In 2011, the World Bank estimated the population of Sub-Saharan Africa (SSA) to be 874,841,049, with 44% of the population being 1–14 years old (http://www.tradingeconomics.com/sub-saharan-africa/population-total-wb-data.html). Millions of children in SSA have hydrocephalus, spina bifida, and other common pediatric neurosurgical conditions.
Kijabe, Kenya, is a village of approximately 5000 people located 65 km northwest of Nairobi, at an altitude of 7220 feet within the Rift Valley Escarpment. It is home to Kijabe Hospital and was founded at that site by missionaries in 1915, because its elevation was too high for malaria-bearing mosquitoes. The hospital is a 283-bed general hospital under the auspices of the Africa Inland Church. Its consultant staff consists of approximately 20 Kenyan physicians and 20 expatriate medical missionary physicians.
Dr. Dick Bransford, a career missionary general surgeon, initiated pediatric neurosurgery (PNS) in Kijabe in 1997, when he began treating children with hydrocephalus and spina bifida. The number of those children increased steadily, and in 2001, Dr. Bransford inserted 119 ventriculoperitoneal shunts and closed 61 myelomeningoceles (MMCs). In 2002, he sent an email to me with the subject “Neurosurgeon Needed,” requesting help in the management of such children. I began going to Kijabe for 2–3 weeks most Januaries thereafter to teach and operate. My wife, Susan Ferson, a pediatric nurse practitioner, and I moved to Kijabe on September 1, 2010, to teach and to perform PNS. We ended our work there in January 2015. In this manuscript, I offer my reflections on developing PNS in Kenya and in SSA in general.
Pediatric Neurosurgical Education in Kijabe
There are no fellowship-trained pediatric neurosurgeons on the University of Nairobi faculty, as is true of almost all other African neurosurgery faculty. Approximately 12 neurosurgery residents from Nairobi came to Kijabe for their PNS rotations, which were structured like resident rotations in the US. Our intent was to teach them how to evaluate patients, to identify therapeutic options, to read scans, to operate, to do clinical research, and to publish manuscripts—basically, how to be neurosurgeons. Eighteen US neurosurgery residents (from 12 American residencies) and two US PNS fellows came for rotations of 2–4 weeks. Residents were usually in the postgraduate Year 5 or 6, so that they could help with the workload as well as learn. They evaluated and helped operate on many children with disorders they would rarely see in their home institutions. For example, approximately 250 children with MMCs were operated on in 2011 and a similar number in each subsequent year, and several children with frontonasal encephaloceles were treated every year. Teaching of fellows and residents was augmented by 14 pediatric neurosurgeons who visited for 2–4 weeks from the US and Canada.
When we moved to Kijabe, my main teaching hope was to train pediatric neurosurgeons. The Medtronic Foundation provided grant support for 3 years for PNS fellowships. The first fellow was Ugandan neurosurgeon Dr. Humphrey Okechi, who had just completed his neurosurgical residency in China and who began his fellowship in November 2010. His fellowship was similar to those offered in the US, with daily rounds, evaluation of outpatients, daily operations, presentation of a paper at the International Society of Pediatric Neurosurgery meeting, and publication of manuscripts. During the year, Dr. Okechi did or assisted on some 750 cases. He was given written examinations every 4 months, based on the second edition of the textbook Principles and Practice of Pediatric Neurosurgery,3 and he authored or coauthored 7 manuscripts. After working with me for 2 years, he became a consultant on the Kijabe Hospital staff and my colleague. Accreditation for the fellowship was requested from the University of Nairobi—such certificates are highly valued in Africa—but was not received. Accreditation of the fellowship from the College of Surgeons of East, Central and South Africa (COSECSA) may be possible in the future.
It was a principle of mine to not offer the fellowship to applicants I had never met or to those whose visits to Kijabe indicated they would probably not become reasonably good pediatric neurosurgeons. The second fellowship was discontinued after a few months because it seemed that goal would not be met. The third fellowship was completed under the tutelage of Dr. Okechi in August 2015.
Research
It was possible to do both retrospective and prospective clinical research in Kijabe, particularly research into disorders such as MMCs that are far less common in developed countries. My wife, Susan, kept a prospective database on every patient, which provided information for multiple publications.1,5,8,9 The main impediment to clinical research was the difficulty in obtaining follow-up. Although mobile clinics staffed by nurses and therapists for patient follow-up were held at 15 sites across Kenya, many patients never returned to clinic and many of their cell phone numbers became nonfunctional.
