The posterior drawer test is a physical exam technique that is done to assess the integrity of the posterior cruciate ligament (PCL). The PCL is attached to the posterior intercondylar area of the tibia and passes anteriorly, medially, and upward to attach to the lateral side of the medial femoral condyle.
This ligament prevents backward displacement of the tibia or forward sliding of the femur. Injury to the ligament allows displacement of the tibia

Indications for a simple mastectomy with a concomitant axillary procedure, which may be either a sentinel node biopsy or an axillary clearance, are as follows:

– Locally advanced breast cancer
– Multifocal breast cancer
– Large tumour relative to the size of the breast, excision of which may compromise final cosmesis
– Extensive ductal carcinoma in situ (DCIS)

Multifocality and multicentricity (MFMC) are frequently used descriptors to assess the extent of disease in patients presenting breast cancer. The presence of two or more foci of cancer within the same breast quadrant is defined as multifocal, while the presence of two or more foci of cancer in different quadrants of the same breast is defined as multicentric. A simple mastectomy involves removing the breast along with an ellipse of skin that encompasses the nipple-areola complex. Unlike a radical mastectomy, it does not involve removal of the underlying muscles and uninvolved lymph nodes.

Patient unsuitability for breast-conserving approaches as a result of contraindications for radiation therapy – Such contraindications include a previous history of chest wall irradiation, either after previous breast-conserving therapy or in mantle field radiation therapy for lymphoma; severe skin disorders, such as scleroderma and psoriasis; and severe pulmonary dysfunction

Patient preference for mastectomy – A patient who is a suitable candidate for breast conservation may opt for a mastectomy instead.

Patients treated with moderate-dose intravenous thrombolysis within 3 hours after the onset of stroke symptoms benefit substantially from therapy, despite a modest increase in the rate of symptomatic haemorrhage. This patient is within 3h of symptom onset of a stroke, therefore he should be urgently referred to the medical team for thrombolysis, before Aspirin is given. According to the current guidelines, in order to limit the
risk of an intracranial haemorrhagic complication, no antiplatelet treatment should be administered in the 24 hours that follow treatment of an ischemic stroke by intravenous thrombolysis.

This patient is likely to have paralytic ileus and the administration of antiemetic drugs, in this situation, will have no effect. It is, therefore, important to decompress the stomach with a wide bore nasogastric tube.

Paralytic ileus is the obstruction of the intestine due to paralysis of the intestinal muscles. It commonly occurs after an abdominal surgery. Irrespective of the cause, paralytic ileus causes constipation, abdominal distention, nausea, and vomiting. It is a severe condition because if left untreated, an ileus can cut off blood supply to the intestines and cause tissue death.

Staphylococcus epidermidis tends to colonise plastic devices (such as the breast implant in this scenario) and forms a biofilm which allows colonisation with other bacterial agents. It is extremely difficult to eradicate the infection once established, and the usual treatment is removal of the device.

Staphylococcus epidermidis is a gram-positive facultative anaerobic bacterium. It is part of the normal human flora, typically the skin flora, and less commonly the mucosal flora. It is also one of the most frequently detected pathogens in infections following implant surgery.

Bowel preparation is a significant aspect of the flexible sigmoidoscopy procedure. Clear visibility of the bowel mucosa is critical for a thorough examination. The combination of a light breakfast in the morning and the application of 1 or 2 phosphate enemas a few hours before the examination is a safe and commonly used method of preparing a patient for a flexible sigmoidoscopy procedure.

A small number of foreign body aspirations are incidentally found after chest radiography or bronchoscopic inspection. Patients may be asymptomatic or may be undergoing testing for other diagnoses. If present, physical findings may include stridor, fixed wheeze, localized wheeze, or diminished breath sounds. If obstruction is severe, cyanosis may occur. Signs of consolidation can accompany post obstructive pneumonia.

