Five year follow-up with Neuropsychologist

On 31 August 2011 we headed along to the Plastic & Maxillofacial Surgery Unit at the Royal Children's Hospital in Melbourne to meet with the Neuropsychologist to whom we had been referred by the Neurosurgeon at Connor's routine five year old check up.  We were basically meeting her prior to meeting the Plastic Surgeon so we would know if there were any developmental delays indicating pressure on the brain.  This would of course add to any possible cosmetic issues such as future facial distortion that might result from the lump on Connor's head.

The appointment was a two hour epic.  We met at 10am and spent about twenty minutes giving some background to the Neuropsychologist on Connor's medical history, personal background and general behaviour and development from a parent's point of view.  She then commenced twenty minutes of tests followed by a break for twenty minutes then a further thirty or forty minutes of additional tests.  

The tests were called the Wechsler Preschool & Primary Scale of Intelligence - Third Edition; NEPSY Developmental Neuropsychological Assessment.  They ranged from verbal tests such as naming pictures, giving definitions for words or describing items, to arranging patterns with blocks or picking patterns in pictures.  The report which she forwarded me later covered Test Behaviours, Intellectual Abilities, Information Processing and Visuomotor Speed, Attention and Executive Functions, New Learning and Memory.

Overall her assessment was that Connor is fine although he was a little impulsive and distractible with subtle difficulty with regulating his responses on testing.  To me it seemed he was just shy performing for a stranger but I guess these might be the subtle issues that are interesting to a neuropsychologist.  She mentioned that we should keep an eye on his school progress and if any decline is noted in behaviour or academic progress we should bring him back for reassessment in case pressure on his brain becomes an issue, I guess.  Apparently children with a history of cranial abnormality are at risk of experiencing mild developmental delays.  So one appointment down, one more to go...

Five year follow-up with Neurosurgeon

Connor is a beautiful looking four and a half year old, progressing like any other child his age.  I had however, been worried about a kind of half egg shaped lump on the top of his head.  Unless I rub his head I cannot notice it.  He has thick hair so any small head shape imprefections probably wouldn't be very obvious anyway.  I did remember though that Dr Jeffrey Wisoff (http://www.med.nyu.edu/biosketch/jhw1), our fabulous Pediatric Neurosurgeon at NYU had mentioned at our one year surgery follow-up appointment that ordinarily, should we remain in the US, we would have a five year follow up appointment with him.  This would be the last appointment required.  It would usually be just a formality to ensure the head was shaping correctly in the growing child.  As we were moving back to Australia, we asked if he could recommend a Neurosurgeon in Australia, knowing that he had recently travelled to a worldwide conference.  He recommended Charlie Teo.  At the time the name meant nothing, but now that I am back in Australia, I know Charlie Teo as a bit of a media personality, probably the reason Dr Wisoff recalled his name as a recommendation also.

In any case, after Connor turned four I mentioned to our family doctor that I would like a referral to a local Pediatric Neurosurgeon in Melbourne, where we are now living in Australia.  He referred me to the Royal Children's Hospital, which has an excellent reputation, particularly in the area of craniofacial work.  The recent separation of conjoined twins from Bangladesh had brought quite a bit of attention (http://www.theage.com.au/national/beginning-life-apart-20091118-ikzz.html).  I had also read an article in the paper about work they had done with children with Scaphacephaly, so I was quite looking forward to the appointment and their view of Connor's New York operation.

I was really thinking of the appointment as a formality.  A final sign-off.  I was quite taken aback when, on meeting Dr Patrick Lo (http://www.laccon.com/about.html), the Pediatric Nurosurgeon to whom I was referred, he seemed concerned about the shaping of Connor's head.  He said that although, as a Neurosurgeon, he can say that the surgery looks fine, it is not the type that they perform at the Royal Children's, as they prefer to insert springs to allow for brain growth.  He said that we really needed a referral to the Craniofacial unit to see a Plastic Surgeon.  Although Connor looks fine now, it is important to confirm that the lump on his head and general skull shaping will not have any detrimental effect on skull or facial shape in future as Connor grows.

