Answer: Transposition of the great arteriesTransposition 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.

Compared to the rest of the options, Meckel’s diverticulum with ectopic gastric mucosa seems to be the most probable diagnosis, as it can lead to fresh bleeding.Fresh red bleeding can be caused by haemorrhoids, polyps or a massive GI bleed.

90% of patients with meconium ileus have cystic fibrosis (CF). Indeed, in 10 – 15% of cases of CF, the patient presents with meconium ileus.

Cardiac notch: is an indentation on the left lung of the heart.
Coronary sinus: a venous sinus on the surface of the heart (the posterior aspect) that receives blood from the smaller veins that drain the heart.
Coronary sulcus: a groove on the heart between the atria and ventricles.
Transverse pericardial sinus: located behind the aorta and pulmonary trunk and anterior to the superior vena cava.
Oblique pericardial sinus: located behind the left atrium. Accessed from the inferior side (or the apex) of the heart upwards.
Horizontal pericardial sinus: this is a made-up term.

Germinal matrix/intraventricular haemorrhage (GM/IVH) is a complication of premature delivery that can result in life-long medical and developmental consequences. Although GM/IVH can occur in term infants, haemorrhage in this group of infants remains distinct from periventricular haemorrhage (PVH)/IVH of the preterm infant. Several acquired lesions of the central nervous system (CNS) specifically affect infants born prematurely and result in long-term disability, including GM/IVH, periventricular white matter injury (e.g., cystic periventricular leukomalacia [CPVL], periventricular haemorrhagic infarction [PVHI]), haemorrhage, and diffuse injury to the developing brain.The physical examination is usually negative in germinal matrix/intraventricular haemorrhage (GM/IVH). Occasionally, severe GM/IVH may present with nonspecific systemic findings suggestive of cardiovascular collapse.One subgroup of infants with GM/IVH presents with the following:- A sudden unexplained drop in haematocrit levels- Possible physical findings related to anaemia (e.g., pallor, poor perfusion) or haemorrhagic shockAnother subgroup of infants with GM/IVH presents with extreme signs, including the following:- A sudden and significant clinical deterioration associated with anaemia, metabolic acidosis, glucose instability, respiratory acidosis, apnoea, hypotonia, and stupor is present.Physical findings related to these signs include poor perfusion, pallor or an ashen colour, irregularities of respiratory pattern, signs of respiratory distress including retractions and tachypnoea, hypotonia, and altered mental status (e.g., decreased responsiveness, coma).Additional neurologic signs, such as fullness of the fontanelles, seizures, and posturing, may also be observed. Progression can be rapid and may result in shock and death.Extradural haemorrhage also known as an epidural hematoma, is a collection of blood that forms between the inner surface of the skull and outer layer of the dura, which is called the endosteal layer. They are usually associated with a history of head trauma and frequently associated skull fracture. The source of bleeding is usually arterial, most commonly from a torn middle meningeal artery.A subdural haemorrhage (or hematoma) is a type of bleeding that often occurs outside the brain as a result of a severe head injury. It takes place when blood vessels burst between the brain and the leather-like membrane that wraps around the brain (the dura mater). The pooling blood creates pressure on the surface of the brain, causing a variety of problems.

An appendicular mass, on the whole, is managed medically with intravenous antibiotics and monitoring for signs of obstruction or on-going sepsis. If the child is not responding to medical management, then surgery is performed. This is due to the high morbidity risk associated with operating on an appendicular mass. Consent for a limited right hemi-colectomy must be taken after explaining the increased risk of complications. The decision whether or not to perform an interval appendicectomy is controversial and currently subject to a multicentre national trial. The likelihood of another episode of appendicitis is 1 in 5. Other options:- Ultrasound and clinical examination is sufficient to confirm the diagnosis, especially in a boy. This may not be the case in females.- Majority of appendicular masses respond to conservative management. – Raised CRP indicates significant inflammatory response and thus intravenous antibiotics are indicated. – Intravenous antibiotics are indicated due to sepsis. Oral antibiotics are not sufficient to tackle sepsis in this scenario.

