Monthly Archives: July 2012
(Photo courtesy of http://www.ehow.com/how_2335892_manage-preeclampsia.html)
Few months ago, one baby was referred to my service because the mother was having a life-threatening, severe pre-eclampsia (if I’m not mistaken, her BP was >180/100 and can’t be controlled), on the verge of having seizure. Baby had to be delivered prematurely because she was already not faring well. Baby then was born at 28 weeks, 815 grams. She was able to survive and was discharged after nearly 2 months in the hospital.
When baby was 3 months old, she was supposed to have her injectable hormonal contraceptive but she missed it because the obstetrician was out of town at that time she had her post-natal visit. Then when baby was 5 months old, mother found out that she is 1-month pregnant again. She was very confused and alarmed because she cannot forget her traumatic experience with her first baby, when she almost had seizure due to the very high blood pressure.
On her 2nd month of gestation on the 2nd baby, she came and talked to me in a soft whisper. She was asking my opinion on her plan to have abortion. I was shocked. But at one point, I got her point, her fear of possibly dying while being pregnant again, especially that her first-born is just 5 months old. I really could sense her confusion. Do I blame her to be afraid? No. The thing she fears is a reality and not just a make-believe. I told her, personally, I would not advice abortion as it is a crime. I tried to talk to her, told her the chances of severe hypertension related with pregnancy decreases on the next pregnancies.
Gladly, she took my and her obstetrician’s advice not to go on with her plan. Now, she jokingly blames her obstetrician for being absent during that prenatal check-up that’s why she got pregnant too soon. She gave birth via a repeat cesarean section. This time, she was able to do the Unang Yakap and she was so grateful that she was able to do this at least to one of her kids. Immediately, she had ligation – she and her husband decided on this before the delivery.
Can you blame them for deciding to have ligation? My take? It was their choice. They were well aware of what the consequences of pregnancy to her health, their kids, their family. It was a well-informed, intelligent choice!
The ventricles. (Image courtesy of http://library.thinkquest.org/28457/csf.shtml)
The brain normally produces cerebrospinal fluid daily via the choroid plexus (a network of vessels) situated over the ventricular system. There are two lateral ventricles, located on each of the brain hemisphere. The fluid from both lateral ventricles enters the 3rd ventricle, then passes through a narrowing, the aqueduct of Sylvius (a common site of obstruction giving rise to obstructive or non-communicating type of hydrocephalus) into the 4th ventricle. From the 4th ventricle, via three openings (foramina of Monroe and Luschka), the CSF empties into the subarachnoid space that covers the entire brain and spinal cord. From the subarachnoid space, the fluid is reabsorbed into the venous system via the arachnoid granulations. Sometimes the latter may also be obstructed by blood clots or fibrosis, such that CSF accumulation leads to the communicating type or non-obstructive type of hydrocephalus.
Diagram: Cerebrospinal fluid circulation via http://securesignupoffers.net/c/
How devastating is it for parents to find out their newly born child has congenital defect? It must be very traumatic, they must feel very distraught. I cannot entirely speak for them but I do partially understand that it is a very painful reality. I will never be able to quantify the pain that they are experiencing at that very moment seeing their child with abnormality. They must be having mixed emotions and their thoughts are running awry. Feeling depressed, mixed with guilt, questions on what did they do wrong to deserve such an abnormal child, why them of all people, among other else.
Cleft lip and palate are one of the most devastating abnormality a child could have. Not only because it is aesthetically displeasing, but it also brings along with it several medical conditions. A child can have recurrent upper respiratory infection, obviously from the defect. In addition, if the cleft palate also affects the opening of the eustachian tube, a child can also have a frequent ear infection and worse deafness.
(image courtesy of http://plasticsurgery.about.com/od/Cleft-Lip-And-Palate/ss/Goals-And-Timing-Of-Cleft-Palate-Repair_3.htm)
Eustachian tube is a communication that clears and drains fluid and debris from the middle ear to the nasopharynx. In a child it is almost horizontal relative to its opening at the nasopharynx, hence the easier access for infection. It is naturally closed at rest, but the muscular opening at the nasopharynx end is disrupted by cleft. This does not allow the muscle opening to open thus fluid accumulates in the middle ear, followed by infection and eventual hearing loss. The incidence of hearing loss is decreased if repair is done early (before 12 months old).
Another problem that cleft palate imposes is feeding difficulty. For one, there is a contiguity of the oral and nasal cavity. This does not allow the baby to create a sufficient suction pressure needed to extract milk from the breast. If baby swallows, he tends to swallow more air than food, and food may regurgitate through the nasal cavity. This is a risk factor for malnutrition.
Speech is another important aspect that must be considered in children with cleft palate. Between two and three months of age, infants will start making sounds. Between six and ten months, babble such as “dadada” and “bababa “will occur. The prelinguistic babbling of infants is ultimately crucial in the development of normal speech. Most children speak their first words (mama, dada) by 13 months of age. True speech — characterized by the ability to put words together to convey meaning and communicate with others – develops by two years of age.
All of these steps on the pathway to intelligible speech require a normal velopharyngeal mechanism. The velopharyngeal mechanism includes the palate and the back of the throat. During speech, the goal is to have air flow out through the mouth instead of out through the nose for most speech sounds. The exceptions are m, n, and ng. In order for air to be directed out of the mouth and not the nose, the soft palate lifts and moves toward the back of the throat. You can see this motion of the palate when you open your mouth and say “ahhh.” This movement closes off the mouth from the nose. If this movement is impaired due to a cleft in the palate, too much air will escape from the nose during speech and the speech becomes hypernasal. Hypernasal speech is speech that sounds overly nasal in quality as if the person is talking through the nose.