Bronchoscopy can be used diagnostically and therapeutically. Most aspirated foreign bodies are radiolucent. Radiologic procedures do not have extreme diagnostic accuracy, and aspiration events are not always detected. Rigid bronchoscopy usually requires heavy intravenous sedation or general anaesthesia. The rigid bronchoscope has important advantages over the flexible bronchoscope. The larger diameter of the rigid bronchoscope facilitates the passage of various grasping devices, including a flexible bronchoscope. A better chance of quick, successful extraction and better capabilities of suctioning clotted blood and thick secretions are offered by the rigid bronchoscope. The paediatric flexible bronchoscope lacks a hollow working channel through which instruments may be inserted or blood and secretions may be aspirated.
Unlike the flexible bronchoscope, the patient can be ventilated through the rigid scope; therefore, ventilation of the patient can be maintained. Rigid bronchoscopy is the procedure of choice for removing foreign bodies in children and in most adults. Success rates for extracting foreign bodies are reportedly more than 98%. Large solid and semisolid objects are best managed emergently in the operating room with a rigid bronchoscope and appropriate grasping instruments.

Acetaminophen, NSAIDs, and opiates are recommended in the basic approach to cancer pain associated with bone metastases. They should provide patients with adequate analgesia when used appropriately. NSAIDs including COX2 inhibitors are especially useful for patients with bone pain. Acetaminophen is an effective analgesic but is a weak anti-inflammatory agent. Ketorolac tromethamine is a potent NSAID capable of relieving moderate to severe acute bone pain.

Ankle fractures most often occur by rotational mechanisms with the external forces transmitted through the foot via the talus to the malleoli. The specific pattern of fracture and ligamentous injury depends on the position of the foot and the direction of the force at the time of injury.

Reduce the ankle fracture as soon as possible once informed consent provided to the patient. Assess the neurovascular status of the limb before and after manipulation. Have splinting materials ready and measured out (use the uninjured, contralateral leg for measuring). Either a short leg splint or cast is applied based on fracture type, patient, surgical urgency, and surgeon preference.

Ensure adequate analgesia for the patient including NSAIDs, IV medications, hematoma block, or procedural sedation. Once reduced and splinted in place, recheck neurovascular status, elevate the leg and obtain a post-reduction X-ray.

There is strong evidence to support haemostatic resuscitation in the setting of massive haemorrhage due to trauma. This advocates the use of 1:1:1 ratio.

Uncontrolled haemorrhage accounts for up to 39% of all trauma-related deaths. In the UK, approximately 2% of all trauma patients need massive transfusion. Massive transfusion is defined as the replacement of a patient’s total blood volume in less than 24 hours or the acute administration of more than half the patient’s estimated blood volume per hour. During acute bleeding, the practice of haemostatic resuscitation has been shown to reduce mortality rates. It is based on the principle of transfusion of blood components in fixed ratios. For example, packed red cells, FFP, and platelets are administered in a ratio of 1:1:1.

The Monteggia fracture refers to a dislocation of the proximal radio-ulnar joint (PRUJ) in association with a forearm fracture, most commonly a fracture of the ulna. Depending on the type of fracture and severity, they may experience elbow swelling, deformity, crepitus, and paraesthesia or numbness. Some patients may not have severe pain at rest, but elbow flexion and forearm rotation are limited and painful.
The dislocated radial head may be palpable in the anterior, posterior, or anterolateral position. In Bado type I and IV lesions, the radial head can be palpated in the antecubital fossa. The radial head can be palpated posteriorly in type II lesions and laterally in type III lesions.

Colles’ fractures have the following 3 features:
– Transverse fracture of the radius
– 1 inch proximal to the radio-carpal joint
– Dorsal displacement and angulation

Smith’s fracture (reverse Colles’ fracture)
– Volar angulation of distal radius fragment (Garden spade deformity)
– Caused by falling backwards onto the palm of an outstretched hand or falling with wrists flexed

Bennett’s fracture
– Intra-articular fracture of the first carpometacarpal joint
– Impact on flexed metacarpal, caused by fist fights
– X-ray: triangular fragment at ulnar base of metacarpal

Galeazzi fracture
– Radial shaft fracture with associated dislocation of the distal radioulnar joint

Pott’s fracture
– Bimalleolar ankle fracture
– Forced foot eversion

Barton’s fracture
– Distal radius fracture (Colles’/Smith’s) with associated radiocarpal dislocation
– Fall onto extended and pronated wrist
– Involvement of the joint is a defining feature

