Answer: Waterhouse- Friderichsen syndrome

Waterhouse-Friderichsen syndrome is a condition characterized by the abrupt onset of fever, petechiae, arthralgia, weakness, and myalgias, followed by acute haemorrhagic necrosis of the adrenal glands and severe cardiovascular dysfunction. The syndrome is most often associated with meningococcal septicaemia but may occur as a complication of sepsis caused by other organisms, including certain streptococcal species. This disorder may be associated with a history of splenectomy.

Fulminant infection from meningococcal bacteria in the bloodstream is a medical emergency and requires emergent treatment with vasopressors, fluid resuscitation, and appropriate antibiotics. Benzylpenicillin was once the drug of choice with chloramphenicol as a good alternative in allergic patients. Ceftriaxone is an antibiotic commonly employed today. Hydrocortisone can sometimes reverse the adrenal insufficiency. Amputations, reconstructive surgery, and tissue grafting are sometimes needed as a result of tissue necrosis (typically of the extremities) caused by the infection.

This patient has a Bankart lesion which is the most common underlying abnormality in recurrent anterior dislocation of the shoulder.

Bankart lesion is an injury of the anterior (inferior) glenoid labrum of the shoulder due to anterior shoulder dislocation. When this happens, a pocket at the front of the glenoid forms that allows the humeral head to dislocate into it. It is usually visualised by CT and MRI scanning and is often repaired arthroscopically.

Shoulder fractures and dislocations usually result from low-energy falls in predominantly elderly females or from high-energy trauma in young males. They can be associated with nerve injury (commonly axillary), and fracture-dislocation of the humeral head. Anterior shoulder dislocation (glenohumeral dislocation) is the most common type of shoulder dislocation (>90%) and is usually traumatic in nature.

Early assessment of shoulder dislocation:
Careful history, examination, and documentation of neurovascular status of the upper limb, in particular the axillary nerve, is important. This should be re-assessed after manipulation. Early radiographs should also be done to confirm the direction of the dislocation.

Initial management of anterior dislocation:
It consists of emergent closed reduction (to prevent lasting chondral damage) under Entonox and analgesia, but often requires conscious sedation. The affected arm should then be immobilised in a polysling. Initial management requires emergent reduction to prevent lasting chondral damage.

Familial adenomatous polyposis (FAP) is an autosomal dominant, hereditary colon cancer syndrome that is characterized by the presence of innumerable adenomatous polyps in the colon and rectum. Gardner’s syndrome is a variant of FAP, which in addition to the colonic polyps, also presents extracolonic manifestations, including desmoid tumours, osteomas, epidermoid cysts, various soft tissue tumours, and a predisposition to the thyroid and periampullary cancers.
Of patients with FAP, 75%-80% have a family history of polyps and/or colorectal cancer at age 40 years or younger.
Mutations of the APC gene are thought to be responsible for the development of FAP, and the location of the mutation on the gene is thought to influence the nature of the extracolonic manifestations that a given patient might develop.
Though patients are often asymptomatic, bleeding, diarrhoea, abdominal pain and mucous discharge frequently occur. Diagnostic tools include genetic testing, endoscopy, and monitoring for extra-intestinal manifestations.
If left untreated, all patients with this syndrome will develop colon cancer by age 35-40 years. Besides, an increased risk exists for the development of other malignancies.
Currently, surgery is the only effective means of preventing progression to colorectal carcinoma. Restorative proctocolectomy with ileal pouch-anal anastomosis (RPC/IPAA) with mucosectomy is the preferred surgical procedure since it attempts to eliminate all colorectal mucosa without the need for an ostomy. Periampullary carcinoma and intra-abdominal desmoid tumours are a significant cause of morbidity and mortality in these patients after colectomy. Frequent endoscopy is needed to prevent the former, while there is no definitive treatment available yet for the latter.

Passage of bloody diarrhoea together with mucus and a short history makes this a likely presentation of inflammatory bowel disease. Rectal malignancy in a young age would be a very unlikely event. Furthermore, the history is too short to be consistent with solitary rectal ulcer syndrome.

Rectal bleeding is a common cause for patients to be referred to the surgical clinic. In the clinical history, it is important to try and localise the anatomical source of the bleeding. Bright red blood is usually of rectal origin, whereas, dark red blood is more suggestive of a proximally located bleeding source. Blood which has entered the gastrointestinal tract from a gastroduodenal source will typically resemble melaena due to the effects of the digestive enzymes on the blood itself.

PR bleeding in ulcerative colitis (UC) is usually bright red and often mixed with stool. It is mostly associated with the passage of mucus as well. Other clinical features reported on history include diarrhoea, weight loss, and nocturnal incontinence. Proctitis is the most marked finding on examination and perianal disease is usually absent. Colonoscopy is carried out which shows continuous mucosal lesions.

