Successful Surgical Management of Spina Bifida (Lumbosacral Meningomyelocele) with Fluid in Brain(Gross Hydrocephalus) in a 47-Day-Old Infant

A 47-day-old baby boy, Eliezer Abraham from Eritrea, East Africa, was brought to Sarvodaya Hospital, Sector 8, Faridabad, in a critical condition.

He was born with Lumbosacral Meningomyelocele, the most severe form of Spina Bifida, a birth defect in which the spine does not develop properly. As a result, the spinal cord and nerves were protruding through an opening in his lower back, forming a swelling. The swelling had ruptured and was leaking Cerebrospinal Fluid (CSF)—the fluid that surrounds and protects the brain and spinal cord, putting him at a very high risk of a serious brain infection.

The baby also had Gross Hydrocephalus, a condition in which excess fluid builds up inside the brain. This caused his head to become unusually large and increased pressure on the brain.

When he arrived at the hospital, the baby weighed only 2.7 kg and was in a critical condition, requiring urgent medical and surgical care to save his life.

Symptoms the Baby Had

When the baby was brought to the hospital, he showed several signs of a serious neurological condition, including:

• A large, balloon-like swelling on the lower back that had been present since birth and was leaking fluid (Lumbosacral Meningomyelocele)
• An unusually large and rapidly growing head due to excess fluid building up in the brain (hydrocephalus).
• Sunset eyes, where the eyes appeared to look downward because of increased pressure inside the brain.
• Weak, floppy legs with reduced movement due to nerve damage (caused by Spina Bifida).
• Poor control over bowel and bladder functions, leading to leakage of urine and stool.
• Poor feeding and weight loss, due to which the bay had become weak and undernourished.
• Signs of dehydration

A High-Risk Situation

During the baby's brain scans, doctors found signs of ventriculitis, an infection of the fluid-filled spaces inside the brain. The pressure inside the skull had also become dangerously high, causing the brain stem to shift (brain stem herniation). This affected the baby's breathing, leading to rapid and noisy breathing. If left untreated, this could have resulted in aspiration (food or fluid entering the lungs), breathing failure, and could have been life-threatening. 

The surgery at this point had become unsafe, and the priority was therefore to first stabilise the baby. He was admitted to the NICU, where he received intensive supportive care, nutritional optimisation, and one week of intravenous antibiotics to control the infection before any surgical intervention could be safely planned.

A Carefully Planned Two-Stage Surgical Approach

Once the infection had settled and the baby was stable enough for surgery, Dr Shweta K Sharma, Senior Consultant of Paediatric Surgery, along with her team, proceeded with a carefully planned treatment strategy.

The first procedure involved placement of a Ventriculoperitoneal (VP) Shunt (a surgically implanted medical device used to treat hydrocephalus by draining excess cerebrospinal fluid), which diverted the excess fluid from the brain to the abdomen, relieving the dangerously high pressure caused by gross hydrocephalus.

Following successful fluid diversion, the team performed meticulous repair of the ruptured Lumbosacral Meningomyelocele ( the bulge on the lower back), carefully repositioning and protecting the exposed neural tissues and achieving a watertight closure of the spinal defect.

The surgery had two primary objectives:

• Anatomical restoration by repairing the spinal defect and preventing further CSF leakage and infection.
• Hydrocephalus management through VP shunt placement to relieve pressure on the developing brain and preserve neurological function.

It was a complex case as the infection had spread to the brain. Both procedures required exceptional surgical precision and close coordination between the team of multidisciplinary experts.

Recovery

Following surgery, the baby was closely monitored in the NICU. His recovery has been encouraging.

The infection has resolved successfully, the VP shunt is functioning well, and the spinal wound is healing satisfactorily. The baby's weight has improved from 2.7 kg to 3.5 kg, reflecting good nutritional recovery.

He is currently undergoing nutritional rehabilitation and multidisciplinary follow-up, with regular assessments by paediatric neurosurgery, paediatrics, physiotherapy, and rehabilitation specialists to support his long-term neurological development.

Although surgery cannot reverse nerve damage that occurred before birth, timely intervention has successfully controlled the infection, protected the brain, repaired the spinal defect, and given the child the best possible chance for improved long-term outcomes.

Understanding Spina Bifida

Spina Bifida is a birth defect in which a baby's spine does not develop properly during pregnancy. Normally, the bones of the spine surround and protect the spinal cord. In babies with spina bifida, a part of the spine remains open, allowing the spinal cord or nerves to bulge out through the opening.

This appears as a soft, balloon-like swelling on the baby's back, usually in the lower back. Depending on how severe the condition is, it can affect the baby's ability to move the legs and control bladder and bowel functions.

