Lymphedema – Management and Treatment

DEFINITION
Lymphedema refers to excessive accumulation of interstitial protein-rich fluid, typically resulting from impaired regional lymphatic drainage.


PHYSICAL FINDINGS AND CLINICAL PRESENTATION
Edema
● Painless and progressive
1. Initially, the edema is pitting and smooth; however, with advanced cases, the edema becomes nonpitting            (this depends on the extent of fi brosis that has occurred).
2. Elevation of the extremity resolves the swelling in the early stages but not in the advanced stages.
● More often unilateral  but, depending on the cause, can be bilateral
● Not always restricted to the lower extremities but may involve the genitals, face, or upper extremities (e.g., arm
swelling after mastectomy)

● Stemmer’s sign (squaring of the toes caused by edema in the digits)
● Buffalo hump appearance of the dorsum of the foot
● Loss of the ankle contour, giving a tree trunk appearance
to the leg .
Skin
● Hard, thick, leathery skin secondary to fi brosis induced
by chronic stasis
● Occasional drainage of lymph
● Infections (cellulitis, lymphangitis, onychomycosis)
CAUSE
Lymphedema is caused by a reduction in lymphatic transport
and is classifi ed into primary and secondary forms.
Primary idiopathic lymphedema is thought to result from
developmental abnormalities such as lymphatic hypoplasia
and functional insuffi ciency or absence of lymphatic valves.
Subclasses of this type of lymphedema include the following:
● Congenital lymphedema
1. Detected at birth or recognized within the fi rst 2 years of life
2. Involving one or both extremities, usually the entire leg
3. May be familial (Milroy’s disease)
● Lymphedema praecox
1. Onset in teenage years
2. Usually unilateral
3. Most common form of primary lymphedema (up to 94% of cases)

4. More common in females (10:1), suggesting that estrogen plays a role in pathogenesis
5. May be familial (Meige’s disease)
● Lymphedema tarda
1. Usually occurs after the age of 30 years
2. Uncommon, accounting for less than 10% of cases of

primary lymphedema
Secondary lymphedema develops after disruption or obstruction of the lymphatic system as a consequence of the following:
● Surgery for malignant tumors (e.g., breast, prostate, lymphoma)
● Edema of the arm after axillary lymph node dissection is the most common cause of lymphedema in the United States.
● Incidence of lymphedema is approximately 14% in post mastectomy patients with adjuvant radiation.


TREATMENT
● Inflammation (streptococci, filariasis)
● Filariasis is most common cause of lymphedema worldwide
● Trauma
● Radiation with lymph node removal
DIFFERENTIAL DIAGNOSIS
● Lymphedema is primarily a clinical diagnosis made on the basis of physical features that distinguish it from other
causes of chronic edema of the extremities, such as the presence of cutaneous and subcutaneous fi brosis (peau d’orange) and the Stemmer sign.
● When physical examination is inconclusive, other available imaging tests can help make the diagnosis: isotopic
lymphoscintigraphy, indirect and direct lymphography, lymphatic capillaroscopy, MRI, CT, ultrasound
● Isoptopic lymphoscintigraphy is currently considered the gold standard for diagnosis of lymphedema.
● Exclude other causes of edema (e.g., cirrhosis, nephrosis, CHF, myxedema, hypoalbuminemia, chronic venous stasis, reflex sympathetic dystrophy, obstruction from abdominal or pelvic malignancy).
LABORATORY TESTS
● BUN, creatinine (Cr), liver function tests, albumin, urine analysis, thyroid function tests (TFTs) are obtained to exclude possible systemic causes of edema.
● Noninvasive venous studies help exclude venous insufficiency.
● Genetic testing may be practical in defining a specific hereditary syndrome with a discrete gene mutation, such as
lymphedema-distichiasis (FOXC2) and some forms of Milroy disease (vascular endothelial growth factor receptor
3 [VEGFR-3]).
IMAGING STUDIES
● Lymphoscintigraphy:
1. Diagnostic image of choice
2. Sensitivity and specifi city of 100% in diagnosing lymphedema
● CT scan: to exclude malignancy leading to obstruction
● Duplex ultrasound to rule out venous obstruction as a cause for edema
● Lymphangiography:
1. Available but rarely used
2. May be requested by surgeons considering repair or excision of tissue for lymphedema
3. Difficult to perform; most information can be obtained
from the nuclear lymphoscintigram.

TREATMENT
Complex decongestive therapy (CDT) is backed by long-standing experience as the primary treatment of choice for lymphedema in children and adults. It involves a two-stage treatment
program:
1. Reduce leg swelling and size:
● Leg elevation
● Limb massage
● Pneumatic leg compression
2. Maintain edema-free state:
● Elastic support stockings that are properly fi tted according to compression pressure and length are essential to
prevent edema from returning.
● Compression pressures are graduated; most of the pressure is distal, with less and less pressure from the stockings
moving proximally.
● Compression pressures range from 20 to 30 mm Hg, 30 to 40 mm Hg, 40 to 50 mm Hg, and 50 to 60 mm Hg. Most prefer 40 to 50 mm Hg for lymphedema.
● The length should cover the edematous site. Choices include below-knee, thigh-high, and pantyhose lengths.
● No drugs have been shown to be benefi cial. Diuretics, in particular, should not be used because they may promote
the development of volume depletion.
● Treat infections, such as lymphangitis (usually caused by group A streptococcus), with antibiotics. Clotrimazole 1%
cream should be applied daily to dried fi ssured areas between the toes to prevent fungal infections.
● In secondary lymphedema, treating the underlying cause is indicated (e.g., prostate cancer, breast cancer). If the cause is filariasis caused by the parasites Wuchereria bancrofti or Brugia malayi, treatment is diethylcarbamazine citrate.
● Surgery for chronic lymphedema should act as an adjunct to CDT or as an alternative if CDT has proved unsuccessful.
● Operative treatment is considered for the following:
● Continued increase in leg size, despite medical treatment
● Impaired leg function
● Recurrent infections
● Emotional lability secondary to the cosmetic appearance

Salivary gland neoplasms-management and prevention

DEFINITION
Salivary gland neoplasms are benign or malignant tumors of a salivary gland (parotid, submandibular, or sublingual).
PHYSICAL FINDINGS AND CLINICAL PRESENTATION
● Parotid gland
● Painless swelling overlying the masseter muscle (under the temporomandibular joint)
● Pain
● Facial nerve palsy
● Cervical lymph nodes
● Mass in oral cavity
● Submandibular gland: swelling under anterior portion of the mandible
● Sublingual gland: intraoral swelling under the tongue, medial to the mandible
● Salivary gland neoplasms most often present as slowgrowing, well-circumscribed masses. Pain, rapid growth,
nerve weakness, fi xation to skin or underlying muscle, and paresthesias usually are indicative of malignancy.

