Difficulties in Preventing Neural Tube Defects in Ethiopia

Ethiopian mother hold her child with lumbar myelomeningocoel

The Mekelle University Multidisciplinary Research Group for Neural Tube Defects has just published its first research paper in Brain & Development Journal July 2018, “Maternal Risk Factors Associated with Neural Tube Defects in the Tigray Region of Ethiopia”. This being the first major prospective study done on neural tube defects in Ethiopia confirmed our worst fears of a very high incidence, significantly higher than the 75 per 10,000 births seen in most of Sub-Saharan Africa. There a many challenges in how this problem can be addressed involving cultural beliefs and practices, poverty, diet diversity, supplementation, and fortification.  Unfortunately, applying a Western style solution for Ethiopia will not be so easy or likely to succeed as well.

For the past decade the development of neurosurgery in Ethiopia has witnessed the high incidence of neural tube defects including myelomeningocoel and anencephaly coming to their clinics first in Addis Ababa but now also in Mekelle, Gondar, Bahir Dar, and Oromia. Previous published reports noted incidences first of greater than 160 per 10,000 births in Addis with a more recent report of 191 per 10,000 births in Addis Ababa. The reports of up 300 per 10,000 in some areas of Tigray are higher than those reported in any other developing countries.

Research replicated in nine countries in the 1960s and 1970s showed that neural tube defects were somewhat but no totally related to lack of folic acid in the diet and that adding folic acid would significantly reduce the incidence of neural tube defects. At first attempts were made with prescribing supplementation for women of childbearing age but this did not have the desired result. The incidence really came down in Europe and the United States when the government mandated the fortification of folic acid in food staples like bread and cereals

One of the factors we identified was that a lack of diet diversity increased the risk for NTDS while increased diversity reduced it. About 65% of the diet of most Ethiopians is enjera bread made at home from teff and boiled chick peas called shiro.   Although a serving of raw chick peas has about 1000 micrograms of folic acid its likely that boiling them reduces the folic acid to basically nothing.   A similar situation exists for spinach which is often boiled and eaten during the rainy season.

Traditionally, Ethiopians avoid eating fresh vegetables and fruits as documented in our study and many previous others. In fact a study of the one hundred most elite Ethiopian runners showed that 20% had a significant folic acid deficiency which correlated with lack of dietary diversity (avoiding greens and fruit). In the countryside where the women may spend many hours a day just to get 5 gallons of water, there is not much water to spare for cleaning produce. The population fears getting diarrheal illness from such produce.

Our study showed that of more than 13,000 women interviewed who were pregnant essentially none of them had any knowledge of preconceptional nutritional needs or about neural tube defects. 

Convincing Ethiopians to take medication for invisible illness such as hypertension has proved difficult. Research in many parts of Ethiopia has shown for example that as few as 50% of those prescribed medication for hypertension actually take it. There exists underlying fears of addiction to “un-natural” substances. Will Ethiopian women be convinced to take supplementation?

Ethiopians especially the 88% who live in country side rarely buy food staples like bread but instead make their own enjera from stored teff. Currently there is only one factory in the country capable of making fortified bread but it is not functioning.

The cost of a months supply of folic acid 4 milligrams per day is about 80 birr or about $2.40 US for a single woman. Given the fact that the average family makes about 250 birr per month to support a family of six, there is little room to allow payment of this expense. There are no current domestic producers of folic acid so considerable hard foreign currency would need to be mobilized to import stock. For the government to provide this for every women of child bearing age would cost tens of millions of dollars to be added to the budget of a country which currently spends the equivalent of about $13 per capita for the 100 million population.

Ultimately addressing the issue of the high incidence of neural tube defects in Ethiopia will be requiring taking into account cultural norms and practices in such a way that changes are seen as consistent with Ethiopian culture.  Widespread public education and a major investment in folic acid purchases by the government will be necessary. This begins with the clear realization that there is a problem.

 

Interpersonal Violence and Head Injury in Tigray:Public Health Issue

The head injury problem especially that due to interpersonal violence in Tigray is a growing and significant problem which requires a public health approach.

