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Midline clefts of the upper lip
MIDLINE
CLEFTS OF THE UPPER LIP
By L. M. IREGBULEM, F.R.C.S.(E).,
F.R.C.S.
Department of Plastic Surgery, Orthopaedic and Plastic Hospital, P.M.B.
1294,
Enugu, Nigeria
In the average plastic surgical unit in Britain MEDIAN clefts of the upper lip are rare. and North America a median upper-lip cleft might be seen once in every 5 to IO years (according to a personal communication from Mr F. V. Nicolle). In a survey of 574 cleft lip and palate patients in North East England, Knox and Braithwaite (1963) reported 3 with median upper-lip clefts. Fogh-Andersen (1965) noted 15 cases of such clefts among 3,940 patients with clefts of lip and palate attending his clinic in Copenhagen over a 3o-year period, an incidence of 0.43 per cent. A diligent search of the literature revealed a total of only 33 reported cases. I present 3 more found among 70 consecutive patients with clefts of lip and palate seen at Enugu in the last 24 months, an incidence of about 4 per cent, IO times greater than the incidence reported previously. For the purpose of this paper, Millard’s remark “that any congenital, vertical cleft through the centre of the upper lip, no matter to what extent, be classified as a median cleft of the lip” is adopted without reservation (Millard and Williams, 1968). Applying Davis’ and Ritchie’s classification, such clefts belong to group I, subgroup 2 (i.e. prealveolar) or group III, subgroup 2, when both the lip and palate are cleft (Davis and Ritchie, 1922). Fogh-Andersen, however, classified all rare atypical facial clefts into a separate group: group IV (Fogh-Andersen, 1942). Embryology. The fundamental abnormality in the causation of midline clefts lies in the behaviour of the mesodermal component of the “primary palate” of Kernahan and Stark (1958) from which the upper lip, premaxilla and nasal septum develop. Such clefts may result from failure of mesodermal penetration or its subsequent breakdown in the median portion of the prolabial element. At an even earlier stage of development mesoderm may be completely absent in this region (Veau, 1938), giving rise to a complete midline cleft. It is easy to visualise how a whole spectrum of midline defects, ranging from a simple vermillion notch to the more severe lesions of median cleft lip with rhinoschisis and hypertelorism, can result from varying degrees of defective development in the mesenchyme of the mid-face area. The 3 patients were unrelated. Case I. A r-year-old girl was admitted with a wide complete midline cleft of the upper lip extending to the base of the columella and a corresponding cleft in the alveolus. She also had a bifid nose with widely separated nostrils and nasal cartilages, total absence of the septum, grade 2 hypertelorism (intercanthal distance 39 mm) and telecanthus (Fig. I). Other relevant abnormalities noted were an alternating convergent strabismus and a cardiac murmur. The child’s mental development was normal and the family history was uninformative. The lip was repaired by the simple Veau technique of direct approximation after freshening the edges. A preliminary correction of the bifid nose by soft tissue excision only was undertaken at the same time. Three weeks later, reduction of the telecanthus by the Mustarde technique (Mustarde, 1966) reduced the intercanthal distance to 31 mm (Fig. 2). Further correction is planned at about the age of 15, when the risk of trauma to the growing nasal skeleton should be minimal. Case 2. A 2-month-old girl was seen with an incomplete median cleft of the upper lip which presented as a furrow running vertically up to the base of the columella with a midline 63
BRITISH
64
JOURNAL
OF
FIG. I.
Case
I. Preoperative view showing wide, bifid nose, hypertelorism,
FIG. 2.
Case
I. Postoperative view 3 weeks after repair of bifid nose with reduction of intercanthal FIG. 3.
FIG. 4.
Case 3. An incomplete
median
PLASTIC
SURGERY
complete median cleft of the upper telecanthus and strabismus.
lip and
of cleft lip and preliminary soft tissue distance from 39 mm to 31 mm.