DIFFERENTIAL DIAGNOSIS
Benign tumors
● Mixed tumor (usually parotid)
● Adenolymphoma (Warthin’s tumor)
● Pleomorphic adenoma
● Capillary hemangioma, lymphangioma (in children)
● Intraductal papilloma
● Other (e.g., myoepithelioma, canalicular adenoma, basal cell adenoma)
Malignant tumors
● Mucoepidermoid carcinoma (most common malignant tumor of the parotid gland)
● Adenoid cystic carcinoma
● Adenocarcinoma
● Malignant mixed tumor
● Squamous cell carcinoma
● Other

WORKUP
● Fine-needle aspiration. The sensitivity, specifi city, and accuracy of parotid gland aspirates are approximately 92%,
100%, and 98%, respectively
● Imaging by CT scan (see Fig. 94–1B) or MRI
● Open biopsy (rarely indicated; see Fig. 94–1C)
TREATMENT
Malignant tumors
● Surgery is the mainstay of treatment; gland resection and neck dissection if lymph nodes are involved
● A lateral lobectomy with preservation of facial nerve should be considered for tumors confi ned to the superfi cial
lobe of the parotid gland. Gross tumor should not be left in situ, but if the facial nerve is able to be preserved by “peeling” the tumor off the nerve, it should be attempted, followed by radiation therapy for microscopic disease.

● Postoperative radiation is indicated for high-grade malignancies demonstrating extraglandular disease, perineural invasion, direct invasion of surrounding tissues, or regional metastases.
● Chemotherapy Benign tumors
● Surgery for tumor resection

Scarlet fever-Management and Treatment

DEFINITION
Scarlet fever is a rash involving the skin and tongue and complicating
a streptococcal group A pharyngitis.

PHYSICAL FINDINGS AND CLINICAL PRESENTATION
● Diffuse erythema, beginning on the face and spreading to the neck, back, chest, rest of trunk, and extremities; most
intense on inner aspects of arms and thighs
● Erythema blanches, but nonblanching petechiae may be present or produced by a tourniquet.
● Strawberry or raspberry tongue
● Rash lasts about 1 week and then desquamates
● Febrile illness with headache, malaise, anorexia, and pharyngitis begins after a 2- to 4-day incubation period
● Scarlatinal rash begins 1 or 2 days after the onset of
pharyngitis.

CAUSE
Caused by group A beta-hemolytic Streptococcus infection, which produces one of three erythrogenic toxins. Note: Some streptococcal species have the ability to cause both scarlet fever and
rheumatic fever.
DIFFERENTIAL DIAGNOSIS
● Viral exanthems
● Kawasaki disease
● Toxic shock syndrome
● Drug rashes

LABORATORY TESTS
● Identifi cation of group A Streptococcus by throat culture
● SLO antibody titers
TREATMENT
● Penicillin

Sinusitis-Management and Prevention

DEFINITION
Sinusitis is infl ammation of the mucous membranes lining one or more of the paranasal sinuses. The various presentations are as folllows:
● Acute sinusitis: infection lasting less than 30 days, with complete resolution of symptoms
● Subacute infection: lasts from 30 to 90 days, with complete resolution of symptoms
● Recurrent acute infection: episodes of acute infection lasting less than 30 days, with resolution of symptoms, which
recur at intervals of at least 10 days apart
● Chronic sinusitis: infl ammation lasting more than 90 days, with persistent upper respiratory symptoms
● Acute bacterial sinusitis superimposed on chronic sinusitis:
new symptoms that occur in patients with residual symptoms from prior infection(s). With treatment, the new
symptoms resolve but the residual ones do not.

 
PHYSICAL FINDINGS AND CLINICAL PRESENTATION
● Patients often give a history of a recent upper respiratory illness with some improvement, then a relapse.
● Mucopurulent secretions in the nasal passage
● Purulent nasal and postnasal discharge lasting more than 7 to 10 days
● Facial tightness, pressure, or pain
● Nasal obstruction
● Headache
● Decreased sense of smell
● Purulent pharyngeal secretions, brought up with cough, often worse at night
● Erythema, swelling, and tenderness over the infected sinus in a small proportion of patients
● Diagnosis cannot be excluded by the absence of such findings.
● These fi ndings are not common, and do not correlate with number of positive sinus aspirates.
● Intermittent low-grade fever in about one half of adults with acute bacterial sinusitis
● Toothache is a common complaint when the maxillary sinus is involved.
● Periorbital cellulitis and excessive tearing with ethmoid sinusitis
● Orbital extension of infection: chemosis, proptosis, impaired extraocular movements.
Characteristics of acute sinusitis in children with upper respiratory tract infections:
● Persistence of symptoms
● Cough
● Bad breath
● Symptoms of chronic sinusitis (may or may not be present)
● Nasal or postnasal discharge
● Fever
● Facial pain or pressure
● Headache
● Nosocomial sinusitis is typically seen in patients with nasogastric tubes or nasotracheal intubation.

