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Background:
Median cleft lip is a rare anomaly consisting of a midline vertical cleft through the upper lip. It can also involve the premaxillary bone, the nasal septum, and the central nervous system. In our current report, we present the clinical features of 6 patients with a median cleft lip and their surgical management according to the accomp...
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This article provides an overview of timeline of interventions and the critical role different providers have in the continuum of cleft lip and palate care. The earliest intervention is the presurgical infant orthopedic treatment, which is initiated in the first few weeks of life. This is followed by several interventions done in a phased manner. T...
Citations
... Cleft surgeons will rarely encounter cases during a career and thus a reliable approach to treatment is important. Although methods of repair have been described [2][3][4][5][6], specific technical details are lacking. ...
Midline clefts of the upper lip are rare, and it is therefore important that surgeons have access to a methodical approach for when these presentations are encountered. We adapted principles of the anatomic subunit approximation for unilateral cleft lip, to the repair of midline clefts. The overt use of anatomic landmarks to define the repair results in a design that inherently adjusts to varying degrees of clefts and can accommodate asymmetries. The "measure twice, cut once" style is an advantage to new surgeons and to surgeons who seldom encounter this presentation. We describe the details of surgical repair in the context of a patient with Pai syndrome and associated nasal hamartomas that resulted in nasolabial asymmetry. This is the first report of surgical outcome following treatment of Pai syndrome and includes early and 5-year follow-up. The system of repair that we describe is applicable to both symmetric and asymmetric midline clefts.
... The developmental error that results in midline facial defects usually occurs during the 3 rd week of gestation. [3] Median cleft lip occurs due to failure of fusion of the frontonasal process with maxillary process. ...
Median cleft lip with nasal dermoid is a very rare occurrence. Midline nasal dermoid results from the abnormal fusion of embryological processes. They can present as asymptomatic midline nasal swelling, infection, or meningitis due to intracranial extension. Median cleft lip also results from failure of fusion of embryological processes and can be complete or incomplete or associated with other congenital anomalies or as a part of syndrome. We present a rare combination of both in a 7-month-old female child who was surgically treated.
... Midfacial clefting is estimated to occur in approximately 5/100,000 live births [35,36]. In human patients, the phenotypic spectrum ranges from a small midline notch of the upper lip to a cleft that separates the central incisors, the medial alveolar ridge, the philtrum, and the nasal septum. ...
Human midfacial clefting is a rare subset of orofacial clefting and in severe cases, the cleft separates the nostrils splitting the nose into two independent structures. To begin to understand the morphological and genetic causes of midfacial clefting we recovered the Unicorn mouse line. Unicorn embryos develop a complete midfacial cleft through the lip, and snout closely modelling human midfacial clefting. The Unicorn mouse line has ethylnitrosourea (ENU)-induced missense mutations in Raldh2 and Leo1. The mutations segregate with the cleft face phenotype. Importantly, the nasal cartilages and surrounding bones are patterned and develop normal morphology, except for the lateral displacement because of the cleft. We conclude that the midfacial cleft arises from the failure of the medial convergence of the paired medial nasal prominences between E10.5 to E11.5 rather than defective cell proliferation and death. Our work uncovers a novel mouse model and mechanism for the etiology of midfacial clefting.
... Therefore, surgery is performed to treat most patients with true median cleft lip. True median cleft lip ranges from severe cases with bifid nose to mild cases limited to only part of the lips [3]; however, surgical procedures reported to date have mostly been designed for midline suture closure [1,[4][5][6][7][8][9]. Although scarring cannot be avoided in patients with severe lip and nose deformation, in mild cases, it is preferable to not scar the midline of the white lip. ...
... Topkara [8] reported a method for patients with cleft lip extending to the lower half of the philtrum, whereby the columella is extended using a V-Y advancement flap without tissue excision, and the upper lip is lengthened upon matching triangular flaps from cleft margin of the bilateral red lips. Koh [9] conducted inverted U excision in four patients and reported that the midline scar was unremarkable. All of these surgical procedures leave a midline suture line, and there are no reports wherein the bilateral cleft lip design has been applied. ...
Objective:
Median cleft lip is an extremely rare congenital abnormality that can be classified into "true" (characterized by true tissue separation) and "false" (caused by holoprosencephaly). We report a patient with true median cleft lip who underwent cheiloplasty using the surgical procedure for bilateral cleft lip.
Methods:
The subject was a girl, who presented with mild orbital hypertelorism. Alveolar cleft with midline cleft lip extending slightly to the white lip, and anterior basal meningocele was observed. The patient was in good general condition and had undergone cheiloplasty using modified DeHaan's method at 5 months of age.
