patient jennyAt just 15 years old, Jenny Gonzales has a profound understanding of conquering life’s adversities. She has endured 17 medical procedures including two open-heart surgeries. In July 2023, she faced another formidable obstacle: an 11-hour surgery to correct a severe curvature in her spine.

Jenny has DiGeorge syndrome, a rare genetic disorder caused by the deletion of a small piece of chromosome 22. It can lead to a range of health issues including heart defects, immune system problems, low levels of calcium in the blood, and developmental delays.

“One of the challenges that comes with it is weakened bones, which put Jenny at an increased risk for scoliosis,” said Stephanie, Jenny’s mom. “I was prepared for the possibility my daughter might develop scoliosis as she grew older, so it wasn’t a total surprise when I received the diagnosis.”

Our scoliosis journey

When Jenny was around 6 years old, she began experiencing discomfort. She would wake up in the middle of the night with severe hip and leg pain, and sometimes her legs would go numb. She’d have frequent back pain, forcing her to sit down to find some relief. During her annual physical exam, Michelle Barratt, MD, adolescent medicine pediatrician affiliated with Children’s Memorial Hermann Hospital, noticed a slight curve in Jenny’s spine when she bent down. She referred Jenny to Shiraz Younas, MD, a pediatric orthopedic and scoliosis surgeon affiliated with Children’s Memorial Hermann Hospital.

“Not all children diagnosed with DiGeorge syndrome develop scoliosis,” explained Dr. Younas. “Patients like Jenny fall into the category of ‘syndromic scoliosis,’ where their spinal curvature is related to a broader medical condition or syndrome. This type of scoliosis behaves differently from idiopathic curves and does not align with the characteristics of a typical neuromuscular curves.”

For patients with syndromic scoliosis, the treatment often requires a multidisciplinary approach because it involves addressing both the spinal curvature as well as the underlying syndrome or condition. The specific treatment and prognosis for individuals with syndromic scoliosis can vary widely depending on the underlying syndrome and the severity of the spinal curvature. Since Jenny’s scoliosis reached a severe 70-degree curve in adolescence, the only alternative was surgical treatment.

“Dr. Younas reassured us about the surgery and what to expect,” Stephanie explained. “Despite my initial nervousness due to Jenny’s underlying health issues, the team diligently prepared for her surgery. They conducted extensive blood work to ensure she had all the necessary vitamins and nutrients to keep her bones strong and prevent bone deterioration during the rod placement.”

A multidisciplinary approach to care

The scoliosis treatment at Children’s Memorial Hermann Hospital offers non-operative and operative management of spine conditions in children of all ages, including scoliosis and other spinal conditions. The team’s multidisciplinary approach uses orthotists (to make spine braces), radiologists, physical therapists, rehabilitation specialists, and even neurosurgeons to provide specialized care through the many phases of treatment. Dedicated care coordinators provide patients and their families with information about what to expect before and after surgery, and instructions on post-operative care. This collaborative approach not only improves the overall quality of care but also enhances patient safety, outcomes and the patient’s experience throughout their scoliosis treatment journey.

Preparing for surgery

The affiliated orthopedic surgical team followed a meticulous pre-surgery preparation process. The team participated in a collaborative discussion to address the merits and concerns about this upcoming case. Every aspect of Jenny’s surgery was thoroughly examined to ensure no detail was left unturned. This involved a detailed review of her X-rays and her underlying medical conditions, ensuring the surgery’s indications were precise, anesthesia levels were accurately determined, and any cardiac or pulmonary issues were addressed. Jenny was also given calcium and Vitamin D supplementation to ensure her bones was strong enough to handle the surgery. Given the complexity of Jenny’s case, the team dedicated three months to prepare for her surgery to determine the optimal course of action.

The orthopedic surgeons took the lead on the surgical team. They assessed Jenny’s condition and planned the delicate surgical procedure ensuring the utmost precision and expertise. Working in tandem with them were the radiologists who carefully reviewed and interpreted Jenny’s X-rays and imaging scans, providing essential insights into the extent and nature of the scoliosis curve. This helped the surgical team plan the procedure effectively while ensuring no intraspinal problems were overlooked. Surgical techs and nurses ensured all equipment and instruments were in order. Dr. Younas and Shahnawaz Dodwad, MD, an orthopedic surgeon affiliated with Children’s Memorial Hermann Hospital, scrubbed into the case to ensure the surgery was done efficiently and safely.

