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Cleft Deformities in Zimbabwe, Africa

Socioeconomic Factors, Epidemiology, and Surgical Reconstruction

Annette M. Pham, MD; Travis T. Tollefson, MD

I n 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 epide- miology 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 mis- sion were referred to the Republic of South Africa. The cooperation among the Zimbabwean admin- istration, physicians, and nurses was integral to the organization and successful execution of this re- constructive 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. Arch Facial Plast Surg. 2007;9(6):385-391

According to ancient folklore, Great Zim- babwe 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 popu- lation 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 Nde- bele. 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 ex- pectancy is 78 years).1

Similar to other African countries, Zim- babwe has been affected by the mass exo- dus of skilled professionals (ie, physicians, engineers, scientists, educators) to more de- veloped countries—the “brain drain.”2 The impact on the health care sector is consid- erable, contributing to increased medical fees and costs, overcrowded central hospi- tals, and lack of access to specialty care.2

In particular, a gap between lack of spe- cialty care in cleft surgery and resources for the indigent population was identified. The incidence of cleft deformities in Zimba- bwe, to our knowledge, is not cited in the literature. However, in Malawi, Africa, the incidence of clefts (all types) has been re- ported to be about 0.67 per 1000 births.3

This is comparable with the incidence rate reported in the United States for the popu- lation of black individuals (0.7:1000).4

Author Affiliations: Department of Otolaryngology–Head and Neck Surgery, University of California, Davis Medical Center, Sacramento.

For editorial comment see page 382

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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 postopera- tive care will also be addressed, including the manage- ment of a unique subset of midfacial clefts.

METHODS

In Zimbabwe, British colonial influence helped to create a par- liamentary government system divided into departments called ministries. The Ministry of Health infrastructure is designed for

an efficient, widespread distribution of information and re- sources. The public health care system is organized based on ge- ography, which consists of rural clinics where village health work- ers (such as midwives and nursing or medical assistants) manage day-to-day health care issues. At the next level, primary care phy- sicians evaluate patients in the ward clinics. If additional levels of care are required, patients are then sent to the district hospi- tals or to the provincial hospitals for specialty care access. Ter- tiary 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 re- garding the planned cleft surgical procedures was disseminated prior to the team’s arrival. Patients and their caregivers (predomi- nantly 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, fami- lies traveled from afar and were given room and board in the hos- pital 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 con- tinuity of care. Surgical residents from the HCH program also evaluated the patients, performing preoperative examinations and laboratory evaluations. In addition, nurses and nursing stu- dents were responsible for ensuring the flow of the screening process, obtaining vital signs, and assisting in translation for non–English-speaking families.

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 par- ents kept a notebook containing physician notes from birth, which served as a personal, portable “medical record.” Over- all, among the 63 patients who were evaluated there was a pre- dominance of cleft deformities of the lip and palate. A variety of other facial deformities were evaluated, as already de- scribed. 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 sev- eral members, including the attending anesthesiolo- gists, residents of different training levels, nurse anes- thetists, 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 pa- tient was sedated, the patient was placed onto the oper- ating table, and a member of the anesthesia team es- corted 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 coun- tries because of its lower cost.7 However, one concern as- sociated with the use of halothane is the sensitization of the myocardium to arrhythmias after exogenous admin- istration of catecholamines. When using halothane, the surgeon must limit the epinephrine level to decrease the risk of cardiac arrhythmias.7 (When using isoflu- rane, enflurane, or desflurane, the subcutaneous dose of

Table. Diagnoses and 42 Procedures Performed for 39 Patients on 5 Consecutive Operating Days

Patient No./ Sex/Age, mo Diagnosis Procedure

1/F/36 Cleft palate Cleft palate repair 2/M/60 Cleft palate Cleft palate repair 3/M/48 Cleft lip and palate Cleft lip and palate repair 4/F/12 Cleft lip Cleft lip repair 5/M/36 Cleft lip Cleft lip repair 6/M/18 Cleft lip Cleft lip repair 7/M/9 Cleft lip Cleft lip repair 8/M/12 Cleft lip Cleft lip repair 9/M/48 Cleft lip Cleft lip repair

