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Pediatric Surgical Procedures – An Updated Guide – Volume I
Tailoring personalized analgesic strategies, considering the age and individual char-
11
acteristics of each patient, reinforces the integral role of pediatric anesthesiologists in
postoperative care. Interdisciplinary collaboration and specific expertise in pediatrics
anesthesiology are essential to address the unique challenges of laparoscopic surgery
in children. The presence of pediatric anesthesiologists not only ensures safety and
stability during the procedure but also contributes significantly to a more comfortable and satisfactory recovery for the pediatrics patient undergoing laparoscopic
procedures.

Importance of Anesthesia in Pediatric Laparoscopic Procedures
DOI: http://dx.doi.org/10.5772/intechopen.115024
12
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Pasqua N, Maggio G, Brunero M, et al.
Pediatric laparoscopy and adaptive
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Pediatric Reports. 2017;(2):7214
[2] Jiang R, Sun Y, Wang H, Liang M,
Xie X. Effect of different carbon dioxide
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Medicine. 2019;(41):e17520
[3] Kim SJ, Barlog JS, Akhavan A.
Robotic-assisted urologic surgery in
infants: Positioning, trocar placement,
and physiological considerations.
Frontiers in Pediatrics. 2019;:411
[4] Brady MC, Kinn S, Ness V, O'Rourke K,
Randhawa N, Stuart P. Preoperative
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[5] Li-Wei L. y cols, influence
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pneumoperitoneum on neonate circulation
and respiration. Journal of International
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[6] Jackson HT, Kane TD. Advances in
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patients. Advances in Pediatrics.
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[7] Tuna AT, Akkoyun I, Darcin S,
Palabiyik O. Effects of carbon dioxide
insufflation on regional cerebral
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Brazilian Journal of Anesthesiology.
2016;(3):249-253
[8] Heuttemann E, Sakka SG, Petrat G,
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[9] Chou CM, Yeh CM, Huang SY, Chen HC.
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[10] Fujimoto T, Segawa O, Lane GJ,
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[11] Shi Y, Hanson AC, Schroeder DR,
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general anaesthesia. British Journal of
Anaesthesia. 2022;(2):294-300
[12] Kangralkar G, Jamale P. Sevoflurane
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[15] Bai W, Golmirzaie K, Burke C, Van
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the emergency department. Pediatric
Anesthesia. 2016;(4):384-391
[16] Napolitano N, Laverriere EK,
Craig N, Snyder M, Thompson A,

Pediatric Surgical Procedures – An Updated Guide – Volume I
13
Davis D, et al. Apneic oxygenation As a
quality improvement intervention in an
academic PICU*. Pediatric Critical Care
Medicine. 2019;(12):e531-e537
[17] Crulli B, Kawaguchi A, Praud JP,
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ventilation. Critical Care. 2021;(1):229
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[23] Papparella A, Noviello C, Ranucci S,
Paciello O, Papparella S, De Biase D,

Chapter 2
14
Cleft Lip/Palate: Hidden Aspects
beyond the Gap
ShilpiKarmakar and Pawan KumarDixit
Abstract
Cle
ft lip/palate is a common congenital anomaly, with 1 in 700 newborns afflicted.
Most surgery textbooks teach standard techniques for cleft lip/palate repair. However,
there is much deeper science and art behind eradicating the stigma of the cleft. The
cleft nose, vermillion, maxilla, and voice are giveaways of a good-but-not-great
surgery. Similarly, the failure to address the maxillary hypoplasia, the dentition,
themaxillary arches, the hearing, and the speech are common occurrences. This
chapter will highlight the broad spectrum of cleft lip/palate conditions and the “why,”
“when”, and “how” to address these deformities. Management of cleft lip/palate is a
multidisciplinary task, with the child’s welfare at its center.
Keywords: maxilla, cleft lip, cleft palate, rhinoplasty, nasal bone,
nasoalveolar molding, orthopedics, malocclusion
. Introduction
Werner Hagedorn said, “Great things are done when art and science meet.”
Cleft care is a discipline where art and science blend to create magic. Adding the
elixir of “timing” and “dedicated teamwork” leads to mesmerizing magic of galactic
proportions.
One in 700 newborn babies are born with cleft lip and palate (CLP) worldwide.
The numbers vary for sex, race, and region [1]. The multifactorial etiology occurs
due to variations during the embryo’s normal development. The regular events of the
development of the face are well described in the literature, and the reader is referred
elsewhere to understand the same.
Besides the visibly apparent defects of the lip and palate, children affected with
CLP also have distortion and dysfunction of the velum, eustachian tubes, hearing
apparatus, speech apparatus, nose, maxilla, and dentition. These all cumulatively lead
to inadequate physical development and psychological trauma. The malady affects not
only the child but also the parents and the family, who struggle with a sense of guilt
and helplessness. The management of CLP, thus, requires a dedicated team of healthcare providers and interventions that are timely and intelligent. The skilled care of the
CLP child occurs throughout the individual’s growth period.