Aspiration is the treatment of choice in thyroid cysts, but the recurrence rates are high (60%–90% of patients), particularly with repeated aspirations and large-volume cysts.
Percutaneous ethanol injection (PEI) has been studied in several large randomized controlled studies, with reported success in 82–85% of the cases after an average of 2 sessions, with a volume reduction of more than 85% from baseline size.
PEI may also be considered for hyperfunctioning nodules, particularly if a large fluid component is present. It has a success rate ranging from 64% to 95%, with a mean volume reduction of 66%, but recurrences are more common and the number of sessions required to achieve good response is higher (about 4 sessions per patient). PEI is a safe procedure, with the most common reported adverse effects being local pain, dysphonia, flushing, dizziness, and, rarely, recurrent laryngeal nerve damage.
Surgery, Lobectomy is also a reasonable therapy for cystic lesions, as an alternative to the previously mentioned procedures.

An anal fissure is a painful linear tear or crack in the distal anal canal, which, in the short term, usually involves only the epithelium and, in the long term, involves the full thickness of the anal mucosa. Anal fissures develop with equal frequency in both sexes; they tend to occur in younger and middle-aged persons.

Treatment should include stool softeners and lifestyle advice. Failure of medical therapy is an indication for surgical therapy. Controversy mostly involves continued efforts to find a medical therapy for anal fissure that is as successful as the surgical therapy for the condition.
Typically, the patient reports severe pain during a bowel movement, with the pain lasting several minutes to hours afterward. The pain recurs with every bowel movement, and the patient commonly becomes afraid or unwilling to have a bowel movement, leading to a cycle of worsening constipation, harder stools, and more anal pain. Approximately 70% of patients note bright-red blood on the toilet paper or stool. Occasionally, a few drops may fall in the toilet bowl, but significant bleeding does not usually occur with an anal fissure.

Children with intussceception usually present at a relatively young age with colicky abdominal pain, together with a mass on clinical examination. The often cited red current jelly type stool is a rare but classical feature.

Juvenile polyps may occur as part of the familial polyposis coli syndromes. The lesions, which are hamartomas, are often cherry red if they protrude externally.

Answer: Rickets

Rickets is a disease of growing bone that is unique to children and adolescents. It is caused by a failure of osteoid to calcify in a growing person.
The signs and symptoms of rickets can include:

pain – the bones affected by rickets can be sore and painful, so the child may be reluctant to walk or may tire easily; the child’s walk may look different (waddling)
skeletal deformities – thickening of the ankles, wrists and knees, bowed legs, soft skull bones and, rarely, bending of the spine
dental problems – including weak tooth enamel, delay in teeth coming through and increased risk of cavities
poor growth and development – if the skeleton doesn’t grow and develop properly, the child will be shorter than average
fragile bones – in severe cases, the bones become weaker and more prone to fractures.

Marfan syndrome (MFS) is a genetic disorder of the connective tissue. The degree to which people are affected varies. People with Marfan tend to be tall and thin, with long arms, legs, fingers and toes. They also typically have flexible joints and scoliosis. The most serious complications involve the heart and aorta, with an increased risk of mitral valve prolapse and aortic aneurysm. Other commonly affected areas include the lungs, eyes, bones and the covering of the spinal cord.

Ehlers-Danlos syndrome is a group of inherited disorders that affect your connective tissues — primarily your skin, joints and blood vessel walls. People who have Ehlers-Danlos syndrome usually have overly flexible joints and stretchy, fragile skin. This can become a problem if you have a wound that requires stitches, because the skin often isn’t strong enough to hold them.
A more severe form of the disorder, called Ehlers-Danlos syndrome, vascular type, can cause the walls of your blood vessels, intestines or uterus to rupture.

Osteoporosis is a disease in which bone weakening increases the risk of a broken bone. It is the most common reason for a broken bone among the elderly. Bones that commonly break include the vertebrae in the spine, the bones of the forearm, and the hip. Until a broken bone occurs there are typically no symptoms. Bones may weaken to such a degree that a break may occur with minor stress or spontaneously. Chronic pain and a decreased ability to carry out normal activities may occur following a broken bone.

