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Pediatric Surgical Procedures – An Updated Guide – Volume I
31
mandibular setback alone [47]. However, the planning and execution of the osteotomies and fixation have to be more meticulous.
.. Secondary deformities
Despite advancements in cleft care and the development of excellent comprehensive care centers, most patients, especially in developing countries with clefts,
still need to receive ideal treatment as per the current international protocols [48].
Surgeons need to understand the longevity and profound impact of poorly performed
surgeries. Secondary deformities of the cleft lips are a rule rather than an exception
[48]. Even after the most perfect initial repair, deformities may occur due to scar
maturation and growth. Many children do not undergo any presurgical orthopedics/
orthodontics or subsequent dental and maxillofacial treatment, thereby aggravating
the secondary deformities [48].
The treatment should ideally start with presurgical orthopedics followed by cheiloplasty, palatoplasty, continued orthodontic treatment, preschool minor revisions,
distraction of midface when necessary, rhinoplasty, and orthognathic surgery as
required in adulthood [48]. Throughout this treatment, the patient’s result is dynamically influenced by growth. The final result of any cleft surgery is hence evident only
after the child’s growth is complete. Therefore, it is difficult to envisage a scenario
without secondary cleft lip deformities.
Secondary deformities of the lip are classified as scar-related, lip-related,
white roll-related, vermillion-related, philtrum-related, muscle-related, or
asymmetry of the structures. Scar-related issues may be a wide scar, hypertrophic
scar, contracted scar, scar with cross hatches/suture marks, and hyperpigmented/
hypopigmented scar. Lip-related problems may be the presence of a long lip,
short lips, or tight lips. The white roll may be misaligned, scarred, or thinned.
Vermillion may be misaligned, notched, thinned, or deformed (“whistle lip deformity”). Besides being unsightly, lack of muscle repair or poor muscle alignment
leads to shallow buccal sulcus or absence of a sulcus, causing an adherent lip.
Philtrum, being the central element of the lip, is very apparent when it is wide,
narrow, or absent. The secondary deformities must be corrected surgically whenever the patient presents, as they cause ridicule of the child in school, and most of
these deformities worsen with the child’s growth.
.. Secondary rhinoplasty
After a balanced and symmetric skeletal base is achieved, secondary rhinoplasty is
performed. No single technique, to date, has provided a definite solution for correcting all the problems accompanying these deformities. Secondary rhinoplasty can be
performed using an open or closed technique. The open approach is preferred for
better exposure and visualization of the nasal elements. An open technique refers
to lifting the skin off the nose to operate, while a closed technique is done through
tiny incisions. Transcolumellar and infracartilaginous incisions are most frequently
employed to visualize the lower lateral cartilages and the nasal septum.
The usual steps required in definitive rhinoplasty are septum straightening, tip
elevation, columella lengthening, alar rim augmentation and dorsum augmentation
with cartilage grafts, alar base augmentation and repositioning, correction of vestibular webs, elevation of nostril sill and nasal bones osteotomy. The extent of surgery
needed is tailor-made to each patient’s deformity.
Cleft Lip/Palate: Hidden Aspects beyond the Gap
DOI: http://dx.doi.org/10.5772/intechopen.115578
32
The usual steps are as follows. The open dorsal approach with tip-takedown is used
to expose the septum. The septum is partially resected, leaving an L-shaped strut
for dorsal caudal support. The remaining septum is repositioned in the midline and
sutured to the periosteum of the anterior nasal spine. Submucous resection of the
hypertrophied inferior turbinate may be required to improve the nasal airway. The
inferiorly displaced cleft-side alar cartilage is advanced medially and sutured to its
counterpart on the non-cleft side to obtain a balanced nasal tip. Placement of columellar strut graft, along with suturing of the medial and middle crura of both the alar
cartilages to the tip of the strut, achieves tip projection, symmetry, and support [11].