Results:
In many reports to date, surgical procedures for median cleft lip could not avoid leaving a scar on the midline; however, in the present case, we were able to achieve a clear Cupid's bow with an unremarkable scar consistent with the philtral columns. Furthermore, a nostril sill had formed in the flat base of the nasal cavity. The morphology of the opened nostril base also improved.
Conclusion:
This procedure is only indicated for patients with mild cleft. However, better esthetic surgical outcomes are generally requested for milder original deformations. This surgical procedure was effective in esthetically improving the median cleft lip.
... Incidence of median cleft of the lip or Tessier number 0 cleft is reported to be about 0.43% to 0.73% of the craniofacial cleft population, and it occurs in 1:1,000,000 live births. [1,2] Median cleft lip is defined as congenital vertical craniofacial cleft through the center of the upper lip. These are classified as Tessier's 0 craniofacial clefts. ...
Median cleft lip is a midline craniofacial cleft through
upper lip. The incidence of craniofacial clefts is about
1:1,000,000 of live births. It indicates the rarity of this
anomaly. Hence, this series is being presented. From
January 2010 to December 2017, eight cases of the
median cleft of upper lip attended the cleft clinic based
in rural India. All eight patients had partial midline cleft
lip. One patient had abnormal soft tissue mass and one
patient had hypertelorism with nasal deformity. All of
them underwent surgical repair of median cleft lip using
inverted‑U incision along with repair of orbicularis
oris muscle in the midline. The postoperative
symmetry was achieved in 100% of patients with
good reconstruction of the philtrum. The associated
deformities were left to be managed in second stage.
A series of eight cases of partial midline cleft lip has
been presented with good surgical outcome.
Objective
Children with hypopituitarism (CwHP) can present with orofacial clefting, frequently in the setting of multiple midline anomalies. Hypopituitarism (HP) can complicate medical and surgical care; the perioperative risk in CwHP during the traditionally lower risk cleft lip and/or palate (CL/P) repair is not well described. The objective of this study is to examine the differences in complications and mortality of CL/P repair in CwHP compared to children without hypopituitarism (CwoHP).
Design
A retrospective cross-sectional analysis.
Setting
The 1997 to 2019 Kids’ Inpatient Databases (KID).
Patients
Children 3 years old and younger who underwent CL/P repair.
Main Outcome Measure(s)
Complications and mortality.
Results
A total of 34 106 weighted cases were analyzed, with 86 having HP. CwHP had a longer length of stay (3.0 days [IQR 2.0-10.0] vs 1.0 day [IQR 1.0-2.0], P < .001) and higher rates of complications and mortality (12.8% vs 2.9%, P < .001) compared to CwoHP. Controlling for demographic factors, CwHP had 6.61 higher odds of complications and mortality than CwoHP (95% CI 3.38-12.94, P < .001).
Conclusions
CwHP can present with a CL/P and other midline defects that can increase the complexity of their care. These data show a significant increase in length of stay, complications, and mortality in CwHP undergoing CL/P repair. Increased multidisciplinary attention and monitoring may be needed for these children peri- and postoperatively, especially if additional comorbidities are present. Further studies on perioperative management in this population are warranted to reduce morbidity and mortality.
Oral-facial-digital syndromes (OFDSs) represent a heterogenous group of embryonic development disorders characterized by malformations of the face, oral cavity, and extremities. Oral-facial-digital syndrome type II is an autosomal recessive disease characterized by median cleft lip, gingival frenula, cleft lobulated tongue, and polydactyly. There are few reports on surgical techniques for correction of incomplete median cleft lip. Here we describe a novel surgical method that we used to correct an incomplete median cleft lip in a 5-year-old girl with oral-facial-digital syndrome type II. She had previously undergone surgery for congenital heart disease, oral anomalies, and polydactyly. Cheiloplasty was performed at 5 years and 8 months using a surgical approach that focused on repair of the median tubercle using lateral labial elements. A reasonably good Cupid's bow and median tubercle were achieved. Our technique for surgical correction of moderate incomplete median cleft lip provides adequate philtral height, vermillion fullness, and a good-shaped Cupid's bow.
The lips are two flaps surrounding the oral orifice. As they are located at the entrance to the digestive tract, the external surface is covered with skin and the internal surface with mucosa. Externally, the appearance of the lip can vary because of syndromes affecting the face. Lip shape is usually defined by the shape of the philtrum. The lateral border of the lip and the nasolabial fold differ among individuals in shape and length. Internally, the labial salivary glands can be affected by disease and aging. The muscles in the lips can also be altered by certain diseases. In this chapter, we describe the anatomy and variations of the lip.
