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Complete bilateral facial cleft (Tessier 4) with corneal staphyloma: a rare association
Journal of Pediatric Surgery (2008) 43, E15–E18
www.elsevier.com/locate/jpedsurg
Complete bilateral facial cleft (Tessier 4) with corneal staphyloma: a rare association Nathalie Portier-Marret a , Judith Hohlfeld a , Merhad Hamedani b , Anthony S. de Buys Roessingh a,⁎ a
Department of Pediatric Surgery, University Hospital Center of the Canton of Vaud, (CHUV), 1011 Lausanne, Switzerland Department of Ophthalmology, University Hospital Jules Gonin, 1004 Lausanne, Switzerland
b
Received 20 April 2008; accepted 6 May 2008
Key words: Oro-ocular cleft; Staphyloma
Abstract The oro-ocular cleft no. 4 according to the Tessier classification is one of the rarest facial cleft, and to this day, few cases have been reported in the literature. We describe the case of a 9-month-old girl with a complete bilateral facial cleft. On the right cornea protruded a hard lesion, a corneal staphyloma. We describe the 3 primary surgical steps used to restore the possibility of satisfactory feeding, to promote language acquisition, and to protect vision in the nonaffected eye. The psychological and social aspects of severe facial deformities in developing countries are also tackled. © 2008 Elsevier Inc. All rights reserved.
1. Case report A 9-month-old girl was born in Africa, sixth child of the family. When she was presented to our team during a consultation mission, she was underweight but otherwise well cared for. The baby presented a complete bilateral Tessier no. 4 cleft at birth. The cleft passed through the high point of the Cupid's bow and extended laterally to the nasal alae up into the inferiomedial canthal region bilaterally. The bony alveolar cleft arose between the central and lateral incisors on both
Presented as a Poster at the XVIII Congress of the European Association for Craniomaxillofacial Surgery, Barcelona, Spain, September 12 to 16, 2006. ⁎ Corresponding author. Service de Chirurgie Pédiatrique, Centre Hospitalier Universitaire Vaudois (CHUV) CH-1011 Lausanne, Switzerland. Tel.: +41 213143126; fax: +41 213143076. E-mail address: [email protected] (A.S. de Buys Roessingh). 0022-3468/$ – see front matter © 2008 Elsevier Inc. All rights reserved. doi:10.1016/j.jpedsurg.2008.05.016
sides, coursing laterally to the piriform aperture, medial to the infraorbital foramen. The lower eyelids were bilaterally cleft with nasolacrymal duct involvement, and the left orbit was severely dystopic, with marked corneal exposure (Fig. 1). On the right cornea protruded a hard cutaneous-like lesion, later histologically diagnosed as a corneal staphyloma with multiple associated malformations of the internal structures of the eye. The hypotrophic premaxilla seemed to be retained only by the vomer. The hard and soft palatal clefts were complete on both sides. The computed tomographic (CT) scan reconstruction revealed that the alveolar cleft ascended through to the anterior maxilla on both sides but did not involve the orbital floors. The medial portion of the orbital rim was hypoplastic bilaterally (Fig. 2). The child also presented multiple bone defects of the maxilla and an almost total absence of hard palate. The CT scan showed no evidence of malformation of the brain structures, and the child appeared normally developed for her age.
E16
N. Portier-Marret et al. nerve, and an abnormal vascularization of the retina with foveal swelling and pigmented fusiform cells of the prepapillary choroid membrane. The child spent 8 months in Switzerland and, in between her 3 short (4-7 days) periods of hospitalization, was cared for by a family. She was equipped with prosthesis to replace the right eye, a simple half-sphere easily slipped into the inferior conjunctival recess and under the upper lid (Fig. 4). Prosthesis care required simple cleaning and saline humidification.
2. Discussion
Fig. 1 Preoperative clinical view of the patient. A complete bilateral Tessier no. 4 cleft with a right corneal lesion is noted.