Pediatric Surgical Procedures – An Updated Guide – Volume I
15
. Health concerns in a child born with CLP
. Lip defect
There is a deficiency of upper lip tissue comprising orbicularis oris, skin, mucosa,
and vermillion. The fibers of orbicularis oris are discontinuous and abnormally
attached to the cleft margin, maxilla, and alar base. These abnormal attachments lead
to widening of the cleft gap as the child grows. The bilateral cleft lip has a deficiency
of orbicularis oris in the central premaxilla segment. Thus, the absence of muscular
reining forces manifests in the protrusion of the premaxilla.
During the repair of the cleft lip, the orbicularis oris is freed from all anomalous
attachments and sutured across the cleft. The height and thickness of the orbicularis
need to be maintained while repairing for adequate cosmesis.
. Defect in palate
There is a deficiency of palate tissue, comprising horizontal plate of maxilla bone,
palatine bone, oral mucosa, nasal mucosa, muscles of soft palate (levator veli palatine
(LVP), tensor veli palatini (TVP), musculus uvula, palatopharyngeus and palatoglossus). As in the cleft lip, the fibers of these muscles are abnormally attached to the cleft
margin and posterior border of the hard palate. During the repair of the hard palate,
closure of oral and nasal mucosa is not enough. The abnormal attachments of the
muscles must be released, and the LVP of both sides must be repaired.
The LVP is a crucial member of the velopharyngeal sphincter complex. While
repairing the LVP, it must also be positioned in the more anatomical posterior part
of the velum. Positioning is vital to prevent nasal air leaks while speaking and fluids
while swallowing.
. Nutrition
Feeding is a significant challenge for the parents of CLP babies. In normal children,
during sucking, the lips form a seal over the mother’s breast as the velum closes the
nasopharynx. In this closed cavity, movements of the tongue and mandible in relation
to the palate generate a suction force. The cleft in the lip prevents the formation of the
lip seal [2]. This is overcome by a child with a cleft lip (CL) by using the alveolar arch. A
cleft palate (CP) precludes the formation of the velopharyngeal seal. This leads to weak
suction pressure while feeding and milk regurgitation through the nose. The attempts
at breastfeeding are also exhausting for the CLP baby. Over time, with inadequate
breast milk expression, there is gradual lactation suppression. The parents resort to
formula feeding or diluted cow’s milk, which does not have sufficient nutrient value.
The CLP child, thus, gets entangled in a vicious circle, where the result is malnutrition, failure to thrive, anemia, hypoproteinemia, and low weight gain. All this is happening while the infant’s brain is undergoing maximal development. Nutritional insult at
this stage has devastating consequences for the physical and mental growth of the child.
. Hearing
The eustachian tube connects the nasopharynx to the tympanic cavity, and its
nasopharyngeal opening is regulated mainly by the TVP. The LVP functions as a tube
dilator. The eustachian orifices usually are closed at rest and open with yawning,

