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Cleft Deformities in Zimbabwe, AfricaSocioeconomic Factors, Epidemiology, and Surgical Reconstruction
Annette M. Pham, MD;
Travis T. Tollefson, MD
Arch Facial Plast Surg. 2007;9:(doi:10.1001/archfaci.9.6.qsp70001).
ABSTRACT
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In the African country of Zimbabwe, a variety of socioeconomic factors have contributed to a lack of specialty care and resources for the indigent population. Although cleft lip and palate has a lower incidence in Africa (0.67 per 1000 births) than in Latin America or Asia, access to reconstructive surgery is often difficult to obtain. A surgical team worked with Zimbabweans at the Harare Central Hospital, Harare, to perform cleft surgery for 39 patients. We review the epidemiology of cleft deformities in Africa, our experience with 39 patients with cleft lip and palate, and the techniques used to address 2 patients with midfacial clefts. To our knowledge, this retrospective case review and epidemiologic literature review is the first review of cleft care in Zimbabwe. Poverty in Zimbabwe, caused in part by the highest inflation rate in the world, has contributed to the emigration of a large number of specialists to other countries. In addition, the health care system is overwhelmed by a high prevalence rate of human immunodeficiency virus (25%), leading to a drastically reduced parental life expectancy (mean life expectancy, 36 years). Primary and secondary cleft lip and palate repairs were completed without complications. Children requiring care beyond the scope of this mission were referred to the Republic of South Africa. The cooperation among the Zimbabwean administration, physicians, and nurses was integral to the organization and successful execution of this reconstructive surgical mission. Ultimately, until the socioeconomic conditions improve in Zimbabwe, training and continuing education of local physicians are imperative to advance the care of children with cleft lip and palate.
INTRODUCTION
According to ancient folklore, Great Zimbabwe was considered to be the capital of one of the world's oldest civilizations. Its name derives from the words zimba (palace) and bwe (stone), referring to the ancient city built from stone. Modern-day Zimbabwe is a land-locked country in south-central Africa, bordered by Botswana, Mozambique, the Republic of South Africa, and Zambia, and slightly smaller than California in area. The population in Zimbabwe is 12 million people, with approximately 2 million living in the capital city, Harare. The official language is English, but the 2 most common native languages spoken are Shona and Ndebele. Zimbabwe has the highest literacy rate (91%) in Africa.1 However, Zimbabwe has the lowest mean life expectancy in Africa, 36 years (in the United States, the life expectancy is 78 years).1
Similar to other African countries, Zimbabwe has been affected by the mass exodus of skilled professionals (ie, physicians, engineers, scientists, educators) to more developed countries—the "brain drain."2 The impact on the health care sector is considerable, contributing to increased medical fees and costs, overcrowded central hospitals, and lack of access to specialty care.2
In particular, a gap between lack of specialty care in cleft surgery and resources for the indigent population was identified. The incidence of cleft deformities in Zimbabwe, to our knowledge, is not cited in the literature. However, in Malawi, Africa, the incidence of clefts (all types) has been reported to be about 0.67 per 1000 births.3 This is comparable with the incidence rate reported in the United States for the population of black individuals (0.7:1000).4
In October 2006, a US surgical team affiliated with the organization Operation of Hope worked with physicians and nurses in Harare Central Hospital (HCH) to evaluate 63 patients (23 patients with cleft lip and palate, 24 with an isolated cleft lip, 10 with an isolated cleft palate, 2 with a median cleft, and 4 with other diagnoses [microstomia, ecotodermal dysplasia, bilateral swelling, and Tessier cleft No. 25-6]). We performed 42 procedures for 39 patients on 5 consecutive operating days (Table). The preoperative preparation, socioeconomic factors, and epidemiology are described herein. Surgical reconstruction and postoperative care will also be addressed, including the management of a unique subset of midfacial clefts.
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Table. Diagnoses and 42 Procedures Performed for 39 Patients on 5 Consecutive Operating Days
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METHODS
In Zimbabwe, British colonial influence helped to create a parliamentary government system divided into departments called ministries. The Ministry of Health infrastructure is designed for an efficient, widespread distribution of information and resources. The public health care system is organized based on geography, which consists of rural clinics where village health workers (such as midwives and nursing or medical assistants) manage day-to-day health care issues. At the next level, primary care physicians evaluate patients in the ward clinics. If additional levels of care are required, patients are then sent to the district hospitals or to the provincial hospitals for specialty care access. Tertiary level care is available at the 2 main central hospitals in the 2 largest cities, Harare and Bulawayo.