Repair of this complex and rare facial cleft requires a multidisciplinary surgical approach and staged procedures. In the case of this child because she was already 9-monthold, we performed a modified von Langenbeck [1] procedure to close the palate in one single operation, creating bipedicled flaps of mucoperiosteum, preserving the greater palatine neurovascular bundles, and dissecting and repositioning the palatal muscles to create a normalized muscular sling to promote speech acquisition. The second surgical procedure involved closure of the left oro-ocular cleft to preserve the sound left eye and its cornea, which was exposed because of coloboma of the inferior eyelid. Closure of the lid was achieved by means of cheek advancement flaps and multiple Z-plasties. The third surgical procedure was performed 6 weeks later. It entailed closure of the right oro-ocular cleft, exenteration of the right ocular globe, and reconstruction of the lower eyelid. This reconstruction was done using a prosthetic guide sutured to the sclera to better support the lower lid flap. After exenteration, a vicryl ball was inserted in the orbit, and the oculomotor muscles were sutured to the conjunctival covering so that the prosthesis would be mobile. Multiple Zplasties lengthened the deficient oro-ocular distance, and additional Z-plasties reconstructed the vestibular fold and vermillion contour. Macroscopically, the eyeball appeared hypotrophic, with a thickened and brownish cornea. Incision of the eyeball showed the retina to be normally placed. A fragment of iris, lens debris with cataract lesions, and a thickly keratinized cornea corresponded to what is defined as a congenital staphyloma (Fig. 3); also noted were a rudimentary pars plicata of the ciliary body, a differentiation abnormality of the pars plana and ora serrata, a hypotrophy of the optical
Craniofacial dysraphia, orbitomaxillary, and laterofacial clefts are rare malformations compared with the cleft lip and palate condition. Orofacial clefts are among the rarest malformations, with an incidence of 1.43 to 4.85 per 100,000 births, and of 1% among children born with cleft lip and/or palate (CL ± P) [2,3]. The etiology of craniofacial cleft is unknown but studies support multifactorial causes. Initial priorities in cases of congenital facial deformities include airway patency, feeding, and protection of the cornea. Restoration of a normal appearance by closing the soft tissues should be considered as soon as possible to facilitate social integration [3]. Many attempts have been made since the 19th century to establish a classification of craniofacial defects. Morian [4] was the first to describe in 1887, 3 types of facial coloboma, depending on the location of the maxillary cleft (between the central incisor and the canina) in relation to the ocular region. Our present case corresponds to a “Morian II.” In 1976, Tessier [5] introduced a classification using the orbit as a
Fig. 2 3-dimensional CT scan reconstruction showing the bilateral cleft sparing the orbital floors.
Tessier 4 with corneal staphyloma
Fig. 3
Histologic examination of corneal staphyloma.
central landmark and following northbound (cranial) and southbound (facial) lines through the lips, maxilla, nose, eyelid, or eyebrows. The true occurrence of the different facial cleft types is difficult to evaluate because of the possibility that different clefts may coexist. This was confirmed by Stretch and Poole [6] in 1990 in a review of records of 170 patients with craniofacial clefts. They showed that midfacial and laterofacial clefts are rarely isolated but often seen overlapping the adjacent cleft area (according to Tessier's classification) or extending downward along their axis [7]. More recent, and on the basis of his studies of cleft embryology and anatomy, van der Meulen et al [8] renamed this cleft medial maxillary dysplasia. Finally the latest review, by Zhou et al [9], proposed a supplement to the Tessier classification that conferred greater importance to the severity and localization of the tissue deformity. This STO subclassification, with S for skin, T for soft tissue, and O for os or craniofacial bone, was proposed to better describe the clinical aspects, to facilitate presurgical planning and to provide standardization of postoperative evaluation. But the Tessier classification remains the most widely used among craniofacial surgeons and will therefore be used in the remainder of this article. The Tessier cleft 4 remains one of the rarest malformations, with only few dozen cases described in the literature for the past 130 years [10,11]. The complete no. 4 oro-oculofacial cleft includes bone and soft tissue defects to a variable degree. It extends vertically from the lacrymal portion of the inferior eyelid medially to the infraorbital foramen on the orbital floor, continuing down through the maxillary sinus and cheek to penetrate the maxillary arch in the classic location of the labiomaxillary cleft, that is, through the superior curve of the Cupid's bow between the lateral incisor and canine [2,3]. The alar cartilages are spared but are often in external rotation, especially when the cleft is unilateral. Extrophy of the maxillary sinus is frequently associated and there is usually a marked decrease in the oculo-oral distance, as was observed in our case. The medial canthal tendon is usually intact, but the canthus itself is usually displaced to a latero-
