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SPECIAL TOPIC The Spectrum of Median Craniofacial Dysplasia Karam A. Allam, M.D. Derrick C. Wan, M.D. Henry K. Kawamoto, M.D., D.D.S. James P. Bradley, M.D. Heddie O. Sedano, D.D.S. Samia Saied, M.D. Los Angeles, Calif.; and Nasser City, Sohag, Egypt Summary: Given the multiple permutations in craniofacial malformations, classification of median craniofacial dysplasia or midline Tessier no. 0 to 14 clefts has been difficult and disjointed. In this review, the authors present a summary of normal embryology, prior terminology, and their proposed new classification system. Median craniofacial dysplasia has tissue agenesis and holoprosencephaly at one end (the hypoplasias), frontonasal hyperplasia and excessive tissue (the hyperplasias) at the other end, and abnormal splitting or clefting and normal tissue volume (dysraphia) occupying the middle portion of the spectrum. These three distinct subclassifications have different forms of anomalies within their groups. (Plast. Reconstr. Surg. 127: 812, 2011.) NORMAL EMBRYOLOGY T horough knowledge of embryologic development in the craniofacial region is of paramount importance for understanding, classifying, and treating median craniofacial defects. In the third week of gestation, primitive tissues of the trilaminar embryo give rise to notochordal and prechordal mesoderm (Fig. 1). Simultaneously, the rostral ectoderm differentiates to form highly specialized neural crest cells, which are responsible for ultimate development of the brain and midline facial structures.1 The dual role of the prechordal mesoderm forms the median facial skeleton and induces the rostral neural ectoderm to develop into the prosencephalon.2 Starting at the fourth week of gestation, various processes appear and the face assumes a more recognizable form.3,4 A bewildering coordination of cell proliferation, migration, and interaction occurs. The correct amount of tissue must be present at the right time in a proper three-dimensional relationship. Any breakdown in this intricate program can produce disastrous consequences.5 By the end of the fourth week, the double-layer stomodeal membrane creates an opening for the primitive mouth. An overhanging frontonasal prominence represents the superior border of the stomodeum.5 On either side of the frontonasal prominence, just above the stomodeum, a localFrom the Division of Plastic and Reconstructive Surgery, Department of Surgery, University of California, Los Angeles Medical Center; the Department of Plastic and Reconstructive Surgery, Sohag University School of Medicine; and the University of California, Los Angeles School of Dentistry. Received for publication June 15, 2010; accepted July 19, 2010. Copyright ©2011 by the American Society of Plastic Surgeons DOI: 10.1097/PRS.0b013e318200aa08 812 ized thickening of ectoderm forms the nasal or olfactory placodes. During the fifth week, the margins of the nasal placodes elevate while the central portion deepens to form the olfactory pits. The olfactory pit is actually a groove because it communicates inferiorly with the stomodeum. The raised margins of the placode become the medial and lateral nasal processes. These processes, together with the frontonasal prominence, constitute the frontonasal process. During the sixth week, the medial nasal processes enlarge and coalesce in the midline. The caudal extensions of the medial nasal processes, the globular processes, are united with the developing maxillary processes to form the upper lip. The medial nasal process gives rise to the nasal tip, the columella, the philtrum, and the premaxilla. The nasal alae are derived from the lateral nasal processes. The frontonasal process contributes the bridge and the root of the nose.5 Forebrain development is closely associated with the midline facial structures, which consist of the ethmoid, crista galli, nasal and vomerine bones, nasal septum, premaxilla with four incisors, triangular component of the primary hard palate, and the philtrum of the upper lip.6 The neural crest cells migrate at the time of neural fold closure and prosencephalon cleavage.7 Most of the median facial structures form from these neural crest cells. Defects or deficiencies in the median Disclosure: The authors have no financial interest to declare in relation to the content of this article. All sources of funds supporting the completion of this article are under the auspices of the University of California, Los Angeles. www.PRSJournal.com Volume 127, Number 2 • Median Craniofacial Dysplasia part of the anterior neural plate and neural crest cells, from whatever cause, intrinsic or extrinsic, result in abnormal development (dysplasia) of the forebrain and/or medial facial structures.8 NEW MEDIAN CRANIOFACIAL DYSPLASIA CLASSIFICATION Classification