3d CT scan of man suffering depressed fracture of skull from stone injury

Research published by Fasika et al showed that 24.8% of head injury admissions came from interpersonal violence from 2011 to 2014 which was before neurosurgery was permanently established at Ayder Comprehensive Specialized Hospital. Currently we are seeing about 10 patients a day and operating on 2 to 3 every day. Most of the surgeries we do are for depressed fractures caused by stone injury received in interpersonal violence. The age range of these injuries ranges from preschool to the eighth decade of life. We know there is a cultural proclivity to this type of injury but it is not well studied.

The hospital burden of head injury includes about 19% of adult ICU admissions and bedspace and 25% of pediatric ICU admissions and bed space. Our average daily census on the adult ward is 5 for head injury and on the pediatric ward also 5. The length of stay can vary from 24 hours to months with most of the surgical patients requiring a least a 5 days stay in the hospital.This burden acts to limit the care those suffering from other maladies can receive.

At the current time there is no public health or government plan to try to prevent these injuries.The World Health Organization has created a Violence Prevention Alliance which sees interpersonal violence as requiring a public health approach.

This public health approach to violence prevention seeks to improve the health and safety of all individuals by addressing underlying risk factors that increase the likelihood that an individual will become a victim or a perpetrator of violence.

The approach consists of four steps:

1 To define the problem through the systematic collection of information about the magnitude, scope, characteristics and consequences of violence.
2 To establish why violence occurs using research to determine the causes and correlates of violence, the factors that increase or decrease the risk for violence, and the factors that could be modified through interventions.
3 To find out what works to prevent violence by designing, implementing and evaluating interventions.
4 To implement effective and promising interventions in a wide range of settings. The effects of these interventions on risk factors and the target outcome should be monitored, and their impact and cost-effectiveness should be evaluated.

 

Diagram of WHO prevention plan

By definition, public health aims to provide the maximum benefit for the largest number of people. Programs for the primary prevention of violence based on the public health approach are designed to expose a broad segment of a population to prevention measures and to reduce and prevent violence at a population-level.

I propose that Mekelle University put together a multidisciplinary team consisting of not only physicians but also social scientists, public health professionals, police and prosecutors ( this was previously discussed with the head of the Tigray police who was interested), and government stakeholders. This type of investigation and policy development is exactly the type of activity which the University with all its resources and knowledge base should be tackling.

Research:Neural Tube Defects in Tigray Ethiopia

Recent research we have done at Mekelle University soon to be published has confirmed that there is a high rate of neural tube defects affecting the brain and spinal cord in Tigray. Experience suggests this is also the case in other parts of Ethiopia as well. At least 131 out of every 10,000 births is affected with some areas having almost twice that number. Defects result in death at birth for about 77% of the pregnancies affected, usually with anencephaly, while the 23% born alive usually have severe paralysis of the lower extremities and often need a operations to close the open spine, closure of myelomeningocoel,  and to control pressure in the brain, ventricular peritoneal shunt, for lumbar and thoracic myelomeningocoel associated with an Arnold Chiari II malformation causing hydrocephalus. Lesser numbers of encephalocoel often requiring closure were encountered as well.

Ethiopian mother hold her child with lumbar myelomeningocoel

The most likely significant cause is lack of diversity in the diet and especially failing to consume foods with the vitamin folic acid. This is usually found in green and leafy vegetables as well as fresh fruits. Cooking foods such as chick peas which may contain folic acid will destroy much of it.

Around the world these birth defects have been reduced about 75% by encouraging women to plan their pregnancy and take 4 milligrams of folic acid daily starting before conception. We are working with our research group at Mekelle University to help the Tigray Regional Health Bureau and the Ethiopian Ministry of Health come up with programs to reduce these defects but this will take time.

In the meantime we encourage all women in Ethiopia to plan their pregnancy, starting folic acid supplementation before they conceive, and practice dietary diversity. They should try to wait one year between pregnancies, and understand that breast feeding increases their need for folic acid. Very young and older women are more susceptible to having children with these defects. Other factors may be involved besides folic acid deficiency but the good news is that folic acid supplementation will likely still reduce these defects.

Alternative Ventricular Peritoneal Shunt Anchoring Method in Pediatric Patients with Chhabra Shunt System

Object: The author reports his experience using the Chhaabra Slit n Spring shunt system SH202 in pediatric patients in Ethiopia using connectors at the entry point of the shunt into the ventricle to anchor the system without anchoring suture. Placing an anchoring suture is often difficult in very young pediatric patients because of a lack of available tissue. This alternative was therefore developed.