Osteoporosis may be due to lower-than-normal maximum bone mass and greater-than-normal bone loss. Bone loss increases after menopause due to lower levels of oestrogen. Osteoporosis may also occur due to a number of diseases or treatments, including alcoholism, anorexia, hyperthyroidism, kidney disease, and surgical removal of the ovaries.

In this case, there is no delay in treatment and the mechanism by which the fracture occurred fits accidental fracture.

A statement from the parent or guardian and any witnesses regarding how the child sustained the injury will help determine whether the injury is accidental or abusive. A statement from the parent or guardian explaining why he or she delayed in seeking medical treatment is important to the investigation because caretakers often postpone medical treatment or fail to provide treatment for an injured child to hide physical abuse. The abusing parent or caregiver may also put a child in oversized clothing or keep the child inside a residence for extended periods of time in an attempt to conceal the child’s injuries.

Parents who inflict fractures on their children tend to minimize the severity of the accident purported to cause the fracture, whereas many parents of children with accidental fractures will relate a history of high-energy events.

Complications of intravenous drug abuse, such as subcutaneous abscess, joint infections, osteomyelitis, overdose, hepatitis, and infective endocarditis, account for an increasing number of admissions in accident and emergency departments throughout the UK. The organisms that usually cause chronic osteomyelitis in intravenous drug users are Gram‐negative rods such as Pseudomonas aeruginosa and Gram‐positive cocci such as staphylococci. Early treatment is essential to prevent progressive bone destruction. TB tends to affect the thoracic spine and in other causes of osteomyelitis the lumbar spine is affected.

Clinical characteristics associated with increased venous thromboembolism (VTE) risk in children (listed alphabetically, owing to the current lack of expert consensus or robust data regarding relative risk contributions)
-Anticipated hospitalization > 72 h
-Cancer (active, not in remission)
-Central venous catheter presence
-Oestrogen therapy started within the last 1 month
-Inflammatory disease (newly diagnosed, poorly controlled, or flaring)
-Intensive care unit admission
-Mechanical ventilation
-Mobility decreased from baseline (Braden Q‐score < 2)
-Obesity (BMI > 99th percentile for age)
-Post pubertal age
-Severe dehydration, requiring intervention
-Surgery > 90 min within last 14 days
-Systemic or severe local infection (positive sputum/blood culture or viral test result, or empirical antibiotics)
-Trauma as admitting diagnosis

Pharmacological prophylaxis may be instituted in the following risk groups:
Children in an ICU with a CVC and one other risk factor fit a high‐risk profile and may benefit from pharmacological thromboprophylaxis in the absence of strong contraindications.
For children with either a CVC or admission to an ICU (but not both), two risk factors are recommended before the initiation of pharmacological thromboprophylaxis.
For children with neither a CVC nor ICU admission, at least three risk factors should be present before the initiation of pharmacological thromboprophylaxis.

Absence of comorbidities and a small procedure with no likelihood of systemic compromise will equate to an ASA score of 1.

The ASA physical status classification system is a system for assessing the fitness of patients before surgery. It has six grades, as described below:

ASA 1: No physiological, biochemical, or psychiatric disturbance. The surgical pathology is localised and has not invoked systemic disturbance.

ASA 2: Mild or moderate systemic disruption caused either by the surgical disease process or through an underlying pre-existing disease.

ASA 3: Severe systemic disruption, not life-threatening, caused either by the surgical pathology or a pre-existing disease.

ASA 4: Patient has severe systemic disease that is a constant threat to life.

ASA 5: Patient is moribund and will not survive without surgery.

ASA 6: A brain-dead patient whose organs are being removed with the intention of transplanting them into another patient.

Indications for IV fluids include:
– Resuscitation and circulatory support
– Replacing on-going fluid losses
– Maintenance fluids for children for whom oral fluids are not appropriate
– Correction of electrolyte disturbances

According to the NICE guidelines, if children and young people need IV fluid resuscitation, use glucose‑free crystalloids that contain sodium in the range 131–154 mmol/litre, with a bolus of 20 ml/kg over less than 10 minutes. Take into account pre‑existing conditions (for example, cardiac disease or kidney disease), as smaller fluid volumes may be needed.

