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Common Paediatric Operations

CIRCUMCISION

What is it? :
Surgical operation to remove the foreskin (or prepuce) of the penis

When is this operation required? :
Infection (balanoposthitis), balanitis xerotica obliterans, phimosis (inability to retract prepuce) with or without scarring.

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How is it performed? :
This is usually performed as an elective day surgery procedure under general anaesthesia. Circumcision is performed routinely with carbon dioxide laser at our centre. Circumcision using the Plastibell technique under local anaesthesia may be chosen for selected newborn babies.

INGUINAL HERNIOTOMY

What is it?:
Surgical operation to correct an inguinal hernia, which is the protrusion of an organ through the internal inguinal orifice (an area of weakness in the abdominal wall). The protruding organ may be a loop of intestine, omental fat (in boys and girls), or ovary and fallopian tube (in girls).

When is this operation required?:
Inguinal hernia (reducible or irreducible) and hydrocele in childhood both of which commonly manifest as groin swellings. For inguinal hernia, it’s recommended that surgery is performed before complications like obstruction or strangulation occurs. These complications may potentially damage the blood supply of the bowel and testis with adverse consequences. Elective inguinal herniotomies are usually indicated for hydroceles in boys of age more than a year-old.

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How is it performed?:
This is usually performed as an elective day surgery procedure under general anaesthesia. A skin crease inguinal incision is made on the side of the swelling. The hernia sac (a patent tract that communicates with the abdomen) is identified in the inguinal canal and sewn (ligated) with absorbable sutures at its origin. Postoperative recovery is usually rapid with early resumption of activities.

ORCHIDOPEXY

What is it?:
Surgical operation to correct cryptorchidism (hidden testis; undescended testis, maldescended testis, ectopic testis)

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When is this operation required?:
This operation is required when a child is diagnosed with cryptorchidism, that is, the testis’ position is too high and suboptimal for normal development. The operation is recommended to be performed as early as the first year of life (preferably 6 to 12 months of age) as evidence supports the need for an ideal environment for development of sperm-producing cells. 

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How is it performed?:
The management strategy depends on whether the testis is palpable before surgery. For palpable testis, conventional orchidopexy is indicated.  This is usually performed as an elective day surgery procedure under general anaesthesia. A skin crease inguinal incision is made on the side of cryptorchidism. The testis, vas deferens, testicular blood vessels are identified. An adjacent patent processus vaginalis is usually identified and ligated. Adequate mobilization of the vas deferens, testicular blood vessels would enable the testis to be relocated into the scrotum. The testis is then fixed to the scrotum with an anchoring stitch.  Postoperative recovery is usually rapid with early resumption of activities. For non-palpable testis, after confirmation with an ultrasonographic scan, diagnostic laparoscopy may be used to locate the testis in the abdomen. Appropriate measures will be advised based on the findings at laparoscopy.

Resources

Childhood Cancers

NEUROBLASTOMA

What is neuroblastoma?
One of the common childhood cancers (in the group of malignant solid tumors). It arises from neural crest elements and may be found in the neck, chest, abdomen or pelvis, in particular, involves the adrenal glands and the sympathetic nervous system.

Is it serious disease?
This is a malignant tumor that is usually aggressive and commonly presents at the advanced stage. Early staged disease tends to be without symptoms and often undiscovered. Most if untreated will lead to life-threatening consequences.

How is neuroblastoma usually detected?
Common symptoms include masses or swellings in the regions involved. Persistent fever, bone pain, swelling and bruise around the eyes and in the head are signs of disease spread. Initial consultation with one’s family doctor or paediatrician when symptoms arise is highly recommended before referral to the relevant specialists (paediatric oncologist or paediatric surgical oncologist). Further tests usually include imaging scans (like ultrasonography, CT scan, MRI) and bone marrow tests.

How is neuroblastoma treated?
Patients diagnosed with neuroblastoma undergo a set of evaluations to be assigned a risk-group and the necessary treatment protocol. Various combinations of surgery, chemotherapy, peripheral stem cell transplantation, radiotherapy and MIBG therapy may be recommended. Most neuroblastomas respond to chemotherapy. When it is not feasible to perform initial surgery to remove the tumor, chemotherapy is used as the first treatment instead. To date, many treatment protocols have been tested with published safety and outcome features.

What is spontaneous regression in neuroblastoma?
This is a unique characteristic in a particular subgroup. Neuroblastoma is well-known of its ability to undergo spontaneous regression (disappears without treatment). This is most often observed in young infants (below 18 months of age) with stages 1, 2 or 4S, and without MYCN amplification. This is a special group of patients where non-treatment with regular surveillance may be chosen. Strict selection criteria has to be applied.