of frontonasal malformations remains confusing. We believe that the median craniofacial malformations should be studied from the embryologic point of view. With tissue agenesis and holoprosencephaly at one end (the hypoplasias), and frontonasal hyperplasia and excessive tissue (the hyperplasias) at the other end, median anomalies with separation or clefting and normal tissue volume (dysraphia) occupy the middle portion of the spectrum. We prefer use of the term “dysplasia” rather than “dysgenesis.” Also, we agree with Noordhoff et al. in limiting the term holoprosencephaly to cases of alobar brain.8 Thus, our modified median craniofacial dysplasia classification is based on both previous descriptions and severity of the anomaly (Table 1). The three main divisions are as follows: Fig. 1. Facial development of the human embryo. Drawings depict (above, left) a 5-week embryo (6.5 mm), (above, right) a 6-week embryo (9 mm), (below, left) a 6.5-week embryo (12 mm), and (below, right) a 7-week embryo (19 mm). I. Median craniofacial hypoplasia (tissue deficiency or agenesis). II. Median craniofacial dysraphia (normal tissue volume but clefted). III. Median craniofacial hyperplasia (tissue excess or duplication). Table 1. Modified Median Craniofacial Dysplasia Classification Subclassification I. Median craniofacial hypoplasia A. Holoprosencephalic spectrum (alobar brain) 1. Cyclopia 2. Ethmocephaly 3. Cebocephaly 4. Primary palate agenesis B. Median cerebrofacial hypoplasia (lobar brain) C. Median facial hypoplasia D. Microforms of median facial hypoplasia II. Median craniofacial dysraphia A. True median cleft B. Anterior encephalocele III. Median craniofacial hyperplasia Description Tissue deficiency Single holistic brain with midline facial hypoplasia or agenesis. Four subclassifications: cyclopia, ethmocephaly, cebocephaly, primary palate agenesis. 1. Single eye in a single orbit, arhinia with proboscis often located above the single orbit and microcephaly (Fig. 2). 2. Severe hypoteloribtism but separate orbits. Arhinia with proboscis located in between the orbits (Fig. 3). 3. Severe hypoteloribtism. Proboscis-like rudimentary nose (Fig. 4). 4. Premaxillary segment missing or hypoplastic (Fig. 5). Separate lobes to brain but with midline cerebral malformation; midline facial hypoplasia (Fig. 6). Midline facial hypoplasia without gross cerebral involvement (Fig. 7). 1. Binder anomaly (maxillonasal dysplasia) (Fig. 8). 2. Central maxillary incisor anomaly (Fig. 9). 3. Absent upper lip frenulum/incisors. Normal tissue volume but clefted Isolated cleft of the upper lip or abnormal split between the median globular process. It can be an incomplete (Fig. 10, left) or complete form (Fig. 10, right). Cystic malformation in which central nervous structures are abnormally displaced or herniated through a defect in the cranium (Fig. 11). Tissue excess or duplication. All forms of excess tissue starting from just thickened or duplicated nasal septum to the more severe forms of frontonasal dysplasia (Fig. 12). 813 Plastic and Reconstructive Surgery • February 2011 Under each division, further subclassifications are used to describe the specific anomalies. I. Median Craniofacial Hypoplasia The first category of median craniofacial dysplasia, median craniofacial hypoplasia, occurs with tissue deficiency or agenesis. A. Holoprosencephalic Spectrum (Alobar Brain) 1. Cyclopia: This is described by a single eye in a single orbit. Arhinia exists with a proboscis often located above the single orbit. Microcephaly is also a component (Fig. 2). 2. Ethmocephaly: Severe hypoteloribtism exists but the orbits are separate anatomically. Again, arhinia is present; however, the proboscis is located in between the orbits (Fig. 3). 3. Cebocephaly: Moderate to severe hypoteloribtism is noted. There is a “proboscis-like” rudimentary nose in a more typical nasal position (Fig. 4). 4. Primary palate agenesis: The primary palate, including the premaxillary segment and associated midline structures, is absent or severely deficient. Hypoteloribtism is seen (Fig. 5). Fig. 3. Severe form of median craniofacial hypoplasia with hypoteloribtism, and proboscis in between orbits. B. Median Cerebrofacial Hypoplasia (Lobar Brain) In this condition, midline facial hypoplasia and midline cerebral malformations exist. Unilateral or bilateral cleft lip and palate may be present (Fig. 6). Fig. 4. A patient with cebocephaly. Fig. 2. A patient with cyclopia. Note the rudimentary proboscis above the single orbit. 