In January 2013, we began a randomized clinical trial, comparing infection rates and shunt complications of Codman Bactiseal shunts and Chhabra shunts. The study was interrupted in June 2014 after 100 patients were enrolled. Although every patient was given a date to return for postoperative follow-up, 13 patients were lost to followup; they either came only once or never returned and their cell phone numbers were either disconnected or incorrect. It seems that such research can only be done successfully if study coordinators are hired to obtain data from home-visit follow-ups.
Equipment and Supplies
Because the mission of Kijabe Hospital is to provide good quality medical care for poor people, its budget for neurosurgical supplies and equipment was zero. If the hospital had to pay for supplies and equipment, its charges would necessarily increase to levels that poor people could not pay. Thus, nearly all our neurosurgical equipment and supplies were donated, used, from colleagues and hospitals in the US and Canada. The hallmark of used equipment is that it breaks. When breaks occurred, repairs were rarely available in Kenya and had to be done—if they were done—in the US. Our only new equipment was a Storz endoscope and monitor purchased with a grant from the Medtronic Foundation, and a Myriad tissue resector donated by the NICO Corporation. Supplies needed on a daily basis, such as Ioban, cottonoids, Dermabond, and microscope drapes, were brought every 2–3 months by short-term visiting pediatric neurosurgeons or neurosurgery residents. Kijabe Hospital, like many if not most hospitals in SSA, had no CT scanner while we were there. A single-slice scanner was donated in 2013, but it broke after 4 months and was not repaired because of cost. (CT and MR scans are available in Nairobi, but at prices most patients cannot afford, approximately $75 for a CT scan and $200 for an MR image.) A CT scanner was procured within a few months after we left Kijabe.
As the work began, I sought to obtain several higher-tech instruments that are routinely used in the States, rather than the instruments that would be available to Kenyan neurosurgeons after their training—instruments such as a brace-and-bit, Gigli saw, and rongeurs. It took approximately 1.5 years to get an adequate, donated, used operating microscope, a functional used Midas Rex drill, and a new endoscope for endoscopic third ventriculostomies (ETVs). Whether such equipment will remain available and functioning in Kijabe in coming years is unknown.
Aspects of Clinical Care in Kijabe
It was helpful to have practiced PNS for many years before moving to Kenya. I used to tell residents in the States that even after doing PNS for many years, I continued to see a child once a month with a condition I had not seen before. In Kenya, that occurred more than once a week. Children presented for care late, partly because their families could not pay for transportation to the hospital, partly because they were taken first to the practitioners of traditional medicine, and partly because they were kept for days or weeks in hospitals that received daily reimbursements from the government for the hospitalization even though no neurosurgical treatment was given.
When we arrived in Kijabe, the average daily PNS census was 15–20 patients. During our 4 years there, the daily census increased to 25–30 patients and on a few occasions, 40 patients. We did an average of 5 operations per day, 100 per month. Although Dr. Okechi and I each operated only 4 days a week, that level of operations, approximately 5000 in the first 4 years, turned out to be unsustainable, from both a personal standpoint and from a financial standpoint. Near the end of our service, we limited the census to 20 patients and developed guidelines about cases that would not be admitted or operated on because of poor prognoses.
Treatment decisions about many patients were difficult, particularly the treatment of hydranencephaly, which some readers would consider to be unethical. Treatment was offered primarily to improve the quality of life for the family, so that they did not have to take care of an infant with a 60-cm, 10-lb head that needed to be manually turned every few hours to prevent scalp necrosis, ulceration, infection, and draining pus. In my 32-year practice in the US, I encountered fewer than 20 cases of hydranencephaly. In the 4 years in Kijabe, we saw 54 cases. They were treated with ventriculoperitoneal shunts (n = 11), endoscopic choroid plexus coagulation (n = 18), choroid plexus resections (n = 15), and “comfort care” with medications (n = 10). Outcomes of the different treatments will be evaluated in the coming year, but decisions at the time were made on an individual basis that took into account factors such as parent preference about treatment, costs, and the functional status of the endoscope, monitor, and Bugbee wire.
We admitted approximately 2 children with hydrocephalus every day. The most common cause of hydrocephalus was MMC and the second was postinfectious. Bacterial meningitis is common in Kenya and is poorly treated. A child with fever, irritability, and poor feeding who is taken to the local governmental health facility is either considered to have malaria and treated with antimalarial medication—without testing for malaria—or given a 3- to 5-day course of antibiotics, usually an oral cephalosporin. Many of those children have meningitis/ventriculitis, but lumbar punctures are rarely done. The children present 1–2 months later with increasing head size, and head ultrasounds often demonstrate multiloculated hydrocephalus (Fig. 1). Thousands of cases of hydrocephalus could be prevented if bacterial meningitis were accurately diagnosed and appropriately treated. Yet, one caveat is that when lumbar punctures are performed and a CSF sample is sent for culture, results are usually negative because specimens are handled improperly. Improper handling includes specimens being delayed in going to the laboratory and then sitting for hours in the sunlight before being inoculated onto agar plates.
