Anorectal malformations (ARM) are a congenital (existing before birth) abnormality resulting in some flaw of the gastrointestinal tract (GIT). These flaws range from an imperforate (closed) anus to a misplaced anus. In the majority of boys, the lower GIT is connected to the urinary system, whilst in females it may be connected to the genital tract. All forms of ARM require surgical management and some may be associated with cardiac (heart), renal(kidney), musculoskeletal (bone), and chromosomal (genetic) abnormalities. All systems will require thorough work up, which may include blood tests, x-rays and specialised ultrasound scans.

Symptoms:
An abnormal anus should be detected before your child is discharged from hospital. A baby with an ARM may present with multiple problems including:
• an anal opening that is too small (stenotic) or abnormally located– this can result in symptoms from constipation to complete bowel obstruction(blockage)
• an absent anal opening
• an anal opening with abnormal connections to the urinary and genital systems

Depending on the nature of the defect, children with ARM may suffer from constipation, and in the most severe cases even incontinence, in spite of the surgical care they receive. Management of these problems requires a long-term partnership between patients and their medical professionals.

Diagnosed:
The diagnosis of an ARM is usually clinical, but may involve x-rays, blood tests, ultrasound scans and even examination in theatre. Special ultrasound scans and x-rays will include:
• X-rays to examine the pelvis and bones of the spine
• Abdominal ultrasound to examine the urinary tract and kidneys
• Ultra sound to check for spinal abnormalities
• Spinal ultrasound to evaluate for spine abnormalities  
• Echocardiogram to check for possible heart defects

Treatment:
ARM requires specialised multistage surgical repair. Surgery does not guarantee are turn to full function. Each child will be individually assessed and managed in a specialised multidisciplinary team.Management of ARM requires a long-standing partnership between patients and medical professionals.

A congenital diaphragmatic hernia (CDH) is a birth defect resulting in a hole in the diaphragm. The diaphragm is an important muscle in the chest that allows the lungs to breathe. CDH allows intestines to pass into the chest, affecting development of the lungs and heart. As with many congenital conditions, CDH maybe identified by antenatal ultrasound scanning. If diagnosed antenatally, you should plan for delivery in a hospital with neonatal intensive care and paediatric surgical services. All forms of CDH require surgical management and some may be associated with cardiac (heart),chromosomal (genetic), intestinal, and neural tube (spinal) abnormalities. All systems will require thorough work up, which may include blood tests, x-rays and specialised ultrasound scans.

Symptoms of CDH:
Signs are usually noticed before birth on antenatal ultrasound and can include abnormal heart position, and protrusions of the stomach, intestines, or liver into the chest. Post-natal symptoms can include difficulty breathing, a barrel shaped chest, and a flat tummy.

Treatment:
All cases of CDH need to be repaired surgically. The timing of the operation will vary depending on each child’s condition. The operation is usually performed withan open approach through the abdomen. In most cases, the hole can be repaired primarily, but occasionally, the defect requires a patch repair. Your child will require to be managed in a multidisciplinary setting and all steps of the process will be thoroughly discussed.

Gastroschisis is a condition in which a hole in the abdominal wall allows intestines and other organs to protrude through to the outside. The problem is congenital and occurs early in gestational life. As such, the baby’s intestines are exposed to the mother’s amniotic fluid throughout the pregnancy. As a result, babies with gastroschisis are often born with swollen intestines that take a long time to work once they are returned to the baby’s abdomen and the abdomen is closed. As gastroschisis is a congenital problem, it may be diagnosed by antenatal ultrasound. If diagnosed antenatally, you should plan for delivery in a hospital with neonatal intensive care and paediatric surgical services. The diagnosis of gastroschisis does not preclude the possibility of undergoing a normal vaginal delivery. The risk for gastroschisis is higher in young pregnant women, undergoing their first pregnancy, and who smoke.

Treatments:
Gastroschisis can only be treated once the baby is delivered. The management of each child with gastroschisis is individualised to each patient’s circumstances. Repair of gastroschisis is broadly classified into 2 groups:

‍Primary Repair:
‍This repair is possible when the bowel is not too swollen and can be easily placed back into the abdomen.

‍Staged Repair:
Staged repair is utilised when the bowel is too swollen to immediately place back into the baby’s abdomen. Several surgeries are usually required to achieve reduction and abdominal closure. Initially, a plastic silo (pouch) is placed around the bowel and fixed to the abdominal wall to protect the bowel and allow for reduction. When the bowel is entirely reduced (placed back into the abdomen),the abdominal wall is closed. All children born with gastroschisis require admission to the neonatal intensive care unit and a central venous catheter. Some children with gastroschisis may require prolonged intubation and ventilation. Gastroschisis is usually not associated with other malformations.

Post-Operative Care:
Children born with gastroschisis are usually smaller than average. In conjunction with their often-long hospital stays this may result in these children taking sometime to catch up to a normal developmental path. Other issues these children encounter include the possibility of intestinal infection, gastrointestinal reflux, umbilical hernia, and in males, undescended testicles. Nevertheless, gastroschisis and its associated problems have an excellent prognosis and once these problems are addressed, children with gastroschisis have normal healthy lives with few complications.