CAUSE
● Each of the four paranasal sinuses is connected to the nasal cavity by narrow tubes (ostia), 1 to 3 mm in diameter; these drain directly into the nose through the turbinates. The sinuses are lined with a ciliated mucous membrane (mucoperiosteum).
● Acute viral infection
● Infection with the common cold or infl uenza
● Mucosal edema and sinus infl ammation
● Decreased drainage of thick secretions, obstruction of the sinus ostia
● Subsequent entrapment of bacteria
a. Multiplication of bacteria
b. Secondary bacterial infection
 Other predisposing factors
● Tumors
● Polyps
● Foreign bodies
● Congenital choanal atresia
● Other entities that cause obstruction of sinus drainage
● Allergies
● Asthma
● Dental infections lead to maxillary sinusitis.
● Viruses recovered alone or in combination with bacteria (in 16% of cases):
● Rhinovirus
● Coronavirus
● Adenovirus
● Parainfluenza virus
● Respiratory syncytial virus
● The principal bacterial pathogens in sinusitis are Streptococcus pneumoniae, nontypeable Haemophilus influenzae, and Moraxella catarrhalis.
● In the remainder of cases, fi ndings include Streptococcus pyogenes, Staphylococcus aureus, alpha-hemolytic streptococci, and mixed anaerobic infections (Peptostreptococcus, Fusobacterium, Bacteroides, Prevotella).

Infection is polymicrobial in about one third of cases.
● Anaerobic infections seen more often in cases of chronic sinusitis and in cases associated with dental infection; anaerobes are unlikely pathogens in sinusitis in children.
● Fungal pathogens are isolated with increasing frequency in immunocompromised patients but remain uncommon
pathogens in the paranasal sinuses. Fungal pathogens include Aspergillus, Pseudallescheria, Sporothrix, phaeohyphomycoses, Zygomycetes.
● Nosocomial infections occur in patients with nasogastric tubes, nasotracheal intubation, cystic fi brosis, or those who are immunocompromised.
● S. aureus
● Pseudomonas aeruginosa
● Klebsiella pneumoniae
● Enterobacter spp.
● Proteus mirabilis
Organisms typically isolated in chronic sinusitis:
● S. aureus
● S. pneumoniae
● H. infl uenzae
● P. aeruginosa
● Anaerobes

DIFFERENTIAL DIAGNOSIS
● Migraine headache
● Cluster headache
● Dental infection
● Trigeminal neuralgia
WORKUP
● Water’s projection: sinus radiograph
● CT scan
● Much more sensitive than plain radiographs in detecting acute changes and disease in the sinuses
● Recommended for patients requiring surgical intervention, including sinus aspiration; it is a useful adjunct to
guide therapy.
● Transillumination
● Used for diagnosis of frontal and maxillary sinusitis
● Place transilluminator in the mouth or against cheek to assess maxillary sinuses, and under the medial aspect of the supraorbital ridge to assess frontal sinuses.
● Absence of light transmission indicates that sinus is filled with fluid.
● Dullness (decreased light transmission) is less helpful in diagnosing infection.
● Endoscopy
● Used to visualize secretions coming from the ostia of infected sinuses
● Culture collection via endoscopy often contaminated by nasal flora; not nearly as good as sinus puncture
● Sinus puncture
● Gold standard for collecting sinus cultures
● Generally reserved for treatment failures, suspected intracranial extension, nosocomial sinusitis.
TREATMENT
Nonpharmacologic therapy
● Sinus drainage
● Nasal vasoconstrictors, such as phenylephrine nose drops, 0.25% or 0.5%

● Topical decongestants should not be used for more than a few days because of the risk of rebound congestion.
● Systemic decongestants
● Nasal or systemic corticosteroids, such as nasal beclomethasone, short-course oral prednisone
● Nasal irrigation, with hypertonic or normal saline (saline may act as a mild vasoconstrictor of nasal blood fl ow)
● Use of antihistamines has no proved benefi t, and the drying effect on the mucous membranes may cause crusting,
which blocks the ostia, thus interfering with sinus drainage.
● Analgesics, antipyretics.
Antimicrobial therapy
● Most cases of acute sinusitis have a viral cause and will resolve within 2 weeks without antibiotics.
● Current treatment recommendations favor symptomatic treatment for those with mild symptoms.
● Antibiotics should be reserved for those with moderate to severe symptoms who meet the criteria for diagnosis of
bacterial sinusitis.

● Antibiotic therapy is usually empirical, targeting the common pathogens.
● First-line antibiotics include amoxicillin, TMP-SMZ.
● Second-line antibiotics include clarithromycin, azithromycin, amoxicillin-clavulanate, cefuroxime axetil, loracarbef, ciprofloxacin, levofloxacin.
● For patients with uncomplicated acute sinusitis, the less expensive first-line agents appear to be as effective as the
costlier second-line agents.

Surgery
● Surgical drainage indicated
● If intracranial or orbital complications suspected
● For many cases of frontal and sphenoid sinusitis
● For chronic sinusitis recalcitrant to medical therapy
● Surgical débridement imperative for treatment of fungal sinusitis

BRAIN TUMOURS-Management and Treatment

Primary intracranial tumours account for 10% of all neoplasms, and in the UK about half of all intracranial tumours are metastatic. Primary intracranial tumours are derived from the skull itself, or from any of the structures lying
within it, or from their tissue precursors. They may be malignant on histological investigation but rarely metastasize outside the brain. The most common intracranial tumours occurring in adults . Pituitary
tumours are discussed separately .

Clinical features
The clinical features of a cerebral tumour are the result of the following:
– Progressive focal neurological deficit
– Raised intracranial pressure
– Focal or generalized epilepsy.
Neurological deficit is the result of a mass effect of the tumour and surrounding cerebral oedema. The deficit depends on the site of the tumour, e.g. a frontal lobe tumour will initially cause personality change, apathy and
intellectual deterioration. Subsequent involvement of the frontal speech area and motor cortex produces expressive aphasia and hemiparesis. Rapidly growing tumours destroy cerebral tissue and loss of function is an early
feature.

Raised intracranial pressure produces headache, vomiting and papilloedema.
The headache typically changes with posture and is made worse by coughing, sneezing, bending and straining.
As the tumour grows there is downward displacement of the brain and pressure on the brainstem, causing drowsiness, which progresses eventually to respiratory depression, bradycardia, coma and death. Distortion of normal structures at a distance from the growing tumour leads to focal neurological signs (false localizing signs). The most common are a third and sixth cranial nerve palsy  resulting from stretching of the nerves by downward
displacement of the temporal lobes. Epilepsy Fits may be generalized or partial in nature. The site of origin
of a partial seizure is frequently of value in localization.