Scans showing multicystic postinfectious hydrocephalus.
We were able to determine appropriate treatment in perhaps 90% of hydrocephalus cases of any origin using ultrasound images, but individuals with complex multiloculated hydrocephalus were usually evaluated further with a CT scan in Nairobi. Most children with hydrocephalus who were less than 6 months old, particularly those younger than 3 months, were treated with a ventriculoperitoneal shunt, usually a Surgiwear Chhabra shunt donated by the International Federation for Spina Bifida and Hydrocephalus. Some 5%–10% of those shunts drained less CSF than desired, and approximately 3% of them were associated with perforation of the gastrointestinal tract or abdominal wall.9 Postoperative shunt complications occurred in 20%–25% of cases, including infections in approximately 15%. Complications were related to a multitude of factors, including severe malnutrition, suboptimal sterilization of linen and equipment, suboptimal sterile technique, and suboptimal wound care.
We performed ETVs and choroid plexus coagulations in many children older than 6 months if the equipment was working. Storz Oi telescopes used for ETVs rarely worked for longer than 3–4 months because their glass rod lens broke from improper care and use. Decisions about whether to treat children with severe macrocephaly (> 60 cm) were made on an individual basis and took into consideration factors such as nutritional status, functional status, and socioeconomic conditions. Reduction cranioplasties on children with head circumferences > 70 cm were done rarely, and only if some cognitive ability was present.
An average of 5 children with unrepaired MMCs were admitted every week. Most were admitted within the first 2 months of life, but some were more than 1 year old (Fig. 2). Their general and neurological conditions were evaluated in an outpatient clinic and their ventricular CSF was analyzed if their anterior fontanels were patent. The care of children with grossly infected CSF (white blood cell counts > 1000 mm3) and the care of children with multiple malformations—e.g., thoracic MMC with chest wall deformities—was considered to be futile, and these children were sent home to receive comfort care. Most children with MMCs underwent distal cordectomies, removing the neural placode.2 Fifteen percent of infants with MMCs had a concomitant kyphotic deformity (Fig. 3), which was often treated by a kyphectomy after the placode was removed. The outcome of infant kyphectomies is unknown and is being evaluated.
Myelomeningoceles 2 days (A), 2 months (B), and 2 years (C) after birth. Figure is available in color online only.
Upper: Lumbar kyphotic deformity associated with a myelomeningocele. Lower: Postoperative radiograph of a patient 6 months after a kyphectomy, demonstrating fusion of adjacent vertebrae.
Management decisions concerning children with brain tumors were likewise difficult. If a child presented with a posterior fossa tumor and a CT scan indicative of a medulloblastoma, preoperative spine MRI was never feasible (because of cost) to see if drop metastases were present. If the child was younger than 3 years (poor-risk medulloblastoma), if the family lived far from Nairobi (the only site for radiotherapy in Kenya), or if the family was too poor to pay for adjuvant therapy, we had no good therapeutic choices. On rare occasions, Susan and I personally paid for adjuvant therapy for children with better prognoses.
Craniopharyngiomas also presented treatment dilemmas. They were more common in Kenya (6–8 cases/year) than in the US, and were often large (Fig. 4). Craniotomies for complete tumor removals were almost never feasible because families were unable to afford the multiple hormone replacements that are needed postoperatively. Many craniopharyngiomas were cystic. Monocystic tumors were treated with intracyst instillations of bleomycin, and multicystic tumors with endoscopic cyst fenestrations. Children with mixed cystic-solid tumors who were younger than 8 years underwent subtotal resections to “buy time” until they were old enough to receive radiotherapy.
Image showing multicystic mixed cystic and solid craniopharyngioma.
The issue of costs affected the clinical care of virtually every patient. Costs had to be considered when we were deciding which laboratory tests to order and which antibiotics to recommend, but costs did not alter decisions about whether to admit a child for care or about whether to operate, if an operation was indicated. Costs of hospitalizations were paid partially by the National Health Insurance Fund (NHIF) if the parents had subscribed to it (approximately $3/month). NHIF paid approximately $26/day. Children with hydrocephalus were often hospitalized for 3 days and their hospital bill was roughly $500, more than many families' annual income. In addition to the portion paid by NHIF, a small proportion of the bill was paid by the parents (sometimes with funds obtained by selling a family animal) or by extended family members, but the majority (approximately 85%) of the bills were paid by BethanyKids (BethanyKids.org), a Christian charitable organization. As the neurosurgical caseload increased, BethanyKids was not financially able to continuously support 100 PNS cases a month in addition to supporting pediatric surgery cases, nor were Dr. Okechi and I physically able to continually do 100 cases a month. The admission and treatment guidelines were therefore developed.