Via this network of public health care levels, information regarding the planned cleft surgical procedures was disseminated prior to the team's arrival. Patients and their caregivers (predominantly mothers) were transported by bus from the rural areas to the district or provincial hospitals and then on to HCH where the surgical procedures were to be performed. In some cases, families traveled from afar and were given room and board in the hospital until the screening clinic, where eligibility for surgery was determined. The mean time for travel from home was 3 hours, although some traveled as long as 8 hours by bus.
Identification numbers and screening cards were assigned and completed for all patients (Figure 1). The surgical team worked closely with the medical staff at HCH to ensure continuity of care. Surgical residents from the HCH program also evaluated the patients, performing preoperative examinations and laboratory evaluations. In addition, nurses and nursing students were responsible for ensuring the flow of the screening process, obtaining vital signs, and assisting in translation for non–English-speaking families.
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Figure 1. An example of the screening card used to record patient data. After the patient is evaluated, the diagnosis and surgical plan are recorded. Screening cards are then used to maintain medical records of all the patients evaluated.
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Our cleft surgical team screened patients based on age, weight, notable medical or surgical history, and complexity of the deformity to determine eligibility for surgery. In most cases, medical and surgical history were easy to obtain because parents kept a notebook containing physician notes from birth, which served as a personal, portable "medical record." Overall, among the 63 patients who were evaluated there was a predominance of cleft deformities of the lip and palate. A variety of other facial deformities were evaluated, as already described. Thirty-nine patients were then chosen for a total of 42 procedures.
SURGICAL PROCEDURES
ANESTHESIA
Working with the anesthesiologists from HCH was a unique experience. The anesthesia team consisted of several members, including the attending anesthesiologists, residents of different training levels, nurse anesthetists, and students. Each child was brought into the operating room with a parent, who held the child while sedation was induced with mask anesthesia. Once the patient was sedated, the patient was placed onto the operating table, and a member of the anesthesia team escorted the parent to the preoperative holding area.
In Zimbabwe, the primary inhalational agent used is halothane, which is less commonly used in the United States. It is most commonly used in underdeveloped countries because of its lower cost.7 However, one concern associated with the use of halothane is the sensitization of the myocardium to arrhythmias after exogenous administration of catecholamines. When using halothane, the surgeon must limit the epinephrine level to decrease the risk of cardiac arrhythmias.7 (When using isoflurane, enflurane, or desflurane, the subcutaneous dose of epinephrine can be up to 4-fold greater than that used with halothane before arrhythmias occur.8)
Also, most arrhythmias associated with halothane use are secondary to hypercapnia or an inadequate anesthetic level.7 To prevent these potential cardiac adverse effects, communication was a key part of the surgical process when carefully injecting local anesthetic using lidocaine, 1%, with epinephrine in a 1:100 000 ratio. Furthermore, the anesthesia team prevented hypoventilation and hypercapnia by manually controlling the patient's respiratory rate.
Once the surgery was completed, every patient was extubated in the recovery room rather than in the operating suite. The turnover of the operating suite was thus quite efficient. All patients were successfully extubated in this manner. There were no anesthetic complications and no need for reintubations.
GENERAL SURGICAL TECHNIQUES
We performed a total of 42 procedures for 39 patients (Table). This included 10 cleft palate repairs as well as 6 cleft palate revisions using a variety of techniques, such as 2-flap, 3-flap, Furlow, or von Langenbeck palatoplasties, based on the type of cleft encountered.4 There were 20 primary cleft lips (Figure 2) and 1 cleft lip revision—all repaired using a modified Millard rotation-advancement flap technique.
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Figure 2. There were 20 primary cleft lips repaired. A, Preoperative and B, postoperative photographs of a patient with a unilateral incomplete cleft lip. C, Preoperative and D, postoperative photographs of a patient with a unilateral complete cleft lip.
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Among the 20 cleft lips, 2 were bilateral. Divergent schools of thought exist regarding bilateral cleft lip repair with or without a primary rhinoplasty at the time of repair.9-10 Historically, the cleft nasal deformity was not addressed during the primary cleft lip repair. The deficient columellar length in the bilateral cleft nasolabial deformity has been addressed with forked flaps (Cronin technique) and V-Y advancement techniques. However, dissatisfied with the surgical results, a group of cleft surgeons shifted their focus from secondary to primary nasal repair, thus developing new strategies to achieve an ideal primary nasolabial repair.9-10
One such strategy in the United States is the use of presurgical orthopedics (eg, nasoalveolar molding) to improve columellar length and to facilitate a primary nasolabial repair. This strategy is, however, not feasible in Zimbabwe because of the lack of resources and/or experienced orthodontists. Primary rhinoplasty during the bilateral cleft lip repair was performed to limit the number of future procedures. The prolabial incisions were continued into the marginal incisions to expose the lower lateral cartilages. Dome-binding sutures (5-0 Prolene absorbable sutures; Ethicon Inc, Somerville, New Jersey) were placed to improve tip projection. Suspension sutures from the lower lateral cartilages to the upper lateral cartilages were placed bilaterally. Silastic nasal conformers (sizes 3 and 4; Porex Surgical Inc, Newnan, Georgia) were used to retain the nostril size and prevent stenosis.