814 C. Median Facial Hypoplasia Midline facial hypoplasia exists without gross cerebral involvement. Unilateral or bilateral cleft lip and palate may again be present (Fig. 7). D. Microforms of Median Facial Hypoplasia Microform variants of median facial hypoplasia may occur when there are mild deficiencies from maldevelopment of the median facial structures. Unilateral or bilateral cleft lip and palate can also be found. This group includes the following: 1. Binder syndrome or anomaly (maxillonasal dysplasia): Binder syndrome patients have a characteristic flat nasomaxillary facial region with deficient or absent anterior nasal spine Volume 127, Number 2 • Median Craniofacial Dysplasia Fig. 5. A patient with primary palate agenesis. Fig. 7. Frontal view of patient with median facial hypoplasia, median cleft, nasal hypoplasia, and no gross cerebral involvement. II. Median Craniofacial Dysraphia Median craniofacial dysraphia describes a midline anomaly that has normal tissue volume but clefting, or when an abnormal separation of midline structures is present. Within the spectrum of median craniofacial dysplasia, there is a group of malformations that have normal tissue volume but have abnormally split (true median cleft lip) or are displaced (encephalocele). This group is better categorized as median tissue defects midway between hypoplasia and hyperplasia.9 Fig. 6. Frontal view of patient with median cerebrofacial hypoplasia with median cleft, rudimentary columella, and absent premaxillary segment. and negative overjet from a class III anterior incisor relationship (Fig. 8). 2. Abnormalities of the maxillary central incisors (Fig. 9): three variants of this include: a. Absent central maxillary incisors. b. Single maxillary central incisor. c. Hypoplastic central maxillary incisors. 3. Absence of upper lip frenulum. A. True Median Cleft A true median cleft may manifest as an isolated cleft of the upper lip “0 cleft” not associated with either hypoplasia or hyperplasia (Fig. 10). Alternatively, a true median cleft may have tissue deficiency or agenesis (e.g., absent nasal septum). A true median cleft may also occur with tissue excess (e.g., duplicated nasal septum). With median craniofacial dysraphia, a true median cleft has separated but retains a normal tissue amount. The upper lip deformity is a true median cleft lip with a split between the median globular processes. This is opposed to a “false” median cleft with agenesis of the globular processes. With true median cleft, the separation passes between central incisors. The cleft may continue posteriorly as a cleft of the primary and/or secondary palate. When the cleft encroaches into the interorbital region, orbital hypertelorbitism may be seen.5 815 Plastic and Reconstructive Surgery • February 2011 Fig. 8. (Left) Frontal and (right) lateral views of a patient with Binder syndrome. phologic classification.12 Basal encephaloceles are associated with a defect at or behind the crista galli and, in some cases, may protrude through a defect in the sphenoid bone and are called transsphenoidal encephaloceles.13 Fig. 9. Patient with a single central maxillary incisor. B. Anterior Encephaloceles An encephalocele is a cystic congenital malformation in which central nervous system structures herniate through a defect in the cranium in communication with cerebrospinal fluid pathways.10 They occur between normally developed zones, where a weakness permits brain to escape. The mass further pushes fields apart.9 Anterior encephaloceles are divided into frontoethmoidal and basal groups (Fig. 11). In frontoethmoidal encephaloceles, the defect occurs at the junction of the frontal and ethmoidal bones (the foramen cecum).11 Nasoethmoidal encephaloceles are considered Tessier no. 14 clefts or frontonasal dysraphia in Mazzola’s mor- 816 III. Median Craniofacial Hyperplasia (Tissue Excess or Duplication) This spectrum of anomalies includes all forms of excess tissue starting from just a thickened or duplicated nasal septum and extends toward the more severe forms of frontonasal dysplasia (Fig. 12). “Frontonasal dysplasia” is the most widely known of these types of hyperplasias.14 Objections are raised about the terminology of this condition. Typically, the term “dysplasia“ refers to the whole spectrum of abnormal tissue development starting from tissue agenesis and hypoplasia all the way to the other end of hyperplasia and excess tissue. The basic defect of median craniofacial hyperplasia is not known. Embryologically, if the nasal capsule fails to develop properly, the primitive brain vesicle fills the space normally occupied by the capsule, thus producing an anterior cranium bifidum occultum. This leads to morphokinetic arrest in the positioning of the eyes and nostrils, which tend to maintain their relative fetal positions.15 Experiments have shown that a reduction in the number of migrating neural crest cells results in these multiple defects.16 Other nasal findings may range from a notched broad nasal tip to completely divided nostrils with Volume 127, Number 2 • Median Craniofacial Dysplasia Fig. 10. Frontal views of (left) a boy with