10/M/18 Cleft lip Cleft lip repair 11/M/19 Cleft lip Cleft lip repair 12/M/5 Cleft lip Cleft lip repair 13/F/36 Cleft lip Cleft lip repair 14/F/36 Median cleft lip Cleft lip repair 15/F/16 Cleft palate Cleft palate repair 16/M/5 Cleft lip Cleft lip repair 17/F/36 Cleft palate Cleft palate repair 18/M/264 Cleft nasal deformity Cleft rhinoplasty 19/M/48 Cleft palate Cleft palate revision 20/M/24 Cleft lip, palate Cleft lip revision and

cleft palate repair 21/M/60 Cleft palate Cleft palate revision 22/F/96 Cleft palate Cleft palate repair 23/F/60 Cleft palate Cleft palate repair 24/M/60 Cleft palate Cleft palate repair 25/F/36 Cleft palate Cleft palate revision 26/F/48 Cleft palate Cleft palate revision 27/F/96 Cleft palate Cleft palate revision 28/F/7 Cleft lip Cleft lip repair 29/M/7 Cleft lip Cleft lip repair 30/M/24 Cleft lip Cleft lip repair 31/F/216 Median cleft lip Cleft lip repair and

rhinoplasty 32/M/13 Cleft lip Cleft lip repair 33/M/96 Cleft lip Cleft lip repair 34/M/36 Microstomia Commissuroplasty 35/F/96 Cleft lip Cleft lip repair 36/F/36 Cleft lip Cleft lip repair 37/M/11 Cleft palate Cleft palate repair 38/M/12 Cleft lip Cleft lip repair 39/F/96 Cleft palate Cleft palate revision

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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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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 anes- thesia 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 oper- ating 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 pal- ate 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.

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 His-

torically, the cleft nasal deformity was not addressed dur- ing 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 second- ary 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 pre- surgical orthopedics (eg, nasoalveolar molding) to im- prove columellar length and to facilitate a primary naso- labial repair. This strategy is, however, not feasible in Zimbabwe because of the lack of resources and/or expe- rienced orthodontists. Primary rhinoplasty during the bi- lateral cleft lip repair was performed to limit the number of future procedures. The prolabial incisions were con- tinued into the marginal incisions to expose the lower lat- eral cartilages. Dome-binding sutures (5-0 Prolene ab- sorbable 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 stig- mata 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 ad- vancement and interdomal sutures. The surgery had a

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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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 ap- pearance, 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 me- dian cleft lips. A rare occurrence, the median, or mid- line cleft lip, has an incidence ranging from 0.43% to 0.73%.11 In 1976, Paul Tessier, MD, classified craniofa- cial 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 oc- cur with extension into the skull base (cranium bifidum occulta).5 Other features may include an alveolar cleft be- tween the 2 central incisors; a broad nasal bridge; hy- pertelorism; a thickened, duplicated, or absent septum; and/or a broad tip with columellar and tip bifidity.5

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 ad- dressed 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 max- illary segment, and the cleft area. The modified fork flaps raised at the nasal sill were then used to achieve colu- mellar lengthening. In another report of the treatment of 2 cases of bifid nose, Miller et al13 suggested that a mid- line nasal incision extending from the midline of the na- sal tip to the nasal root optimized exposure of the struc- tural 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 tech- nique. Previously, a cleft lip repair had been unsuccess- ful. 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 tri- angle flap technique, the nose was addressed by extend- ing the midline incision just onto the columella. The me- dial crura were accessed, and suture technique was used to narrow the columella and to improve the subtle bifid-

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.

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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ity created by the divergent lower lateral cartilages. An im- mediate postoperative photograph illustrates the inci- sions used and preliminary results (Figure 4C).

An 18-year-old woman with a median cleft lip and a bi- fid 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 buc- cal sulcus within the bony cleft (Figure 8).

