patient location and the worrying history would place this retrieval as a high priority.

Foetal fibronectin is a protein released when separation of chorion and decidua starts to occur. It can be detected by a simple point of care test.

• It is a useful negative predictor of preterm delivery within next 7-10 days. In other words, if the test is negative, mother is unlikely to proceed to deliver within 7-10 days.
• Unfortunately a few things mess up the test and one of them is blood. Therefore it is not helpful in this case and really the concern here is not with preterm delivery but bleeding to death!

The three most important causes of PV bleeding to consider are:

1. placenta (abruption, praevia, abnormal implantation)
2. uterus (uterine rupture)
3. fetus (vasa praevia)

Uterine blood flow at term is approximately 500ml/min. This is important when considering the need for blood products… Which brings us to part c.

Anyone doing this retrieval should want to take blood. How much? As much as possible — if you’re dealing potential blood losses of 500ml/min, you’ll want as much as your service can take on a retrieval!

No!

You are not treating preterm labour.. Active obstetric haemorrhage is an absolute contraindication to tocolysis!

Definitive care requires an obstetrically skilled surgeon, an obstetric anesthetist, a surgical suite, and a blood bank and pathology service capable of massive blood transfusion therapy.

Clearly, this cannot be provided by the retrieval team.

The management priority is resuscitation with emphasis on:

• uterine displacement with lateral tilt of the pelvis
• aggressive fluid therapy with early blood product replacement

A role for permissive hypotension in the resuscitation of the bleeding obstetric patient is unproven and may contribute to a poor fetal outcome. I suggest avoiding it in this scenario.

Australian guidelines for critical bleeding/ massive transfusion are considered in Hematology Hoodwinker 003 — Managing the Critical Bleeder!!

Haemostatic agents have actions that are either:

• Systemic — inhibit fibrinolysis or promote coagulation, or
• Local — cause vasoconstriction or promote platelet aggregation

Let’s consider FRecombinant factor VII first. We must remember that pregnancy is already a pro-coagulant state. Furthermore, in the retrieval setting, prior to knowing what the coagulation status is, giving Recombinant factor VII is not proven for traumatic bleeding let alone obstetric bleeding! The definitive care is surgical control of the anatomy. Factor VIIa is generally reserved for situations that involve a salvageable patient, bleeding that cannot be surgically corrected, and there has been adequate replacement of blood products (e.g. platelets >80, INR <1.5), and correction of acidosis (e.g. PH>7.2) and hypothermia (e.g. T>34C).

For a detailed discussion of Recombinant Factor VIIa check out: Hematology Hoodwinker 002 — Factor VIIa to the Rescue!?

What about tranexamic acid?

• The CRASH 2 trial indicates early use under 3hrs from injury improves outcomes in haemorrhaging trauma patients
• There are recommendations for prehospital use in absence of liberal blood product availability and long transport times

Tranexamic acid is an intriguing potential prehospital haemostatic agent with current studies looking at its role in obstetric bleeding and reducing need for transfusion. It is also cheap and easy to administer.

Prothrombinex is another option:

• Freeze dried human clotting factors
• Licensed for warfarin overdose or congenital clotting deficiency
• Off label for trauma
• Theoretical clot risk combined with tranexamic acid

Foetal tachycardia

• This is not a good sign in the setting of obstetric bleeding. It implies foetal distress, probably from acute placental blood loss.
• The baby is bleeding to death! 
• It does matter but in retrieval setting little can be done apart from resuscitation of the mother as best as possible. Ideally baby should be delivered emergently.

Abnormal vasculature of the placenta

• Abnormal vasculature on placental USS in setting of major obstetric haemorrhage must make one suspiscious of abnormal placenta such as an accreta or percreta.
• The likelihood of the patient needing emergency peripartum hysterectomy is high.

This is a difficult decision to make. It is akin to letting parents into the resuscitation room with their critically ill/injured child. My personal view is that if you are going to die, you should  have your family with you if possible.

Furthermore, leaving country for Indigenous folk is a big deal and dying out of country is an even bigger cultural issue. Thus, I believe we should try to ensure that the next of kin is transported with all critically ill/injured patients from remote areas.

This is what happened:

• RFDS flies in another surgeon from Townsville
• Brings Novoseven ($30000 worth) as well as 12 PRBC and 12 FFP and 10 PLT
• Surgeon goes straight to OT and performs a hysterectomy

• It is not ideal to subject a post operative patient who has had a massive blood transfusion to the added stress of aeromedical transport. However, it also not ideal to leave them in a hospital that has exhausted it blood supplies and its staff!
• I also was asked to return the surgeon back to Townsville so he could go home… a request that is hard to refuse. Indeed, this the first I had a surgeon escort on a retrieval flight!

What is needed is basically good critical care with some minor adjustments for aeromedicine such as checking ETT cuff pressure with a manometer during climb, cruise and descent. Among supportive care measures, good analgesia and sedation are important minimise the stress from vibration and noise.