E17 inferior position. The nasolacrymal duct is abnormal in the terminal portion in 12% of the cases described by Whitaker et al [12] (in ours as well) and can be atretic or bifid [9]. The eye can be functional, microphthalmic, or anophtalmic [13]. The operating calendars for cleft reconstructions vary in different specialized centers according to their philosophical and practical priorities. In our center, a simple unilateral complete cleft lip and palate is closed in 2 procedures. First a veloplasty at 3 months of age and then, at 5 to 6 months of age, the closure of the hard palate associated with labioplasty. In the time between these 2 operations, the palatal plates take a more horizontal position, and the width of the cleft diminishes. This allows one-layer closure of the cleft without lifting the periosteal flaps, which we know has a negative impact on subsequent palatal growth. The urgent primary concerns in our case were the closure of the palatal cleft to allow proper feeding (and a better chance of normal language acquisition), and the repair of the left lower eyelid to protect the remaining and dystopic eye. But severe midface hypoplasia is to be expected in the longterm in any case of bilateral type 4 Tessier cleft, which means that secondary bone grafting and possibly osteotomy may later prove necessary. Because our patient was already 9 months old and because we wanted to limit the time, she remained in Switzerland away from her family; we did not follow our usual surgical schedule. We were therefore motivated to close the palate after the one step modified von
Fig. 4
Postoperative results with prosthesis in place.
E18 Langenbeck procedure to ensure closure and normalize feeding and language acquisition [1,14-16]. We decided that a prompt repair of the coloboma was indicated to preserve the cornea as much as possible, as the exposure of the cornea, combined with an abnormal nasolacrymal duct, usually sets the stage for chronic conjunctivitis and ulcerations or permanent scarring of the cornea [17]. The surgical technique used was multiple Z according to Mustardé [18], which included cheek advancement, lengthening of the oroocular distance, and closure of the cleft soft tissues without tension, thus providing a good primary aesthetic result. Recently, Menard et al [19] have proposed the use of tissue expansion to provide good texture and color as a technique for cases where retractile scar tissue is observed. In many developing countries, children with clefts have a high neonatal mortality [20]. The reasons are multiple— some babies have associated malformations, but most neonates die of starvation or bronchoaspiration because of the early feeding difficulties that occur in populations where breast-feeding is the only available option. There are also cultural factors, and in some areas, children with clefts are rejected, the facial malformation being taken as a sign of malediction of the family [21], and the mother and child are often cast out by the community [22]. We have learned, in the course of our 20-year period of collaboration, that even when surgery has been successful, many children have difficulty being reintegrated in their families after a long absence, especially when there are subsequent siblings. The follow-up therapy after surgery also must be planned so that it is compatible with the local, often precarious living conditions, or the long-term result is doomed to failure. In the case of our patient, before beginning the treatment, we evaluated the probability that she would receive adequate care when she returned home. The child was 9 months old and, in spite of her appearance, was well cared for. The mother obviously had the capacity and the desire to care for her child. The child was brought to our university hospital in Switzerland. In between her 3 periods of hospitalization, lasting from 4 to 7 days, she was placed in the care of a volunteer family, which belonged to “Terre des Hommes” an international nongovernmental organization based in Switzerland. She spent 8 months in Switzerland and returned home equipped with prosthesis for her right eye, a simple half-sphere easily slipped into the inferior conjunctival recess and under the upper lid (Fig. 4). The care of this prosthesis consists in a simple washing in boiled water and humidification with salt water. Her family takes very good care of this little girl, who is seen yearly by the surgical team in regular follow-up and for the planning of prosthesis change, bone grafting, and further aesthetic procedures. Without the prosthesis, familial and social integration would certainly have been more difficult. Severe facial malformations require a careful planning of their staged reconstruction, and corrections of these anomalies in developing countries add additional challenges to this process, as is also the case with all pediatric surgery.