Differential diagnosis
The main differential is from other intracranial mass lesions (cerebral abscess, tuberculoma, subdural haematoma and intracranial haematoma) and a stroke, which may have an identical clinical presentation. Benign (idiopathic)
intracranial hypertension presents with headache and papilloedema in young obese females. Neuroimaging is normal but at lumbar puncture there is raised CSF pressure.

Investigations
CT and MRI are useful in detecting brain tumours. MRI is of particular value in investigation of tumours of the posterior fossa and brainstem. MR angiography is sometimes necessary to define the site or blood supply of a mass, particularly if surgery is planned. Positron emission tomography (PET) is sometimes helpful to locate an occult primary tumour with metastasis. Plain skull X-rays have no value in brain tumour
diagnosis.
– Other investigations. These include routine tests, e.g. chest X-ray if metastatic disease is suspected. Lumbar puncture and examination of the CSF is contraindicated with the possibility of an intracranial mass
lesion because of danger of immediate herniation of the cerebellar tonsils, impaction within the foramen magnum and compression of the brainstem (‘coning’).

Management
– Surgery. Surgical exploration, and either biopsy or removal of the mass, is usually carried out to ascertain its nature. Some benign tumours, e.g. meningiomas, can be removed in their entirety without unacceptable
damage to surrounding structures.
– Radiotherapy is given for gliomas and radiosensitive metastases and improves survival slightly.
– Medical treatment. Cerebral oedema surrounding a tumour is rapidly reduced by corticosteroids; i.v. or oral dexamethasone. Epilepsy is treated with anti-convulsants. Chemotherapy has little real value in the majority
of primary or secondary brain tumours. The prognosis is very poor in patients with malignant tumours, with only 50% survival at 2 years for high-grade gliomas.

THE LYMPHOMAS(Hodgkin’s lymphoma and Non-Hodgkin’s lymphoma)

The lymphomas are neoplastic transformations of normal B or T cells which reside predominantly in lymphoid tissues. They are commoner than the leukaemias and are increasing in incidence for reasons that are unclear. The
disease is classified on the basis of histological appearance into Hodgkin’s and non-Hodgkin’s lymphoma (NHL).
Hodgkin’s lymphoma
Hodgkin’s lymphoma is primarily a disease of young adults. Previous infection with Epstein–Barr virus (EBV) is thought to play a role in pathogenesis in some patients.

Clinical features
Painless lymph node enlargement (often cervical nodes) is the most common presentation. These lymph nodes have a rubbery consistency on examination. There may be hepatosplenomegaly. Systemic ‘B’ symptoms
are fever, drenching night sweats and weight loss (>10% in previous 6 months). Other constitutional symptoms such as pruritus, fatigue, anorexia and alcohol-induced pain at the site of the enlarged lymph nodes also occur.
Investigations
– Blood count may be normal or show a normochromic, normocytic anaemia.
– The erythrocyte sedimentation rate (ESR) is usually raised and is an indicator of disease activity.
– Liver biochemistry may be abnormal, with or without liver involvement.
– Serum lactate dehydrogenase (LDH) if raised is an adverse prognostic marker.
– Chest X-ray may show mediastinal widening from enlarged nodes.
– Diagnosis is by lymph node biopsy and histological examination showing Reed–Sternberg cells (binucleate or multinucleate malignant B lymphocytes) in a background rich in benign small lymphocytes and histiocytes.
– Disease staging is by computed tomography (CT) scan, which may show intrathoracic, abdominal and pelvic nodes. Positron emission tomography (PET) (p. 828) is increasingly being used for staging, assessment of response and direction of treatment.

Differential diagnosis
This includes any other cause of lymphadenopathy (Table 6.3). Persistently
enlarged lymph nodes must always be excised for histological and microbiological
examination for diagnostic purposes.
Management
Treatment is given with curative intent. The choice of treatment depends on:
– Involved sites
– ‘Bulk’ of lymph nodes involved
– Presence of ‘B’ symptoms.
– ‘Early’ stage disease (Stage IA, IIA with no bulk) is treated with brief
chemotherapy (ABVD, doxorubicin, bleomycin, vinblastine, dacarbazine) followed by involved field irradiation.
– Advanced disease (all other stages) are treated with cyclical combination chemotherapy (8 cycles of ABVD) with irradiation at sites of bulk disease. PET/CT is used to detect disease activity after treatment and to distinguish
between active tumour (PET-positive) and necrosis or fibrosis (PETnegative) in residual masses. Irradiation, with its attendant complications, can be omitted in PET-negative masses after chemotherapy.
Prognosis is related to the stage of disease at presentation, with a 5-year survival rate of over 90% in stage I declining to 60% in stage IV. The presence of B symptoms indicates more severe disease with a worse prognosis.
Non-Hodgkin’s lymphoma
These are malignant tumours of the lymphoid system classified separately from Hodgkin’s lymphoma. Most (70%) are of B cell origin with 30% of T cell origin. There is a malignant clonal expansion of lymphocytes, which occurs
at different stages of lymphocyte development. In general, neoplasms of non-dividing mature lymphocytes are indolent whereas those of proliferating cells (e.g. lymphoblasts, immunoblasts) are much more aggressive. The B
and T/natural killer (NK) cell lymphomas are each further divided on this basis, e.g. precursor B cell lymphoma, and then again subdivided based on cytogenetics and immunophenotyping. The aetiology is unknown in most
cases but some are associated with a specific infection, e.g. Helicobacter pylori and gastric mucosa-associated lymphoid tissue (MALT) lymphoma.
Clinical features
Presentation is rare before the age of 40. Most patients present with painless peripheral lymph node enlargement. Systemic symptoms as in Hodgkin’s lymphoma may occur. Extranodal involvement is more common that in Hodgkin’s lymphoma and almost any organ in the body can be involved. Bone marrow infiltration leads to anaemia, recurrent infections and bleeding. Skin involvement with T cell lymphoma presents as mycosis fungoides and Sézary
syndrome.