Congenital duodenal atresia is one of the more common intestinal anomalies treated by paediatric surgeons, occurring 1 in 2500-5000 live births. In 25-40% of cases, the anomaly is encountered in an infant with trisomy 21 (Down syndrome).Presenting symptoms and signs are the results of high intestinal obstruction. Duodenal atresia is typically characterized by the onset of vomiting within hours of birth. While vomitus is most often bilious, it may be nonbilious because 15% of defects occur proximal to the ampulla of Vater. Occasionally, infants with duodenal stenosis escape detection of an abnormality and proceed into childhood or, rarely, into adulthood before a partial obstruction is noted. Nevertheless, one should assume any child with bilious vomiting has a proximal GI obstruction until proven otherwise, and further workup should be begun expeditiously.Once delivered, an infant with duodenal atresia typically has a scaphoid abdomen. One may occasionally note epigastric fullness from dilation of the stomach and proximal duodenum. Passing meconium within the first 24 hours of life is not usually altered. Dehydration, weight loss, and electrolyte imbalance soon follow unless fluid and electrolyte losses are adequately replaced. If intravenous (IV) hydration is not begun, a hypokalaemic/hypochloraemic metabolic alkalosis with paradoxical aciduria develops, as with other high GI obstruction. An orogastric (OG) tube in an infant with suspected duodenal obstruction typically yields a significant amount of bile-stained fluid.

Based on the clinical scenario provided, this patient most probably has impacted gall stones. Gall stones in children can be caused by haematological diseases such as sickle cell anaemia and thalassemia. Cholesterol stones are also becoming more prevalent. A dilated common bile duct (> 10mm in adults) suggests gall stone impaction. The presence of pyrexia indicates cholecystitis.

Inguinal hernias are a common surgical condition in preterm male infants. Surgical repair is usually suggested shortly after birth due to a fear of incarceration or strangulation. These hernias are often indirect as they pass though the processes vaginalis, rather than the posterior wall of the inguinal canal. Recurrence of the hernia is the most common post operative complication. While other complications can occur if left un-repaired, hydrocele and testicular atrophy are not as common, neither is spontaneous resolution.

Adhesion formation might be reduced by minimally invasive surgical techniques and the use of adhesion barriers. Non-operative treatment is effective in most patients with ASBO. Contraindications for non-operative treatment include peritonitis, strangulation, and ischemia. When the adhesive aetiology of obstruction is unsure, or when contraindications for non-operative management might be present, CT is the diagnostic technique of choice. The principles of non-operative treatment are nil per os, naso-gastric, or long-tube decompression, and intravenous supplementation with fluids and electrolytes. When operative treatment is required, a laparoscopic approach may be beneficial for selected cases of simple ASBO.

Beckwith-Wiedemann Syndrome (BWS) was first characterized by Patients having abdominal wall defects, macrosomia, macroglossia, and enlarged adrenal glands. Since then, the clinical presentation has expanded to recognize hemihypertrophy/lateralized overgrowth, hyperinsulinism, omphalocele, and organomegaly as classic features of BWS.About 50% of children with BWS have hypoglycaemia and therefore patients with diagnosed BWS should be evaluated for hypoglycaemia. Hypoglycaemia in most BWS new-borns generally resolves within the first few days of life. However, in about 5% of patients that have hyperinsulinism, the severe prolonged hypoglycaemia requires escalated therapy ranging for medication (diazoxide) to partial pancreatectomy.

Answer: Surgery over the next few daysInguinal hernia is a type of ventral hernia that occurs when an intra-abdominal structure, such as bowel or omentum, protrudes through a defect in the abdominal wall. Inguinal hernias do not spontaneously heal and must be surgically repaired because of the ever-present risk of incarceration. Generally, a surgical consultation should be made at the time of diagnosis, and repair (on an elective basis) should be performed very soon after the diagnosis is confirmed.The infant or child with an inguinal hernia generally presents with an obvious bulge at the internal or external ring or within the scrotum. The parents typically provide the history of a visible swelling or bulge, commonly intermittent, in the inguinoscrotal region in boys and inguinolabial region in girls. The swelling may or may not be associated with any pain or discomfort.

The most worrying condition is testicular torsion and to exclude it exploratory surgery is required.