The placement of cartilage grafts for support and reinforcement is a significant
element of the cleft rhinoplasty surgery. Autologous cartilage grafts, from septum,
costa, and auricle, are used for augmenting deficient areas and restoring structural
support in the nose. Splinting the alar rim with cartilage grafts is required, as the cleft
cartilage is too weak to withstand the deforming forces of the depressed dorsal skin
mold, gravity, and growth. Patients with internal nasal valve dysfunction may benefit
from a spreader graft. Patients may need lateral osteotomies to narrow the dorsum, or
straighten a deviated nasal pyramid. If lateral osteotomies do not adequately narrow
the dorsum, a central segment is removed to create an open roof via paramedian osteotomies [11]. The nasal bones are, then, in-fractured to achieve adequate narrowing.
Nasal bone osteotomies are performed either percutaneously with a 2-mm osteotome
or transnasally.
. Outcome
. Outcome analysis
CLP impacts many patient life domains, including appearance, speech, social
integration, self-image, and physical and psychological functioning. Poor facial
esthetics significantly affect health-related quality of life in people with repaired
CLP. Investigators have used questionnaires about quality of life to highlight the
disparity between functional outcomes and patient satisfaction. Young adult women
report poorer satisfaction with their appearance, without any significant difference
in functional outcomes of biting, chewing, swallowing, and speech comprehensibility. Presently, instruments related to one domain, such as speech, are being used to
evaluate the impact of CLP on a person [49]. A free-to-use cleft-specific instrument
that considers all CLP domains is sorely needed.
More comprehensive, valid, and reliable questionnaires for CLP surgery must be
created [49]. The measurement instruments that have been validated in CLP are as
follows: craniofacial-specific measures (Youth Quality of Life–Facial Differences),
voice-related measures (Patient Voice–Related Quality of Life and Cleft Audit
Protocol for Speech–Augmented), and oral health-related measures (Child Oral
Health Impact Profile and Child Oral Health Quality of Life) [49].
The Youth Quality of Life–Facial Differences questionnaire consists of 48 questions across five realms: stigma, negative self-image, positive consequences, negative
consequences, and coping. It is applied to children 11 to 18years old. The Pediatric
Voice-Related Quality of Life survey is a parent proxy instrument (Table ). It is a
10-item instrument that measures social-emotional and physical-functional aspects
of voice and speech issues. The Cleft Audit Protocol for Speech–Augmented is
designed for parents of CLP children and measures 14 speech parameters, including
Pediatric Surgical Procedures – An Updated Guide – Volume I
33
Because of my child’s voice, how much of a problem is this?
S. No. Question None, not
1 My child has trouble
speaking loudly or being
heard in noisy situations.
2 My child runs out of
air and needs to take
frequent breaths when
talking.
3 My child does not know
what will come out when
he or she begins speaking.
4 My child is sometimes
anxious or frustrated
(because of his or her
voice.)
5 My child sometimes gets
depressed (because of his
or her voice).
6 My child has trouble
using the telephone or
speaking with friends in
person.
7 My child has trouble
doing his or her job or
schoolwork (because of
his or her voice).
8 My child avoids going out
socially (because of his or
her voice).
9 My child has to repeat
himself or herself to be
understood.
10 My child has become less
outgoing (because of his
or her voice)
a problem
1 2 3 4 5 6
A small
amount
1 2 3 4 5 6
1 2 3 4 5 6
1 2 3 4 5 6
1 2 3 4 5 6
1 2 3 4 5 6
1 2 3 4 5 6
1 2 3 4 5 6
1 2 3 4 5 6
1 2 3 4 5 6
A moderate
amount
A lot Problem is
“as bad as
it can be”
Not
applicable
Table 4.
Pediatric voice-related quality of life survey.
intelligibility, voice, hypernasality, hyponasality, nasal emission, nasal turbulence,
nasal friction, grimace, non-cleft speech errors, specialist intervention.