Investigations
– Blood count may show anaemia. An elevated white cell count or thrombocytopenia suggests bone marrow involvement. The ESR may be raised.
– Liver biochemistry may be abnormal if the liver is involved.
– Serum lactate dehydrogenase and β2-microglobulin are prognostic indicators.
– Chest X-ray, CT, PET and gallium scans are of help in staging.
– Bone marrow aspiration and trephine biopsy will confirm marrow involvement.
– Lymph node biopsy is required for definitive diagnosis and subtype classification.
Management
Treatment depends on the lymphoma type and stage (similar to Hodgkin’s lymphoma). Diffuse large B cell lymphoma is the commonest lymphoma and first-line treatment is with cyclical combination chemo-immunotherapy
(CHOP + R, cyclophosphamide, hydroxydaunorubicin, vincristine, prednisolone and rituximab) with field irradiation for those with bulky disease.

Between 60–70% of those with early-stage disease will achieve a cure with this regimen.
– Primary gastric lymphoma is many cases is associated with H. pylori infection. Treatment to eradicate the infection (p. 80) is usually all that is required provided there is no evidence of disease outside the stomach.
This is followed by close endoscopic surveillance.
– Burkitt’s lymphoma occurs mainly in African children, and is associated with EBV infection. Jaw tumours are common, usually with gastrointestinal involvement. Treatment is with cyclical combination chemotherapy.

Fever of Unknown Origin-Management

Introduction
Fever is common with many acute illnesses. The fever usually resolves spontaneously. However, certain
patients develop prolonged fever without clear cause, posing a diagnostic challenge for their physicians.
Petersdorf and Beeson established the classic defi nition of fever of unknown origin (FUO) in 1961. They
defi ned FUO as (1) illness of more than 3 weeks’ duration, (2) temperature of 38.3º C (101º F) on several occasions,
and (3) uncertain diagnosis after 1 week of study in hospital. Although this defi nition of FUO has been
very useful, changes in the practice of medicine have signifi cantly altered the approach to patients with persistent
fever. In 1991, Durack and Street proposed revisions to the defi nition, including the substitution of
three outpatient visits or 3 hospital days for the third criterion. Alternatively, the third criterion can be replaced
with uncertain diagnosis after 1 week of evaluation. In addition, Durack and Street proposed distinguishing
classic FUO from nosocomial, neutropenic, or HIV-associated FUO, given diff erences in etiologies and underlying
conditions.

Etiology and Pathogenesis

More than 200 diseases have been linked to FUO. The five primary categories of FUO are infection, malignancy,
noninfectious inflammatory diseases, miscellaneous, and undiagnosed . Infections represent about 30%
to 50% of cases; malignancies, 20% to 30%; noninfectious inflammatory diseases, 10% to 30%; miscellaneous, 15%
to 25%; and undiagnosed, 5% to 15%. Most case series on FUO report infections as the most common diagnosis;
however, some studies have shown an increase in cases due to noninfectious infl ammatory diseases. The prevalence of febrile illnesses varies geographically, with infections being more common in developing countries and tropical regions.
Causes also vary according to host factors such as underlying immunosuppression and hospital and health service
exposures. The most commonly identified infections are abscesses, tuberculosis, and viral infections. Abscesses, predominantly intra-abdominal, have remained a leading cause of FUO over the past three decades. Hepatic, subhepatic, and subdiaphragmatic abscesses are common. Other locations include the retroperitoneal, splenic, appendiceal, pericolonic, perinephric, and pelvic areas. Underlying conditions such as diabetes mellitus, immunosuppressive medications, prior surgery, and cirrhosis have been associated with the
development of occult abscesses.
Tuberculosis is the most common source of FUO in most case series and often presents with miliary or extrapulmonary involvement. Viral infections have been increasingly recognized because of the availability of tests for cytomegalovirus (CMV) and Epstein-Barr virus (EBV). HIV infection, either with acute or previously undiagnosed established infection with concurrent opportunistic infection, also may cause FUO. Acute HIV infection as a cause of fever is frequently not considered but should be included in the differential diagnosis of individuals presenting with a mononucleosis-like illness, particularly with high-risk sexual or injection drug use exposure. Acute HIV infection is also among the most common causes of FUO among returned travelers in addition to malaria and typhoid fever. Many other infections have been associated with FUO, including cat-scratch disease (Bartonella species infection), brucellosis, histoplasmosis, leishmaniasis, malaria, psittacosis, relapsing fever, and leptospirosis.

Clinical Presentation
By definition, the clinical presentation of FUO includes fever. The fever may be high or low and relatively constant
or intermittent. Generally, the more prolonged the fever, the less likely that an infection is the cause. Other symptoms and signs may or may not be present. If present, these can provide crucial clues to the diagnosis. Relatively minor findings should not be overlooked or dismissed

Differential Diagnosis
As discussed, a wide variety of diseases can cause FUO. In approaching the patient meeting criteria for FUO, one
should first consider if they have classic, nosocomial, neutropenic, or HIV-associated FUO. Classic FUO occurs
in immunocompetent hosts, with onset as an outpatient and fever documented on at least three outpatient visits or
persistent for at least 3 weeks. Nosocomial FUO represents all patients with fever onset after admission to the
hospital, and persisting for at least 3 days with no diagnosis after initial workup. Differential diagnosis of nosocomial
FUO includes drug-resistant bacteria, drug fever, deep venous thrombosis secondary to immobility, Clostridium
difficile colitis, noninfectious postoperative fever due to inflammatory responses, and increased risk for infections
secondary to surgical procedures, urinary and respiratory instrumentation, and intravascular devices. In addition,
endotracheal intubation and gastric and enteral feeding tubes increase the risk for nosocomial sinusitis, which can
present as FUO in intensive care units.