Since Jenny had a history of congenital heart disease, she required specialized care. Cardiac anesthesiologists ensured her heart was well-supported during the surgical procedure and they monitored cardiac function throughout the surgery. Additionally, scoliosis anesthesiologists, who specialize in administering anesthesia tailored to scoliosis surgery, used techniques that allowed Jenny to remain asleep while still monitoring brain signals to the limbs.

Rehabilitation specialists also participated in the pre-surgery collaborative discussion, preparing for their role in post-surgery mobility and walking assistance.

“We customize our treatment and care options with an unwavering commitment to patient safety and optimal outcomes,” said Dr. Younas. “Our services are built upon the principles of safety, patient empowerment and a united, multidisciplinary approach – all defining features of our pediatric spine care services. By harnessing advanced technologies, like low-dose radiation imaging and 3D navigation, we can precisely refine our care, further enhancing the safety of surgical procedures. These principles help distinguish our hospital as a trusted source for doctors seeking to refer their patients in need of spinal treatment.”

Surgery day

On July 19, 2023, Jenny underwent spinal fusion surgery at Children’s Memorial Hermann Hospital.

Since Jenny’s 70-degree spinal curvature extended into her lumbar region, the surgical process involved exposing Jennifer’s spine from the upper thoracic area to the pelvis. To enhance precision, Dr. Younas and his team utilized a specialized table at Children’s Memorial Hermann Hospital that is connected to a cutting-edge imaging device capable of generating a 3D representation of Jenny’s spinal structure. This real-time technology enabled the orthopedic surgeons to navigate instruments with a precision-enhancing navigation system, ensuring accurate screw placement by relying on minute reference points on the spine.

“We strategically inserted screws at various levels of the spine,” said Dr. Younas. “They acted as anchors that allowed us to gradually pull the spine towards rods that are contoured in the normal shape of the spine to correct the deformity. Neuromonitoring ensured Jenny’s signals from the brain to her limbs remained stable during the procedure. Once we got to the desired position, we conducted another imaging assessment while still in the operating room to verify the correct positioning of all the components. Finally, we introduced bone graft material and then completed the closure of the surgical site.”

Surgeries like this one have traditionally relied on fluoroscopy, X-rays and two-dimensional imaging. However, Dr. Younas says the introduction of advanced navigational technology has revolutionized the field.

“Today we can perform surgeries with far greater precision, thanks to the incorporation of three-dimensional imaging in the OR, all while minimizing radiation exposure. Our state-of-the-art navigation systems leverage this 3D imagery to help us visualize the surgical site and assist us in the precise placement of surgical instruments. As a result, we can confidently perform surgical procedures while maintaining a heightened level of control and oversight for the patient.”

Jenny spent six days recovering from her spine surgery before she was ready to go home.

patient jenny“The surgery went really well,” said Stephanie. “Dr. Younas showed me the X-ray to demonstrate how they straightened Jenny’s back. When Jenny woke up, tears of joy filled her eyes, however she was afraid to move around and had discomfort that she wasn’t used to of course. Jenny’s Child Life specialists brought in facility dog Dexter and he made all the difference. He was her motivation to get up out of bed and move around. He was her hero.”

Jenny is thriving

It has been several months since Jenny’s scoliosis surgery, and she is doing exceptionally well. She’s regained her independence and is virtually pain-free and back to her usual self.

“I was so happy to see how much taller I got," said Jenny. "I feel stronger and more confident everyday as I continue to heal, and I look forward to learning tap dance as soon as I can. I am very grateful to Dr. Younas and the surgical team and thankful to Dexter because I couldn’t have done it without him,” said Jenny.

“Providing education to both the patient and their families about all aspects of the surgery improves their engagement and post-surgery care,” said Dr. Younas. “This, in turn, increases the likelihood of a successful recovery and a positive post-operative experience. Jenny serves as a shining example of a patient who emerged from surgery with a smile. Her prognosis looks good.”

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