Presented at the 21st Meeting of the Surgical Society of Ethiopia at the African Union, Addis Ababa, Ethiopia on February 3, 2017.

Methods: The consecutive results of patients was retrospectively studied from 10/7/2014 to 9/21/2016 in 76 patients. Follow-up ranged from 26 months to 3 months through December 2016. Most the patients were 6 months of age or less (49) while (16) were between 6 to 12 months and (11) were greater than 1 year of age.

The ventricular catheter was connected to a straight connector which was connected to silicone tube which bent at the entry into the brain. The silicon tube proximal end was connected via connector the shunt valve. All connections were secured with 2-0 silk. Otherwise the procedure is as described by B.C. Warf in J Neurosurg (Pediatrics 4) 102:358–362, 2005. Complications were 7% shunt revision , 4% minor wound revision, and 5% shunt infection.

Conclusion:This alternative method of anchoring the shunt without suture compares favorably with other previous reports of the Chaabra shunt system. There were no intraventricular migrations or migrations out of the ventricle as has been reported in the literature. When revision was necessary it was slightly more complicated than with the ventricle single connector setup but still could be accomplished without too much effort. Longer follow-up is necessary to more fully evaluate this alternative.

PDF of slide presentation

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Emergency Percutaneous External Ventricular Drainage for Infants

For the past 18 months we have used a novel technique for emergency percutaneous external ventricular drainage (EPEVD) in young infants. This is taught to pediatric residents and general surgery residents under the direction of the neurosurgical unit at Ayder Comprehensive Specialized Hospital, Mekelle University College of Health Sciences. Acute obstructive or communicating hydrocephalus presenting in young infants with patent anterior fontanels is a common occurrence in teaching hospitals of developing countries. This life saving procedure can allow a rapid decompression of intracranial pressure and the immediate safe acquisition of cerebrospinal fluid analysis for diagnosis.

The following case is an example of the procedure. A 6 month old child was seen by the parents to have progressive head growth and lack of development over several months. Examination showed a tense fontanel, macrocephaly, and developmental delay. A cranial ultrasound showed severe hydrocephalus with viscous intraventricular content consistent with infection.

We suspected tuberculosis of the ventricles and urgent decompression and diagnosis was necessary.

6 month old child with hydrocephalus due to chronic tuberculosis of the lateral ventricles
6 month old child with hydrocephalus due to chronic tuberculosis of the lateral ventricles

With the mother holding the child so no sedation or anesthesia was necessary, the scalp was prepped with betadiene. Then an 18 gauge angiocatheter was placed perpendicular to the mid-pupillary line  in the anterior fontanel.

Perpendicular placement of angiocath into lateral ventricle on mid-pupillary line in lateral anterior fontanel
Perpendicular placement of angiocath into lateral ventricle on mid-pupillary line in lateral anterior fontanel

The needle stylet is removed and the angiocatheter seated to scalp. Several gauze are used to wrap the angiocathether which will then hold it firm when a protective dome is placed.

The drainage system is connected and the catheter wrapped with sterile gauze to prevent movement laterally
The drainage system is connected and the catheter wrapped with sterile gauze to prevent movement laterally

The protective dome made out of the top of common water bottle that has been cleaned with alcohol is slipped over the drainage tube. By securing the catheter to prevent dislodgement or lateral movement the protective dome prevent secondary brain injury and displacement of the catheter.

The protective dome is placed over the catheter wrapped in gauze
The protective dome is placed over the catheter wrapped in gauze

The drainage tube and dome are then secured with tape to the skull. In this case because of the high viscosity of the intraventricular fluid only a small amount of elevation of the drainage tube over the head was used.

Final dressing of ventricular catheter drainage system
Final dressing of ventricular catheter drainage system

This is a useful temporary measure which does not require an operating room or advanced skill of a neurosurgeon to perform.  It does have the disadvantage of not being tunneled but is not intended for long term use. The use of easily available material in the setting of a developing country and teaching it to pediatricians and general surgeons in a country setting where few neurosurgeons are present will allow access of emergency treatment for more infants.