Management and Therapy
The appropriate management of FUO depends primarily on the identification of the underlying etiology.
However, certain general rules of management should be considered.
Early in the evaluation of FUO, especially in older patients, all nonessential medications should be discontinued,
including medications that have been used for a lengthy period of time. Drug fevers can be caused by longstanding
medications. A costly investigation may be avoided if fever resolves after discontinuation of medications.
Empirical therapeutic trials should be avoided whenever possible, particularly in the setting of immunocompetent
hosts with classic FUO. Given the frequent spontaneous resolution of the FUO, cure cannot be ascribed to the treatment with certainty. Furthermore, use of therapeutic agents, especially antibiotics, can obscure
findings or tests that might lead to a definitive diagnosis.
In contrast, prompt empirical antibiotics, and sometimes antifungals, are the cornerstone of management for
patients with neutropenic FUO. These patients are routinely taking antibiotics and antifungals as prophylaxis
before the onset of fever and necessitate consideration of broader antimicrobial coverage while the diagnostic
workup is underway. Fever often resolves following the recovery of neutrophil counts, with no clear diagnosis.
Empirical antibiotics also may be warranted in the case of nosocomial or HIV-associated FUO, depending on clinical
severity and likely infectious causes.

Optimum Therapy
The treatment of FUO is specifi c to the cause of fever, and prognosis is determined by both the cause and the presence of underlying immunosuppression or comorbidities.
Avoiding Treatment Errors
Failure to obtain a thorough medical history and to perform a comprehensive physical examination is likely the most
critical error in identifying possible causes of FUO. Empirical therapeutic trials should be avoided except for cases in
which all diagnostic approaches have failed and the clinical condition of the patient does not allow for careful observation.
If empirical antimicrobial therapy is initiated, cultures should be obtained before administration if possible,
to allow subsequent targeted and optimized therapy to cultured pathogens.

Choking from a foreign body and Paediatric basic life support

Choking from a foreign body
Despite preventative measures (eg making pen tops with holes in them), children continue to die each year from airway obstruction due to FB impaction. FB aspiration produces a sudden onset airway problem and must be distinguished from other causes of airway obstruction (epiglottitis, bacterial tracheitis see b Stridor: upper respiratory tract infections, which may be worsened by the basic measures described below.
The majority of choking events in children are witnessed and occur during play or whilst eating. FB airway obstruction is characterized by sudden onset of respiratory distress associated with coughing, gagging or stridor
with no other signs of illness. If the child is coughing effectively (fully responsive, loud cough, able to take a breath before coughing, crying or verbal response to questions), encourage coughing and observe for the cough becoming ineffective.

Conscious, but ineffective cough
If conscious with an ineffective cough, give 5 back blows. In the infant, support in a head downwards prone position and in the child aim for a head down or forward leaning position. Deliver 5 sharp back blows with the heel of one hand centrally between the shoulder blades. If ineffective, turn to supine position and give 5 chest thrusts to infants (using the same landmarks as for cardiopulmonary resuscitation (CPR), but thrusts are sharper and delivered at a slower rate) and abdominal thrusts to children > 1 year. Perform abdominal thrusts from behind the child, placing your fist between the umbilicus and xiphisternum, and grasping it with your other hand, then pulling sharply inwards and upwards — repeat up to 5 times. Following chest or abdominal thrusts, if the object has not been expelled and the victim is still conscious then repeat the sequence of back blows and chest (for infant) or abdominal (for children) thrusts.
Do not use abdominal thrusts for infants.
Unconscious from foreign body airway obstruction If a child with FB airway obstruction is or becomes unconscious, place him on a flat surface, then open the mouth and look for any obvious object. If one is seen, use a single fi nger sweep to remove it. It may be possible to remove the FB with Magill’s forceps under direct laryngoscopy. Do not
attempt blind or repeated fi nger sweeps. Open the airway and attempt 5 rescue breaths. If a breath does not make the chest rise, reposition the head before making the next attempt. If there is no response whilst
attempting the 5 rescue breaths, proceed to chest compression with ventilation using a ratio of 15:2. Each time the airway is opened, check for a foreign body and if visible, try to remove it .
If it appears that the obstruction has been relieved, open and check the airway. If the child is not breathing, deliver rescue breaths. If initial measures prove unsuccessful and the child is hypoxic, oxygenate via a surgical airway until senior help arrives. Perform needle cricothyroidotomy in children aged <12 years, surgical cricothyroidotomy in older children.

Paediatric basic life support

Evaluate responsiveness
Check the child’s responsiveness — gently stimulate and ask loudly ‘are you alright?’ Do not shake if you suspect cervical spine injury. If the child does not respond, shout for help ± get someone to go for assistance.
Open airway
Open the airway by head tilt and chin lift. Desirable degrees of tilt are neutral <1 year and ‘sniffing the morning air’ > 1 year. Do not press on thensoft tissues under the chin as this may block the airway. If it is still difficult to open the airway, try a jaw thrust. If there is any suspicion that there may have been a neck injury, instruct a second rescuer to manually immobilizemit, and use either chin lift or jaw thrust alone. If this is unsuccessful, add the smallest amount of head tilt needed to open the airway.

Check breathing
Whilst keeping the airway open, look listen and feel for breathing for 10sec. If the child is not breathing or is making infrequent irregular breaths, carefully remove any obvious obstruction, give 5 initial rescue breaths
(with the rescuer taking a breath between each rescue breath).
Rescue breaths
For children > 1 year , whilst maintaining head tilt and chin lift, give breaths mouth to mouth, pinching off the nose. Blow steadily for 1–1.5sec watching for the chest to rise. Take your mouth away, watch the chest fall
and repeat this sequence 5 times. For the infant (<1 year) ensure the neutral position of the head and apply
chin lift. Give mouth to mouth and nose breaths, ensuring a good seal. Blow steadily for 1–1.5sec watching for chest rise. Take your mouth away, watch the chest fall and repeat this sequence 5 times.
Difficulty achieving an effective breath suggests airway obstruction. Open the mouth and remove visible obstruction (no blind fi nger sweep), ensure appropriate head tilt/chin lift and neck position. Try a jaw thrust if head
tilt/chin lift has not worked. Try up to 5 times to give effective breaths. If still unsuccessful, move to chest compression.