POSTOPERATIVE CARE

Our team worked with the nurses and pharmacists to un- derstand standardized order sets and medicine formula- tions for optimization of postoperative issues such as fluid management, antibiotic use, and pain control. The par- ents also played a vital role in the patients’ postopera- tive care. Although breastfeeding of children with cleft lips is a controversial issue among cleft surgeons, we en- couraged the mothers of the patients with cleft lip to nurse postoperatively for several reasons. Breastfeeding sim- plified postoperative management of oral intake for moth- ers and nurses and also allowed the mothers to be inti- mately involved in their children’s care—a practical as

Figure 5. An 18-year-old woman with a median cleft lip and nasal bifidity (A) that is accentuated when smiling (B).

Figure 6. An intraoperative photograph depicting exposure of the duplicated septum (black arrow) and divergent lower lateral cartilages using an external rhinoplasty approach.

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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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 addi- tion, 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 postop- erative 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, trans- portation was arranged for patients and their caregivers to return home. Patients whose deformities had been re- paired 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 re- warding and a challenging experience. Although pro bono missions for these reconstructive surgical procedures con- sist 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 coordi- nation among the people of the hosting country. In Zim- babwe, 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 accom- modating and flexible. They organized nursing staff, operating room personnel, and even hospital rooms to create an efficient working environment. Of utmost im- portance, the interaction between the Zimbabwean sur- geons and our team in the operating room facilitated the exchange of cleft repair techniques. This is important be- cause ultimately these surgeons will be treating the chil- dren independently.

In conclusion, a long-term relationship between Op- eration 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 em- power the medical staff dedicated to staying in Zimba- bwe despite the “brain drain.”

Accepted for Publication: June 1, 2007. Published Online: October 22, 2007 (doi:10:1001 /archfaci.9.6.qsp70001). 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@yahoo.com). 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 sup- port: 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.

REFERENCES

1. World Health Organization Global Health Atlas. 2003. http://globalatlas.who.int /globalatlas/. Accessed November 9, 2006.

2. Chetsanga CJ, Muchenje TB. An Analysis of the Cause and Effect of the Brain Drain in Zimbabwe. Harare, Zimbabwe: Scientific and Industrial Research and Development Centre; 2001.

3. Msamati BC, Igbigbi PS, Chisi JE. The incidence of cleft lip, cleft palate, hydro- cephalus and spina bifida at Queen Elizabeth Central Hospital, Blantyre, Malawi. Cent Afr J Med. 2000;46(11):292-296.

4. Sykes JM, Senders CW. Cleft palate. In: Cotton RT, Myer CM, eds. Practical Pe- diatriatric 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.

6. Tessier P. Anatomical classification facial, cranio-facial and latero-facial clefts. J Maxillofac Surg. 1976;4(2):69-92.

7. Cote CJ. Pediatric anesthesia. In: Fleisher LA, Johns RA, Savarese JJ, Wiener- Kronish J, Young WL, eds. Miller’s Anesthesia. 6th ed. New York, NY: Churchill Livingstone; 2005:2374-2375.

8. Savino JS, Floyd TF, Cheung AT. Cardiac anesthesia. In: Cohn LH, Edmunds LH Jr, eds. Cardiac Surgery in the Adult. 2nd ed. Columbus, OH: McGraw-Hill; 2003: 249-281.

9. Mulliken JB, Wu JK, Padwa BL. Repair of bilateral cleft lip: review, revisions, and reflections. J Craniofac Surg. 2003;14(5):609-620.

10. Mulliken JB. Bilateral cleft lip. Clin Plast Surg. 2004;31(2):209-220. 11. Apesos J, Anigian GM. Median cleft of the lip: its significance and surgical repair.

Cleft Palate Craniofac J. 1993;30(1):94-96. 12. Darzi MA, Chowdri NA, Bhat AN. Breast feeding or spoon feeding after cleft lip

repair: a prospective randomized study. Br J Plast Surg. 1996;49(1):24-26. 13. Miller PJ, Grinberg D, Wang TD. Midline cleft: treatment of the bifid nose. Arch

Facial Plast Surg. 1999;1(3):200-203. 14. Weatherley-White RCA, Kuehn DP, Mirrett P, et al. Early repair and breast-

feeding for infants with cleft lip. Plast Reconstr Surg. 1987;79(6):879-885. 15. Cohen M, Marschall MA, Schafer ME. Immediate unrestricted feeding of infants

following cleft lip and palate repair. J Craniofac Surg. 1992;3(1):30-32.

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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