Lumbar Myelomeningocoel Surgery in Northern Ethiopia

This a review of some of the principals I follow when performing myelomeningocoel surgery in young infants in Northern Ethiopia. More than a third of all the neurosurgery operations is made up by this pediatric neurosurgical condition at our hospital.

It is estimated by the World Health Organization that over 300,000 children suffer neural tube defects which results in improper formation of the spinal cord, spine, and brain leading to absence of bowel, bladder, and sexual function control, spinal deformity, reduced mental development, and paralysis. More common in underdeveloped countries then in developed countries, the occurrence has been mostly strongly related to preconception maternal folate deficiency and some genetic factors. Unfortunately in Ethiopia there are thousands of these children born every year, we see several a week in our clinic at Ayder Comprehensive Specialized Hospital at the Mekelle University College of Health Sciences in Northern Ethiopia.

Myelomeningocoel is a disorder where the neural tube which develops in the first month after conception from a flat plate of specialized tissue fails to fully close into a tube. This results in neural elements being exposed directly to the atmosphere or only a thin membranous layer. The lowest nerves below the defect often do not completely form leading to paralysis in the lower extremities and difficulties with the functions normally performed by the sacral nerve roots involving the bladder, rectum, and sexual organs.

Pre-Operative Assessment

Pre-operatively many of these children come to the hospital from long distances in poor nutritional condition. If they weigh less than 3 kilograms we delay surgery until they reach this weight because of problems with temperature control in the operative and immediate post-operative period. Children who have cerebrospinal fluid leaks, chronic bleeding from the neural placode, progressive macrocephaly, symptomatic hydrocephalus, symptomatic Arnold-Chiari symptoms such as weak cry, weak upper extremities or multiple pneumonias from aspiration, or meningitis are given priority. Because of the large number of cases and limited resources there is often a several month waiting period for those in stable condition with well epithelialized neural tube defects.

To undergo surgery they must be fit for general anesthesia with normal hemoglobin, kidney function, cardiovascular status and be free of active infection. Ultrasound evaluations of the head to assess for hydrocephalus and of the abdomen to assess the kidneys is routinely done.

They are kept nothing per oral (NPO) for several hours before surgery and started on pediatric maintenance intravenous fluid containing glucose during this time which is continued in the operating room and after until the child is breast-feeding normally.

We do not routinely prepare blood for the surgery unless the child has a low hemoglobin/hematocrit preoperatively. If so then a pre-operative transfusion will be given.

Surgery

The child is placed under general inhalation anesthesia using isofluorane rather then halothane is the most common anesthetic agent used in Ethiopia. We use a electric heater in the room, warm the saline irrigation used during the case, intravenous fluid heaters, and keep the child covered as much as possible to avoid hypothermia which can occur easily in our mountain environment.

Pre-operative ceftriazone at 50mg/kg is given for prophylaxis and continued for 48 hours post-operatively.  This is given not just to prevent wound infection but also because these children are at risk of developing post operative pneumonia from the co-existing Arnold-Chiari malformation also present.

myelom-closure1

The child is placed in prone position with an intravenous fluid bag under the chest and under the groin which leaves the abdomen free of pressure thus reducing venous bleeding during the surgery. In the picture is seen a child with a large membranous lumbar myelomeningocoel. The large size taking up most of the width of the back and the mostly membranous covering predicts that closure will be difficult.

myelom-closure2

The dissection is done under loupe magnification. The neural elements are dissected free of the epithelium, fat, scar tissue, and fibrous bands. In the picture above the spinal cord with attached neural placode and the lumbar dorsal fascia have been identified.

myelom-closure3

In many cases the lumbar dorsal fascia can be carefully dissected at the lateral extant of the wound and then reflected medially to be closed as a dural layer. In the picture above this “dural” layer is closed first by several interrupted 3-0 vicryl sutures and the oversewn with a running suture.

myelom-closure4

Key to being able to close the wound is mobilizing the subcutaneous fascia superiorly and inferiorly and bilaterally. This must be generously done by careful digital dissection to free up the skin edges. It is generally better to over mobilize then undermobilize to reduce tension on the skin closure. If the a primary midline closure cannot be done then the primary skin fascial incision can be extended to superior and inferior z-plasty incisions which must be longer than the original incision. Alternatively a rhomboid type flap can also be used. It is important in the post-operative period to support the flap by giving supplemental oxygen, maintaining hydration, checking the post-operative hemoglobin/hematocrit, and avoid undo pressure on the flap. Most of the cases can be primarily closed.