Needless to say, I was a little concerned and my husband was very worried about the possibility of the prospect of new surgery.  I have since received my referral to the Plastic Surgeon for an assessent on 9 September 2011.  In the meantime a Pediatric Neuropsychologist from the Royal Children's Hospital called me up to determine if we should make an appointment with her prior to the Plastic Surgeon appointment.  This appointment would be to conduct a developmental assessment to ensure that the head shaping issue is not also causing an kind of brain pressure affecting development.  This would also, of course influence any further surgery decisions - being in addition to cosmetic concerns.  This appointment will be on 31 August.  She sounded interesting as she works with children with craniosynostosis and had a few developmental traits that she said were common.  Language, concentration and self-regulation of moods were some areas she said she had noticed. 

I figure the Neuropsychologist is an interesting thing to do anyway.  No harm really.  But I am not looking forward to the Plastic Surgeon.  Oh well, here we go again.

Speech progress

I thought it might be relevant to include an update on any developmental issues we had encountered with Connor.  I have no idea if this is related to Connor's scaphacephaly and any possible pressure on the brain.  But nonetheless, I mention it in case it resonates with anyone else reading who knows a cranio child.

At 3 years and 10 months, I decided to take Connor to a speech pathologist recommended by our local Maternal Health nurse, for the purpose of having his progress assessed prior to his commencing pre-school the following year.  At that stage Connor's speech definitely lagged behind his peers at playgroup.  I could understand him but people outside the family had difficulty.  The speech pathologist was kind enough to send me an email report summarising her opinion following our visit.  I have pasted it in below, together with her six month follow up opinion. 

Speech Pathologists opinion - First visit - 8 December, 2010 - age 3 years, 10 mths:

Thankyou for bringing Connor to speech pathology today for assessment.

I assessed Connor for speech articulation but we also discussed some other areas of his development.

1. Speech Articulation

Connor is presenting with a mild delay in phonological development. It is not clear whether the tongue tie is contributing to this delay but is possibly mildly affecting 'l' at this time. Errors noted were:

deleting consonants at the end of words (eg. bus to buh)
deleting consonants in the middle of words ( eg. window to wi - oh)
deleting the weak syllable (eg. flower to flow, matches to mat)
substituting 'w' or 'y' for 'l' (age appropriate but consistent)

I did feel that Connor is not yet attending well enough socially to benefit fully from therapy for these errors and it is still possible that some self correction may occur. Connor was able to produce an approximation of 'l' which is encouraging as far as future progress is concerned.

We agreed that I will review Connor in 6 months.

2. Social development

Connor presented with mild delays in this area and this was evident early in the session. He was hypersensitive to my approach and showed limited eye contact throughout the session. He was also less responsive to my direct questions and praise feedback than would typically be expected. However, he appeared to enjoy the task, he know many of the words and did complete the testing with help. He was generally compliant which was great! You also mentioned that Connor has some history of hesitations or sensitivity in his social peer group.

I would recommend that we discuss this area again when you return in 6 months. At that time he will have had an opportunity to show how he is doing within his age peer group. His teacher may also be able to make observations about this area as she will have extended observation opportunities. I would recommend that you consider an OT assessment at any time if you feel that his sensory processing is making things more difficult than they need to be and I can direct you to my preferred colleagues if you wish.

Please contact me if you have any questions or feedback following our consultation today. I look forward to seeing you again in 6 months and for the time being, I have place Connor on my waiting list for therapy.

Speech Pathologists opinion - Follow-up visit - 3 June, 2011 - age 4 years 4 months
 
Thankyou for bringing Connor back for review today. He has shown rapid improvement over the last 6 months in his speech clarity and falls within the average range now. He has corrected his pattern of dropping syllables and end sounds from words and has all of the sounds present (except L) which we would expect for his age. He replaces L with W or Y which is very common in this age group. This will probably self correct but if still present in 12 months, you are welcome to bring him back for another review.


