I need your professional opinion. I have a little daughter (13 years old), with a lot of medical problems. She has Hydrocephalus(fourth entrapped ventricle-slit ventricle syndrome), Cerebral Palsy, spastic diplegia, etc. We went to our Neurosurgeon for e check up since she was having a lot of headaches(3 years until now). He said her physical exam was normal and the shunts are ok.(pumping them/without a CT scan). She is double shunted,(programmable valve shunts).His opinion was that headaches caused by her situation of the slit ventricle syndrome and advised to take by her a little dose of acetazolamide(Diamox). If it’s not work he will change the program of the shunts (both shunts). As he said and I have read, when the patient with slit ventricle syndrome lying down he feels better. That doesn’t work in my daughter’s headaches(even she is sleeping with her hand in the front place of her head). When she have a sneeze, or a suddenly movement she touch the front place of her head. Is it really the problem now, the slit ventricle syndrome, or is there a new problem with the fourth entrapped ventricle? Is any relationship between her situation and her oral hydration?(her hydration is about 1350-1500 ml daily winter or summer/her weight is 22kg). Is it possible her headaches caused by the other medical problems?(cerebral palsy-they are not seizures-,osteoarthritis, bad vision, bad and longtime position on the wheelchair).
Thank you for your time.
“Worried about My Daughter’s Headache”,
Dear “Worried About My Daughter’s Headache”,
I hate to hear about anyone who is suffering and in pain, especially when it is a child. It sounds like you have invested a lot of time and energy taking care of her and it must be very frustrating knowing that she’s in pain and not having a answer. Unfortunately children with Hydrocephalus and Slit Ventricle Syndrome tend to experience headaches. As with most chronic medical conditions, children experience symptoms differently. Children with Slit Ventricle Syndrome can present with variations in the type of headaches. Yes there are some that find relief with change of positioning, but others may experience a more cyclical pattern.(1) In general the common symptoms found in Slit Ventricle Syndrome include headache, vomiting and drowsiness.
Just to make things more confusing, these same symptoms occur when there is a shunt malfunction. Shunt malfunction may occur because of chronic or acute inflammation, accumulation of cellular debris or blood, or blockage of one of the ends of the shunt.(2) A child’s growth may also affect the functioning of the shunt. Unfortunately the need for shunt revision occurs at some point in almost all children treated for Hydrocephalus from infancy (2) Therefore, whenever a child experiences symptoms it is important to make sure the shunt is functioning properly.
Many times the functioning of a shunt is checked by measuring the pressure in the skull through Intracranial Pressure ICP monitoring. (1) As you already know the functioning of a shunt is also checked through imaging studies such as a Scan of the head. You mentioned that your Neurosurgeon checked the shunts by pumping them, said they were okay and reported that your daughter's physical examination was normal. These are all good signs. It would be important to follow up with your Doctor if your daughter’s symptoms persist or worsen, in case he may want to order further studies such as a Cat Scan or MRI.
Your daughter’s complaints of pain at night and with movement or activity are typically considered warning signs for a problem that originates in the brain. Headache that awaken a child or present when the child first awakes in the morning are classic symptoms of increased intracranial pressure. (3) In particular pain in the occipital area (back of head) and the inability of the patient to describe the quality of the head pain are associated with problems inside the skull such as a shunt malfunction.(3)
Your daughter's Neurosurgeon knows her medical history, findings from her physical examination and results of tests done so far. He is in the best position to advise you regarding the most beneficial course of treatment. It seems that your child’s Neurosurgeon has pin pointed Ventricular Slit Syndrome as the cause of your child’s headaches. Accepting a chronic condition as the reason for a longstanding painful symptom in your child is very difficult and normally results in a lot of questioning and doubt. It is also very common to want to rule out other causes of a headache since headaches can be caused by many factors. This is a very thorough and reasonable approach, especially when it comes to the health of your child.
