Our son Nate was born in March 2013 with a large lump on his skull above his left eye and no palpable fontanelle. Within hours of his birth, following an X-ray and MRI, we had a preliminary diagnosis of craniosynostosis and a referral to a specialist at the Children’s Hospital of Philadelphia, which is about an hour from our home in New Jersey. At nine days old Nate’s craniofacial plastic surgeon examined him and diagnosed multisuture craniosynostosis, which was confirmed with a subsequent CT scan.

The CT scan showed closure of the right coronal and sagittal sutures, which caused the lump over Nate’s left eye and the elongated shape of his skull. We were immediately referred to a geneticist, since most cases of multisuture cranio are related to genetic syndromes such as Pfeiffer, Crouzon or Apert syndromes. All of the genetic testing came back negative, which was a relief but made Nate’s case all the more unusual.

The original plan was to wait until Nate was around 6 months old to cut open the sutures and expand his skull using either springs or distractors, but at six weeks old his doctor saw evidence of increased intracranial pressure and recommended that we proceed with a strip craniectomy and installation of cranial distractors.

On June 3, 2013, at the age of nine weeks old, Nate had his first surgery. In fact, his first haircut was done by the team prepping him for surgery. They gave us his hair in a biohazard bag as a keepsake. The neurosurgeon cut open his sutures and the craniofacial plastic surgeon placed two distractors—one to expand the sagittal suture and one to expand the right coronal suture. The surgery was long and the wait was agonizing, but it was a success. Nate spent two nights in the ICU and one more night on the surgical floor before being discharged.

At home we had to turn the “arms” of the distractors twice a day to achieve more expansion of Nate’s skull. As the doctor explained to us before surgery, distractors work like mini car jacks. As you turn more space is created between the opposing skull plates. At first Nate didn’t seem bothered when we turned them, but as the weeks went by he seemed to experience some discomfort.

About a month after surgery we returned to CHOP to have the “arms” of the distractors removed, since we no longer needed to turn them. The surgeon was happy with the expansion achieved by the distractor placed at the sagittal suture, but the one placed at the right coronal suture malfunctioned and didn’t expand that suture quite as much as he had hoped.

In mid-August Nate had his second surgery, which was to remove the distractors completely. The surgery was uneventful and we spent just one night in the hospital. The only thing we didn’t expect was returning home with a drain placed under Nate’s skin to drain any excess fluid caused by the removal of the distractors. Trying to keep a 5-month-old baby from tugging on a tube protruding from his scalp was a bit of a challenge.

In September Nate was fitted for a helmet. Though it was assumed that Nate would need a second surgery at some point in the next year or two to correct the shape of his skull cosmetically, his doctor thought it was worth trying a helmet to see if enough cosmetic correction could be achieved without surgery. Nate wore the helmet for about 6 months before it was concluded that the helmet wouldn’t be sufficient, and he’d need surgery anyway. We planned for cranial vault reconstruction (CVR) surgery in November 2014, when Nate would be about 20 months old.

One of the mixed blessings of the severity of Nate’s multisuture cranio is that it was so evident at birth, and therefore it was diagnosed and treated early before it hampered his development. In fact, Nate had quite advanced language skills by September 2014, when he was 17 months old. It was in early September that he came to us a few times, held his hand to head and whimpered that it “hurt.” The first time I didn’t think much of it, but when it happened again his doctor told us to bring him in. He had been seeing the ophthalmologist regularly, since it was already identified that Nate had trouble with his vision, and it was his ophthalmologist who saw signs of papilledema—swelling of the optic nerve due to increased intracranial pressure.

Nate was scheduled for surgery the very next day and was admitted to CHOP immediately for pre-op testing. My husband stayed with him in Philadelphia and I drove home to pack some things and arrange care for our daughter, who was almost 4 at the time. I returned to CHOP very early the next morning to await Nate’s surgery.

Nate’s third surgery was even longer than the first—about 9 hours from start to finish. His surgeons found that all of his sutures had prematurely fused once again, not just the sagittal and right coronal, which his surgeon termed “progressive pansynostosis.” In cutting apart and reconstructing his skull they tried to create as much space as possible for his brain to grow, and also tried to address the cosmetic issues.

Despite the urgent and unplanned nature of this third surgery, everything in the hospital went smoothly, though it was a different experience with a 17-month-old as compared with a 9-week-old. After his first surgery Nate slept nearly the whole time we were in the hospital. At 17-months he still slept for most of the first 24 hours following surgery, but after that he was much more alert and aware. By the time we moved out of the ICU to the surgical floor he was feeling well enough that he wanted to walk around and visit the playroom, so my husband or I trailed after him holding the drain that was attached to his scalp. Nate was discharged after 5 days in the hospital and within a week we went apple picking and he attended a birthday party.

Following Nate’s third surgery the hope was that it would be his last, but given the unique nature of his case the surgeons couldn’t make any assurances. We continued seeing his craniofacial plastic surgeon and ophthalmologist regularly.

At a routine visit in August 2016, when Nate was almost three-and-a-half, his ophthalmologist once again saw papilledema. A CT confirmed that some of his sutures were again prematurely fused. Nate was scheduled for his fourth surgery on September 8. Fortunately this time we had a couple of weeks notice, so we were able to arrange for my mother to visit and care for our two other children, Nate’s older sister who was almost 6 and his younger brother who had just turned 6 months.

Nate’s fourth surgery was another CVR and it also went well, though it was harder for him being older and more aware. He developed a hatred of medical personnel and would scowl as soon as they walked in the room. He didn’t like having his blood pressure taken or taking medications, so he would lie and say he wasn’t in pain, though we could always tell when he was in pain by the change in his mood. We thought Nate might be disturbed by his shaven head the first time he looked in a mirror after surgery, but he seemed to like it, and said he looked just like one of his friends from daycare.

Nate was discharged after just 3 days in the hospital and insisted on playing soccer in the backyard when we got home.

Nate is now 6 years old and has just finished Kindergarten. Nate’s surgeon says that if Nate can make it a few more years without increased ICP he should be in the clear, though he has suggested that we may want to consider another surgery when Nate is around 12 for additional cosmetic correction.

We see Nate’s surgeon annually and the ophthalmologist 2-3 times per year, both to monitor his vision, since he now wears glasses, but also to check for papilledema. Aside from these visits to CHOP, which we use as an excuse to treat ourselves to cheesesteaks, Nate is a healthy kid who loves all sports, plays chess competitively, and thinks it’s pretty neat that he has a “million dollar head,” as we call it.

P.S. Nate’s medical team published a case study in a medical journal about his first surgery, since it was the first time distraction osteogenesis was used at such a young age to correct nonsyndromic multisuture craniosynostosis. The article is “Simultaneous Unicoronal and Sagittal Distraction Osteogenesis for the Treatment of Nonsyndromic Multisutural Craniosynostosis” and it was published in the Journal of Craniofacial Surgery (Volume 26, Number 1, January 2015).