incomplete isolated cleft of the upper lip and (right) of a boy with complete isolated midline cleft of the upper lip. orbitism is severe, or when extracephalic anomalies occur, mental deficiency appears to be more likely and more severe.14,17 PREVIOUSLY DESCRIBED TERMINOLOGY AND CLASSIFICATIONS Fig. 11. Frontal view of a patient with a frontonasal encephalocele and hypertelorbitism. hypoplasia and even absence of the prolabium and maxilla with a median cleft lip. In addition, variable notching of the ala is described. Occasional associated abnormalities include accessory nasal tags, lowset ears, conductive hearing loss, mild to severe retardation, basal encephalocele, and agenesis of the corpus callosum. Importantly, a high incidence of ocular abnormalities is described. When hypertel- Historically, Kundrat was the first to use the term “arhinencephaly” to describe absence of olfactory buds and failed development of the prosencephalon and associated structures.18 DeMyer and Zeman later restricted the term arhinencephaly to absence of olfactory bulbs and designated the undifferentiated prosencephalon as holoprosencephaly.19 Brucker et al. suggested the term “median cerebrofacial dysgenesis” to encompass the whole spectrum of anomalies affecting the central part of the face and forebrain.20 DeMyer proposed the “median cleft face syndrome” and classified patients into four facial types based on orbital hypertelorbitism and other associated craniofacial abnormalities: cranium bifidum occultum, V-shaped frontal hairline, primary telecanthus, median cleft nose, median cleft prolabium, median cleft premaxilla, and median cleft palate.21 Sedano et al. modified DeMyer’s classification, preferring the designation of “frontonasal dysplasia” and categorized abnormality into four types depending on embryologic derivation.22,23 They considered frontonasal dysplasia and holoprosencephaly to be at the opposite ends of the median facial anomaly spectrum.5 817 Plastic and Reconstructive Surgery • February 2011 Fig. 12. (Left) A patient with a thickened nasal septum representing a mild form of median facial hyperplasia. (Center) A child with a bifid nose and hypertelorbitism. (Right) A patient with a severe form of median facial hyperplasia, hypertelorbitism, and bifid nose associated with other facial clefts. In contrast, Pfeifer offered a morphologic classification dividing the head of the embryo into three topographic areas: a frontonasal region and two lateral-posterior regions.12 Mazzola subsequently modified Pfeifer’s classification, with anomalies in (i) upper face region including holoprosencephaly, frontonasal dysplasia, nasal duplication, and nasal aplasia; and (ii) midcephalic borderline region including paramedian anomalies.12 Tessier considered a cleft to be at the basis of each malformation. He established a numeric system for each malformation depending on its relationship to the sagittal midline.24 The no. 0 to no.14 Tessier facial cleft passes through the midline of the face and embraces most of the midline deformity described in the other classifications.21,22,25,26 Based on Tessier’s classification, Kawamoto separated the median facial anomalies into two broad categories: (1) those in which the volume of tissue is deficient (hypoplasia), and (2) those in which the amount of tissue is near normal or in excess (hyperplasia).5 Tessier’s classification is a descriptive, clinical classification that is related to the embryopathogenesis. Use of the term “cleft,” however, creates confusion where no cleft exists, as in hypertelorbitism and nasal aplasia.27 For these reasons, van der Meulen et al. proposed a new classification based on embryology and introduced new nomenclature.27 They preferred to use the term “dysplasia” instead of 818 “cleft.” They distinguished two main groups of malformations: (1) cerebral craniofacial defects involving the brain and/or eyes, and (2) defects of the face and cranium (“craniofacial defects”). In 1993, Noordhoff et al., based on the classification of van der Meulen et al., introduced the term “median cerebrofacial dysplasia” to describe the holoprosencephalic dysplasia spectrum.8,27 Their classification primarily distinguishes the group of patients with deficient midline tissues and did not include the other end of the midfacial spectrum with excessive tissue. After Kundrat first described arhinencephaly, DeMyer and Zeman coined the term “holoprosencephaly” to describe the arrest in cleavage of the prosencephalon into two cerebral hemispheres.18,19 The resulting various skull and facial defects with brain abnormalities were classified into five categories: I. II. III. IV. Cyclopia. Ethmocephaly. Cebocephaly. Median cleft lip with total absence of the primary palate (premaxillary agenesis). V. Facial dysmorphism, median philtrumpremaxilla