All the others are contraindicated according to the guidelines as they are either made of glucose or are colloids.
Gelofusine is a 4% w/v solution of succinylated gelatine (also known as modified fluid gelatine) used as an intravenous colloid, and behaves much like blood filled with albumins.
Dextrose solution is a mixture of dextrose (glucose) and water. It is used to treat low blood sugar or water loss without electrolyte loss. Intravenous sugar solutions are in the crystalloid family of medications. They come in a number of strengths including 5%, 10%, and 50% dextrose.
0.45% saline/ 5% glucose solution also contains glucose hence it is contraindicated.

Duct ectasia is the cause of nipple discharge, with different percentages recorded in different articles (>10%, >30% and >50%). Duct ectasia differs from cystic disease since it is an inflammatory process, usually affecting the ducts below the nipple. It develops gradually, either through the ductal system or the breast lobes. The acute inflammation may be caused by epithelial rupture and diffusion of the contents into the fibrous duct-wall and the underlying structures. These contents consist of neutral fat and lipid crystals that are typical of duct ectasia. The chronic granulation-type reaction may develop foreign body-like giant cells and a multiform inflammatory cell population. Since the whole process in duct ectasia is long in duration, plasma cells are sometimes dominant in the inflammatory infiltrations. Other findings in duct ectasia include the following:
-Serous or green-white nipple discharge in 20% of cases;
-Nipple inversion that leads to fibrosis and development of ring or tubular calcification (this finding is typical in the mammogram and especially when the disease is in its advanced stages);
-Apocrine metaplasia with or without epithelial hyperplasia. The epithelial layer consists of widened atrophic cells.
The condition may be managed symptomatically and/or with antibiotics, but in persistent or recurrent cases it is managed with surgical excision of the ducts below the nipple. A focused excision is preferred to a complete subareolar excision since the later technique is associated with higher rates of seroma formation, nipple numbness and nipple inversion. The Hadfield’s procedure (major duct excision) is an option.

A pancreatic pseudocyst is a circumscribed collection of fluid rich in pancreatic enzymes, blood, and necrotic tissue, typically located in the lesser sac of the abdomen. Pancreatic pseudocysts are usually complications of pancreatitis, although in children they frequently occur following abdominal trauma. Pancreatic pseudocysts account for approximately 75% of all pancreatic masses.
Signs and symptoms of pancreatic pseudocyst include abdominal discomfort and indigestion.

Diagnosis of Pancreatic pseudocyst can be based on cyst fluid analysis:
Carcinoembryonic antigen (CEA) and CEA-125 (low in pseudocysts and elevated in tumours);
Fluid viscosity (low in pseudocysts and elevated in tumours);
Amylase (usually high in pseudocysts and low in tumours)

The most useful imaging tools are:

-Ultrasonography – the role of ultrasonography in imaging the pancreas is limited by patient habitus, operator experience and the fact that the pancreas lies behind the stomach (and so a gas-filled stomach will obscure the pancreas).
-Computerized tomography – this is the gold standard for initial assessment and follow-up.
-Magnetic resonance cholangiopancreatography (MRCP) – to establish the relationship of the pseudocyst to the pancreatic ducts, though not routinely used.

Hernia containing Meckel’s diverticulum is termed as Littre’s hernia.

Hernias occur when a viscus or part of it protrudes from within its normal anatomical cavity. A Littre’s hernia is an abdominal wall hernia that involves the Meckel’s diverticulum which is a congenital outpouching or bulge in the lower part of the small intestine and is a leftover of the umbilical cord.

Management of Littre’s hernia includes resection of the diverticulum followed by herniorrhaphy.

Acromioclavicular joint (ACJ) dislocation normally occurs secondary to direct injury to the superior aspect of the acromion. Loss of shoulder contour and prominent clavicle are the key features.

An ACJ dislocation, or AC separation, is a very frequent injury among physically active people. It is commonly caused by a fall directly on the shoulder or a direct blow received in a contact sport. Disruption of the ACJ results in pain and instability in the entire shoulder and arm. The pain is most severe when the patient attempts overhead movements or tries to sleep on the affected side.