Whenever a parent has a chronically ill child, so much time is spent at specialist’s office that sometimes the value of the primary care physician or Pediatrician is overlooked. It is through your daughter’s Pediatrician that other causes for a headache can be investigated. This is not only a very essential part of her overall care, but it will reassure you all areas were investigated. Headaches in children have many causes and an evaluation by your daughter’s Pediatrician can guide you in the right direction.
Cervical spine abnormalities can present with musculoskeletal headaches and also with headaches compatible with migraines.(4) You mentioned that your daughter has Cerebral Palsy, Osteoarthritis and is wheelchair bound. All three of these conditions may cause or contribute to an abnormality in her cervical spine which could lead to chronic headaches. This may be an area that you want to investigate.
Chronic Sinusitis is another condition that is commonly associated with headaches. (4) Your pediatrician will be able to ascertain if your daughter’s symptoms are caused s by chronic Sinusitis. Sometimes a child can have a chronic headache and Sinusitis at the same time. If this occurs, the Sinus infection can make the chronic headaches worse and more difficult to control. Once a Sinus infection is treated, the chronic headaches are much easier to treat. (4)
Another consideration is Temporomandibular Joint problems or TMJ which can also present with a chronic daily headache. TMJ is often associated with clicking or popping and problems moving the jaw. Other dental conditions such as malocclusion or an uneven bite, chronic gum infections and abscesses can lead to chronic headaches.(4) An evaluation by a dentist can identify and address these issues if necessary.
You mentioned that your daughter has bad vision. Bad vision is usually not the cause of the type of chronic headaches that you are describing. But your daughter’s health condition can affect her vision. Eye problems are often found when a child is initially diagnosed with Hydrocephalus, as well as in children with functioning shunts and controlled Hydrocephalus. (2) The increased intracranial pressure that children develop with Hydrocephalus can causes changes in their eye examination. Because of this yearly visits with a Pediatric Ophthalmologist are typically recommended.(2)
Your child’s evaluation at the Pediatrician’s Office can rule out any other possible causes headaches that may apply to your daughter. Many times a head ache evaluation includes blood work and a headache diary. The Headache diary is a record that is taken over the span of 1 to 2 weeks and includes information such as the intensity of pain on a scale of 1 to 10, the location of the pain, other associated symptoms such as vomiting or visual disturbances, the time of day that the pain occurs, the duration of the pain, any association between the pain and activities, its relation to food and the time of the month. I’m not sure if your daughter is able to eat food or if the fluids she is taking is a nutritional supplement. It would be important to report any changes in her dietary intake and use of medications and herbal supplements. In some cases certain foods or herbal remedies cause headaches.(5)
I wish you and your daughter a speedy resolution to this problem. If she continues to have pain you should bring her back to the Neurosurgeon’s office in order to discuss other treatment options. In some cases an adjustment in the medication or the addition of a new type of medication is all that is needed.
For more information about Split Ventricle Syndrome; log on to:
The International Federation for Spina Bifida and Hydrocephalus.
(1)The International Federation for Spina Bifida and Hydrocephalus. Split Ventricle Syndrome. Available at: http://www.ifglobal.org/hydrocephalus.asp?lang=1&main=7&sub=1#Slit_Ventricle_Syndrome. Accessed September 2006.
(2)Jackson P, Vessey J. Primary Care of the Child with a Chronic Condition. St. Louis Missouri: Mosby –Yearbook, Inc. 1992:304-305.
(3)Rubin D, Suecoff S, Knupp K. Headaches in Children. Pediatric Annals. 2006;35(5)345-354.
(4)Linder S .Understanding the Comprehensive Pediatric Headache Examination. Pediatric Annals. 2005;34(6):442-446.
(5)Lewis D, Yonker M, Winner P, Sowell M. The Treatment of Pediatric Migraine. 2005;34(6):449-460.
Lisa-ann Kelly R.N., P.N.P.,C.
Certified Pediatric Nurse Practitioner
Pediatric Advice About Sick Kids