Hypoxia is when the whole body or a region is deprived of adequate oxygen supply. Different types of hypoxia include the following:
– Hypoxic hypoxia, which occurs due to poor oxygen supply, as a result of low partial pressure of oxygen in arterial blood. This could be due to low partial pressure of atmospheric oxygen (e.g., at high altitude), sleep apnoea, poor ventilation because of chronic obstructive pulmonary disease or respiratory arrest, or shunts. The other types of hypoxia have a normal partial pressure of oxygen.
– Anaemic hypoxia occurs due to low total oxygen content of the blood, with a normal arterial oxygen pressure.
– Hyperaemic hypoxia occurs due to poor delivery of oxygen to target tissues, such as in carbon monoxide poisoning or methemoglobinemia.
– Histotoxic hypoxia results due to inability of the cells to use the delivered oxygen due to disabled oxidative phosphorylation enzymes.
– Ischaemic (or stagnant) hypoxia occurs due to local flow restriction of well-oxygenated blood, seen in cases like cerebral ischaemia, ischaemic heart disease and intrauterine hypoxia.

Under basal conditions, most of the energy needed by cardiac muscle for metabolism is derived from fats (60%), 35% by carbohydrates, and 5% by ketones and amino acids. However, after intake of large amounts of glucose, lactate and pyruvate are mainly used. During prolonged starvation, fat acts as the primary source. 50% of the used lipids are sourced from circulating fatty acids.

An upper motor neuron lesion affects the neural pathway above the anterior horn cell or motor nuclei of the cranial nerves, whereas a lower motor neurone lesion affects nerve fibres travelling from the anterior horn of the spinal cord to the relevant muscles. An upper motor neurone lesions results in the following:
– Spasticity in the extensor muscles (lower limbs) or flexor muscles (upper limbs).
– ‘clasp-knife’ response where initial resistance to movement is followed by relaxation
– Weakness in the flexors (lower limbs) or extensors (upper limbs) with no muscle wasting
– Brisk tendon jerk reflexes
– Positive Babinski sign (on stimulation of the sole of the foot, the big toe is raised rather than curled downwards)

Aortic injuries not resulting in immediate death may be due to a contained haematoma. Clinical signs are subtle, and diagnosis may not be apparent on clinical examination. Without prompt treatment, the haematoma usually bursts and the patient dies.

Traumatic aortic disruption, or aortic transection, is typically the result of a blunt aortic injury in the context of rapid deceleration. This condition is commonly fatal as blood in the aorta is under great pressure and can quickly escape the vessel through a tear, resulting in rapid haemorrhagic shock and death. A temporary haematoma may prevent the immediate death. Injury to the aorta during a sudden deceleration commonly originates near the terminal section of the aortic arch, also known as the isthmus. This portion lies just distal to the take-off of the left subclavian artery at the intersection of the mobile and fixed portions of the aorta. As many as 80% of the patients with aortic transection die at the scene before reaching a trauma centre for treatment.

A widened mediastinum may be seen on the X-ray of a person with aortic rupture. Other findings on CXR may include:
1. Deviation of trachea/oesophagus to the right
2. Depression of left main stem bronchus
3. Widened paratracheal stripe/paraspinal interfaces
4. Obliteration of space between aorta and pulmonary artery
5. Rib fracture/left haemothorax

Diagnosis can be made by angiography, usually CT aortogram.

Treatment options include repair or replacement. The patient should, ideally, undergo endovascular repair.

A congenital heart disease, ASD or atrial septal defect leads to a communication between the right and left atria due to a defect in the interatrial septum. This leads to mixing of arterial and venous blood from the right and left side of the heart. The hemodynamic significance of this defect depends on the presence of shunting of blood. Normally, the left side of the heart has higher pressure than the right as the left side has to pump blood throughout the body. A large ASD (> 9 mm) will result in a clinically significant left-to-right shunt, causing volume overload of the right atrium and ventricle, eventually leading to heart failure. Cardiac catheterization would reveal very high oxygen saturation in the right atrium, right ventricle and pulmonary artery. Eventually, the left-to-right shunt will lead to pulmonary hypertension and increased afterload in the right ventricle, along with the increased preload due to the shunted blood. This will either cause right ventricular failure, or raise the pressure in the right side of the heart to equal or more than that in the left. Elevation of right atrial pressure to that of left atrial pressure would thus lead to diminishing or complete cessation of the shunt. If left uncorrected, there will be reversal of the shunt, known as Eisenmenger syndrome, resulting in clinical signs of cyanosis as the oxygen-poor blood form right side of the heart will mix with the blood in left side and reach the peripheral vascular system.

The abdomen is divided by theoretical anatomic lines into four quadrants. The median plane follows the linea alba and extends from the xiphoid process to the pubic symphysis and splits the abdomen in half. The transumbilical plane is a horizontal line that runs at the level of the umbilicus. This forms the upper right and left quadrants and the lower right and left quadrants. Structures in the right lower quadrant include: caecum, appendix, part of the small intestine, ascending colon, the right half of the female reproductive system, right ureter. Pain in this region is most commonly associated with appendicitis.