Hypoplasia Surgery: Everything You Need to Know

Hypoplasia surgery can include a number of different surgical interventions aimed at rectifying hypoplasia, which is incomplete development of an organ in the body. While hypoplasia is usually congenital and can involve any part of the body, corrective surgery is most frequently used to treat hypoplasia of the heart, blood vessels, lungs, or gastrointestinal (GI) system. Typically, these procedures are done during childhood, and optimal correction might involve more than one separate operation.

What Is Hypoplasia Surgery?

There are many types of hypoplasia surgery, with methods that may include restructuring underdeveloped organs or transferring tissue from another area of the body to the malformed organ.

Hypoplasia is often a feature of congenital syndromes that involve multiple structural defects. For example, congenital heart disease can involve a few anatomical abnormalities, and hypoplasia may be one of several coexisting problems. In these situations, the corrective procedure may involve hypoplasia repair as well as other surgical interventions done at the same time.

Hypoplasia surgery may be minimally invasive or done as an open procedure. This surgery can be done as an outpatient procedure or it may require staying in the hospital for several days. Typically, hypoplasia surgery requires pain control with general anesthesia.

Contraindications

There are several situations in which hypoplasia surgery might not be the right option. If the defect can't be surgically corrected, or if surgery would pose a life-threatening risk due to underlying health issues, then hypoplasia surgery would be contraindicated.

Sometimes the procedure has to be delayed until a target milestone is reached. For example, if a child's anticipated growth would alter the anatomical correction of hypoplasia surgery, the surgery might be postponed until the child has reached a certain size.

Also, an acute illness, like severe infection or respiratory distress, may need to be fully treated before surgery can proceed.

Potential Risks

This surgery involves risks normally associated with general anesthesia and surgery. Additionally, hypoplasia surgery can result in:

• Excessive bleeding
• Inadequate healing
• Organ damage
• Post-operative infection
• Blood clots

Sometimes complications of hypoplasia surgery can cause noticeable symptoms, like swelling or pain. But issues aren't always immediately obvious and might only be detected with postoperative imaging studies or other diagnostic tests.

Purpose of Hypoplasia Surgery

Hypoplasia surgery is done to alleviate the effects of hypoplasia, which can range from negligible to life-threatening. The impact of hypoplasia is determined by which part of the body is underdeveloped and what type of alterations in physical function occur as a result.

Generally, specific parts of an organ, such as the valves of the heart or the blood vessels of the lungs, are more likely to be affected by hyperplasia than other regions.

A few types of hypoplasia that can be surgically treated include:

• Hypoplastic left heart syndrome
• Hypoplastic right heart syndrome
• Hypoplastic coronary artery disease
• Hypoplasia of the carotid artery
• Pulmonary hypoplasia
• Esophageal atresia
• Stomach or intestinal hypoplasia
• Jejunal atresia
• Duodenal atresia
• Omphalocele
• Prune belly syndrome
• Hypoplasia of the liver
• Renal (kidney) hypoplasia
• Turner syndrome
• Ovarian hypoplasia
• Testicular hypoplasia
• Hypoplasia of the bones or muscles

Symptoms of hypoplasia of the heart and lungs can occur during infancy and may include pale, bluish skin and lethargy. Hypoplasia affecting organs of the GI system can cause abdominal distension (enlargement), vomiting, and inadequate growth.

Sometimes hypoplasia of the ovaries or testes might not be detected until late childhood due to the absence of signs of puberty. These issues may lead to infertility if they aren't treated.

Hypoplasia may also cause physical deformities in a person's appearance, such as an uneven appearance of the face or short stature.

How to Prepare

Hypoplasia surgery is a major procedure that requires detailed planning. Ultrasound testing during pregnancy can detect some forms of hypoplasia, and some types of hypoplasia surgery are performed shortly after a baby's birth.

Surgical preparation involves diagnostic tests that assess the structure and function of the affected organ.

An echocardiogram might be used to examine the heart's anatomy prior to surgery. And an ultrasound or computerized tomography (CT) scan might be used to visualize the abdominal organs. Additionally, an evaluation to identify commonly associated anatomical malformations might be carried out as well. This can include imaging tests or blood tests.

As part of pre-anesthesia and presurgical testing, a complete blood count (CBC), blood chemistry tests, and a chest X-ray will be done.

Location

Hypoplasia surgery is done in a hospital operating room or in a surgical center operating room.

What to Wear

Your child can wear anything comfortable for the surgery appointment. They will need to wear a hospital gown during the procedure.

Food and Drink

It is necessary to abstain from food and drink after midnight the night before surgery.

Medications

Medication instructions are specific to the type of surgery being done. Sometimes medication is needed to stabilize the medical condition or to prepare for surgery. Be sure you are clear about what your child can take before surgery, what they can't, and when any medications should be stopped.

What to Bring

Be sure to bring a form of identification, health insurance information, and payment for any portion of the surgery that you are responsible for.

If you are the one having the surgery, bring someone along to drive you home upon discharge.

Pre-Op Lifestyle Changes

Depending on the type of surgery, some dietary or activity restrictions may be needed in the days or weeks before surgery.

What to Expect on the Day of Surgery

When you arrive at to the surgery appointment, you need to register and sign a consent form. If your young child is having surgery, you might be able to go to the pre-operative area with them after that.

Preparation includes checking temperature, pulse, blood pressure, respiration, and oxygen level. An intravenous (IV, in a vein) line is placed in the hand or arm.

The surgeon and anesthesiologist may conduct an examination before surgery. And same-day pre-operative tests like a CBC, blood chemistry tests, and urinalysis might be done. If these tests show an acute issue, like an infection, surgery may need to be postponed until it resolves.

Before the Surgery

Before surgery begins, the skin is prepared and cleaned, and anesthesia is started. A urinary catheter might be placed as well.