I was pleased to hear that Connor is settling well socially at kinder. He certainly listened well to me during the session and responded more rapidly than when I saw him late last year. Of course if you or his teacher have any more concerns about his social use of language you can get back in touch anytime.

One year follow-up with the Neurosurgeon

Today we had Connor's one year follow up with Dr Wisoff, the excellent Neurosurgeon who performed Connor's Craniosynostosis operation. He asked if Connor was meeting his normal development milestones for a 15 month old and we reported that he is. Then he measured Connor's head which was 48cm and therefore is in the normal range - 50th percentile. As usual, which is something I love, he gave us a copy of his report to our pediatrician. To paraphrase (and translate the medical terms in brackets) his letter said:
The extensive craniectomy has completely reossified (his skull has grown back where the panel was cut out on top), there is resolution of the scaphocephaly (correction of an abnomally long narow skull) with a normal anterior-posterior diameter (head length if measured front to back), resolution of the frontal bossing (his forehead does not bulge anymore) and normalisation of the bitemporal (temple to temple), biparietal (top and sides of the skull from one side to the other) and bioccipital (back of the head) width. He also said the facies was unremarkable (meaning there was no unusual appearance or expression of the face) and that there was no new craniofacial (skull and face) or neurological (brain) abnormality. He said Connor has had an excellent functional and cosmetic outcome following his sagittal craniosynostosis correction and he did not believe there was any need for further neurosurgical follow-up.
I mentioned to Dr Wisoff about the fact that he landed on his head after being dropped when he was around seven months and he said nicely that, well,these things happen with kids and it probably won't be the last time he bumps his head. I also let him know about the seizure last week. He said that it most likely would be an impact seizure related to falling over and hitting his cheek on the magazine basket. I let him know we are seeing the neurologist next week also.
So overall a good report as we expected for Connor. Hard to imagine that this time last year we were huddled in the NYU Pediatric ICU and I was about the spend a terrible sleepless night by his bed. Now he is a beautiful and totally normal looking little boy. Of course he was always beautiful to us anyway.