In addition, a 22-year-old man presented with the stigmata of a previously repaired bilateral cleft lip, which had affected his self-esteem. During rhinoplasty, poor nasal tip projection was addressed with a V-Y columellar advancement and interdomal sutures. The surgery had a profound effect on the young man and his family. On our return to the United States, his mother related:
Words fail me to extend/express my heartfelt gratitude at the love and commitment shown by yourself and Team you travelled [sic] with to Zimbabwe. . . . Thanks to you all, by the grace of God, he now has a good looking nose, the change has done wonders to his outward appearance, its truelly [sic] amazing. With time I believe he'll get over the complex and build up a confidence in himself and who he ought to be and not what circumstance tried to make him.
A FEW RARE CASES
Of 63 patients screened, there were 2 patients with median cleft lips. A rare occurrence, the median, or midline cleft lip, has an incidence ranging from 0.43% to 0.73%.11 In 1976, Paul Tessier, MD, classified craniofacial clefts according to an ordered numbering system that could involve the soft tissue, the skeletal framework, or both (Figure 3).5-6 Within this classification system, there is a spectrum of cleft severity. For instance, a Tessier No. 0 cleft can present with variations such as minimal lip notching in the midline to a complete cleft midline cleft lip and possible bifid nasal deformity. Severe cases occur with extension into the skull base (cranium bifidum occulta).5 Other features may include an alveolar cleft between the 2 central incisors; a broad nasal bridge; hypertelorism; a thickened, duplicated, or absent septum; and/or a broad tip with columellar and tip bifidity.5
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Figure 3. A young girl presented with a rare Tessier No. 2 cleft anomaly.5, 12 She also had a congenital nasal mass with an associated pit (black arrow), which would require further workup prior to excision.
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Several techniques have been described to address the bifid nose associated with the median cleft lip. Using a combined intraoral approach with a modified fork flap incision at the base of the columella, Turkaslan et al5 addressed the base of the nose and the duplicated septum. The intraoral approach allowed wide exposure of the base of the nose and the piriform apertures, the anterior maxillary segment, and the cleft area. The modified fork flaps raised at the nasal sill were then used to achieve columellar lengthening. In another report of the treatment of 2 cases of bifid nose, Miller et al13 suggested that a midline nasal incision extending from the midline of the nasal tip to the nasal root optimized exposure of the structural relationships. Unfortunately, this technique leaves a midline nasal scar that does not fall within the borders of aesthetic units.
We chose a modified external rhinoplasty approach. The median cleft lip and nasal bifidity of a 3-year-old girl were repaired with a modified V-Y columellar advancement technique. Previously, a cleft lip repair had been unsuccessful. In addition to the cleft lip and bifid nose (Figure 4A), she had a diastema between the 2 central incisors as well as an alveolar cleft noted on imaging (Figure 4B). After the cleft lip was repaired using a modified white roll triangle flap technique, the nose was addressed by extending the midline incision just onto the columella. The medial crura were accessed, and suture technique was used to narrow the columella and to improve the subtle bifidity created by the divergent lower lateral cartilages. An immediate postoperative photograph illustrates the incisions used and preliminary results (Figure 4C).
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Figure 4. A 3-year-old with a previous attempt at repair of her median cleft lip. A, Along with a median cleft lip, the patient had a bifid nose and a diastema at the 2 central incisors. Note also the hypertelorism. B, A plain radiograph in the anterior-posterior view demonstrates a median cleft through the skeletal framework (yellow lines). C, The midline cleft lip was repaired using a modified V-Y columellar advancement technique. An immediate postoperative result demonstrates the incisions for the cleft lip revision with extension onto the midline columella.
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An 18-year-old woman with a median cleft lip and a bifid nose also presented for repair (Figure 5). An external rhinoplasty approach was used to address the duplicated septum and divergent lower lateral cartilages (Figure 6). The fibrofatty intradomal tissue was rotated superiorly into the supratip and secured as a vascularized flap prior to dome-binding suture placement (Figure 7). Both patients had mucosalized tracts extending in the midline maxillary buccal sulcus within the bony cleft (Figure 8).
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Figure 5. An 18-year-old woman with a median cleft lip and nasal bifidity (A) that is accentuated when smiling (B).
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Figure 6. An intraoperative photograph depicting exposure of the duplicated septum (black arrow) and divergent lower lateral cartilages using an external rhinoplasty approach.