In summary, when there is a cleft in the palate, air escapes out from the nose instead of from the mouth when certain sounds are made. Repair of the cleft palate is necessary for the development of normal speech. Speech therapy may still be required after repair of a cleft palate. This is especially true if a cleft palate is closed later in life, after a person has established compensatory habits.
Timing of Repair
The optimal timing for cleft palate repair is controversial. Repair is considered early when it is done before 12 months of age. Early repair is associated with better speech development and less hearing loss. On the other hand early closure of the palate can impair facial growth. Specifically both nasal growth and palatal growth can be restricted with early repair. Delayed repair is associated with better facial growth, but at the cost of better speech and hearing outcomes.
In order to glean the advantages of both early and late repair while minimizing the disadvantages, a two-stage repair has been proposed. A two-stage repair involves closure of the soft palate between three to six months of age and closure of the hard palate between 15 to 18 months. With this approach, the part of the palate necessary for normal speech development is intact, but without the growth restriction of closing the hard palate.
I have a newly born baby who presents with cleft lip and palate. At the same time, the baby is small for gestational age (the weight is below 10th %ile rank for gestational age). At present, my dilemma is how to feed the baby. There is no pediatric orthodontist around whom I could refer the baby for denture. (When I was still a fellow, we had a similar case. We referred this to a pediatric orthodontist who created an artificial palate that we put on the baby every time he feeds. This creates a barrier between oropharynx and nasopharynx thus milk do not regurgitate into the nasal cavity. While it may not be able to create the same suction pressure as a normal child does, it still enables the baby to suckle well).
There is no available special nipple, specifically designed for cleft palate babies, locally. Sure, I could try breastfeeding but as stated above, the baby might not be able to create enough suction pressure to extract milk from his mother’s breast. This may bring about malnutrition and remember the baby is already small for gestational age. This will present a big risk indeed for baby to be malnourished. Babies who are small for gestational age need their weight to be corrected before the reach 6 months old so as to avoid their risk of acquiring metabolic syndrome later during adult life. Meanwhile, I have inserted an orogastric tube but I will have to let baby suckle before going home soon.
I have an interesting case that I’d like to share. Maybe someone as well would be able to help me identify any syndrome appropriate to describe it.
One time, the mother was standing on one foot on a chair, while the other foot was over a stone wall. Suddenly she slipped, forcing her to fall to the ground, buttocks first. She did not consult nor informed anyone about this matter.
(Image courtesy of http://www.epubbud.com/read.php?g=XWL9CAZ3&p=1)
When the mother was at the labor room, on a dorsal lithotomy position, suddenly a sharp severe pain struck her right hip. When the obstetrician asked what was the matter, that was the only time she mentioned about slipping from a chair hitting the ground with her buttocks first. Because of this, she was instead scheduled for emergency cesarean section as she cannot bear down to deliver her baby.
When the baby was born, and when he was stimulated to cry, one thing struck me instantly. His cry was very different, very very peculiar. It was in a staccato. Later, I also noted baby to have jerky movements. This then told me that something was wrong with the baby. I worked up the baby considering the revelation of the mother at the labor room. First, I took serum electrolytes including magnesium – all were within normal values, so it means that the jerky and seizure-like movements were not because of electrolyte imbalance.
Next thing, we did an electroencephalogram studies (EEG) to see if there are epileptiform discharges from the brain that’s causing those abnormal, seizure-like movements. Lo and behold, an epileptiform discharges were being emitted by the right occipital lobe of the brain.
Then, to be able to document if this seizure is because of organic in origin (structural defect), I requested for a CT scan to check for presence of bleeding, tumor or any other brain structural abnormality. What’s your guess? Consider the history that happened to the mother before she gave birth. Yes, there was a dense mass at the brain that suggests a bleeding, an epidural hematoma. Naturally, you would think that the epidural hematoma would be located at the right occipital lobe as well, since that’s where the seizure discharges come from, right? Surprisingly, it isn’t. The epidural hematoma is located at the left fronto-temporal area, an area on the opposite side of the other half of the brain.
Left epidural hematoma pointed by 2 small white arrows (not the actual case). Image courtesy of http://www.urmc.rochester.edu/smd/Rad/neurocases/Neurocase17.htm
This suggests a contrecoup brain injury that happened during the mother’s fall. It should be remembered that uterus will always protect the baby from external harm, trauma included. I was surprised of this finding in our patient. The patient was co-managed with a neurologist and when discharged he was maintained on anti-convulsant.
As months went by, and with serial follow-up with the neurologist, a sad development happened. He was diagnosed to be cortically blind. This means that the eyes can capture light (the eyes can see) and send it to the brain for interpretation. Unfortunately, because of the injury back then during pregnancy, the brain cannot interpret the lightwaves that the eyes see. Also, he was noted to be aphasic. He can not talk in words, but follows some commands by actions. His posture is that of tilting his head towards back and look at the source of the sound. Yes, this is something unfortunate event indeed. For good, he is very dependent on someone for his daily needs.
Not the actual patient. This is just to illustrate the child’s posture. (Photo courtesy of www.cpfamilynetwork.org/patient-care/how-i-got-medicaid-to-pay-for-disposable-diapers)