Answer: MittelschmerzMittelschmerz is midcycle abdominal pain due to leakage of prostaglandin-containing follicular fluid at the time of ovulation. It is self-limited, and a theoretical concern is treatment of pain with prostaglandin synthetase inhibitors, which could prevent ovulation. The pain of mittelschmerz usually occurs in the lower abdomen and pelvis, either in the middle or to one side. The pain can range from a mild twinge to severe discomfort and usually lasts from minutes to hours. In some cases, a small amount of vaginal bleeding or discharge might occur. Some women have nausea, especially if the pain is very strong.Diagnosis of pelvic pain in women can be challenging because many symptoms and signs are insensitive and nonspecific. As the first priority, urgent life-threatening conditions (e.g., ectopic pregnancy, appendicitis, ruptured ovarian cyst) and fertility-threatening conditions (e.g., pelvic inflammatory disease, ovarian torsion) must be considered. Many women never have pain at ovulation. Some women, however, have mid-cycle pain every month, and can tell by the pain that they are ovulating.As an egg develops in the ovary, it is surrounded by follicular fluid. During ovulation, the egg and the fluid, as well as some blood, are released from the ovary. While the exact cause of mittelschmerz is not known, it is believed to be caused by the normal enlargement of the egg in the ovary just before ovulation. Also, the pain could be caused by the normal bleeding that comes with ovulation.Pelvic inflammatory disease can be ruled out if the patient is not sexually active.

Immediate needle decompression of pneumoperitoneum is necessary to avoid tension pneumoperitoneum. Air reduction enema is the main stay of treatment for intussusception and is successful in around 90% of cases. A serious potential risk of this procedure is perforation of the colon and a pneumoperitoneum, leading to rapid distension of the abdomen and splinting of the diaphragm.

Based on the clinical scenario, the most probable diagnosis is acute appendicitis.Acute appendicitis presents typically with central abdominal pain migrating to the right iliac fossa, anorexia, nausea and fever. The temperature usually does not go beyond 40C. Although appendicitis is a clinical diagnosis, an ultrasound scan showing an appendix width of greater than 6mm is diagnostic.Pyrexia, guarding, and pain are all in keeping with appendicitis.Other options:- Cholecystitis is very rare in children, and although gall stones are present, there is no jaundice or biliary tree dilatation suggested by the scan.- Impacted Gall Stone: There is no mention of common bile duct dilatation in the clinical scenario.- Sickle Cell Crisis: A sickle cell crisis should not cause guarding, or an ultrasound scan finding of a dilated appendix base.- Gastroenteritis: Gastroenteritis would not produce the ultrasound scan findings described above with a thickened appendix.

The most probable cause for the patient’s presentation would be intra-abdominal adhesions, and the most appropriate management for this patient would be nasogastric decompression and intravenous fluids.Malrotation typically presents in the first month of life with bilious vomiting. There is a lifetime risk of intra-abdominal adhesions. This presents with bilious vomiting and dilated bowel loops on plain abdominal film. 2/3 of adhesional obstructions resolve by conservative management with nasogastric decompression and intravenous fluids. If this fails to resolve after 24-48 hours or if there are signs of peritonism, a laparotomy is indicated.Other options:- Recurrent volvulus, urgent laparotomy required: The child is now 8-years-old and recurrent volvulus is unlikely as bowel should be fixed by intra-abdominal adhesions.- Intra-abdominal adhesions, surgery for division of intra-abdominal adhesions: Although surgery may be required, the majority of adhesional obstructive cases respond to conservative management.- Gastroenteritis with incompetent pylorus, intravenous fluids: While it is true that patients with malrotation can have bilious vomiting in gastroenteritis, the abdominal X-ray is suggestive of obstruction.- Likely sepsis with bilious vomiting, intravenous fluids and antibiotics: While a septic ileus can give bilious vomiting, this patient has no inflammatory markers suggestive of sepsis.

The trend in the management of splenic injury continues to favour nonoperative or conservative management. In Paediatrics, Blunt splenic injuries with hemodynamic stability and absence of other internal injuries requiring surgery should undergo an initial attempt of Non-operative Management (NOM) irrespective of injury grade.In hemodynamically stable children with isolated splenic injury, splenectomy should be avoided.NOM is contraindicated in the presence of peritonitis, bowel evisceration, impalement or other indications to laparotomy.The vast majority of paediatric patients do not require angiography/angioembolization (AG/AE) for CT blush or moderate to severe injuries.AG/AE may be considered in patients undergone to NOM, hemodynamically stable with sings of persistent haemorrhage not amenable of NOM, regardless with the presence of CT blush once excluded extra-splenic source of bleeding.