anlage with bilateral complete lateral cleft of the primary palate with rudimentary intermaxillary segment. Volume 127, Number 2 • Median Craniofacial Dysplasia Groups I to IV were described as alobar, whereas group V was described as lobar. The term “holoprosencephaly,” however, refers to cerebral defects and makes no reference to the invariably present median facial deformity.2 Brucker et al. suggested the term “median cerebrofacial dysgenesis” to describe this spectrum of anomalies that ranges from severe (e.g., cyclopia) to cerebral and facial development approaching normality.20 Gruss and Matthews added group VI to the above five categories for patients with a unilateral complete cleft of the primary palate with a prolabium-premaxillary anlage.2 In 1983, van der Meulen et al. introduced the term “interophthalmic dysplasia” as a subdivision of “cerebral craniofacial dysplasia” to describe agenesis or hypodevelopment of the midline structures of the face and brain.27 They recommended changing the term dysgenesis (which is abnormal morphogenesis) to dysplasia to denote abnormal development or growth. In 1992, Elias et al., with the help of new advances in computed tomography and magnetic resonance imaging, proposed a revised classification system taking into account the wide range of midline facial anomalies seen in those patients with otherwise normal brains.1 Proposed primarily from a prognostic point of view to aid treatment planning, Elias and colleagues left DeMyer groups I through III unchanged, with surgical intervention being contraindicated because of severe brain abnormality.21 A redefined group IV included patients with a false median cleft, hypoteloribtism, and abnormal cerebral morphology. Further subdivision of group IV separated those with severe brain malformation (group IVA) from those with moderate (group IVB) involvement. Group V was changed to include only those patients with anatomically normal brains. Subdivision of group V distinguished those with false median clefts (group VA) from those without a false median cleft but still with hypoteloribtism and midface hypoplasia. Patients in group IVA may live several years but are severely neurologically compromised and rarely benefit from operation. Group IVB patients are candidates for lip and palate repair. Groups VA and VB are also surgical candidates for more definitive procedures. Noordhoff et al. preferred to limit the term “holoprosencephaly” to those malformations with a single holistic brain.8 They used the term “median cerebrofacial malformations,” and based on the recommendations of van der Meulen et al., classified these malformations into four groups (one alobar and three lobar brain anomalies): I. “Holoprosencephalic dysplasias” included those with alobar brain and agenesis of the primary palate and associated midline structures. Patients with a lobar brain were further classified into: I. Median cerebrofacial dysplasia: if there is dysplasia of the median cerebral structures associated with dysplasia of the midline facial structures. II. Median facial dysplasia: if there is no gross anomalies of the brain. III. Microforms of median facial dysplasia: included microforms of deficient development of the median facial structures. Unilateral or bilateral cleft lip and palate can be found in any of the last three groups. The essential features of maxillonasal dysplasia were first described by Noyes and then later considered a true clinical entity by Binder.28,29 Although he originally described it as a form of arhinencephaly, it actually does not have brain abnormalities, and patients have a normal sense of smell. The hypoplastic midface has characteristic features including absent nasofrontal angle, nasal hypoplasia, flattened nose, short columella, palpable depression in the anterior nasal floor, localized alar base maxillary hypoplasia, and a convex upper lip with a broad philtrum. Radiographically, there is aplasia or hypoplasia of the anterior nasal spine, increase in the nasomaxillary angle, and often hypoplastic/absent frontal sinuses. Cephalometrically, sella-nasion distance and anteroposterior maxillary length are reduced.17 The cause is considered to be a disturbance of the prosencephalic induction center during embryonic growth.30,31 Basal encephalocele (transsphenoidal and transethmoidal) is rare, and its incidence has been estimated at one in 35,000 live births.32 The true incidence of basal encephalocele is probably higher because it may be asymptomatic and is not detectable externally.33,34 Previously asymptomatic basal encephaloceles with an intact palate may even be detected by imaging for examination of headache in adults.35 Basal encephaloceles may be reported in association with median cleft face anomaly, accompanied by ocular hypertelorbitism in 90 percent of cases, median