Cleft Lip/Palate: Hidden Aspects beyond the Gap
DOI: http://dx.doi.org/10.5772/intechopen.115578
16
swallowing, and speech. In CP children, the anomalous insertions of the TVP and
LVP result in the replacement of the isotonic contractions by isometric contractions.
Thus, the eustachian tube opening and middle ear ventilation are affected [3, 4].
The abnormal nasal reflux of feeds instigates an inflammatory reaction, resulting in
edema around the tubal orifice. The variations of the craniofacial skeleton, intrinsic abnormalities of the tubal cartilage framework, misaligned and dysfunctional
peritubal musculature, repeated respiratory infections, and hypertrophied adenoids
all add insult to injury and further block the orifice of the eustachian tube [5–7].
Thus, when absorbed, the gases in the middle ear are not replaced, resulting in a
negative pressure and tympanic membrane retraction. The negative pressure leads to
fluid secretion from the mucous membrane into the middle ear, causing an effusion.
Recurrent effusions may get infected and lead to suppurative otitis media. Recurrent
or chronic middle ear disease can eventually result in conductive hearing loss [2].
Conductive hearing loss further exacerbates speech and learning problems in CLP
children.
The incidence of middle ear disease is as high as 90% in children with CP, 45% of
whom suffer from recurrent ear infections [8]. More than 50% of adolescents and
adults with unoperated CP have developed a hearing loss of more than 15dB [8].
. Speech
The motor aspect of speech includes phonation (involving the larynx), articulation, and resonance. Articulation results from muscular activity of the lip, tongue,
and soft palate. Resonance involves the mouth, nose, paranasal sinuses, pharynx, and
chest cavity [2]. The consonants are categorized depending on whether the articulators are closed (plosives) or narrowed (fricatives) at a specific point.
The cleft of the lip and alveolus only affects the related consonants. The gap in the
palate results in nasal emission of air, incompetence of the velopharyngeal sphincter,
and defects in articulation. This is responsible for the nasal intonation, primarily of
the vowels—the typical hypernasal cleft palate speech or audible nasal emissions or
frictations [2, 9]. Extensive scars (due to messy surgery) in the soft palate also interfere with the closure of the velopharyngeal apparatus. The fistula in the hard or soft
palate allows nasal air to escape with speech, leading to altered vowel quality, audible
nasal emission, indistinct consonant production, and substitution mechanisms [10].
. Maxilla and alveolus
The affected side maxilla is generally underdeveloped and hypoplastic, and surgical intervention negatively influences further growth. InCLP, the alveolar segment
is displaced laterally—pushed by the tongue and pulled by the anteriorly attached
muscle. In addition, the posterior arch is pushed posteromedially by cheek pressure.
These forces misalign the alveolar arch [11]. Thus, the maxillary segment is displaced
in the frontal, sagittal, and vertical planes, with the larger segment retroposition
upward and outward and the lateral segment collapsing and displaced medially [2].
In bilateral CLP, the excessive premaxillary protrusion is directed more horizontally, resulting in a prominent central prolabial segment of the lip. This is due to the
forward expansion of the alveolar process by unrestrained bone growth at the suture
between the vomer and premaxilla, the growth of cartilaginous nasal septum due to
facial muscle and maxillary bone disruption, and associated underdevelopment of the
maxillary segments [2].