The most appropriate diagnosis for this patient is appendicular mass. The initial treatment, according to the Ochsner-Sherren regimen recommended by Hamilton Bailey, would be the initiation of intravenous antibiotics.RationaleThe presentation is highly suggestive of appendicular mass. The correct management is broad-spectrum intravenous antibiotics such as co-amoxiclav and amikacin plus observation. Conservative management is the preferred treatment as surgical exploration at this stage can result in increased morbidity.The child should be allowed to eat and drink. If there are on-going temperature spikes, signs of obstruction or severe colicky abdominal pain, then surgery is required. The majority of patients respond to conservative management.Other options:- This is a partially treated appendicitis which has formed an appendix mass. Rather than planning appendectomy immediately| Hamilton Bailey recommended interval appendectomy after 6 weeks post-discharge. However, the need for interval appendectomy is still under debate.- Drainage via interventional radiology is not recommended in this patient as it can lead to swinging pyrexia secondary to peritonitis secondary to the collection.- The patient would have high-grade pyrexia and be constitutionally unwell if the patient was a patient of pyelonephritis. The presentation of the patient is more suggestive of appendicular mass.- The history would be more extended with symptoms of weight loss and altered bowel habit if the patient had Crohn’s disease.

Intussusception is a condition in which one segment of intestine telescopes inside of another, causing an intestinal obstruction. Although intussusception can occur anywhere in the gastrointestinal tract, it usually occurs at the junction of the small and large intestines. Most describe the symptoms of intussusception as a triad of colicky abdominal pain, bilious vomiting, and currant jelly stool. The primary symptom of intussusception is described as intermittent crampy abdominal pain.

The most probable diagnosis for this patient would be external angular dermoid.External angular dermoids typically form where there are overlapping tissue planes such as the midline. They contain a caseous material. The cysts are essentially benign in nature and are excised for cosmetic purposes or when they encroach on vision.Other options:- Branchial remnant typically presents as a supraclavicular pit.- Sebaceous cysts tend to be present on the scalp.- There is nothing in the clinical scenario to suggest a vascular malformation such as pigment change or ‘bag of worms’.- Lymphatic malformations are typically found in the neck or under the axilla.

Children who present with ruptured appendices are at increased risk of intra abdominal collections or abscesses. A swinging temperature is the first clue in indicating an intra abdominal abscess in a patient who had recently undergone surgery for a perforated appendix. The best course of action is therefore to get an ultrasound of the fluid collection before proceeding to rule out any other complication.

Perforated appendices are common in children as it is more difficult to surgically assess an unwell child due to poor localisation of abdominal pain, and their inability to express discomfort. They are therefore prone to a greater risk of post operative complications including would infections, intra abdominal fluid collections, and chest infections. In the above scenario the spiking temperature points to an abscess, which characteristically presents with a swinging temperature. The fever is unlikely due to bacterial resistance as blood tests performed post surgery would have indicated any resistance, and a UTI is also unlikely since the child is already on antibiotics. As his chest is clinically clear, a severe chest infection may be ruled out, leaving intra-abdominal collection as the most likely explanation.

The chance of having appendicitis again after appendix mass is around 17% in children. While the traditional teachings by Hamilton Bailey recommend following the conservative Ochsner-Sherren regimen followed by an interval appendicectomy six weeks after the discharge of the patient, there remains a looming controversy whether to perform an interval appendicectomy or not.

Answer: Do not operate and review the patient several weeks laterAir weapon injuries in children should be managed in the same way as any low velocity gun shot injury. Subcutaneous pellets are best removed. Urgent specialist referral is indicated for cranial, ocular, chest, abdominal, or vascular injuries as they may require emergency surgery. Cardiac injuries may be rapidly fatal. Penetrating abdominal injuries involving hollow viscera or major blood vessels need prompt exploration and repair. Intracranial air weapon pellets should be removed if possible. A pellet in lung parenchyma or muscle may be safely left in situ but there is a risk of infection. A pellet that has penetrated a joint or is associated with a fracture requires skilled orthopaedic management. A pellet lodged near a major blood vessel or nerve should ideally be removed. The possibility of intravascular embolism must be considered if the pellet is absent from a suspected entry site and there is no exit wound| numerous examples of arterial and venous embolism of an air weapon pellet in children have been described.In this case, the child seems well so there is no need to operate. He should be reviewed several weeks later.