Case z. An incomplete
median
cleft with a left columellar
alveolus, correction
cleft. cyst and notching
of the left ala nasi.
notch in the alveolus (Fig. 3). There was also a small papilloma on the dorsum of the tongue. No family history of cleft lip or palate was elicited. The lip was repaired at 3 months by simply paring the cleft edges and suturing in 3 layers. Case 3. A girl of 3 months was referred because of a lump in the left nostril which interfered with breathing during sleep and caused intolerable snoring. She had a median notch in the upper lip vermillion and a midline notch of the alveolus. A soft cystic swelling measuring 1.5 x 1.5 cm was found in the left margin of the columella almost completely occluding the left nostril (Fig. 4). A notch defect of the left ala nasi was also noticed. The family history was again uninformative. At operation a few days later, the cyst was enucleated and the lip cleft was repaired by simple diamond shaped excision; the “cyst” proved to be a myolipoma. Further surgery at about the age of 5 years is contemplated to correct the notch in the left alar margin.
MIDLINE
CLEFTS
OF THE
UPPER
LIP
W
SUMMARY Three severest
females
being
with
median
one of median
cleft
upper-lip face
clefts
of varying
degree
are presented,
syndrome.
REFERENCES DAVIS, J. S. and RITCHIE, H. P. (1922). Classification of congenital palate. Journal of the American Medical Associatiort, 79, 1323.
clefts of the lip and
Inheritance of harelip and cleft palate. Thesis. !\!vt iVordis/z Copenhagen. FOGH-ANDERSEN,P. (x965). Rare clefts of the face. Acra Chimrgica Scmdinavica, 129, 275. KERNAHAN,D. and STARK,R. B. (1958). A new classification for cleft lip and cleft palate. FOGH-ANDERSEN,I’. (1942). Forlag-Arnold
Buck.
Plastic and Reconstructive Surgery, 22, 435. KNOX, G. and BRAITHWAITE,F. (1963). Cleft lips and palates in Northumberland and Durham. Archives of Diseases itt Childhood, 38, 66. MILLARD, D. R. and WILLIAMS, S. (1968). Median clefts of the upper lip. Plasric md Reconstrzutive Surgery, 42, 4.
MUSTARDE,J. C. (1966). “Repair and Reconstruction in the Orbital Region”. Livingstone. VEAU, V. (1938). “Bet de Lievre.” Paris: Masson et Cie.
31:1--E
Edinburgh:
the
CLEFTS OF THE UPPER LIP
By L. M. IREGBULEM, F.R.C.S.(E).,
F.R.C.S.
Department of Plastic Surgery, Orthopaedic and Plastic Hospital, P.M.B.
1294,
Enugu, Nigeria
In the average plastic surgical unit in Britain MEDIAN clefts of the upper lip are rare. and North America a median upper-lip cleft might be seen once in every 5 to IO years (according to a personal communication from Mr F. V. Nicolle). In a survey of 574 cleft lip and palate patients in North East England, Knox and Braithwaite (1963) reported 3 with median upper-lip clefts. Fogh-Andersen (1965) noted 15 cases of such clefts among 3,940 patients with clefts of lip and palate attending his clinic in Copenhagen over a 3o-year period, an incidence of 0.43 per cent. A diligent search of the literature revealed a total of only 33 reported cases. I present 3 more found among 70 consecutive patients with clefts of lip and palate seen at Enugu in the last 24 months, an incidence of about 4 per cent, IO times greater than the incidence reported previously. For the purpose of this paper, Millard’s remark “that any congenital, vertical cleft through the centre of the upper lip, no matter to what extent, be classified as a median cleft of the lip” is adopted without reservation (Millard and Williams, 1968). Applying Davis’ and Ritchie’s classification, such clefts belong to group I, subgroup 2 (i.e. prealveolar) or group III, subgroup 2, when both the lip and palate are cleft (Davis and Ritchie, 1922). Fogh-Andersen, however, classified all rare atypical facial clefts into a separate group: group IV (Fogh-Andersen, 1942). Embryology. The fundamental abnormality in the causation of midline clefts lies in the behaviour of the mesodermal component of the “primary palate” of Kernahan and Stark (1958) from which the upper lip, premaxilla and nasal septum develop. Such clefts may result from failure of mesodermal penetration or its subsequent breakdown in the median portion of the prolabial element. At an even earlier stage of development