Check pulse
Over the next 10sec check for signs of life: any movement, coughing or normal breathing and check for a pulse (use carotid for > 1yr and brachial for those <1 year). If there are no signs of life and/or no pulse or pulse
<60/min with poor perfusion or you are unsure: start chest compression.
Chest compression
For infants , perform chest compressions (100–120/min) by placing both thumbs flat side by side on the lower third of the sternum with the tips pointing towards the infant’s head. Encircle the rib cage with tips of fingers
supporting the infant’s back. Press down with thumbs at least one third of the depth of the chest.
In children > 1 year using the heel of one hand, compress the lower half of the sternum by at least one-third of the depth of the chest at a rate of 100–120/min. Use two hands if necessary to achieve the depth required.

Injuries to the Neck-Management and Basic Life Support

Overview 

A blunt or penetrating neck injury can rapidly become a life-threatening emergency because the neck
houses many critical structures. The neck is bordered by the head superiorly and the sternal notch and
clavicles inferiorly . The anterior area of the neck contains the pharynx and trachea. The trachea is kept open by a series of cartilages, including the thyroid and cricoid cartilages. The larynx lies behind the thyroid cartilage. The thyroid gland lies just below the larynx and in front of the upper part of the trachea. The esophagus lies behind the trachea.
Posteriorly, the neck is bordered by the cervical spine.
The neck contains several large muscles that provide support and enable movement of the head. The carotid, vertebral, and subclavian arteries are the major arteries of the neck. Injury to any of these vessels
can result in massive bleeding or the formation of a hematoma that can compromise the airway. About onehalf
of patients with blunt trauma to the neck involving the carotid artery will have no external signs of neck
injury. The internal jugular veins can be found lateral to the carotid arteries . Air may enter the
circulatory system if there is penetrating injury to a large blood vessel in the neck.

Mechanism of Injury

A neck injury can be caused by a hanging, impact with a steering wheel, a knife or gunshot wound, strangulation,
a sports injury, or a “clothesline” injury, in which a person runs into a stretched wire or cord that strikes
the throat. The patient with a neck injury may also have an underlying spinal injury.
Patient Assessment

Conduct a scene size-up and ensure your safety. Evaluate the mechanism of injury before approaching the
patient. Put on appropriate PPE. Assess the patient’s airway, breathing, and circulation while maintaining
spinal stabilization. Work quickly, keeping in mind that an open wound to the neck may bleed profusely and cause death. Bubbling from a neck wound suggests involvement of the respiratory tract, such as injury to the
larynx or trachea. Swelling of the structures in the neck can cause an airway obstruction. The patient may experience
shortness of breath, difficulty breathing, and/or a hoarse voice.
Examine the neck for DCAP-BTLS. Assessment findings and symptoms of blunt trauma to the neck include
hoarseness, bruising, deformity, and subcutaneous emphysema. The presence of subcutaneous emphysema suggests involvement of the respiratory tract. Stridor may be present, suggesting an upperairway obstruction. Look for handprints from a blow or possible choking and rope marks on the neck that may be caused by hanging. Palpate the trachea to determine if it is in its normal midline position. While making sure that the head and neck remain in a neutral, in-line position, gently palpate the cervical spine for tenderness and deformity.

Laryngeal injuries may be accompanied by hematomas, swelling, or hemorrhaging, increasing the risk
of airway obstruction. Crepitus in the laryngeal area, subcutaneous emphysema, stridor, hoarseness, or an
inability to speak may indicate a fracture of the larynx. Remember that the cricoid cartilage is the only complete
ring of cartilage in the larynx. A fracture of the cricoid cartilage can result in death due to an airway obstruction.
Injuries to the esophagus are rare. Assessment findings and symptoms can include blood in the saliva,
hematemesis, hoarseness, stridor, neck tenderness, and difficulty swallowing. Frequent suctioning may be
needed to maintain an open airway. Monitor the patient’s airway throughout your care.
Emergency Care

To treat a patient with a neck injury, perform the following steps:
– Patients with a neck injury should be transported to a trauma center. Call for additional EMS resources as soon as possible.
– If the mechanism of injury suggests a head or spinal injury, continue manual stabilization of the patient’s head and neck until additional EMS resources arrive and the patient has been completely stabilized on a long backboard.
– Establish and maintain an open airway. If you must open the patient’s airway, use a modified jaw-thrust maneuver. Monitor the patient’s airway closely while the patient is in your care. Suction as necessary. If the entire patient has been secured to a long backboard, the backboard may need to be tilted to adequately clear the airway.

-= Give oxygen. If the patient’s breathing is inadequate, assist her breathing with a bag-mask device connected to 100% oxygen.                                                                                                                                                                                – Control bleeding. To care for an open neck wound, immediately place a gloved hand over
the wound to control bleeding. Cover the wound with an airtight (occlusive) dressing, and apply a bulky dressing over the occlusive dressing. If blood soaks through the dressing, apply additional dressings on top and continue to apply pressure. The application of pressure using one gloved finger to control bleeding from a carotid
artery or jugular vein may be necessary. Do not apply pressure to both carotid arteries at the same time. Doing so can compromise blood flow to the brain. If signs of shock are present or if internal bleeding is suspected, treat for shock.
– Do not remove a penetrating object. Instead, stabilize it in place with bulky dressings.
– Dress and bandage any open wounds.
– Comfort, calm, and reassure the patient and family members. Reassess as often as indicated. Closely monitor the patient’s airway, breathing, pulse, and mental status for deterioration.
– Record all patient care information, including the patient’s medical history and all emergency care given, on a PCR.

Gastrointestinal Bleeding-Management and Treatment

Introduction
Gastrointestinal (GI) bleeding refers to bleeding from a source in the esophagus, stomach, small intestine,
colon, anus, or rarely the liver and pancreas. In 2002, there were 116,724 hospital discharges with a principal
diagnosis of GI hemorrhage (ICD-9 code 578.9), which was the 51st among all discharge diagnoses, and 74,717
hospital discharges with a principal diagnosis of diverticulosis with hemorrhage (ICD-9 code 562.12). Various
descriptors have been used to categorize GI bleeding including the following: acute, chronic, active, occult,
and obscure. In addition, GI bleeding is usually classifi ed as upper versus lower. Upper GI bleeding refers to
bleeding from a source from the esophagus to the duodenum at the ligament of Treitz. Lower GI bleeding
refers to bleeding from the jejunum to the anus. Because of the breadth of this topic, the focus here is primarily
on the causes of GI bleeding and the management strategies for acute bleeding.