facial cleft in 79 percent of cases, and bifid nose in 25 percent.11,23 Association of basal encephaloceles with cleft lip and palate is well documented in the literature. A large anterior encephalocele has been reported with frontonasal 819 Plastic and Reconstructive Surgery • February 2011 dysplasia.23,36 Guion-Almeida et al. reported 10 cases of basal encephaloceles in a series of 21 patients with frontonasal dysplasia.14 The anterior basal encephalocele is frequently associated with coexisting anomalies in the face, optic system, and brain. The most common anomalies are median cleft facial syndrome, optic dysplasia, and agenesis of the corpus callosum.37 Hilaire proposed a theory for development of basal encephaloceles, postulating that the anterior neuropore of the neural tube does not close, and the anterior part of neuroectodermal layer remains attached to the germinal layer of surface ectoderm. This adhesion interferes with the mesoderm’s interposition between neuroectodermal and ectodermal layers, which is necessary for the formation of skull base bones.38 “Median craniofacial hyperplasia” (tissue excess or duplication) has also been called “median cleft face syndrome, “frontonasal syndrome,” “frontonasal dysostosis,” and “frontonasal malformation.”17,21,22,39 The term “median face cleft syndrome” implies clefting, which may or may not be present in any given case. The condition may also not present as a syndrome. The term “dysostosis” is usually defined as a malformation of bones; thus, the term “frontonasal dysostosis” overemphasizes the bony aspects without consideration of the brain. Finally, the term “frontonasal malformation” implies a developmental field defect or sequence. However, the condition may be pathogenetically heterogeneous and better thought of as a regional defect.14 CONCLUSIONS As can be witnessed through the historical classification of median craniofacial dysplasias, the terminology and descriptions have been confusing and somewhat convoluted. This revised classification system has attempted to clarify these midline anomalies by providing three main subclassifications of (1) hypoplasias, (2) dysraphias, and (3) hyperplasias with related subtypes. Henry K. Kawamoto, M.D., D.D.S. Division of Plastic and Reconstructive Surgery University of California, Los Angeles Medical Center 1301 20th Street, Suite 460 Santa Monica, Calif. 90404 [email protected] PATIENT CONSENT Parents or guardians provided written consent for the use of patient images. 820 REFERENCES 1. Elias DL, Kawamoto HK Jr, Wilson LF. Holoprosencephaly and midline facial anomalies: Redefining classification and management. Plast Reconstr Surg. 1992;90:951–958. 2. Gruss JS, Matthews DN. Median cerebrofacial dysgenesis: The syndrome of median facial defects with hypotelorism. Cleft Palate J. 1978;15:275–281. 3. Hamilton WJ. Human Embryology: Development of Form and Function. 3rd ed. Baltimore: Williams & Wilkins; 1962. 4. Pattern BM. Human Embryology. 3rd ed. New York: McGrawHill; 1968. 5. Kawamoto HK Jr. The kaleidoscopic world of rare craniofacial clefts: Order out of chaos (Tessier classification). Clin Plast Surg. 1976;3:529–572. 6. Kurlander GJ, DeMyer W, Campbell JA, Taybi H. Roentgenology of holoprosencephaly (arhinencephaly). Acta Radiol Diagn (Stockh.) 1966;5:25–40. 7. Slavkin HC. Regulatory issues during early craniofacial development: A summary. Cleft Palate J. 1990;27:101–109. 8. Noordhoff MS, Huang CS, Lo LJ. Median facial dysplasia in unilateral and bilateral cleft lip and palate: A subgroup of median cerebrofacial malformations. Plast Reconstr Surg. 1993;91:996–1005; discussion 1006–1007. 9. Ewings EL, Carstens MH. 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J Pediatr. 1982;101:865–869. Formica F, Iannelli A, Paludetti G, Di Rocco C. Transsphenoidal meningoencephalocele. Childs Nerv Syst. 2002;18:295–298. Tada M, Nakamura N. Sphenoethmoidal encephalomeningocele and midline anomalies of face and brain. Hokkaido Igaku Zasshi 1985;60:48–56. Sueldo G, Fernandes MC. Fronto-nasal dysostosis, callosal agenesis, crossed-fused ectopia, tibial hemimelia, and preaxial polydactyly of feet: Severe expression of the acrocallosal syndrome? Am J Med Genet. 1993;46:355–357. Contribute to Plastic Surgery History The Journal seeks to publish historical photographs that pertain to plastic and reconstructive surgery. We are interested in the following subject areas: • Departmental photographs • Key historical people • Meetings/gatherings of plastic surgeons • Photographs of operations/early surgical procedures • Early surgical instruments and devices Please send your high-resolution photographs, along with a brief picture caption, via email to the Journal Editorial Office ([email protected]). Photographs will be chosen and published at the Editor-inChief’s discretion. 821