mesoderm may be completely absent in this region (Veau, 1938), giving rise to a complete midline cleft. It is easy to visualise how a whole spectrum of midline defects, ranging from a simple vermillion notch to the more severe lesions of median cleft lip with rhinoschisis and hypertelorism, can result from varying degrees of defective development in the mesenchyme of the mid-face area. The 3 patients were unrelated. Case I. A r-year-old girl was admitted with a wide complete midline cleft of the upper lip extending to the base of the columella and a corresponding cleft in the alveolus. She also had a bifid nose with widely separated nostrils and nasal cartilages, total absence of the septum, grade 2 hypertelorism (intercanthal distance 39 mm) and telecanthus (Fig. I). Other relevant abnormalities noted were an alternating convergent strabismus and a cardiac murmur. The child’s mental development was normal and the family history was uninformative. The lip was repaired by the simple Veau technique of direct approximation after freshening the edges. A preliminary correction of the bifid nose by soft tissue excision only was undertaken at the same time. Three weeks later, reduction of the telecanthus by the Mustarde technique (Mustarde, 1966) reduced the intercanthal distance to 31 mm (Fig. 2). Further correction is planned at about the age of 15, when the risk of trauma to the growing nasal skeleton should be minimal. Case 2. A 2-month-old girl was seen with an incomplete median cleft of the upper lip which presented as a furrow running vertically up to the base of the columella with a midline 63
BRITISH
64
JOURNAL
OF
FIG. I.
Case
I. Preoperative view showing wide, bifid nose, hypertelorism,
FIG. 2.
Case
I. Postoperative view 3 weeks after repair of bifid nose with reduction of intercanthal FIG. 3.
FIG. 4.
Case 3. An incomplete
median
PLASTIC
SURGERY
complete median cleft of the upper telecanthus and strabismus.
lip and
of cleft lip and preliminary soft tissue distance from 39 mm to 31 mm.
Case z. An incomplete
median
cleft with a left columellar
alveolus, correction
cleft. cyst and notching
of the left ala nasi.
notch in the alveolus (Fig. 3). There was also a small papilloma on the dorsum of the tongue. No family history of cleft lip or palate was elicited. The lip was repaired at 3 months by simply paring the cleft edges and suturing in 3 layers. Case 3. A girl of 3 months was referred because of a lump in the left nostril which interfered with breathing during sleep and caused intolerable snoring. She had a median notch in the upper lip vermillion and a midline notch of the alveolus. A soft cystic swelling measuring 1.5 x 1.5 cm was found in the left margin of the columella almost completely occluding the left nostril (Fig. 4). A notch defect of the left ala nasi was also noticed. The family history was again uninformative. At operation a few days later, the cyst was enucleated and the lip cleft was repaired by simple diamond shaped excision; the “cyst” proved to be a myolipoma. Further surgery at about the age of 5 years is contemplated to correct the notch in the left alar margin.
MIDLINE
CLEFTS
OF THE
UPPER
LIP
W
SUMMARY Three severest
females
being
with
median
one of median
cleft
upper-lip face
clefts
of varying
degree
are presented,
syndrome.
REFERENCES DAVIS, J. S. and RITCHIE, H. P. (1922). Classification of congenital palate. Journal of the American Medical Associatiort, 79, 1323.
clefts of the lip and
Inheritance of harelip and cleft palate. Thesis. !\!vt iVordis/z Copenhagen. FOGH-ANDERSEN,P. (x965). Rare clefts of the face. Acra Chimrgica Scmdinavica, 129, 275. KERNAHAN,D. and STARK,R. B. (1958). A new classification for cleft lip and cleft palate. FOGH-ANDERSEN,I’. (1942). Forlag-Arnold
Buck.
Plastic and Reconstructive Surgery, 22, 435. KNOX, G. and BRAITHWAITE,F. (1963). Cleft lips and palates in Northumberland and Durham. Archives of Diseases itt Childhood, 38, 66. MILLARD, D. R. and WILLIAMS, S. (1968). Median clefts of the upper lip. Plasric md Reconstrzutive Surgery, 42, 4.
MUSTARDE,J. C. (1966). “Repair and Reconstruction in the Orbital Region”. Livingstone. VEAU, V. (1938). “Bet de Lievre.” Paris: Masson et Cie.
31:1--E
Edinburgh:
the