The outcome

• The patient survived after 3 days in ICU.
• The patient’s husband was understandably very traumatised by whole ordeal
• Sadly, their baby suffered hypoxic brain injury

The key learning points

• Unexplained obstetric PV bleeding is an emergency till proven otherwise
• Beware the multigravid patient in a remote community
• Never forget the baby during resuscitiation
• Haemorrhagic resuscitation in the retrieval setting = preserve blood volume + coagulation
• Always consider the trauma of retrieval for the patient and family

High-pressure injection injury to the hand.

• High-pressure injection injuries are caused by the accidental injection of fluid from high pressure industrial equipment such as hydraulic systems or paint spraying devices.
• High-pressure injection injures are a surgical emergency.
• Most commonly occur in occupational settings.
• High pressure injection guns are used for many tasks such as painting, lubricating, greasing, cleaning and the mass inoculation of animals.
• Injury generally occurs in young males and affect the non-dominant hand: commonly the index finger, followed by middle finger and palm. Injury generally results in people with limited experience using these devices.
• Common materials found in high-pressure injuries are:
  • Paint (60%)
  • Grease (25%)
  • Water
  • Oil
  • Diesel
  • Paint thinner
  • Animal vaccines

• High-pressure guns emit jet streams at pressures of up to thousands of psi. At these extreme pressures, material is forced through the skin and may track along fascial planes, tendon sheaths, and neurovascular bundles.
• Although high-pressure injection injuries are relatively uncommon, they may occur easily because as only 100psi is sufficient pressure to breach the skin.
• Even small amounts of contaminants injected into the skin can lead to compartment syndrome, poor perfusion and closed spaced infection of the anatomical spaces and tendon sheaths.

Tissue injury from high-pressure injection is dependent on the following factors:

• Type of material injected
• Amount injected
• Anatomical location
• Velocity of injected material

• Patients generally give a history of coming into close contact with the jet stream during either the clearing or cleaning of the nozzle, or during general use of the device.
• Early examination generally reveals no apparent break in the skin or a benign-appearing pinhole-sized puncture wound with minimal symptoms.
• Symptoms generally develop over 4-6 hours as the digit/extremity becomes swollen, painful and pale.
• Symptoms are generally classified into 3 stages:

1. Acute Stage

• • Occurs with 4-6 hours
  • Symptoms include swelling, anaesthesia and vascular insufficiency
  • Without prompt treatment the digit/extremity will succumb to tissue ischaemia and necrosis by 12 hours
  • Rarely systemic absorption can occur from the foreign material causing a generalised toxic reaction characterised by lymphagitis, leukcytosis and fever.

2. Intermediate Stage

• • Is characterized by the development of oleomas (lipogranulomas) at the injection site and throughout the affected tissues.
  • The reaction to the foreign material in the tissues make the body prone to the development of these nodular tumors.
  • The oleomas generally remain dormant, however can become fibrosed resulting in functional loss.

3. Late Stage:

• • The late stage is characterized by the breakdown of the skin integrity over the oleomas leading to ulcer and sinuses formation resulting in secondary infection.

Image Source: www.medscape.com

Emergency department management starts with the clinician being able to recognise the seriousness of this injury, initiate prompt aggressive management and arrange for urgent review by a hand surgeon.

“The mainstay of treatment involves surgical decompression, removal of the foreign material,and debridement of the necrotic tissue.”

The following steps can be taken to improve outcomes while the patient is in the emergency department:

• Developing of pain is a worrying sign provide effective analgesia via the oral or parenteral route. Digital nerve blocks are not recommended as will increase compartment pressures and alter ongoing examination of the digit.
• Initiate broad spectrum antibiotics to cover for both gram negative & positive organisms (consult local/institutional guidelines)
• Arrange for imaging:
  • X-ray is the imaging of choice
  • Help determine dispersion and thus extent of exploration required
  • Lead-based paints appear as soft tissue densities (avoid confusing with calcifications)
  • Non-lead-based paints appear as subcutaneous emphysema
  • Grease will appear as a lucency
• The use of steroids to reduce inflammation from the foreign material has shown promise in animal studies, however some clinicians are concerned regards the risk of increasing infection rates from the use of steroids.
• Check patients tetanus status and update accordingly
• Urgent consultation with a hand specialist for early decompression, debridement and washout is required.
• Keep patient NBM and prepare for theatre
• Even if debridement occurs within 6 hours for paint thinner, jet fuel, petrol, or oil the amputation rate is still 38%, compared to 58% amputation rate when treatment occurs over 6 hours. High caustic agents like terpentine and paint solvents, along with higher injection pressures have an amputation rate of about 80% even with appropriate care.
• Of the injuries that don’t result in amputation most will have some form of long-term functional impairment and patients should be counselled accordingly.

Image Source: www.medscape.com

• Never judge a book by its cover!!! Don’t be fooled by benign-appearing high-pressure injection wounds.
• Provided broad spectrum antibiotics early
• Consult hand surgeon as soon as you suspect high-pressure injection injury.
• Educate patient of the high likelihood of some level of dysfunction despite adequate treatment.