What is the Diagnosis?
Barrett's oesophagus is a condition in which there is gastric or intestinal metaplasia of the mucosa of the distal oesophagus thought to be caused by damage from chronic acid exposure, or reflux esophagitis
It is considered to be a premalignant condition because it is associated with an increased risk of esophageal cancer (more specifically, adenocarcinoma)
Barrett's oesophagus is most common in elderly adults
Sometimes people with Barrett's esophagus will have no heartburn symptoms at all.
Barrett's esophagus does not cause any particular symptoms.But WARNING SIGNS are
Retrosternal pain that radiates to the neck
Endoscopic visualisation and biopsy are diagnostic.
Colonic metaplasia is usually identified by finding goblet cells in the epithelium
For the defenitive identification of globlet cells
Histochemical stain Alcian blue pH 2.5
Immunohistochemical analysis with antibodies to CDX-2 can be used
Regular endoscopy monitoring and biopsy
If two endoscopies and biopsy sessions performed within 12 months are negative for dysplasia then surveillance can be performed every 3 years
Intensive anti-reflux measures (long term proton pump inhibitor therapy)
High grade dysplasia - laser ablation of the site of metaplasia
Malignancy may require surgery, radiation therapy, or systemic chemotherapy
- Bulge left anterior hemithorax
- Systolic thrill is felt along the left sternal border
- Loud systolic murmur most intense at the left sternal border
- Systolic ejection murmur at the upper sternal border
37 year old male patient was admitted to the Surgery ward complaining of abdominal and loin pain and haematuria for 2 days duration.No fever or Dysuria.
He was not pale.His blood Pressure was 180/110mmhg.On abdominal examination this patient,two ballotable masses found in both sides.
Ultrasonography of the patient is given here.
Polycystic kidney disease (PKD)also known as polycystic kidney syndrome is a genetic disorder characterized by the growth of numerous cysts in the both kidneys
PKD cysts can profoundly enlarge the kidneys while replacing much of the normal structure, resulting in reduced kidney function and leading to kidney failure.
PKD can also cause cysts in the liver ,the pancreas,blood vessels in the brain(intracranial berry aneurysms) and heart
Two major inherited forms of PKD exist:
Autosomal recessive PKD
- Is a rare inherited form.
- Symptoms of autosomal recessive PKD begin in the earliest months of life, even in the womb.
AUTOSOMAL DOMINANT PKD
- Is the most common inherited form.
- About 90% of all PKD cases are autosomal dominant PKD.
- Important cause of renal failure
- Mutations in either the PKD-1 or PKD-2 gene
- Cyst formation begins in utero
- Cysts arise from the tubules throughout the nephrons but initially, involve only a portion of them
- Symptoms usually develop between the ages of 30 and 40, but they can begin earlier, even in childhood.
- Autosomal dominant PKD is asymptomatic for several tears and hence it is often called “adult polycystic kidney disease"
- Abdominal pain and early satiety and gastroesophageal reflux symptoms - due to mass effect of enlarging kidneys
- Loin and back pain
- Gross Hematuria
- Symptoms of UTI
- Features of uraemia
- Palpable, bilateral abdominal masses
* Liver and pancreatic cysts
* Abnormal heart valves
* High blood pressure
* Kidney stones
*Intracranial berry aneurysms - subarachnoidal hemorrhage
* Diverticulosis—small pouches bulge outward through the colon
*Thoracic aortic aneurisms
Before the age of 30 years, CT scan or T2-weighted MRI is more sensitive
- Diagnostic Criteria
In younger patients
- Two or more cysts in one kidney
- At least one cyst in the contralateral kidney
- With a positive family history of the disease
Blood Pressure control(ACE inhibitors or angiotensin II receptor blockers )
Pain management (percutaneous aspiration, sclerotherapy with alcohol or, rarely, surgical drainage)
Lipid-soluble antimicrobials for UTI
End-stage renal disease - Dialysis or Transplantation
In patients with ESRD optimizing nutritional status is important.
This is a non contrast(T1 Weighted) sagital MRI of a 3days old male baby who developed apnoea and respiratory distress in the baby care unit who had prominent occiput .This condition was prediagnosed during the antenatal period.