Pediatric Surgical Procedures – An Updated Guide – Volume I
17
Cleft of the alveolus affects the number, position, shape, size, time of eruption,
and crown/ root formation of the deciduous and permanent teeth in and adjacent to
the cleft. Also, the alveolar cleft between the maxillary lateral incisor and the canine
tooth roots increases from the incisal edge to the apices of the teeth and is widest at
the nasal cavity [2].
. Dentition
InCL, the incisors may be excessively proclined due to incontinence of the orbicularis muscle or retroclined due to “too tight” lip closure [2]. The number and position
of teeth are affected. CLP children are more prone to develop caries. Compounded to
it is the insult induced by surgical procedures. Palate repair procedures are known to
reduce the vascularity of the alveolar mucoperiosteum.
Malocclusion is common in CLP patients and is somewhat related to the severity
of the cleft and alignment of the arches. Intrinsic maxillary sagittal growth inhibition leads to anterior crossbite. Transverse deficiency presents with crowding, lateral
crossbite, and open bite. Surgical scars greatly restrict the sagittal and transverse
growth [2].
. Nose
Along with CLP, there is an innate deficiency of the maxilla and cleft in the floor
of the nose. The bone platform, cartilage support, lining, and skin are inherently
dissimilar. There is an inherent discrepancy in the skin cover, cartilage support, nasal
lining, and bony platform. Added to it is the anomalous insertion of the transverse
muscle of the nose and the orbicularis oris around the nasal septum and nasal spine
and the contractions of the contralateral normal muscle [2]. These cause the development of a peculiar set of nasal abnormalities, described below. To these deficiencies is
added the insult of growth.
In unilateral CLP, the nasal tip is deviated to the non-cleft side (
Figure ). The nasal
spine is positioned on the floor of the normal nostril, as the inferior edge of the nasal
septum may be anteriorly dislocated from the vomerine groove. The vertical height of
the columella on the cleft side is reduced and is slanted obliquely. The alar cartilage is
attenuated, flattened, and dislocated from the tip [2]. The lateral crus is spread at an
obtuse angle with respect to the medial crus. The medial crus lies lower in the columella.
The dome is retroplaced. The overhanging alar rim forms a web as it lacks cartilage. Due
to a lack of bony support, the alar base is asymmetrical, lying inferior and posterior, with
an outward flare and no alar-facial groove definition. The cleft side nostril has a transverse axis and a circumference greater than the normal nostril [2, 12].
In bilateral cleft lip nasal deformity, the nose is flared with a broad, depressed, flat
nasal tip and a markedly shortened columella (Figure ) [11]. The columella, caudal end
of the septum, and the anterior nasal spine are displaced inferiorly in relation to the level
of the alar bases. The bilateral alar cartilages are severely deformed and dislocated off the
septum [11]. The medial crura is short and widely separated at the tip, the lateral crura is
flat and elongated, and there is an obtuse angle between the crura and the dome [2, 12].
The alar bases are displaced laterally and sometimes inferiorly and posteriorly. The often
asymmetric nostrils are horizontally oriented, and the nasal floor may be absent [2].

Cleft Lip/Palate: Hidden Aspects beyond the Gap
DOI: http://dx.doi.org/10.5772/intechopen.115578
18
Figure 1.
Nasal deformity in unilateral cleft lip.
Figure 2.
Nasal deformity in bilateral cleft lip.