In general, most AC injuries do not require surgery. There are certain situations, however, in which surgery may be necessary. Most patients recover with full function of the shoulder. The period of disability and discomfort ranges from a few days to 12 weeks depending on the severity of the separation.

Answer: Fitz Hugh Curtis syndrome

Fitz-Hugh-Curtis syndrome is a rare disorder that occurs almost exclusively in women. It is characterized by inflammation of the membrane lining the stomach (peritoneum) and the tissues surrounding the liver (perihepatitis). The muscle that separates the stomach and the chest (diaphragm), which plays an essential role in breathing, may also be affected. Common symptoms include severe pain in the upper right area (quadrant) of the abdomen, fever, chills, headaches, and a general feeling of poor health (malaise). Fitz-Hugh-Curtis syndrome is a complication of pelvic inflammatory disease (PID), a general term for infection of the upper genital tract in women. Infection is most often caused by Neisseria gonorrhoeae and Chlamydia trachomatis.

Fitz-Hugh-Curtis syndrome is characterized by the onset of sudden, severe pain in the upper right area of the abdomen. Pain may spread to additional areas including the right shoulder and the inside of the right arm. Movement often increases pain. The upper right area may be extremely tender.

Additional symptoms may occur in some cases including fever, chills, night sweats, vomiting and nausea. Some affected individuals may develop headaches, hiccupping, and a general feeling of poor health (malaise).

Some affected individuals may have symptoms associated with pelvic inflammatory disease including fever, vaginal discharge, and lower abdominal pain. Lower abdominal pain may precede, follow, or occur simultaneously with upper abdominal pain.

Most cases of Fitz-Hugh-Curtis syndrome are caused by infection with the bacterium Chlamydia trachomatis, which causes Chlamydia or the organism Neisseria gonorrhoeae, which causes gonorrhoea. Chlamydia and gonorrhoea are common sexually transmitted diseases (STDs). Researchers believe that more cases of Fitz-Hugh-Curtis syndrome are caused by infection with Chlamydia trachomatis than with Neisseria gonorrhoeae.

The exact process by which such infections cause Fitz-Hugh-Curtis syndrome (pathogenesis) is not completely understood. Some researchers believe that it occurs because of infection of the liver and surrounding tissue, which may result from bacteria traveling from the pelvis directly to the liver or via the bloodstream or lymphatic system. Fitz-Hugh-Curtis syndrome is characterized by the developed of string-like, fibrous scar tissue (adhesions) between the liver and the abdominal wall or the diaphragm.

Laparoscopy is the gold standard for diagnosing FHCS and PID. In the setting of PID, laparoscopy can show oedema with exudates on tubal surfaces, ectopic pregnancy, or tubo-ovarian abscess. FHCS can be diagnosed directly via visualization of adhesions between the diaphragm and liver or liver and the anterior abdominal wall.

Discrete masses graded P3 or R3/M3 or U3 and above should be triple assessed using core biopsy rather than FNAC unless core biopsy is not feasible.
When cytology or biopsy has been reported as C1 or B1 it will require a further biopsy to delineate the pathology in most instances.
The exceptions are when the lesion is suspected to be a lipoma or hamartoma following imaging and discussion. Following triple assessment, if there is non-concordance by more than one grade then further biopsy and or investigations should be undertaken.
In this case, Excisional biopsy is the next required biopsy to delineate the pathology.

Lesions bearing normal dermal appendages and regular borders are likely to be benign pigmented naevi. Therefore diagnostic and not radical excision is indicated. Incision biopsy should not be done.

Melanocytic naevi are pigmented moles. Some moles are present at birth or appear within the first two years of life. These are known as congenital melanocytic naevi. Most develop during childhood and early adult life and are, consequently, called acquired melanocytic naevi. The number of moles increase up to the age of 30–40. Thereafter, the number of naevi tend to decrease. New moles appearing in adulthood need to be monitored and checked if growing or changing. Moles can be found anywhere on the skin, including on the hands and feet, genitals, eyes, and scalp.