Dandy-Walker Syndrome is a rare congenital abnormality in which there is usually an absence of the foramina of Magendie and Luschka.
Occurs more frequently in females than in males.
Dandy-Walker malformation is characterized by
- Agenesis or hypoplasia of the cerebellar vermis
- Cystic dilatation of the fourth ventricle
- Dilatation of the third and lateral ventricles
- Enlargement of the posterior fossa with a high tentorium cerebelli and transverse sinuses
- Brainstem compression and hypoplasia
- Thinning and bulging of the occipital bones
- Approximately 70-90% of patients have hydrocephalus, which often develops postnatally
Other Causes of posterior fossa cystic malformations are Dandy-Walker variant, mega cisterna magna, and posterior fossa arachnoid cyst
Predisposing factors for Dandy-Walker malformation include gestational exposure to rubella (in the first trimester), cytomegalovirus, toxoplasmosis, warfarin, alcohol, and isotretinoin.
- Enlarged head circumference
- Developmental delay
- Signs of cerebellar dysfunction
- Signs and symptoms of hydrocephalus
- Interference with respiratory control centers in the brainstem may cause respiratory failure
- Hearing or visual difficulties
- Intellectual impairment has been observed in patients with agenesis of the corpus callosum
MRI for detailed evaluation of Dandy-Walker malformation lesions
Overall mortality rates of 12-50%
This is a sagital T2 weighted MRI of a 28 years old worker who came to the Neurology unit complaining of headache in the back of head and the nape associated with paresthesia and weakness of the hands and limbs.
On examination cerebellar signs were elicited but there was no significant muscle wasting.
What is the Diagnosis?
The Arnold-Chiari malformation is a congenital malformation of the base of the skull with varying degrees of herniation of cerebellum and medulla into the spinal canal.
Mainly three types
- type I - cerebellar ectopia
- type II
- type III
Arnold-Chiari malformation TYPE 1 is the most common form.It is generally asymptomatic during childhood.
In symptomatic patients, it is associated with:
spinal diseases - 50% of cases: syringomyelia
cranial disorders - 10% of cases: obstructive hydrocephalus
- Gait ataxia
- Spastic quadraparesis
- Downbeat nystagmus or rotatory nystagmus
- Bulbar symptoms
The blockage of CSF flow may also cause a syrinx to form, eventually leading to syringomyelia and eventually patient will develop symptoms of cord compression
- MRI - the investigation of choice
- CT - best with contrast
- Decompression surgery - removes the lamina of the first cervical vertebrae and part of the occipital bone of the skull to relieve pressure
- Ventricular peritoneal shunting if there is evidence of hydrocephalus or raised intracranial pressure.
It is a outpouching of the mucosa of the posterior pharyngeal wall through Killian's triangle(also known as Laimer's triangle, and the Killian-Laimer triangle)an area of weakness between the two parts of the inferior pharyngeal constrictor - the thyropharyngeus and the cricopharyngeus - at their posterior margin.Zenker diverticulum has male predominence and is observed almost exclusively in older individuals.
- Uncoordinated swallowing
- Impaired relaxation and spasm of the cricopharyngeus muscle
Zenker diverticula extend into the left neck 90% of the time
- Palpable swelling in the neck in two-thirds of cases
- Involuntary gurgling noises when swallowing
- Regurgitation of undigested food hours after eating
- Sensation of food sticking in the throat
- Coughing after eating
- Aspiration of organic material
- Episodes of pulmonary infection
- Unexplained weight loss
- Bleeding from the mucosa
- Esophageal obstruction
- Tracheal Fistulation
- Squamous cell carcinoma - but very rare
What is the most propable Diagnosis?
An acoustic neuroma(or vestibular schwannoma), is a slow growing benign primary intracranial tumor of the myelin-forming cells of the vestibular portion of the 8th cranial nerve.
It comprises about 6% of all intracranial tumors
lesions are usually located in the internal auditory canal or the cerebellopontine angle causing compression of the vestibular nerve and resulting eventually in deafness
Bilateral acoustic neuromas are the hallmark in patient with type 2 neurofibromatosis
2. VIII nerve damage - unilateral sensorineural deafness developing gradually over a period of months or years. vertigo with associated nausea and vomiting,tinnitus.Caloric paresis may be demonstrable.