Answer: Spontaneous bacterial peritonitisThe presence of periorbital and facial oedema with normal blood pressure in this toddler indicates that he has nephrotic syndrome. Nephrotic syndrome can be associated with a series of complications that can affect an individual’s health and quality of life:Spontaneous bacterial peritonitis can develop where there is ascites present. This is a frequent development in children but very rarely found in adults.Spontaneous bacterial peritonitis (SBP) is the development of a bacterial infection in the peritoneum, despite the absence of an obvious source for the infection. It is specifically an infection of the ascitic fluid – an increased volume of peritoneal fluid. Ascites is most commonly a complication of cirrhosis of the liver. It can also occur in patients with nephrotic syndrome. SBP has a high mortality rate.The diagnosis of SBP requires paracentesis, a sampling of the peritoneal fluid taken from the peritoneal cavity. If the fluid contains large numbers of white blood cells known as neutrophils (>250 cells/µL), infection is confirmed and antibiotics will be given, without waiting for culture results. In addition to antibiotics, infusions of albumin are usually administered.Signs and symptoms of spontaneous bacterial peritonitis (SBP) include fevers, chills, nausea, vomiting, abdominal pain and tenderness, general malaise, altered mental status, and worsening ascites. Thirteen percent of patients have no signs or symptoms. In cases of acute or chronic liver failure SBP is one of the main triggers for hepatic encephalopathy, and where there is no other clear causal indication for this, SBP may be suspected.These symptoms can also be the same for a spontaneous fungal peritonitis (SFP) and therefore make a differentiation difficult. Delay of diagnosis can delay antifungal treatment and lead to a higher mortality rate.

Although all of the options may lead to an acute red scrotum, torsion of the hydatid of Morgagni is the most common cause at this age (age 12-16 years).

Umbilical hernias are common in young children and they carry a low risk of incarceration as compared to inguinal hernias. About 90% umbilical hernias resolve by the age of 4 years. The best management for young children is to wait for resolution without surgical repair, unless there are complications. Parents should be assured that the child does not need to be referred to the general surgeon, nor should any home remedies such as taping a coin over the hernia be encouraged. If after the age of 4 it has not resolved, then surgery is indicated. This also applies to girls to prevent an unsightly hernia should she get pregnant.

Mittelschmerz is midcycle abdominal pain due to leakage of prostaglandin-containing follicular fluid at the time of ovulation. It is self-limited, and a theoretical concern is treatment of pain with prostaglandin synthetase inhibitors, which could prevent ovulation. The pain of mittelschmerz usually occurs in the lower abdomen and pelvis, either in the middle or to one side. The pain can range from a mild twinge to severe discomfort and usually lasts from minutes to hours. In some cases, a small amount of vaginal bleeding or discharge might occur. Some women have nausea, especially if the pain is very strong.Diagnosis of pelvic pain in women can be challenging because many symptoms and signs are insensitive and nonspecific. As the first priority, urgent life-threatening conditions (e.g., ectopic pregnancy, appendicitis, ruptured ovarian cyst) and fertility-threatening conditions (e.g., pelvic inflammatory disease, ovarian torsion) must be considered. Many women never have pain at ovulation. Some women, however, have mid-cycle pain every month, and can tell by the pain that they are ovulating.As an egg develops in the ovary, it is surrounded by follicular fluid. During ovulation, the egg and the fluid, as well as some blood, are released from the ovary. While the exact cause of mittelschmerz is not known, it is believed to be caused by the normal enlargement of the egg in the ovary just before ovulation. Also, the pain could be caused by the normal bleeding that comes with ovulation.Pelvic inflammatory disease can be ruled out if the patient is not sexually active.

Answer: Acute Epstein Barr virus infectionThe Epstein–Barr virus is one of eight known human herpesvirus types in the herpes family, and is one of the most common viruses in humans. Infection with Epstein-Barr virus (EBV) is common and usually occurs in childhood or early adulthood.EBV is the cause of infectious mononucleosis, an illness associated with symptoms and signs like:fever,fatigue,swollen tonsils,headache, andsweats,sore throat,swollen lymph nodes in the neck, andsometimes an enlarged spleen.Although EBV can cause mononucleosis, not everyone infected with the virus will get mononucleosis. White blood cells called B cells are the primary targets of EBV infection.Petechiae on the palate are characteristic of streptococcal pharyngitis but also can be seen in Epstein–Barr virus infection, Arcanobacterium haemolyticum pharyngitis, rubella, roseola, viral haemorrhagic fevers, thrombocytopenia, and palatal trauma.