Why is neuroblastoma surgery unique?
Surgery to remove neuroblastoma is usually very challenging because of its proximity to important critical blood vessels like aorta, inferior vena cava, arteries supplying the kidneys, intestines, stomach, liver etc. These blood vessels are often encased or trapped within the tumor. Complete surgical resection may risk injury to these blood vessels, leading to end-organ damage. Improved surgical techniques coupled with better understanding of the biological characteristics of neuroblastoma, complete tumor removal can be achieved safely. Good surgery is believed to be beneficial to these patients’ survival.

What are the factors used to predict the patient’s outcome?
Age at diagnosis of less than 18 months, low stages, favourable histology and absence of NMYC amplification are associated with good outcome.

Will my child’s siblings develop neuroblastoma as well?
The probability of inherited neuroblastoma is extremely low. Antenatal ultrasound scan may sometimes be able to detect a neuroblastoma in the unborn child. You are advised to discuss your concerns with your oncologist.   

WILMS TUMOR (NEPHROBLASTOMA)

What is Wilms tumor?
Wilms tumor is the most common childhood malignant tumor of the kidney. Another name is nephroblastoma.

Is this a serious condition?
This is a malignant tumor. Treatment would usually imply the loss of the involved kidney. Treatment results have been very good with many long term survivors. If the disease is not controlled, tumor relapse and death may result.

How is it detected?
Patients commonly present with a large abdominal mass, abdominal pain and/or blood in the urine. As the kidney is a deep structure, patients who present with abdominal mass usually have large tumors. Further evaluations would include ultrasonography, CT scan or MRI. 

How is it treated?
These tumors are routinely treated with surgery and chemotherapy. Patients with advanced stage disease and presence of anaplasia (aggressive cells found at histopathology) require more treatment, that is, more chemotherapy and radiotherapy. The decision of whether to perform surgery first followed by chemotherapy or in the reverse order would depend on the patient’s disease status at presentation.  

Can the child have a good quality of life with only one kidney?
Treatment of Wilms tumor often leaves the child with only one kidney at the end of his successful treatment. This is due to the need to remove the tumor-laden kidney as part of the treatment regime. The remaining kidney should be able to compensate for all necessary daily functions. A normal lifestyle is recommended for these children.  

Which patients are associated with a higher chance of success at treatment?
Patients with Stage I and II disease in the absence of anaplasia have the best chance for cure. Other patients without these features are experiencing improved cure rates with new treatment protocols.

For patients with bilateral Wilms tumors, how is treatment possible with preservation of kidney function?
Wilms tumor may occur in both kidneys. Recent studies have shown that bilateral kidney-sparing Wilms tumor resection after chemotherapy obviated the need for renal transplantation and provided good quality post-treatment function. Specialized surgical techniques enabled removal of tumors whilst preserving normal kidney structure. Such treatment should be performed in specialized centres. 

Is it possible to remove the tumor without removing the entire kidney for unilateral Wilms tumor?
With the experience from bilateral Wilms tumor, kidney-sparing tumor resection in unilateral Wilms tumor may seem feasible. This option is still controversial and should not be taken easily. Such a decision should be carefully discussed with your paediatric surgeon. The disadvantages are many and include the need for preoperative chemotherapy, the possibility of upstaging the disease at surgery and the increase in amount of postoperative treatment.

Will my child’s siblings develop Wilms tumor?
Majority of Wilms tumors are sporadic and only minority are inherited. It is unlikely to be diagnosed before birth.

HEPATOBLASTOMA

What is hepatoblastoma?
It is the most common malignant liver tumor in childhood.

Is this a serious condition?
This is a malignant tumor. Treatment in most cases would usually imply the loss of a proportion of liver with some requiring total liver resection and liver transplantation. Treatment results have been very good with many long term survivors.

How is it detected?
Patients commonly present with a large abdominal mass or abdominal pain. Hepatoblastoma usually produces alpha-fetoprotein which is identified in a simple blood test. Serum alpha-fetoprotein is a reliable tumor marker for diagnosis and follow-up assessment. Further evaluations would include ultrasonography, CT scan or MRI that help in the assessment of tumor resectability. Image-guided needle biopsy may be required to determine the tumor’s histopathology.

How is it treated?
These tumors are routinely treated with a combination of surgery and chemotherapy. A complete surgical removal of the liver tumor with a rim of normal liver cells gives the best outcome. Preservation of sufficient normal liver would ensure adequate liver function. Patients with unresectable disease may receive preoperative chemotherapy. When the tumor has responded, surgical resection may proceed. Selected patients with multifocal liver tumors may consider liver transplantation after preoperative chemotherapy.  

Will the child live a normal life with a smaller liver after successful treatment?
Most certainly. One of the key considerations besides removing a complete tumor is to leave the child with sufficient liver for him to function normally.

Is hepatoblastoma an inherited disease?
Most hepatoblastomas are sporadic and not inherited. However, hepatoblastoma has been found to be present in families with Familial Adenomatous Polyposis (FAP) syndrome.