Perspectives on PNS in Sub-Saharan Africa
In the second anniversary message of the Walter Dandy Society (wedns.org), president Saleem Abdulrauf estimated that there would be 183,917 new cases of hydrocephalus in SSA in 2013 and advocated for a 10-year goal of the Society that no child with hydrocephalus in SSA would be untreated by 2024. That goal is not attainable from either a personnel or an economic standpoint.
There are fewer than 15 fellowship-trained pediatric neurosurgeons in Africa, and there are at least 5 major reasons for that dearth. 1) There are almost no academic pediatric neurosurgeons on African neurosurgery faculties who can offer fellowships. 2) To become a pediatric neurosurgeon requires an additional year of training. 3) Funding for a fellowship year is rarely available. 4) Pediatric neurosurgery pays less than adult neurosurgery. 5) Neurosurgeons prefer—as is true of doctors in general—not to treat disabled people, and many children with pediatric neurosurgical disorders are disabled by hydrocephalus or spina bifida and die within a few years.
Because of the large numbers of infants with hydrocephalus and MMCs in SSA, Park6 has recommended that general surgeons be trained to treat hydrocephalus with shunts and to close MMCs, but there are several drawbacks to that option. 1) Academic neurosurgery training programs have little interest in training general surgeons or in treating complications of their neurosurgical operations. 2) There is minimal evidence that African surgeons seriously want such training. 3) It would take a minimum of 6 months to learn how to treat hydrocephalus well, primarily because there is far more to treating hydrocephalus than inserting shunts. Evaluating whether a shunt is functioning appropriately can be difficult, and treating shunt complications is even more complex, not something that can be learned in a 2- to 3-month tutorial. The appropriate management of shunts that have perforated the gastrointestinal tract or the abdominal wall is rarely taught to surgeons desiring to treat hydrocephalus with shunts. 4) Some hydrocephalus cases require the ability to interpret CT scans, scans that most general surgeons would not know how to interpret, and about which even pediatric neurosurgeons sometimes debate the best therapeutic option.
General surgeons, particularly missionary general surgeons in remote areas, sometimes see children with hydrocephalus and have a compassionate desire to treat these children with shunts. Such operations might be improved if free, online text and video tutorials became available. A tutorial video of the MMC repair developed in Kijabe will be available in the online Masters of Neurosurgery project in the coming months.
“Solutions” to the problems of hydrocephalus and spina bifida in SSA will never come from increased neurosurgical manpower. The high frequency of spina bifida is related to at least 4 factors that would take generations to change: 1) high birth rates (women in all Kenyan counties outside of Nairobi have an average of more than 3 children each); 2) absence of folic acid at conception (the Kenyan government legislated 2 years ago that flour/maize be fortified with vitamins and folic acid, but few Kenyan families can afford to buy fortified flour); 3) ingestion of fumonisin (most Kenyans grow their own maize and have it ground by the local miller; the flour is often stored in humid conditions and becomes infected with fungi that produce fumonisin, a mycotoxin that inhibits fetal DNA metabolism); and 4) absence of prenatal screening and abortion (abortions are illegal in Kenya and that legality is unlikely to change). Each of these factors would be difficult to change individually, and, in the aggregate, probably impossible.