Pediatric Surgical Procedures – An Updated Guide – Volume I
19
. Growth of the child
Poor feeding, recurrent ear and airway infections, intrinsic growth deficiency,
decreased levels of growth hormone, and repeated surgeries contribute to unsatisfactory growth of the child. There is poor weight gain in early life and a general lag in
the growth curve, with a transient growth retardation. In late childhood, the height
and weight are decreased compared with normal children, and puberty is delayed by
6months with reduced velocity of skeletal growth [11]. However, longitudinal studies
reveal that the average growth usually returns to normal by 4years of age initially and
again after puberty, which may be prolonged by up to 1year [2].
. Confidence
Teasing over facial appearance is commonly reported and has been found to result
in poor psychological functioning in individuals with CLP [13]. Anxiety, depression,
and low self-esteem are common among affected adult individuals [14]. Job opportunities and the marriage market are relatively constricted for CLP individuals when
compared to the average population [14].
. Parental distress
After the birth of an infant with CLP, the parents are usually shocked at the first
sight of their child’s face [15]. The family’s situation is classified as a “psychosocial
emergency,” characterized by disappointment, helplessness, and desperation, which
may lead to a period of severe emotional crisis for the parents [15]. Parents often feel
guilty about the malformation and are concerned about their child’s future. Parents
of CLP may develop a feeling of chronic disappointment, which parents of physically
disabled children are often seen to struggle with [15].
. Classification systems of CLP
Davis and Ritchie gave the first classification for CLP, considering the alveolar
process as the foundation. Group I was Prealveolar, Group II was Postalveolar, Group
III was Unilateral alveolar cleft, and Group IV was Bilateral alveolar cleft. This classification was neither based on anatomy nor embryological development. Kernahan
and Stark designated the incisive foramen as the dividing point between primary and
secondary palates. This correctly described the deformity. Vilar-Sancho classified
clefts based on Greek nomenclature. Lip was shown by “K” (keilos), alveolus by “G”
(gnato), hard palate by “U” (urano), and soft palate by “S” (stafilos). Complete cleft
was represented in capitals and partial in small letters. “2” was used to represent
bilateral, “d” indicated right, “l” indicated left, an “I” indicated incomplete, and “o”
indicated operated. Being in Greek, it could not be used by the rest of the world [16].
Spina used the incisive foramen as a reference point and divided clefts into four
groups: Group I – Preincisive foramen clefts, Group II – Transincisive foramen
clefts, Group III – Postincisive foramen clefts and Group IV – Rare facial clefts.
Each group had unilateral, bilateral, and median; each group was subdivided
into total and partial. The International Society adopted this for Plastic and
Reconstructive Surgery. Kernahan proposed the “Y” classification, drawing nine
boxes with nasopalatine foramen as the central point. This classification was a

Cleft Lip/Palate: Hidden Aspects beyond the Gap
DOI: http://dx.doi.org/10.5772/intechopen.115578
20
diagrammatic or symbolic representation of the cleft deformity and was used very
effectively for documentation or charting. It could not be used for writing the diagnosis in the case file, verbal communication for the text’s description, or computer
archiving [16].
In 1989, Kriens proposed LAHSHAL, an abbreviated documentation system. Lip
(L), alveolus (A), hard palate (H), and soft palate (S) were used to form LAHSHAL.
The Indian classification was given by Balakrishnan and is widely accepted now. It
classifies almost all the combinations of clefts encountered, and a brief notation can
represent it. Gp 1 indicates cleft lip, with the suffix “a” indicates cleft alveolus. Gp 2
indicates cleft palate. Gp 3 indicates cleft of lip and palate.
. Management of CLP
A specialized team manages the CLP throughout the individual’s growth period.
The team of healthcare providers for the CLP child incorporates many specialists,
including a counselor, pediatrician, nutritionist, geneticist, plastic surgeon, maxillofacial surgeon, ear, nose and throat specialist, orthodontist, prosthodontist, speech
pathologist, speech therapist, social worker and nurse (Table ) [17]. The quality
of life of the family on the whole, as measured by the “Impact on Family Scale”, is
Age Issues Management Specialities
Antenatal Detection Family and genetic counseling Pediatrician
Parental distress Geneticist
Counselor
Birth to
1month
0 to 3months Feeding Feeding and growth assessment Pediatrician
3 to 6months Cleft lip Cheiloplasty, primary rhinoplasty and anterior
6 to 12months Cleft palate Cleft palatoplasty with soft palate muscle
1 to 5years Speech and language
Parental adaptation Family and genetic counseling Pediatrician
Feeding Feeding counseling Geneticist
Other congenital
malformations
Growth and
development
Gap in lip tissues and
nasal deformity
Speech development Speech evaluation Speech therapist
Middle ear effusion Hearing evaluation =/− ventilatory tube
development
Dental abnormalities Velopharnygeal dysfunction assessment and
Hearing abnormalities Dental evaluation Ent surgeon
Complete medical assessment Counselor
Nasoalveolar molding Orthodontist
Presurgical orthopedics Plastic surgeon
Nutritional assessment Nutritionist
Plastic surgeon
palatoplasty (or gingivoperiosteoplasty)
Plastic surgeon
repair
ENT surgeon
insertion
Speech and language evaluation Speech therapist
Plastic surgeon
management
Hearing evaluation Orthodontist
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