Answer: Acute Epstein Barr virus infectionThe Epstein–Barr virus is one of eight known human herpesvirus types in the herpes family, and is one of the most common viruses in humans. Infection with Epstein-Barr virus (EBV) is common and usually occurs in childhood or early adulthood.EBV is the cause of infectious mononucleosis, an illness associated with symptoms and signs like:fever,fatigue,swollen tonsils,headache, andsweats,sore throat,swollen lymph nodes in the neck, andsometimes an enlarged spleen.Although EBV can cause mononucleosis, not everyone infected with the virus will get mononucleosis. White blood cells called B cells are the primary targets of EBV infection.

The most probable cause for the patient’s symptoms is intussusception.Intussusception is a condition in which part of the intestine folds into the section next to it. It most commonly involves the small bowel and rarely the large bowel. Intussusception doesn’t usually require surgical correction. 80% of cases can be both confirmed and reduced using barium, water-soluble or air-contrast enema. However, up to 10% of cases can experience recurrence within 24 hours after reduction, warranting close monitoring during this period.Other options:- Enteric duplication: a duplication cyst could give all these features, although it may contain sufficient gastric epithelium to produce a positive Meckel’s scan. However, this is comparatively rare. – Meckel’s diverticulum: Scintigraphy has poor sensitivity making the possibility of a bleed from a Meckel’s diverticulum a genuine differential for this patient. However, considering the presence of features of bowel obstruction in the patient, a Meckel’s diverticulum bleeding and obstructing at the same time would be a rare phenomenon. – Midgut volvulus: It is also a plausible differential in this patient. However, for the given age group, a midgut volvulus is relatively uncommon compared to intussusception. – Acute appendicitis: While it is true that appendicitis can present acutely with a palpable mass and bowel obstruction, it rarely presents with rectal bleeding.