The treatment of children with severe hydrocephalus and/or MMCs in SSA countries presents a social and ethical dilemma and raises the question, “Is it appropriate to try to develop pediatric neurosurgery throughout Sub-Saharan Africa?” Three factors to consider are the following: 1) Medical budgets in Sub-Saharan countries are meager, and funds are needed for the vastly greater number of people with malaria, AIDS, and tuberculosis. There are insufficient funds within those budgets to treat the thousands of children newly diagnosed with hydrocephalus in Eastern and Sub-Saharan Africa each year. Warf et al. evaluated the costs and benefits of neurosurgical intervention for infant hydrocephalus in SSA.10,11 Their treatment in 215 of 297 patients was ETV, with or without choroid plexus coagulation. They concluded that neurosurgical intervention has a cost-averted disability-adjusted life-years ratio comparable to other surgical interventions and a favorable benefit-cost ratio. However, it is unlikely that any SSA country would pay the millions of dollars needed to treat these children, many of whom will die within 5 years. Although 50%–60% of the children were successfully treated in Mbale, Uganda, a center with considerable expertise, the likelihood is low that children with postinfectious hydrocephalus or hydrocephalus secondary to spina bifida could be treated with comparable results elsewhere. Although Piquer et al. recently reported a 51% success rate treating hydrocephalus with ETVs (without choroid plexus coagulations) and mobile endoscopic treatment in several SSA countries, 6-month follow-up was available in only 22% of cases.7 2) Pediatric neurosurgery relies on high-tech equipment such as CT scanners and operating microscopes and endoscopes, equipment that is expensive to purchase and difficult to maintain. Head ultrasound units are more widely available but the images can be difficult to interpret. 3) Neurosurgical patients are often disabled, and many, if not most, societies in Sub-Saharan countries do not value disabled people. Thus, it is probably not appropriate economically or socially for countries in SSA to have a goal of treating the thousands of children with the two most common PNS disorders, hydrocephalus and spina bifida. Indeed, we would not have been able to treat the few thousand children with those disorders at Kijabe Hospital in the past 4 years without the support of BethanyKids.org.
It seems reasonable for academic neurosurgical centers in SSA countries to have a pediatric neurosurgeon on their faculty to teach residents how to treat children with hydrocephalus and MMC, but more importantly, how to treat the multitude of other common PNS disorders that occur less frequently but whose outcomes are better—disorders such as tethered spinal cords, frontonasal encephaloceles, spasticity, some brain tumors, and some brain abscesses.
Sustainability
Lastly, one of the hallmarks of PNS, and one of its features that so many pediatric neurosurgeons value highly, is the opportunity to care for children from infancy into adulthood. Unless PNS is done well, children in SSA with hydrocephalus may be treated with a shunt but then lost to follow-up and die of shunt complications. Children with spina bifida may have their MMCs repaired but then die of renal failure because they do not receive chronic care for neurogenic bladders. As Ibrahim and Bernstein write, “It may be ethically dubious—and indeed a disservice to patients—to facilitate the provision of neurosurgery without augmenting the capacity to care for patients following it.”4 PNS done in academic medical centers or in mission hospitals with continual pediatric neurosurgical coverage seems most likely to provide the sustained care, often multidisciplinary care, the children with these disorders need. Is it ethical to offer them less?
References
- 1↑
Albright AL, , Ferson SS, & Okechi H: Cerebrospinal fluid white blood cell counts in infants with myelomeningoceles. J Neurosurg Pediatr 13:189–191, 2014
- 2↑
Albright AL, & Okechi H: Distal cordectomies as treatment for lumbosacral myelomeningoceles. J Neurosurg Pediatr 13:192–195, 2014
- 3↑
Albright AL, , Pollock IF, & Adelson PD: Principles and Practice of Pediatric Neurosurgery ed 2 New York, Thieme, 2008
- 4↑
Ibrahim GM, & Bernstein M: Models of neurosurgery international aid and their potential ethical pitfalls. Am Med Assoc J Ethics 17:49–55, 2015
- 5↑
Ochieng' N, , Okechi H, , Ferson S, & Albright AL: Bacteria causing ventriculoperitoneal shunt infections in a Kenyan population. J Neurosurg Pediatr 15:150–155, 2015
- 6↑
Park BE: The African experience: a proposal to address the lack of access to neurosurgery in rural sub-Saharan Africa. World Neurosurg 73:276–279, 2010
- 7↑
Piquer J, , Qureshi MM, , Young PH, & Dempsey RJ: Neurosurgery education and development program to treat hydrocephalus and to develop neurosurgery in Africa using mobile neuroendoscopic training. J Neurosurg Pediatr 15:552–559, 2015
- 8↑
Shitsama S, , Wittayanakorn N, , Okechi H, & Albright AL: Choroid plexus coagulation in infants with extreme hydrocephalus or hydranencephaly. J Neurosurg Pediatr 14:55–57, 2014
- 9↑
Thiong'o GM, , Luzzio C, & Albright AL: Ventriculoperitoneal shunt perforations of the gastrointestinal tract. J Neurosurg Pediatr 16:36–41, 2015
- 10↑
Warf BC: Pediatric hydrocephalus in East Africa: prevalence, causes, treatments, and strategies for the future. World Neurosurg 73:296–300, 2010
- 11↑
Warf BC, , Alkire BC, , Bhai S, , Hughes C, , Schiff SJ, & Vincent JR, : Costs and benefits of neurosurgical intervention for infant hydrocephalus in sub-Saharan Africa. J Neurosurg Pediatr 8:509–521, 2011
Disclosures
The author reports no conflict of interest.