Intestinal atresia is a complete blockage or obstruction anywhere in the intestine. As with many congenital conditions, intestinal atresia may be identified by antenatal ultrasound scanning. If diagnosed antenatally, you should plan for delivery in a hospital with neonatal intensive care and paediatric surgical services. All forms of intestinal atresia require surgical management and some may be associated with cardiac (heart), renal (kidney),musculoskeletal (bone), and chromosomal (genetic) abnormalities. All systems will require thorough work up, which may include blood tests, X-rays and specialised ultrasound scans. The risk for intestinal atresia is higher in mothers who smoke.

Treatment:
Children with intestinal atresia require an operation, and the exact type of operation differs depending on the location of the obstruction. Removing air and fluid from the intestinal tract can prevent vomiting and reduce the risk of bowel damage. It also provides babies with some comfort as abdominal swelling is relieved. All children with intestinal atresia will require intensive care admission, intravenous nutrition and placement of a central venous catheter. Once your child is stabilized, surgery is performed to repair the obstruction. Dr Patel and a team of multidisciplinary healthcare providers including a specialist paediatrician and paediatric anaesthesiologist will individualise your child’s treatment according to their specific complexities.

The oesophagus is the swallowing pipe that leads to the stomach.In oesophageal atresia, there is some blockage of the swallowing pipe. This obstruction is often associated with an abnormal connection between the oesophagus(swallowing pipe) and the trachea (breathing pipe). Oesophageal atresia (OA) is usually diagnosed before your baby is born in the course of antenatal ultrasound scanning. OA is also associated with cardiac (heart), renal(kidney), intestinal, musculoskeletal (bone), and chromosomal (genetic)abnormalities. All systems will require thorough work up, which may include blood tests, X-rays and specialised ultrasound scans.

Symptoms:
If OA is not diagnosed antenatally, it may present soon afterbirth with coughing or choking when feeding, difficulty breathing, a round full abdomen, and excessive salivation. Making the diagnosis of OA is relatively easy, and your baby’s paediatrician will request specific X-rays in order to make the diagnosis. If your child is suspected to have OA, he/she will not be fed until the diagnosis is excluded.

Treatment:
Surgery is the treatment of oesophageal atresia. In certain instances, repair of OA may not be possible at birth and your child will require a feeding tube (gastrostomy tube) so he/she can be fed until he/she is old enough for definitive surgery. Sometimes oesophageal atresia requires more than one surgery. DrPatel and a team of multidisciplinary healthcare providers including a specialist paediatrician and paediatric anaesthesiologist will individualise your child’s treatment according to their specific complexities.

Atresia refers to complete occlusion or obliteration of part of the intestinal tract. It occurs when the intestine, which should be an open tube, closes off in one or more places. It can affect any part of the gastrointestinal tract, from the oesophagus (swallowing pipe) to the intestines. The obstruction that results from an atresia prevents fluids and solids from passing normally through the digestive system. Atresia is a congenital condition and may be noticed on ultrasound before your child is born, or soon after birth as feed intolerance. If diagnosed antenatally, you should plan for delivery in a hospital with neonatal intensive care and paediatric surgical services.

Omphalocele (also known as exomphalos) is another form of congenital abdominal wall defect.Unlike gastroschisis, in omphalocele the intestines and other organs are covered by a membrane though the hole in the abdominal wall which allows these organs to exist outside of the abdominal cavity. Due to their membranous covering, children with omphalocele are not born with swollen intestines that take a long time to work properly.

This said, omphalocele is associated with multiple other congenital abnormalities. Between one third and one half of all children with omphalocele will have an associated cardiac (heart), chromosomal(genetic), neural tube (spinal), renal (kidney), or intestinal abnormality.Usually these associated problems are not life threatening and can be safely managed. As omphalocele is a congenital problem, it may be diagnosed by antenatal ultrasound. If diagnosed antenatally, you should plan for delivery in a hospital with neonatal intensive care and paediatric surgical services. The diagnosis of omphalocele does not preclude the possibility of undergoing a normal vaginal delivery.

Treatment:
Omphalocele can only be treated once the baby is delivered. The management of each child with omphalocele is individualised to each patient’s circumstances. If your child’s omphalocele is small, surgery may be done soon after birth. The bowel and other organs in the sac are placed into the belly and the abdominal opening is closed. If the omphalocele is larger, your baby’s belly will need to grow or be stretched enough before the surgery can be done. Skin will grow to cover the sac with the help of medication, good skin care and nutrition. If this happens, your baby will then have surgery to close the belly muscles in 12 to 24 months when the belly is larger.

Necrotizing enterocolitis (NEC) is a condition that affects the intestines of newborns. NECis more common in preterm, formula fed, low birth weight children that may have undergone a stressful delivery. In NEC, bacteria invade the body through the intestine. NEC is a dangerous condition that can have severe consequences.

Symptoms:
NEC can present with feed intolerance, abdominal bloating, irritability, difficulty breathing, lethargy and bloody stool. As the disease progresses, NEC may result in redness and swelling of the abdominal wall, and may even require children to be intubated and mechanically ventilated.

Treatment:
All children with NEC require admission to the neonatal intensive care unit, and some may require a central venous catheter. Initial management of NEC includes bowel rest, fluid resuscitation, broad spectrum antibiotics and intra venous nutritional supplementation. For those children for whom conservative management fails, surgery may be necessary. Most children who develop NEC recover completely and do not have any further problems. Treatment for your baby will be based on the findings by Dr Patel and the specialist multidisciplinary team who will take into account the baby’s age, overall health and tolerance for medication and procedures.