Answer: Bochdalek herniaA Bochdalek hernia is one of two forms of a congenital diaphragmatic hernia, the other form being Morgagni hernia. A Bochdalek hernia is a congenital abnormality in which an opening exists in the infant’s diaphragm, allowing normally intra-abdominal organs (particularly the stomach and intestines) to protrude into the thoracic cavity. In the majority of patients, the affected lung will be deformed, and the resulting lung compression can be life-threatening. Bochdalek hernias occur more commonly on the posterior left side (85%, versus right side 15%).In normal Bochdalek hernia cases, the symptoms are often observable simultaneously with the baby’s birth. A few of the symptoms of a Bochdalek Hernia include difficulty breathing, fast respiration and increased heart rate. Also, if the baby appears to have cyanosis (blue-tinted skin) this can also be a sign. Another way to differentiate a healthy baby from a baby with Bochdalek Hernia, is to look at the chest immediately after birth. If the baby has a Bochdalek Hernia it may appear that one side of the chest cavity is larger than the other and or the abdomen seems to be scaphoid (caved in).Situs inversus (also called situs transversus or oppositus) is a congenital condition in which the major visceral organs are reversed or mirrored from their normal positions. The normal arrangement of internal organs is known as situs solitus while situs inversus is generally the mirror image of situs solitus. Although cardiac problems are more common than in the general population, most people with situs inversus have no medical symptoms or complications resulting from the condition, and until the advent of modern medicine it was usually undiagnosed. In the absence of congenital heart defects, individuals with situs inversus are homeostatically normal, and can live standard healthy lives, without any complications related to their medical condition. There is a 5–10% prevalence of congenital heart disease in individuals with situs inversus totalis, most commonly transposition of the great vessels. The incidence of congenital heart disease is 95% in situs inversus with levocardia.Cystic fibrosis is a progressive, genetic disease that causes persistent lung infections and limits the ability to breathe over time. In people with CF, mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene cause the CFTR protein to become dysfunctional. When the protein is not working correctly, it’s unable to help move chloride — a component of salt — to the cell surface. Without the chloride to attract water to the cell surface, the mucus in various organs becomes thick and sticky. In the lungs, the mucus clogs the airways and traps germs, like bacteria, leading to infections, inflammation, respiratory failure, and other complications. Necrotizing enterocolitis (NEC) is a medical condition where a portion of the bowel dies. It typically occurs in new-borns that are either premature or otherwise unwell. Symptoms may include poor feeding, bloating, decreased activity, blood in the stool, or vomiting of bile.The exact cause is unclear. Risk factors include congenital heart disease, birth asphyxia, exchange transfusion, and premature rupture of membranes. The underlying mechanism is believed to involve a combination of poor blood flow and infection of the intestines. Diagnosis is based on symptoms and confirmed with medical imaging.Morgagni hernias are one of the congenital diaphragmatic hernias (CDH), and is characterized by herniation through the foramen of Morgagni. When compared to Bochdalek hernias, Morgagni hernias are:-anterior-more often right-sided (,90%)-small-rare (,2% of CDH)-at low risk of prolapseOnly ,30% of patients are symptomatic. New-borns may present with respiratory distress at birth similar to a Bochdalek hernia. Additionally, recurrent chest infections and gastrointestinal symptoms have been reported in those with previously undiagnosed Morgagni hernia.The image of the transverse colon is herniated into the thoracic cavity, through a mid line defect and this indicates that it is a Morgagni hernia since the foramen of a Morgagni hernia occurs in the anterior midline through the sternocostal hiatus of the diaphragm, with 90% of cases occurring on the right side.Clinical manifestations of congenital diaphragmatic hernia (CDH) include the following:Early diagnosis – Right-side heart| decreased breath sounds on the affected side| scaphoid abdomen| bowel sounds in the thorax, respiratory distress, and/or cyanosis on auscultation| CDH can often be diagnosed in utero with ultrasonography (US), magnetic resonance imaging (MRI), or bothLate diagnosis – Chest mass on chest radiography, gastric volvulus, splenic volvulus, or large-bowel obstructionCongenital hernias (neonatal onset) – Respiratory distress and/or cyanosis occurs within the first 24 hours of life| CDH may not be diagnosed for several years if the defect is small enough that it does not cause significant pulmonary dysfunctionCongenital hernias (childhood or adult onset) – Obstructive symptoms from protrusion of the colon, chest pain, tightness or fullness the in chest, sepsis following strangulation or perforation, and many respiratory symptoms occur.

Answer: Obtain intravenous access, administer fluids and antibiotics.Intussusception is a condition in which one segment of intestine telescopes inside of another, causing an intestinal obstruction (blockage). Although intussusception can occur anywhere in the gastrointestinal tract, it usually occurs at the junction of the small and large intestines. The obstruction can cause swelling and inflammation that can lead to intestinal injury. The patient with intussusception is usually an infant, often one who has had an upper respiratory infection, who presents with the following symptoms:Vomiting: Initially, vomiting is nonbilious and reflexive, but when the intestinal obstruction occurs, vomiting becomes biliousAbdominal pain: Pain in intussusception is colicky, severe, and intermittentPassage of blood and mucus: Parents report the passage of stools, by affected children, that look like currant jelly| this is a mixture of mucus, sloughed mucosa, and shed blood| diarrhoea can also be an early sign of intussusceptionLethargy: This can be the sole presenting symptom of intussusception, which makes the condition’s diagnosis challengingPalpable abdominal massDiagnosis:Ultrasonography: Hallmarks of ultrasonography include the target and pseudo kidney signs.For all children, start intravenous fluid resuscitation and nasogastric decompression as soon as possible.

Answer: Undertake a laparotomyIntussusception, which is defined as the telescoping or invagination of a proximal portion of intestine (intussusceptum) into a more distal portion (intussuscipiens), is one of the most common causes of bowel obstruction in infants and toddlers.Intussusception may be ileoileal, colocolic, ileoileocolic, or ileocolic (the most common type).Most infants with intussusception have a history of intermittent severe cramping or colicky abdominal pain, occurring every 5-30 minutes. During these attacks, the infant screams and flexes at the waist, draws the legs up to the abdomen, and may appear pale. These episodes may last for only a few seconds and are separated by periods of calm normal appearance and activity. However, some infants become quite lethargic and somnolent between attacks.Infants with intussusception require surgical correction. Prompt laparotomy following diagnosis is crucial for achieving better outcomes. Primary anastomosis can be performed successfully, and stomas can be created in the critically ill patients or those with late detection and septicaemia.