N. Portier-Marret et al. The comprehension of the social and psychological aspects of therapy, as well as regular follow-up during the growingup years, are necessary for a successful result, that is, a happy, integrated individual.
Acknowledgments The authors are grateful to Annette Wagnière for reviewing the English text.
References [1] von Langenbeck B. Operation der angeborenen totalen spaltung des harten Gaumens nach einer neuen Methode. Dtsch Klein 1886(24):471. [2] Kawamoto Jr HK. The kaléidoscopic world of rare craniofacial clefts: order out of chaos (Tessier classification). Clin Plast Surg 1976;3: 529-72. [3] Thorne CH. Craniofacial clefts. Clin Plast Surg 1993;20:803-14. [4] Morian R. Über die schräge Gesichtsspalte in Tessier anatomical classific. Arch Klein Chir 1887(35):245. [5] Tessier P. Anatomical classification of facial, cranio-facial and laterofacial clefts. J Maxillofac Surg 1976;4:69-92. [6] Stretch JR, Poole MD. Nasolacrimal abnormalities in oblique facial clefts. Br J Plast Surg 1990;43:463-7. [7] Moore MH. Rare craniofacial clefts. J Craniofac Surg 1996;7:408-11. [8] Van der Meulen JC, Mazzola R, Vermey-Keers C, et al. A morphogenetic classification of craniofacial malformations. Plast Reconstr Surg 1983;71:560-72. [9] Zhou YQ, Ji J, Mu XZ, et al. Diagnosis and classification of congenital craniofacial cleft deformities. J Craniofac Surg 2006;17:198-201. [10] Resnick JI, Kawamoto Jr HK. Rare craniofacial clefts: Tessier no. 4 clefts. Plast Reconstr Surg 1990;85:843-9. [11] Longaker MT, Lipshutz GS, Kawamoto Jr HK. Reconstruction of Tessier no. 4 clefts revisited. Plast Reconstr Surg 1997;99: 1501-7. [12] Whitaker LA, Katowitz JA, Randall P. The nasolacrimal apparatus in congenital facial anomalies. J Maxillofac Surg 1974;2:59-64. [13] David DJ, Moore MH, Cooter RD. Tessier clefts revisited with a third dimension. Cleft Palate J 1989;26:163-84. [14] Chaudré F, Garabédian EN. In: Elsevier SAS, editor. Chirurgie des fentes labio-vélo-palatines: techniques chirurgicales-Tête et Cou; 2003. p. 46-220. Encycl Med Chir. [15] Rohrich RJ, Gosman AA. An update on the timing of hard palate closure: a critical long-term analysis. Plast Reconstr Surg 2004;113: 350-2. [16] Trier WC, Dreyer TM. Primary von Langenbeck palatoplasty with levator reconstruction: rationale and technique. Cleft Palate J 1984;21: 254-62. [17] Coruh A, Gunay GK. A surgical conundrum: Tessier number 4 cleft. Cleft Palate Craniofac J 2005;42:102-6. [18] Mustardé JC. Repair and reconstruction in the orbital region. 2nd ed. Edinburgh: Churchill Livingstone; 1980. p. 316-25. [19] Menard RM, Moore MH, David DJ. Tissue expansion in the reconstruction of Tessier craniofacial clefts: a series of 17 patients. Plast Reconstr Surg 1999;103:779-86. [20] Hujoel PP, Bollen AM, Mueller BA. First-year mortality among infants with facial clefts. Cleft Palate Craniofac J 1992;29:451-5. [21] Oyemade A, Olugbile A. Barriers to the rehabilitation of the handicapped in Nigeria. Public Health 1981;95:82-6. [22] Strauss RP. Culture, rehabilitation, and facial birth defects: international case studies. Cleft Palate J 1985;22:56-62.