There are three main types of acquired melanocytic naevi:
1. Junctional melanocytic naevi: flat and usually circular.
2. Compound melanocytic naevi: raised brown bumps most of which are hairy, some have a slightly warty surface.
3. Intradermal melanocytic naevi: raised and often hairy bumps, similar to compound naevi but more pale coloured (often skin-coloured).

Answer: Transposition of the great arteries

Transposition of the great arteries (TGA) is the most common cyanotic congenital heart lesion that presents in neonates. The hallmark of transposition of the great arteries is ventriculoarterial discordance, in which the aorta arises from the morphologic right ventricle and the pulmonary artery arises from the morphologic left ventricle.

Infants with transposition of the great arteries (TGA) are usually born at term, with cyanosis apparent within hours of birth.

The clinical course and manifestations depend on the extent of intercirculatory mixing and the presence of associated anatomic lesions. Note the following:

Transposition of the great arteries with intact ventricular septum: Prominent and progressive cyanosis within the first 24 hours of life is the usual finding in infants if no significant mixing at the atrial level is evident.

Transposition of the great arteries with large ventricular septal defect: Infants may not initially manifest symptoms of heart disease, although mild cyanosis (particularly when crying) is often noted. Signs of congestive heart failure (tachypnoea, tachycardia, diaphoresis, and failure to gain weight) may become evident over the first 3-6 weeks as pulmonary blood flow increases.

Transposition of the great arteries with ventricular septal defect and left ventricular outflow tract obstruction: Infants often present with extreme cyanosis at birth, proportional to the degree of left ventricular (pulmonary) outflow tract obstruction. The clinical history may be similar to that of an infant with tetralogy of Fallot.

Transposition of the great arteries with ventricular septal defect and pulmonary vascular obstructive disease: Progressively advancing pulmonary vascular obstructive disease can prevent this rare subgroup of patients from developing symptoms of congestive heart failure, despite a large ventricular septal defect. Most often, patients present with progressive cyanosis, despite an early successful palliative procedure.

Answer: Intestinal malrotation

During normal abdominal development, the 3 divisions of the GI tract (i.e., foregut, midgut, hindgut) herniate out from the abdominal cavity, where they then undergo a 270º counter clockwise rotation around the superior mesenteric vessels. Following this rotation, the bowels return to the abdominal cavity, with fixation of the duodenojejunal loop to the left of the midline and the cecum in the right lower quadrant.

Intestinal malrotation refers to any variation in this rotation and fixation of the GI tract during development. Interruption of typical intestinal rotation and fixation during foetal development can occur at a wide range of locations; this leads to various acute and chronic presentations of disease. The most common type found in paediatric patients is incomplete rotation predisposing to midgut volvulus, requiring emergent operative intervention.

Acute midgut volvulus

Usually occurs during the first year of life

Sudden onset of bilious emesis

Diffuse abdominal pain out of proportion to physical examination

Acute duodenal obstruction

This anomaly is usually recognized in infants and is due to compression or kinking of the duodenum by peritoneal bands (Ladd bands).

Patients present with forceful vomiting, which may or may not be bile-stained, depending on the location of the obstruction with respect to the entrance of the common bile duct (ampulla of Vater).

Malrotation may occur as an isolated anomaly or in association with other congenital anomalies; 30-62% of children with malrotation have an associated congenital anomaly. All children with diaphragmatic hernia, gastroschisis, and omphalocele have intestinal malrotation by definition. Additionally, malrotation is seen in approximately 17% of patients with duodenal atresia and 33% of patients with jejunoileal atresia.

The scaphoid bone is the most commonly fractured carpal bone. Fractures are most often localized in the middle third of the scaphoid bone.
Generally, scaphoid bone fractures result from indirect trauma when an individual falls onto the outstretched hand with a hyperextended and radially deviated wrist. Pain when applying pressure to the anatomical snuffbox is highly suggestive of a scaphoid bone fracture.
X-ray is the initial test of choice for diagnosis. Computer tomography and magnetic resonance imaging may be indicated, if x-ray findings are negative but clinical suspicion is high.
Treatment can be conservative (e.g., wrist immobilization) or in certain cases surgical (e.g., proximal pole fracture). Complications include non-union and avascular necrosis.

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.