Seizure Episode

We had a bit of a fright this week. This whole episode I think is completely unrelated to Connor's Craniosynostosis, but as it is head related I thought I would write about it.
Last Sunday we had a lovely day wandering around Park Slope and playing with Connor in the Old Stone House playground. Afterwards we walked down the street to grab some dinner. When we sat down to eat, Connor started getting upset. I thought maybe he was just fidgety and hungry so I tried to breastfeed him, but he wasn't interested. I decided to take him to the bathroom to change him. It was quite hot in there and he started crying and was not happy at all. He had dungarees on so I took off his shirt, washed his face and then took him back into the restaurant.
He was very hot and got very upset and then all of a sudden a giant rush of vomit came out of him and all over me. Two gushes and it was over. Naturally he was still upset. I took him to the bathroom to clean him and myself up and change his clothes, then walked outside with him in the cooler air. He eventually calmed down and cooled down. Consequently we decided it was time to go home. A pretty stinky subway ride home for me, covered in vomit.
Little did we know but this was the start of a virus I guess that the whole family picked up. A 48 hour stomach bug with vomiting and diarrhoea. Not nice - especially when one of us was supposed to be on babysitting duty. Connor was in better spirits than all of us despite the diarrhoea. Suddenly Monday at 5pm he started getting a little upset again. I think maybe he had a slight fever but I don't think it was anything too bad. Everything happened so quickly so I can't say for sure. He was crying a little and tripped over a cushion in our lounge room and hit his cheek on a wicker basket we keep magazines in. He now has a bruise there so I guess he hit it pretty hard but at the time it just seemed like another tumble for a new walker.
Unfortunately, it quickly progressed to something more concerning. He gave himself a shock and other times when something like this has happened he might start crying and maybe even hold his breath, which is worrying. This time I don't think he held his breath but he did pretty quickly stiffen up and start having what I guess was a seizure. His arms and legs rhythmically tensed and untensed and his eyes rolled up and he started gasping rhythmically. We lay him out on the floor and Michael put a small plastic spoon between Connor's teeth (although we now know that officially we should roll him on his side and not put anything in his mouth). After about two minutes I called 911. The seizure went on for seven minutes and ended just as the ambulance guys arrived at the door. He did a small vomit and then went limp in my arms.
We went in the ambulance to Jersey City Medical Center. They took the details of my description in the ambulance but did not take any of Connor's stats - temperature, pulse etc. When we got to the hospital we were taken into the Pediatric Emergency room but were then told by the nurse it wasn't an emergency. So we were taken to the general ER waiting room. And we waited. Connor had livened up and got a little upset at being in the waiting room for so long. I breastfed him and he fell asleep for half an hour or so. An hour and a half later no one had spoken to us. Michael went up to the desk to see what was happening and shortly they called us so a nurse could take Connor's stats. His pulse I think was 105/ 99 and temperature rectally was in the 98/99 region I think. Certainly not a fever by then. After I repeated the story of his seizure they sent us back to the waiting room to be called to be admitted. When nothing further seemed to be happening we decided to give up and go home and put Connor to bed rather than wait there any longer as it was already around 7.30pm.
The next day we were all feeling healthier and we took Connor to see his pediatrician, Dr Parikh at Riverside Pediatrics in Jersey City. She really is an excellent doctor with a lovely manner. She took all the details from us and checked Connor out and said she felt it was some kind of seizure and she would like us to take Connor to see a Pediatric Neurologist and have an EEG (Electroencephalogram - http://www.webmd.com/epilepsy/electroencephalogram-eeg-21508). Since it didn't seem that he had a fever at the time, she thought it was necessary to try to figure out the reason for the seizure.
When Connor was diagnosed with Scaphacephaly we had taken him to Mount Sinai Hospital in New York to see Dr Aron, a well respected pediatric neurologist. This was mostly because I hadn't appreciated the difference between a neurologist and neurosurgeon and when researching the best doctors in New York I had come up with his name. He checked Connor out at the time and told us he was fine, brain-wise and actually referred us to Dr Wisoff who did Connor's operation. He was a very nice doctor, so we decided it made sense to go back to see him. So far we have an appointment on the 29th of May. But for now we just have to wait to see him and find out the cause of the seizure.
I haven't done much research yet other than a quick read of what was said about seizures in "What to Expect The Toddler Years". But so far the only things that seem relevant are that he had the 48 hour stomach bug, had a possible fever, has had an operation on his skull and when he was around seven months he accidentally was dropped and landed onto the back of his head but didn't have a concussion and his CT scans were fine. Nevertheless we will take the CT scan from that hospital visit to show Dr Aron as well as his original CT scans from prior to his operation.
Connor at least is back to his old happy healthy self. For a few days he was quieter than usual and was very wary of any scenario where he might fall over - such as walking on the bathroom floor in case it was wet or walking around obstacles. He seems to have gotten over that now.

The First Diagnosis, Xray and Specialist Song and Dance

Two months passed and we had become pretty used to Connors looks and hadn't noticed any worsening of the squished head issue. My friend came to stay for a visit and asked if his head shape was normal. She hadn't seen many babies, I said it was apparently normal for new babies and forgot about it. The next week however, when I visited the pediatrician for Connor's two month checkup, his first words were "what's happened to his head?". After a brief worried inspection he left us alone in the room to wonder while he went off, I guess to look up something. He came back with a scrap of paper he gave me that said Plagiocephaly (an incorrect diagnosis as it turned out). He gave us a brief description of what he thought was going on, which at the time was very concerning. We knew nothing about the condition and anything that might include surgery as an option when discussing a baby is bad in my books.