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Figure 7. A vascularized fibrofatty tissue flap is rotated into the supratip region to augment the deficient area in the bifid tip secondary to the divergent lower lateral cartilages. Interdomal sutures (5-0 Prolene; Ethicon Inc, Somerville, New Jersey) were used to address the nasal bifidity.
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Figure 8. Patient 31. Both patients with midfacial clefts (patients 14 and 31) demonstrated a mucosalized tract (black arrow) between the bony maxillary cleft.
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POSTOPERATIVE CARE
Our team worked with the nurses and pharmacists to understand standardized order sets and medicine formulations for optimization of postoperative issues such as fluid management, antibiotic use, and pain control. The parents also played a vital role in the patients' postoperative care. Although breastfeeding of children with cleft lips is a controversial issue among cleft surgeons, we encouraged the mothers of the patients with cleft lip to nurse postoperatively for several reasons. Breastfeeding simplified postoperative management of oral intake for mothers and nurses and also allowed the mothers to be intimately involved in their children's care—a practical as well as a psychological advantage. Multiple studies have reported that early postoperative breastfeeding did not increase the risk of wound complications.12, 14-15 In addition, breastfeeding was not only more economical than spoon-feeding, it simplified the postoperative regimen and even enhanced weight gain.12, 14
A discharge clinic was held after the last operating day. Patients who lived in Harare or neighboring towns were discharged when appropriate and returned for a postoperative visit at the discharge clinic. The team removed the sutures, and local wound care was emphasized.
Patients who had traveled far from home remained in the hospital until the discharge clinic. Afterward, transportation was arranged for patients and their caregivers to return home. Patients whose deformities had been repaired later in the week had absorbable sutures placed. There was also another discharge clinic 1 week later for patients still needing additional postoperative care prior to the last team members' return to the United States.
COMMENT
Performing cleft surgical procedures can be both a rewarding and a challenging experience. Although pro bono missions for these reconstructive surgical procedures consist of a short time period spent screening, operating, and performing early postoperative care, the success of the mission is determined by the planning and preparation prior to the trip as well as the participation and coordination among the people of the hosting country. In Zimbabwe, the Ministry of Health distributed information prior to our arrival as well as during our stay to facilitate our outreach efforts.
The physicians, nurses, and staff at HCH were accommodating and flexible. They organized nursing staff, operating room personnel, and even hospital rooms to create an efficient working environment. Of utmost importance, the interaction between the Zimbabwean surgeons and our team in the operating room facilitated the exchange of cleft repair techniques. This is important because ultimately these surgeons will be treating the children independently.
In conclusion, a long-term relationship between Operation of Hope and the Zimbabweans will ensure that the gap between a lack of specialty care in cleft surgery and needs of these patients can be overcome. Future goals will include education and surgical training to empower the medical staff dedicated to staying in Zimbabwe despite the "brain drain."
AUTHOR INFORMATION
Correspondence: Travis T. Tollefson, MD, Cleft and Craniofacial Program, Facial Plastic and Reconstructive Surgery, Department of Otolaryngology–Head and Neck Surgery, University of California, Davis School of Medicine, 2521 Stockton Blvd, Suite 7200, Sacramento, CA 95817 (travis.tollefson{at}yahoo.com).
Accepted for Publication: June 1, 2007.
Published Online: October 22, 2007 (doi:10:1001/archfacial.9.6.qsp70001).
Author Contributions: Study concept and design: Pham and Tollefson. Acquisition of data: Pham and Tollefson. Analysis and interpretation of data: Pham and Tollefson. Drafting of the manuscript: Pham. Critical revision of the manuscript for important intellectual content: Pham and Tollefson. Administrative, technical, and material support: Pham. Study supervision: Tollefson.
Financial Disclosure: None reported.
Additional Contributions: The directors of Operation of Hope—Joseph Clawson, MD, Jennifer Trubenback, and Stephen Clawson—arranged this mission. We thank them for their diligent work to make this surgical mission possible.
Author Affiliations: Department of Otolaryngology–Head and Neck Surgery, University of California, Davis Medical Center, Sacramento.
REFERENCES
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1. World Health Organization Global Health Atlas. 2003. http://www.who.int/globalatlas/. Accessed November 9, 2006.
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4. Sykes JM, Senders CW. Cleft palate. In: Cotton RT, Myer CM, eds. Practical Pediatriatric Otolaryngology. Philadelphia, PA: Lippincott Williams & Wilkins;1998:809-824.5. Turkaslan T, Ozcan H, Genc B, et al. Combined intraoral and nasal approach to Tessier No:0 cleft with bifid nose. Ann Plast Surg. 2005;54(2):207-210.
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6. Tessier P. Anatomical classification facial, cranio-facial and latero-facial clefts. J Maxillofac Surg. 1976;4(2):69-92.
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