Answer: Congenital diaphragmatic herniaCongenital diaphragmatic hernia (CDH) occurs when the diaphragm muscle — the muscle that separates the chest from the abdomen — fails to close during prenatal development, and the contents from the abdomen (stomach, intestines and/or liver) migrate into the chest through this hole.Examination in infants with congenital diaphragmatic hernias include the following findings:Scaphoid abdomenBarrel-shaped chestRespiratory distress (retractions, cyanosis, grunting respirations)In left-sided posterolateral hernia: Poor air entry on the left, with a shift of cardiac sounds over the right chest| in patients with severe defects, signs of pneumothorax (poor air entry, poor perfusion) may also be foundAssociated anomalies: Dysmorphisms such as craniofacial abnormalities, extremity abnormalities, or spinal dysraphism may suggest syndromic congenital diaphragmatic herniaIleal atresia is a congenital abnormality where there is significant stenosis or complete absence of a portion of the ileum. There is an increased incidence in those with chromosomal abnormalities. Ileal atresia results from a vascular accident in utero that leads to decreased intestinal perfusion and subsequent ischemia a segment of bowel. This leads to narrowing, or in the most severe cases, complete obliteration of the intestinal lumen. In the postnatal period, an abdominal radiograph will show air in the dilated loops of proximal bowel. An ileal atresia is often discovered prenatally at a routine prenatal ultrasound scan or following the development of polyhydramnios. On ultrasound, there is frequently a proximal dilated intestinal segment.Meconium Ileus (MI) is a condition where the content of the baby’s bowel (meconium) is extremely sticky and causes the bowel to be blocked at birth. In most cases the bowel itself is complete and intact but it is just the inside that is blocked.In some cases there has been a twist of the bowel before birth, which has caused the bowel to be blind ending (an atresia). Most babies with meconium ileus (90%) have Cystic Fibrosis (CF) and it is this that has caused the sticky meconium. Meconium ileus is a rare condition affecting only 1 in 25,000 babies. There is normally a delay in your baby passing meconium (black sticky stool normally passed within 24 hours of delivery) and your baby may also be reluctant to feed and may vomit a green fluid called bile which would normally pass through the bowel.Your baby may be uncomfortable because of constipation and trapped air in the bowel and the abdomen (tummy) will become distended. Some babies present at delivery with a distended abdomen and may be unwell due to infection around the bowel.Pyloric stenosis is a problem that affects babies between birth and 6 months of age and causes forceful vomiting that can lead to dehydration. It is the second most common problem requiring surgery in new-borns. The lower portion of the stomach that connects to the small intestine is known as the pylorus. In pyloric stenosis, the muscles in this part of the stomach enlarge, narrowing the opening of the pylorus and eventually preventing food from moving from the stomach to the intestine.

An incarcerated indirect inguinal hernia presents with abdominal pain, bloating, nausea, vomiting, and intestinal obstruction. It is characterized by the appearance of a tender mass in the inguinal area. Manual reduction in children requires analgesia and sedation.

The most appropriate procedure in the surgical management of this child would be to perform an appendicostomy for anterior continence enemas (Malone procedure).Idiopathic constipation leading to faecal incontinence is managed in a stepwise progression, first with laxatives such as movicol, enemas and stronger laxatives and in younger children inter-sphincteric injection of botox may be performed. Following this either anal irrigation or antegrade continence enemas are performed. Appendicostomy for anterior continence enemas allow colonic washouts and thereby rapid achievement of continence.Other options:- Defunctioning Ileostomy: Although an option in extreme cases, an ACE stoma would be more appropriate in this child.- Laparotomy for resection of the megarectum is performed if ACE stoma fails due to megarectum.- Left hemicolectomy is a procedure reserved for slow-transit colons to increase transit time.- Bishop-Koop stoma: It is a procedure of historical significance. It is a way of washing out and managing meconium ileus.

A surgeon will observe the patient and will decide which procedure he needs.

The boy suffers from balanitis xerotica obliterans (BXO) which is surgically treated with circumcision and urethral calibration. In fact, the condition is the only true medical indication for circumcision.