Etiology and Pathogenesis
There are many different causes of GI bleeding . Several factors can increase an individual’s risk for GI
bleeding. These include cirrhosis, coagulopathy, the use of aspirin or nonsteroidal anti-infl ammatory drugs, and the
use of antiplatelet or anticlotting drugs.
Clinical Presentation
The clinical presentation depends on the rate, source, and volume of blood loss. Upper GI bleeding can present in several ways: hematemesis, vomiting of bright red blood; coffee ground emesis, vomiting of blood that has been
digested by gastric acid; and melena, the passage of black tarry stool. Upper GI blood loss can also be occult, with
no visible evidence of blood to the naked eye.
Lower GI bleeding can present as hematochezia, the passage of bright red blood per rectum, and less commonly
as melena. Lower GI bleeding does stop spontaneously in most cases (about 85%). It can also be occult.
A person who is experiencing a rapid, large-volume GI bleed will manifest hemodynamic instability. Orthostatic
blood pressure changes (a decrease in blood pressure of 20 mm Hg systolic or 10 mm Hg diastolic with an associated
20-point rise in heart rate after standing) can be seen with a 15% reduction in blood volume. Hypotension
usually occurs with a 20% blood volume reduction.
Slow chronic blood loss can result in a significant reduction in hemoglobin without a change in hemodynamic stability.

These patients often present with weakness, fatigue, shortness of breath, or chest pain as a result of anemia.
All patients presenting with GI bleeding should be checked for physical examination fi ndings suggestive of
chronic liver disease, including palmar erythema, spider angiomas, splenomegaly, ascites, and scleral icterus.
Underlying cirrhosis with portal hypertension is a common cause of upper GI hemorrhage from gastric and esophageal varices.
Some etiologies of GI bleeding are associated with abdominal pain, but many are not. A careful abdominal
examination looking for distention, the presence of bowel sounds, and tenderness is a critical step in patient
evaluation.
Differential Diagnosis
Numerous lesions can cause upper GI bleeding  as well as lower GI bleeding . The following questions yield very important historical information that helps to narrow a very broad differential list of diagnoses:
• Have there been similar prior episodes?
• Did nonbloody emesis precede bloody emesis?
• What medications, both prescription and over the counter, have been taken recently?

Diagnostic Approach
When a patient presents with GI bleeding, the urgency of evaluation is highly dependent on an initial assessment of
hemodynamic stability and on whether there is significant ongoing bleeding. Monitoring vital signs, obtaining good
intravenous access with large-bore intravenous catheters, and ordering a complete blood count, prothrombin time
and partial thromboplastin time, and a type and crossmatch for blood products should occur early in the evaluation.
Hypotension requires aggressive intravenous fluid resuscitation and blood transfusions as indicated. The correction
of an underlying coagulopathy is also a key initial therapeutic step. If a patient is having ongoing hemateme sis, airway protection may require tracheal intubation. The patient should not receive anything by mouth except
essential oral medications.
An experienced endoscopist should see the patient as soon as possible, and the medical care team should ask for
a surgical consultation if the patient’s hemodynamic state remains unstable with evidence of continuing blood loss.
If an upper GI bleed is suspected, the patient should have an urgent upper endoscopy. Endoscopy provides diagnostic and prognostic information and also allows for therapeutic intervention. If the patient can be stabilized hemodynamically and if it is clear that a lower GI bleed is occurring, a polyethylene glycol purge should be given over 3 to 4 hours.
If the patient presents with evidence of chronic occult GI bleeding and is hemodynamically stable, an outpatient
upper and lower endoscopy is a reasonable approach.
When there is no definite diagnosis after these studies, the next step is capsule endoscopy of the small bowel or other small bowel imaging modality.

Management and Therapy
In 2001, the American Society for Gastrointestinal Endoscopy published algorithmic approaches to upper and acute
lower GI bleeding . These approaches remain relevant. A 250-mg dose of intravenous erythromycin
is often given before upper endoscopy to help empty blood clots from the stomach. Also, most endoscopists now
try to remove all adherent clots and provide endoscopic therapy to the lesions found beneath them, if indicated.
In upper GI bleeding, the following are predictors for rebleeding: older age; shock, hemodynamic instability, and
orthostasis; comorbid disease states (e.g., coronary artery disease, congestive heart failure, renal and hepatic diseases, cancer); specifi c endoscopic diagnosis (e.g., GI malignancy); use of anticoagulants and coagulopathy; and
presence of a high-risk lesion (e.g., arterial bleeding or nonbleeding, visible vessel).
Optimum Treatment
For upper GI bleeding, optimum treatment involves a combination of endoscopy and medications. The findings
on endoscopy identify lesions at risk for rebleeding, which affect the length of hospitalization. Helpful adjunctive
medications are available. Proton pump inhibitors, given in high-dose oral or intravenous forms, have decreased
rebleeding rates and the need for surgery in patients with peptic ulcer disease. Somatostatin or its synthetic analogue
octreotide is used to help control variceal bleeding along with the use of endoscopic therapeutic techniques.
For acute lower GI bleeding, the optimum treatment strategy is shown in the algorithm . Stabilization
of the patient must be the first priority, followed by evaluation for a specific bleeding source. Some lower GI
bleeding lesions are amenable to endoscopic hemostatic therapy, including a culprit diverticulum, arteriovenous
malformation, polypectomy sites, and radiation proctitis. Angiography should be reserved for those with massive
bleeding that precludes colonoscopy and those in whom colonoscopy was nondiagnostic but bleeding persists. If an
extravasation site is identified, arterial embolization is an alternative. Surgery is reserved for those failing endoscopic
and angiographic therapy.