Despite the best techniques, about 20 percent of cases have some type of wound problem which can range from mild stitch abscess or dehisicience to severe dehisicience. I always tell the surgical residents that we close the dura thinking that should the flap come apart with a sound dural closure we have a good chance for secondary healing.

myelom-closure5

This was a very large myelomeningocoel that we attempted to close with a modified z-plasty.  Unfortunately a week after surgery there was some necrosis and breakdown. However, with aggressive daily wound care and good nutritional support a good result was obtained by secondary intention healing over a month’s time because of the good dural closure underneath.

Careful monitoring of the head circumference, anterior fontanel, eye movements, mental status, and feeding is necessary in the immediate and later post-operative period. Most of these children will go on to develop progressive hydrocephalus requiring a second operation for insertion of a ventricular peritoneal shunt. A few of them if they have persistent stridor, weak cry, aspiration will need to go undergo an upper cervical laminectomy and decompression of the brainstem for treatment of the Arnold-Chiari malformation.

What Causes Low Back Pain and Neck Pain?

 

spinesegmentweb

Back pain or neck pain can severely limit one’s ability to function in everyday life and often causes great anxiety that a serious threatening condition may have arrived. Fortunately for the vast majority of those afflicted, no surgery will be necessary usually and there is often a good recovery with conservative measures.

The patient and doctor must work together in understanding the mechanism of the pain affecting the patient to effect the best treatment plan.

Traditionally it was once believed that low back pain especially if it was severe was due to pressure on the cauda equina or the spinal cord. However studies over the past twenty years have shown there are pain receptors in the facet joint and it’s capsule, disc, muscles and even bone which can cause back pain. Somatically these are poorly locatized to primitive pain centers in the brain which localize to the back, hip or leg in a poorly defined area similar to radiculopathy which is the classical pain associated with nerve compression. Aging changes to the facet joints, chronic inflammation, infection, tumor, or trauma can lead to this pseudoradiculopathy. Characteristically it is not associated with neurological deficit or straight leg raising sign which are findings suggestive of nerve root compression.

These receptors are stimulated or altered by biochemical factors including cytokines such as matrix metalloproteinases, phospholipase A2, nitric oxide, and tumor necrosis factor-alpha are thought to contribute to the development of low back pain. Inflammatory responses and sensitivity changes to these substances can occur with repetitive motion.

coxinhibitorweb

Cox-2 inhibitors such as nonsteroidal anti-inflammatory drugs have been shown to be more effective than acetopminophen in helping to relieve pain. Additionally neurophysiological mechanisms exist in the midst of stress and/or depression, for example, which can mediate or amplify pain responses.

Nerve root inflammation with and without direct mechanical irritation of the spinal nerve roots in the lumbar spine is thought to occur from the release of substances leaking from the intervertebral disk including phospholipases, leukotrienes, and thromboxane. Chronic bony stenosis causes an reduction in the blood flow to nerves causing inflammatory responses and neural element ischemia. Theoretically corticosteroids can inhibit this inflammatory response, reduce pathological capillary permeability, and decreasce nociceptive input from C-fibers. This has led to widespread use of epidural steroid use for radicular pain in the United States but studies have been mixed as to the results. Poor technique and patient selection was found to contribute to poor outcomes. Although some report this treatment may temporarily aleviate pain allowing the patient to avoid surgery the evidence of long term benefit is lacking.

Recent studies have found that oral administration of gabapentin gave similar results as epidural steroids for nerve root pain or claudication type of pain seen with spinal stenosis.

Since the development of MRI and CT Scan, however, it has been clearly recognized that although most the population will have disc degeneration and facet changes beginning in their thirties and progressing through aging this changes do not always correlate with pain. Thus the medical practioner has to be careful about ascribing pain to these changes of aging and patients should avoid fixating on these findings.