He sent us to an x-ray place in West New York to have some skull x-rays done (unnecessary according to our eventual specialist who could tell from look and feel). After a day driving to and from doctors and clinics, Connor was not entirely pleased at being held in place for an x-ray. We took the x-rays straight back to the pediatrician who explained that it looked like the sagittal suture of the skull that runs from front to back down the top of the skull, was prematurely fused. Normally it should still be open to allow for the first year of brain growth and initially for the molding of the skull needed to pass through the birth canal. He told us that he had some good news as he had a colleague who could pass the x-rays to a pediatric neurosurgeon in the area that could help us and hopefully avoid the queues for specialists in New York. Sounded helpful.

A strange week passed. I tried to track own this elusive colleague who had been given the x-rays. At the same time I had been calling the pediatric neurosurgeon at his office at the University of Medicine and Dentistry in Newark, New Jersey to make an appointment. He was booked up for weeks and his secretary, of course had not heard of us. The mysterious colleague had apparently done nothing with the x-rays. I eventually got in contact with the colleague, whose name I now forget and arranged to pick up the x-rays at the concierge at his apartment in Union, New Jersey. I was actually a little nervous they would be lost and I'd have to have Connor go through the x-ray process again. The secretary for the pediatric neurosurgeon softened after hearing from me too often and found us an appointment the next week.

This pediatric neurosurgeon was named Dr Jeffrey Catrambone (http://www.umdnj.edu/cgi-bin/cgiwrap/quinnaj/76a9-cdb5.cgi?Last_Name=Catrambone&First_Name=Jeffrey) and he made us wait for three hours for our appointment. His secretary kindly bothered to tell us that he had wandered off for lunch at some point and that we could do the same and she would call us when he decided to grace us with his presence. We took her up on the offer to leave the depressing, large and busy pediatric neurosurgery waiting room. More odd shaped heads and brain damaged children than I had wished to see at one time. A sad place. I particularly remember a distraught mother and her child with a very odd shaped head. She was yelling at the receptionist that someone had to make an appointment for her because her baby needed help and she had no insurance and she wasn't leaving until she saw someone.

When she finally called us to tell us to return for our appointment, we still waited another hour. He was one of the more arrogant people I have ever met. He stood throughout the appointment, which was in a tiny room with a bed and a sink - not a doctors office or treatment room - more like the kind of room where the nurse would take blood. He informed us that his approach was to work in concert with a plastic surgeon, Dr Samuel Rhee (http://www.theuniversityhospital.com/plastics/html/physicians/rhee.htm). They would perform a craniofacial reconstruction at nine months of age. As the head would have distorted through the brain growing and pushing the fused skull out of shape, the plastic surgeon would also assist with the complete reconstruction. It sounded epic and he sent us off to obtain cat scans to return to him for a second appointment and discussion of the process.

I'm still pretty angry at the late and missed diagnosis and the initial palaver of specialist referral that followed. Since corrective surgery isn't done until after three months usually, perhaps we were saved from weeks of worry by not knowing. But still.

The New Baby in Town

At 6.15am on 8 February 2007 in Hoboken University Medical Center, New Jersey, USA after many hours of labour (of which ten hours were following being induced with pitocin) and an emergency caesarean (due to full dialation but failure to progress), the beautiful Connor arrived weighing 9lbs 9oz and 21 inches long. He had a thick head of dark brown hair. He was of course gorgeous to us. One of my first questions to his pediatrician, Dr Manuel Hugo, in the hospital was however, about his squishy head. It's normal, I was told. All babies look a bit bashed up to start with. Seemed reasonable.

As the weeks passed, he became even more lovely but I still thought perhaps his forehead was a little large or his hairline a little receded and the back of his head a little pointy. At his first pediatrician appointment in Union, New Jersey, amongst my list of questions for Dr Hugo and pretty standard concerns (he sneezes a lot and seems congested; he's unsettled and burpy between 6pm and 11pm; is he eating too much?) was one about the bump on his head - was it OK? Once again I was told it would round out with time.