In many babies, Spina Bifida is also associated with Hydrocephalus. In this condition, excess fluid builds up in the brain, causing the head to become unusually large and increasing pressure inside the skull.

What Causes Spina Bifida?

Spina bifida develops very early during pregnancy, usually within the first 3–4 weeks after conception, when the baby's neural tube (the structure that later forms the brain and spinal cord) does not close completely. As a result, part of the spine remains open, allowing the spinal cord and nerves to protrude through the opening.

Several factors increase the risk, including:

• Folic Acid deficiency before and during early pregnancy
• The previous pregnancy was affected by neural tube defects
• Certain medications
• Poor maternal nutrition
• Genetic predisposition
• High fever or excessive heat exposure during early pregnancy

Fortunately, many cases can be prevented with adequate folic acid supplementation before conception and during early pregnancy.

Symptoms of Spina Bifida

Depending on severity, babies may present with:

• Swelling over the back has been present since birth
• Fluid leakage from the swelling
• Weakness or paralysis of the legs
• Abnormal foot deformities
• Enlarged head due to hydrocephalus
• Difficulty passing urine
• Loss of bowel control
• Recurrent urinary infections

If you find any unusual signs and symptoms in the baby, immediately consult with a paediatric surgeon

Lumbosacral Meningomyelocele with Gross Hydrocephalus

Lumbosacral Meningomyelocele with Gross Hydrocephalus is a severe birth defect that affects both the spine and the brain.

Here's what each term means:

• Lumbosacral: Refers to the lower part of the spine, where the lumbar (lower back) and sacral (pelvic) bones meet.
• Meningomyelocele: This is the most severe form of spina bifida, in which the bones of the spine do not close completely during pregnancy, leaving an opening in the back. Through this opening, the protective coverings of the spinal cord (meninges), the spinal cord itself, spinal nerves, and cerebrospinal fluid (CSF) bulge out, forming a soft, sac-like swelling. The exposed nerves can become damaged, leading to weakness or paralysis of the legs and loss of bowel and bladder control.
• Gross Hydrocephalus: Hydrocephalus means there is an excessive build-up of cerebrospinal fluid (CSF) inside the brain. The word "gross" indicates that the fluid accumulation is very severe, causing the head to become abnormally enlarged and increasing pressure inside the skull. If left untreated, this pressure can damage the brain and become life-threatening.

Why is Early Treatment Required?

Lumbosacral Meningomyelocele with Gross Hydrocephalus is a medical emergency. Early treatment is essential to:

• Prevent life-threatening infections such as meningitis caused by leakage of cerebrospinal fluid (CSF).
• Protect the exposed spinal cord and nerves from further damage and infection.
• Relieve pressure on the brain caused by excess fluid (hydrocephalus), preventing permanent brain injury.
• Improve long-term outcomes by preserving neurological function and reducing complications related to movement, bowel, and bladder control.

Although surgery cannot reverse nerve damage that has already occurred before birth, timely intervention can save the baby's life, prevent further deterioration, and significantly improve the quality of life.

Importance of Antenatal Scans and Early Paediatric Surgical Intervention

Most cases of spina bifida can be detected during pregnancy through routine antenatal ultrasound scans, usually between 18 and 22 weeks of gestation. Early diagnosis gives parents time to understand the condition, receive counselling, and plan delivery at a hospital equipped with neonatal intensive care and paediatric surgical services.

After birth, early intervention by a paediatric surgeon is crucial. Prompt surgical treatment helps close the spinal defect, prevent serious infections such as meningitis, protect the exposed spinal cord from further damage, and manage associated conditions, giving the child the best chance at life.

A Message for Parents

Although spina bifida is a challenging condition, it is not without hope.

The good news is that many cases of spina bifida can be prevented. Women planning a pregnancy are advised to take 400 micrograms (0.4 mg) of folic acid daily, starting at least one month before conception and continuing through the first trimester.

For women who have previously had a pregnancy affected by a neural tube defect, doctors may recommend a higher dose of folic acid.

Regular antenatal check-ups and routine ultrasound scans also help detect the condition early, allowing timely planning and treatment. If you notice any unusual signs during pregnancy scans, consult with an expert, a paediatric surgeon, immediately, so that the treatment can be planned long before the baby is born.

Though severe, if the condition is managed on time by experienced paediatric surgeons, children with Spina Bifida and Hydrocephalus can live a normal life; their limbs might be affected due to nerve damage, but their speech and cognitive skills can be improved with proper treatment, therapy and rehabilitation.