 

Mechanical Spine Pain:

Injury or inflammation in the soft tissues of the body can cause a release of chemical substances which can incite a further inflammatory reaction which propagates itself locally. Injury to the muscles or tendons connected to the bony elements of the spine can trigger this reaction causing severe spasm of the spine and pain. This type of pain often responds to NSAIDs (Non-Steroidal Anti-Inflammatory Drugs) which can reduce this chemical response and hence bring down the pain.
This pains gets worse with weight bearing and movement of the spine. Typically it is limited to the vicinity of the spine and does not radiate to the extremities. Limitation of weight bearing and movement may provide some temporary relief.
Like sprains and strains in other parts of the body, the initial period of immobility should be followed by a gradual stretching and strengthening program to facilitate restoration of function. Most often there is no significant anatomical damage to the spine. Patients can be treated conservatively.

Pseudoradiculopathy:

The spine is a collection of joints supporting the body while at the same time allowing movement. This movement can occur because the the spine is composed of many bones which can move relative to one another in bending, twisting, and extending directions. However, since there are nerves passing between the bones this movement must be safely restricted to prevent compression of the nerves which could cause pain, weakness, numbness or even paralysis.
There are two main structures which control this movement. The facet joints are bony plates attached to the vertebrae which block excessive movement in some directions while allowing more movement in other directions. Situated between the vertebrae is a shock absorber, the intervertebral disc, made of a tough outer layer of fibrous tissue and an inner layer of normally well hydrated matrix of protein and carbohydrate.
Both the facet joints and intervertebral disc, like other joints in the body can generate pain from tiny sensory nerves that detect injury, inflammation, or aging changes. These nerves are branches of the nerves that go to the extremities so that the brain may read a signal from these nerves as coming from the leg in the lumbar spine or the arm in the cervical spine.
Pain in the extremity coming from the facet or disc typically is not as severe as the associated mechanical pain. Thus the neck or low back pain will be worse than the arm or leg pain present. Patients usually report that they cannot localize the pain exactly and it tends not to follow known distributions of nerve sensation called dermatomes. As the nerve itself is functioning normally and just doing its job, clinical examination and testing will not show an abnormality.

degenerativediscweb

This type of pain is often associated with tears of the outer disc or soft tissue injury or inflammation of the facet capsule covering the facet. It tends to be long lasting than mechanical pain and may require more intense rehabilitation such as physical therapy and learning to use one’s body properly. For some the onset of this pain may necessitate some permanent life style changes if they are involved in jobs like heavy weight lifting. Surgery for this type of pain is rarely successful in making patients totally pain free.

Radiculopathy and Myelopathy:

herniateddiscweb

Compression of a nerve going to the arm in the cervical spine or the leg in the lumbar spine can lead to pain radiating from the spine to the extremities. Often this pain will be greater than the pain localized to the spine. Clinical examination of nerve function will often reveal a disturbance in sensation, strength or reflexes. Specific clinic maneuvers such as compression of the head for neck pain or lifting the leg passively for the lumbar spine worsen the pain due to radiculopathy.
The spine has two channels through which the neural elements (nerves) pass. There is a large central canal for the spinal cord carrying all nerves from the brain to the rest of body and in between the vertebrae on each side, a smaller channel called the foramen, for the individual nerves going to the arm in the cervical spine and to the leg in the lumbar spine. The spinal cord ends at the upper level of the lumbar spine so that compression of the spinal cord occurs mostly in the cervical and thoracic regions.
Compression of the spinal cord (myelopathy) generally results in an increase in reflexes while compression of nerve roots causes a loss or decrease in the reflex of the affected nerve root or roots. If the central canal is involved there will usually be symptoms of both sides of the body including weakness, numbness, or difficulty in controlling urination or bowel movement whereas compression in the foramen (radiculopathy) causes one sided and often single nerve root involvement. Combinations of central and foraminal compression also can occur.
The vast majority of adult spine pain patients have degenerative conditions due to aging and repetitive injury from heavy occupations which cause the disks and facet joints to wear out. Rarely these processes may result in nerve compression but usually are limited to causing only mechanical pain or pseudoradiculopathy which do not require surgery.
Following a thorough history and physical examination, which does not elucidate any warning signs or neurological involvement most adult patients can safely be treated conservatively for at least a month before undergoing expensive imaging such as an MRI. However, it should be noted that in regions where tuberculosis is endemic it may be wise to go for an x-ray of the spine and an ESR (Erythrocyte Sedimentation Rate), which together approach a 90 percent effective low cost screening for tuberculosis of the spine.