www.elsevier.com/locate/jpedsurg
Complete bilateral facial cleft (Tessier 4) with corneal staphyloma: a rare association Nathalie Portier-Marret a , Judith Hohlfeld a , Merhad Hamedani b , Anthony S. de Buys Roessingh a,⁎ a
Department of Pediatric Surgery, University Hospital Center of the Canton of Vaud, (CHUV), 1011 Lausanne, Switzerland Department of Ophthalmology, University Hospital Jules Gonin, 1004 Lausanne, Switzerland
b
Received 20 April 2008; accepted 6 May 2008
Key words: Oro-ocular cleft; Staphyloma
Abstract The oro-ocular cleft no. 4 according to the Tessier classification is one of the rarest facial cleft, and to this day, few cases have been reported in the literature. We describe the case of a 9-month-old girl with a complete bilateral facial cleft. On the right cornea protruded a hard lesion, a corneal staphyloma. We describe the 3 primary surgical steps used to restore the possibility of satisfactory feeding, to promote language acquisition, and to protect vision in the nonaffected eye. The psychological and social aspects of severe facial deformities in developing countries are also tackled. © 2008 Elsevier Inc. All rights reserved.
1. Case report A 9-month-old girl was born in Africa, sixth child of the family. When she was presented to our team during a consultation mission, she was underweight but otherwise well cared for. The baby presented a complete bilateral Tessier no. 4 cleft at birth. The cleft passed through the high point of the Cupid's bow and extended laterally to the nasal alae up into the inferiomedial canthal region bilaterally. The bony alveolar cleft arose between the central and lateral incisors on both
Presented as a Poster at the XVIII Congress of the European Association for Craniomaxillofacial Surgery, Barcelona, Spain, September 12 to 16, 2006. ⁎ Corresponding author. Service de Chirurgie Pédiatrique, Centre Hospitalier Universitaire Vaudois (CHUV) CH-1011 Lausanne, Switzerland. Tel.: +41 213143126; fax: +41 213143076. E-mail address: [email protected] (A.S. de Buys Roessingh). 0022-3468/$ – see front matter © 2008 Elsevier Inc. All rights reserved. doi:10.1016/j.jpedsurg.2008.05.016
sides, coursing laterally to the piriform aperture, medial to the infraorbital foramen. The lower eyelids were bilaterally cleft with nasolacrymal duct involvement, and the left orbit was severely dystopic, with marked corneal exposure (Fig. 1). On the right cornea protruded a hard cutaneous-like lesion, later histologically diagnosed as a corneal staphyloma with multiple associated malformations of the internal structures of the eye. The hypotrophic premaxilla seemed to be retained only by the vomer. The hard and soft palatal clefts were complete on both sides. The computed tomographic (CT) scan reconstruction revealed that the alveolar cleft ascended through to the anterior maxilla on both sides but did not involve the orbital floors. The medial portion of the orbital rim was hypoplastic bilaterally (Fig. 2). The child also presented multiple bone defects of the maxilla and an almost total absence of hard palate. The CT scan showed no evidence of malformation of the brain structures, and the child appeared normally developed for her age.
E16
N. Portier-Marret et al. nerve, and an abnormal vascularization of the retina with foveal swelling and pigmented fusiform cells of the prepapillary choroid membrane. The child spent 8 months in Switzerland and, in between her 3 short (4-7 days) periods of hospitalization, was cared for by a family. She was equipped with prosthesis to replace the right eye, a simple half-sphere easily slipped into the inferior conjunctival recess and under the upper lid (Fig. 4). Prosthesis care required simple cleaning and saline humidification.
2. Discussion
Fig. 1 Preoperative clinical view of the patient. A complete bilateral Tessier no. 4 cleft with a right corneal lesion is noted.