The Child Oral Health Impact Profile assesses self-reported oral-facial well-being
in children aged 8–15. It consists of 34 items in five domains: oral health, functional
well-being, social/emotional well-being, school environment, and self-image [49]. The
Child Oral Health Quality of Life questionnaire measured self-reported oral healthrelated quality of life in children aged 11–14, including oral symptoms, functional
limitations, emotional, and social well-being (Table ). A shorter 16- and 8-item
questionnaire, of the original 37-item questionnaire, is used in clinical settings [49].
Cleft Lip/Palate: Hidden Aspects beyond the Gap
DOI: http://dx.doi.org/10.5772/intechopen.115578
34
S. No. Questions Never Hardly
1 Have you had to avoid eating some foods? 0 1 2 3 4
2 Have you found it difficult to relax? 0 1 2 3 4
3 Have you felt depressed? 0 1 2 3 4
4 Have you been upset? 0 1 2 3 4
5 Have you felt uncomfortable about the
appearance of your teeth, mouth or
dentures?
6 Have you been worried about dental
problems?
7 Have you had trouble getting along with
other people?
8 Have you avoided going out? 0 1 2 3 4
9 Have you been unable to function? 0 1 2 3 4
10 How often did you feel nervous or self-
conscious because of problems with your
teeth, gums or dentures?
11 How much pain or distress has your teeth
or gums caused you?
12 Have you had uncomfortable dentures? 0 1 2 3 4
0 1 2 3 4
0 1 2 3 4
0 1 2 3 4
0 1 2 3 4
0 1 2 3 4
ever
Occassionally Fa irl y
often
Table 5.
Child oral health quality of life questionnaire.
Ve r y
often
Figure 9.
Blue dashed lines indicate brow length and red dashed lines indicate WIZDOM. Yellow solid lines are for
WIZDOM-lateral brow and eye length. Blue solid lines are for WIZDOM-Medial Canthus and Medial Canthusnasal ala. Green line depicts WIZDOM-chin diagonal.
Pediatric Surgical Procedures – An Updated Guide – Volume I
35
. Midface beauty
The extent of abnormal growth of the mid-face varies from mild to severe. It is
affected by genetics, cleft severity, timing of surgery, skills of the surgeon, and the
number of revision surgeries performed.
There are no universally accepted parameters of mid-face beauty. Various surgeons
have given some ways to describe an esthetically pleasing face. Little described the
face as an ogee (a curvilinear silhouette in art and architecture). The soft tissues of the
ideal youthful mid-face form an ogee, in the oblique view. Ramirez muses the whole
youthful face looks similar to a double ogee curve, with the convexity of the upper
ogee at the lateral brow and the lower ogee at the upper mid-face.
Bartlett and Whitaker describe the bizygomatic distance as the broadest portion of
the face [50]. The bitemporal and bigonial distances are approximately equal and 10%
less than the bizygomatic distance [50]. Linkov et al. have defined the line connecting
the right and left zygomaxillary points as WIZDOM (Width of the Interzygomatic
Distance of the Midface), where 1.6xWIZDOM was the facial length (hairline to the
chin in the midline) (Figure ) [50].
Figure 10.
Stepwise management plan.
Cleft Lip/Palate: Hidden Aspects beyond the Gap
DOI: http://dx.doi.org/10.5772/intechopen.115578
. Conclusion
36
CLP is a common congenital anomaly. It is the cause of many esthetic, functional,
and psychosocial issues [11]. The needs of the affected children must be acknowledged, and measures must be tailored to address them. This chapter summarizes the
relevant clinical information for the diagnosis and management of children born with
cleft anomalies. Table
in children born with CLP. It has been shown in multiple studies that the quality of
life of families of children born with CLP is impacted negatively, the degree worsening with the severity of cleft [51]. Other studies show that the family’s quality of life
improves significantly with appropriate management [52]. The holistic care of the
cleft child and the family and their harmonious integration into society is the ultimate
goal of all treatment.
and Figure summarize the age-wise management plan
Pediatric Surgical Procedures – An Updated Guide – Volume I
37
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