The anesthetic medication is injected into the IV line to control pain, paralyze the muscles, and induce sleep. A breathing tube is placed into the throat for mechanical breathing assistance during surgery.

Surgical draping is used to cover the surgical area(s). The specific spots where the incision(s) will be made are left exposed. (There will be more than one if tissue is going to be removed from another area of the body for the repair.)

The skin in those locations is then cleansed again with a disinfectant solution.

During the Surgery

Surgery is started when the surgeon makes an incision into the skin. A deeper incision is then made into the mesothelium, which is a layer of tissue that encloses the organs of the chest, abdomen, and pelvis.

After accessing the organ that's affected by hypoplasia, the surgeon may take a variety of next steps depending on the purpose of the surgery. For example:

• A procedure used to repair hypoplasia of the heart might involve correcting underdeveloped heart valves or blood vessels.
• Procedures for repairing intestinal hypoplasia might involve the creation of connections or opening the lumen of the intestines.
• Surgery for hypoplasia of the kidneys might involve transferring blood vessels to the kidney from elsewhere in the body.

After the underdeveloped organ is operated on, the surgeon closes the mesothelium and the skin, and covers the wound with surgical dressing.

The anesthesia is then stopped and the breathing tube removed. The anesthesia team will ensure that the patient is breathing independently before moving to the post-operative recovery area.

After the Surgery

You might be able to be with your child in the recovery area. They will be groggy and slowly wake up. If they have a urinary catheter, it will be removed. Your child may be able to start eating and drinking. If they are old enough to use the toilet on their own, they will be able to do so with assistance.

Sometimes patients are discharged to go home on the day of hypoplasia surgery. But often, patients need to stay in the hospital for one or more days for post-operative testing. For example, a child who has had intestinal surgery might have abdominal imaging studies, and a child who has had heart surgery might have an echocardiogram.

Upon discharge, you will receive guidance regarding diet, activity, wound care, signs of surgical complications, and other special instructions. You may be given a prescription for medication, such as a painkiller. And you may also receive prescriptions to manage issues related to your surgical organ functioning, such as heart medication or hormone therapy. A follow-up appointment will need to be scheduled with the surgeon.

Recovery

Recovery differs tremendously from one type of hypoplasia surgery to another, and the short-term and long-term recovery process depend on which organ or organs are hypoplastic, the extent of pre-operative hypoplasia, as well as organ function after surgery.

Regardless, this will definitely involve a period of healing, and it might also involve interventions like physical therapy or cardiac or respiratory rehabilitation. You may have instructions for when to start eating and drinking during recovery (and what to consume), as well as what dietary changes should be continued longer term.

Sometimes surgery can result in completely normal organ functioning after recovery is complete; other times, there may be only partial improvement.

You and your child will have to get used to the new way the body is working post-surgery. For instance, learning to eat and use the toilet to have a bowel movement are major adjustments that are necessary when healing from esophageal hypoplasia surgery or duodenal hypoplasia surgery. Once full healing has taken place, long-term diet changes may be required, especially if the gastrointestinal system is not completely normal.

On a more positive note, some changes are more welcome. Eating without pain, running without getting short of breath, and a variety of other outcomes are possible as well. This may open up opportunities that had been avoided before.

Healing

Healing may take several weeks or months. Sometimes pain medication is needed to control pain. Your healthcare provider will recommend an over-the-counter pain medication or prescribe one, and you should use it as directed.

During this time, it is important to keep wounds clean and dry, and to change dressings as directed to promote healing and prevent infection.

Signs of surgical complications include:

• Fever of 100 degrees F or higher
• Severe, unmanageable, and/or long-lasting pain
• Irritability or unusual behavior
• Vomiting
• Shortness of breath
• Pale, or bluish skin
• Lethargy or excessive sleeping

Be sure to discuss any concerning signs with your child's doctor.

Coping With Recovery

As the surgically repaired or restructured tissue heals, your child might need to limit certain activities. Recommendations are based on the type of surgery performed. For example, cardiac surgery might require limited physical strain. Your child's doctor will provide guidance regarding what activities should be avoided and when/if they can be resumed.

Sometimes children may have a brace or bandages during recovery. For young babies, these are positioned to stay put, but toddlers and school-age children may find them annoying and try to push them off or dislodge them when being active.

Keep a watchful eye to make sure that these items are kept in place and that discomfort is minimized as much as possible. Try to explain the need to limit certain activities to your child (as best as you can) and schedule fun activities that won't interfere with healing.
Long-Term Care

Often, congenital problems involve more than just correctable structural issues. Depending on the medical condition, your child might need to have follow-up imaging studies to monitor for new problems that are expected to develop years after hypoplasia surgery.

Your child might have a medical syndrome that requires treatment with prescription medication for several years or for the rest of their life.

And some congenital syndromes that cause hypoplasia might be associated with medical concerns like an immune deficiency that requires special attention as well.

Possible Future Surgeries

Often hypoplasia repair is one step along the path of a series of surgical procedures that will take place over time. Your child may need to have another procedure when certain milestones are met—such as healing after surgery or reaching a certain age or weight.

If your child is old enough to understand, they might have questions about each individual procedure and its objectives. Your child's doctors should be able to explain these things in a way your child can understand.

Lifestyle Adjustments

Over time, healing should improve your child's quality of life. But there may be some limitations due to the underlying medical illness that you and your child will need to adjust to.

A Word From Verywell

Hypoplasia surgery can improve a child's quality of life. And for some congenital conditions, it can be lifesaving. Repair of hypoplasia might involve a multi-step process, with several sequential surgical operations. Since this surgery is usually done during early childhood, parents need to take on the responsibility of postoperative care as kids heal and recover after hypoplasia surgery.