3. V nerve damage - depression of corneal reflex occurs early. Facial pain, paraesthesia and numbness develop.
4. VII nerve damage - facial weakness is unusual.If involved it may lead to ipsilateral facial weakness, sensory impairment, impairment of glandular secretions and loss of taste sensation in the anterior two-thirds of the tongue
5. IX, X, XI nerve damage - rare; presents as dysphagia, change in voice, palatal weakness.
6. Compression effects of large tumours:
* cerebellum - ataxia, loss of co-ordination on ipsilateral side, nystagmus
* pons - contralateral hemiparesis,gaze palsies
* aqueduct and 4th ventricle - raised intracranial pressure, e.g. headache
Pure tone audiometry - demonstrates unilateral sensorineural hearing loss
Brainstem audiometry - to distinguish cochlear deafness from retrocochlear disease
Caloric test - expect depressed or absent response
Contrast CT scan as acoustic neuromas are often isodense
MRI with gadolinium enhancement - especially for small intracanalicular tumours
Microsurgery - is useful in treating large acoustic neuromas. three surgical methods are used in approaching the CP angle
1. retrosigmoid approach
2. translabyrinthine approach
3. middle fossa approach
RUPTURED BAKER'S CYST(PSEUDOTHROMBOPLEBITIS SYNDOME)
Baker's cyst(popliteal cyst), a collection of synovial fluid which has escaped from the knee joint or a bursa and formed a new synovial-lined sac in the popliteal space.The term 'cyst' is a misnomer because it is in fact a synovial sac bulging from the back of the joint.Baker's cysts arise between the tendons of the medial head of the gastrocnemius and the semimembranosus muscles.
Eg : Osteoarthritis, juvenile arthritis of the knee, rheumatoid arthritis
Baker's cysts can be associated with Lyme disease also.
Cartilage (particularly the meniscus) tear
If it is large it becomes palpable particularly on standing ,soft and minimally tender
Ruptured Baker's cyst can produce acute pain behind the calf and knee,redness and swelling of the calf that may mimic thrombophlebitis or a potentially life-threatening deep vein thrombosis (DVT)
- Diagnosis is confirmed by Ultrasonography
- Rest and leg elevation
- Removal of excess knee fluid in conjunction with cortisone injection.
- Surgical excision is reserved for cysts that cause a great amount of discomfort to the patient
A lung abscess is a confined area of suppuration within the lung parenchyma. It is more common in alcoholics and in elderly, debilitated patients who are prone to aspiration, and in intravenous drug abusers. Males are affected slightly more often than females.The incidence of lung abscess has declined in the recent past due to the greater efficacy of antibiotics in the treatment of lower respiratory tract infection.
* Anaerobic bacteria: Peptostreptococcus, Bacteroides, Fusobacterium species,
* Microaerophilic streptococcus : Streptococcus milleri
* Aerobic bacteria: Staphylococcus, Klebsiella, Haemophilus, Pseudomonas, Escherichia coli, Streptococcus, Mycobacteria
* Fungi: Candida, Aspergillus
* Parasites: Entamoeba histolytica
Vasculitis: Wegener's granulomatosis
Necrotizing tumors:primary squamous carcinoma of the lung is the commonest
In pneumonia or bronchiectasis are usually multiple, basal and diffusely scattered
Septic emboli and pyemic abscesses are usually multiple and may affect any region of the lung
- pleuritic chest pain
- malaise and fatigue
Discharge of the abscess into a bronchus:
- the sputum is copious and foul smelling
- the sputum is initially blood-stained but may become green or brown with time
Signs of a pleural effusion or consolidation
High ESR, CRP
Sputum or Transtracheal of Transbronchial aspirates can also be cultured
Chest radiography:may reveal one or more circular lesions of almost any size which may cavitate and show fluid levels Chest ultrasound:can identify a fluid collection
Fibre-optic bronchoscopy:permits sampling for bacteriology and for histological diagnosis of any obstructive lesions
Percutaneous drainage of a lung abscess
Lung resection in selective patients
- bronchial stenosis
- rupture with spread of infection
- haemorrhage and a bronchopleural fistula
- overdistension of the alveoli
- secondary infection
Distant spread: sepsis, brain abscesses, mediastinitis
Mortality from lung abscess is about 5%