Repair of this complex and rare facial cleft requires a multidisciplinary surgical approach and staged procedures. In the case of this child because she was already 9-monthold, we performed a modified von Langenbeck [1] procedure to close the palate in one single operation, creating bipedicled flaps of mucoperiosteum, preserving the greater palatine neurovascular bundles, and dissecting and repositioning the palatal muscles to create a normalized muscular sling to promote speech acquisition. The second surgical procedure involved closure of the left oro-ocular cleft to preserve the sound left eye and its cornea, which was exposed because of coloboma of the inferior eyelid. Closure of the lid was achieved by means of cheek advancement flaps and multiple Z-plasties. The third surgical procedure was performed 6 weeks later. It entailed closure of the right oro-ocular cleft, exenteration of the right ocular globe, and reconstruction of the lower eyelid. This reconstruction was done using a prosthetic guide sutured to the sclera to better support the lower lid flap. After exenteration, a vicryl ball was inserted in the orbit, and the oculomotor muscles were sutured to the conjunctival covering so that the prosthesis would be mobile. Multiple Zplasties lengthened the deficient oro-ocular distance, and additional Z-plasties reconstructed the vestibular fold and vermillion contour. Macroscopically, the eyeball appeared hypotrophic, with a thickened and brownish cornea. Incision of the eyeball showed the retina to be normally placed. A fragment of iris, lens debris with cataract lesions, and a thickly keratinized cornea corresponded to what is defined as a congenital staphyloma (Fig. 3); also noted were a rudimentary pars plicata of the ciliary body, a differentiation abnormality of the pars plana and ora serrata, a hypotrophy of the optical
Craniofacial dysraphia, orbitomaxillary, and laterofacial clefts are rare malformations compared with the cleft lip and palate condition. Orofacial clefts are among the rarest malformations, with an incidence of 1.43 to 4.85 per 100,000 births, and of 1% among children born with cleft lip and/or palate (CL ± P) [2,3]. The etiology of craniofacial cleft is unknown but studies support multifactorial causes. Initial priorities in cases of congenital facial deformities include airway patency, feeding, and protection of the cornea. Restoration of a normal appearance by closing the soft tissues should be considered as soon as possible to facilitate social integration [3]. Many attempts have been made since the 19th century to establish a classification of craniofacial defects. Morian [4] was the first to describe in 1887, 3 types of facial coloboma, depending on the location of the maxillary cleft (between the central incisor and the canina) in relation to the ocular region. Our present case corresponds to a “Morian II.” In 1976, Tessier [5] introduced a classification using the orbit as a
Fig. 2 3-dimensional CT scan reconstruction showing the bilateral cleft sparing the orbital floors.
Tessier 4 with corneal staphyloma
Fig. 3
Histologic examination of corneal staphyloma.
central landmark and following northbound (cranial) and southbound (facial) lines through the lips, maxilla, nose, eyelid, or eyebrows. The true occurrence of the different facial cleft types is difficult to evaluate because of the possibility that different clefts may coexist. This was confirmed by Stretch and Poole [6] in 1990 in a review of records of 170 patients with craniofacial clefts. They showed that midfacial and laterofacial clefts are rarely isolated but often seen overlapping the adjacent cleft area (according to Tessier's classification) or extending downward along their axis [7]. More recent, and on the basis of his studies of cleft embryology and anatomy, van der Meulen et al [8] renamed this cleft medial maxillary dysplasia. Finally the latest review, by Zhou et al [9], proposed a supplement to the Tessier classification that conferred greater importance to the severity and localization of the tissue deformity. This STO subclassification, with S for skin, T for soft tissue, and O for os or craniofacial bone, was proposed to better describe the clinical aspects, to facilitate presurgical planning and to provide standardization of postoperative evaluation. But the Tessier classification remains the most widely used among craniofacial surgeons and will therefore be used in the remainder of this article. The Tessier cleft 4 remains one of the rarest malformations, with only few dozen cases described in the literature for the past 130 years [10,11]. The complete no. 4 oro-oculofacial cleft includes bone and soft tissue defects to a variable degree. It extends vertically from the lacrymal portion of the inferior eyelid medially to the infraorbital foramen on the orbital floor, continuing down through the maxillary sinus and cheek to penetrate the maxillary arch in the classic location of the labiomaxillary cleft, that is, through the superior curve of the Cupid's bow between the lateral incisor and canine [2,3]. The alar cartilages are spared but are often in external rotation, especially when the cleft is unilateral. Extrophy of the maxillary sinus is frequently associated and there is usually a marked decrease in the oculo-oral distance, as was observed in our case. The medial canthal tendon is usually intact, but the canthus itself is usually displaced to a latero-
E17 inferior position. The nasolacrymal duct is abnormal in the terminal portion in 12% of the cases described by Whitaker et al [12] (in ours as well) and can be atretic or bifid [9]. The eye can be functional, microphthalmic, or anophtalmic [13]. The operating calendars for cleft reconstructions vary in different specialized centers according to their philosophical and practical priorities. In our center, a simple unilateral complete cleft lip and palate is closed in 2 procedures. First a veloplasty at 3 months of age and then, at 5 to 6 months of age, the closure of the hard palate associated with labioplasty. In the time between these 2 operations, the palatal plates take a more horizontal position, and the width of the cleft diminishes. This allows one-layer closure of the cleft without lifting the periosteal flaps, which we know has a negative impact on subsequent palatal growth. The urgent primary concerns in our case were the closure of the palatal cleft to allow proper feeding (and a better chance of normal language acquisition), and the repair of the left lower eyelid to protect the remaining and dystopic eye. But severe midface hypoplasia is to be expected in the longterm in any case of bilateral type 4 Tessier cleft, which means that secondary bone grafting and possibly osteotomy may later prove necessary. Because our patient was already 9 months old and because we wanted to limit the time, she remained in Switzerland away from her family; we did not follow our usual surgical schedule. We were therefore motivated to close the palate after the one step modified von
Fig. 4
Postoperative results with prosthesis in place.
E18 Langenbeck procedure to ensure closure and normalize feeding and language acquisition [1,14-16]. We decided that a prompt repair of the coloboma was indicated to preserve the cornea as much as possible, as the exposure of the cornea, combined with an abnormal nasolacrymal duct, usually sets the stage for chronic conjunctivitis and ulcerations or permanent scarring of the cornea [17]. The surgical technique used was multiple Z according to Mustardé [18], which included cheek advancement, lengthening of the oroocular distance, and closure of the cleft soft tissues without tension, thus providing a good primary aesthetic result. Recently, Menard et al [19] have proposed the use of tissue expansion to provide good texture and color as a technique for cases where retractile scar tissue is observed. In many developing countries, children with clefts have a high neonatal mortality [20]. The reasons are multiple— some babies have associated malformations, but most neonates die of starvation or bronchoaspiration because of the early feeding difficulties that occur in populations where breast-feeding is the only available option. There are also cultural factors, and in some areas, children with clefts are rejected, the facial malformation being taken as a sign of malediction of the family [21], and the mother and child are often cast out by the community [22]. We have learned, in the course of our 20-year period of collaboration, that even when surgery has been successful, many children have difficulty being reintegrated in their families after a long absence, especially when there are subsequent siblings. The follow-up therapy after surgery also must be planned so that it is compatible with the local, often precarious living conditions, or the long-term result is doomed to failure. In the case of our patient, before beginning the treatment, we evaluated the probability that she would receive adequate care when she returned home. The child was 9 months old and, in spite of her appearance, was well cared for. The mother obviously had the capacity and the desire to care for her child. The child was brought to our university hospital in Switzerland. In between her 3 periods of hospitalization, lasting from 4 to 7 days, she was placed in the care of a volunteer family, which belonged to “Terre des Hommes” an international nongovernmental organization based in Switzerland. She spent 8 months in Switzerland and returned home equipped with prosthesis for her right eye, a simple half-sphere easily slipped into the inferior conjunctival recess and under the upper lid (Fig. 4). The care of this prosthesis consists in a simple washing in boiled water and humidification with salt water. Her family takes very good care of this little girl, who is seen yearly by the surgical team in regular follow-up and for the planning of prosthesis change, bone grafting, and further aesthetic procedures. Without the prosthesis, familial and social integration would certainly have been more difficult. Severe facial malformations require a careful planning of their staged reconstruction, and corrections of these anomalies in developing countries add additional challenges to this process, as is also the case with all pediatric surgery.
N. Portier-Marret et al. The comprehension of the social and psychological aspects of therapy, as well as regular follow-up during the growingup years, are necessary for a successful result, that is, a happy, integrated individual.
Acknowledgments The authors are grateful to Annette Wagnière for reviewing the English text.
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