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Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_537_Библиотеки_им_академика_М_И_Перельмана
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providing an opportunity for the surgeon to simultaneously reconstruct any
nasal defects. The resultant scarring is disguised within the recreated nasal
philtrum using Millard’s technique. It should be noted that wider clefts are
difficult to close with this technique, especially for inexperienced surgeons,
and patients incur a risk of nostril stenosis as the soft tissues heal [38–40].
Tennison’s triangular flap technique can be precisely pre-planned, and
as such is relatively easier for inexperienced surgeons. Surgeons calculate
the difference between the columellar base and Cupid’s Bow peak between
the noncleft and cleft sides, which is equivalent to the cleft width. The
width indicates how much lengthening of the lip is required, and two
triangular flaps should be created (one on each side) when the cleft width is
greater than 6 mm. In patients with cleft width <6mm, a triangular flap is
created from the cleft side, which is then brought medially and sutured to
the incised noncleft side. Wider clefts can be sufficiently reduced with this
technique, although the results tend to be less optimal regarding aesthetic
appearance. Nasal reconstruction is not accomplished within this plane, and
the scar cannot be camouflaged between cosmetic subunits [41, 42] (Fig. 4).
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Fig. 4 An illustrative overview of Millard’s unilateral cleft lip repair [43]
3.3.2 Bilateral Cleft Lip
There are various techniques used for repair of the bilateral cleft lip,
including two-stage procedures and one-stage procedures. Tennison’s
triangular flap technique discussed previously can be utilized here as a one
or two stage correction. Alternatively, Millard introduced a single-stage
repair, which will be detailed here [29].
Incisions are marked along the vermilion border of the clefted lateral lip
segments, which will be used to create bilateral muscle flaps containing oral
mucosa and submucosa. The lip segments are dissected from the maxilla at
their muscular attachments, allowing for medial advancement. At this point,
the alar cartilages of the nose are sutured to the anterior nasal spine (ASA)
medially to reconstruct the nostrils. Upper lip volume at the medial aspect is
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increased by excising and reattaching excess vermilion to the premaxillary
tissues [44, 45].
The lateral lip flaps are fastened to the ASA just below the alar
cartilages, and the orbicularis oris muscle layers are attached medially to
create the perioral sphincter. After the muscles are reattached, a prolabial
flap is advanced inferiorly and sutured deep to the sphincter, allowing for
creation of the philtral dimple. Bilateral forked prolabial flaps are attached
at the midline to create the nasal floor [44, 45].
After surgery, the incisions are cleaned with hydrogen peroxide and
protected with antibiotic ointment. Elbow splints or arm restraints may be
employed to prevent the patient from contaminating or causing harm to the
surgical wound, and feeding should be performed with a bulb syringe to
avoid suckling movement of the healing tissues. After 1week, the sutures
can be removed [29].
3.3.3 Palatoplasty
Palatoplasty for isolated cleft palate repair and cleft lip/palate palatoplasty
is generally performed at 9–15months of age, usually after the lip repair
has been completed. There are a few techniques used, including VeauWardill-Killner V-Y pushback, straight line repair, and Furlow double Zplasty. All of these techniques incorporate levator veli palatini (LVP)
muscle reconstruction, which is of critical importance for its role in palatal
elevation with swallowing. Additional caution is required to ensure the
secure reattachment of LVP, as this helps to avoid velopharyngeal closure
and resultant nasal reflux and speech defects [35, 46, 47].
The Veau-Wardill-Killner V-Y Pushback technique is performed to
lengthen the palate using bilateral mucoperiosteal flaps, which are elevated
and reapproximated along the midline. The nasal mucosa is left exposed
and will close on its own by secondary intent [35, 47].
Straight Line Repair (Two-flap palatoplasty) uses intraoral incisions to
reconstruct mucoperiosteal and mucosal flaps, which will be elevated and
repositioned without lengthening the palate. Mucoperiosteal flaps on either
side of the cleft are dissected from the vomer, and nasal and oral mucosal
flaps are elevated away from the maxillary alveolus, leaving the soft palate
intact. The flaps will be advanced posteriorly toward the soft palate and
rotated medially, after which they are fixated in multiple sutured layers to
the midline. LVP muscles should be dissected from the hard palate and
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reattached posteriorly to the soft palate to reconstruct the muscle sling [35,
47].
Furlow’s palatoplasty utilizes multiple palatal flap layers, allowing for
recreation of the uvula with mucosal flap transposition and levator sling
reconstruction with soft palatal muscle flaps. Hard palate reconstruction is
accomplished using the previously discussed straight line technique. The
transposition of multiple flap layers, also referred to as Z-plasty, allows for
palatal lengthening [48].
After palatoplasty, patients should avoid bottle feeding until the wound
is healed to prevent damage from the nipple. Breastfeeding and spoon or
dropper feeding can commence four hours after the operation, and water is
recommended after any meal to reduce food contamination at the suture
line. Patients should be kept in a lateral or prone position while feeding in
the early postoperative stages, and caregivers should monitor for aspiration,
especially after isolated cleft palate repair [49, 50] (Fig. 5).
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Fig. 5 The various stages of bilateral cleft palate repair are shown in the images [51]
3.4 Outcomes and Complications
Cleft lip and palate repair involves subsequent reconstruction and intensive
orthodontic work later on in life to maintain the surgical results. The
patient’s age at the time of reconstruction is significant, and it is
recommended to perform surgery before age 2 and/or the patient’s language
acquisition [52].
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When performed in younger patients, the risk of palatine fistula
development was relatively lower in some studies compared with
individuals older than 2 [52]. Other studies associate greater degree of cleft
laterality with fistula formation [53]. Palatine fistulas are a relatively
common complication of palatoplasty and present with symptoms of nasal
regurgitation, food particle lodging, and speech impairments. Fistulas
increase the risk of conductive hearing loss compared with individuals that
did not develop this complication [54] (Fig. 6).
Fig. 6 Development of fistula after primary cleft palate repair, indicating a secondary reconstruction
for this patient [55]
The detrimental impact of cleft palate repair on the mid-facial or
maxillary growth is measured by the decreased maxillary arch growth and
circumference trends. It should be noted that the V-Y pushback method
provides patients with relatively successful midfacial growth [56, 57].
Many patients that have undergone surgical repair of cleft lip and/or
palate require subsequent follow up to assess for related functional
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impairments, including difficulty with speech and feeding, hearing loss, and
poor dentition [58, 59]. Criteria used to assess postoperative outcomes are
centered around the patient’s functional abilities, as well as aesthetic
appearance. Earlier studies have focused on the clinician’s assessment to
determine surgical success, although the psychosocial impact on the patient
and their family is equally important [60]. As of 2016, the cleft lip and
palate group of the ICHOM (International Consortium for Health Outcomes
Measurement) introduced specific outcome measures (CLEFT-Q) to assess
the patient and caregiver’s reported outcomes after surgery, which have
been implemented in various care facilities to date [58, 61–64].
CLEFT-Q contains parent-reported and patient-reported subsets of
questions to assess facial function (eating, drinking, breathing), speech,
psychosocial function, health-related quality of life, and appearance. It is
used in conjunction with other surveys, including the NOSE questionnaire
(Nasal Obstructive Symptom Evaluation), Intelligibility in Context
Scale/ICS to assess speech difficulties, and the Child Oral Health Impact
Profile-Oral Symptoms Scale (COHIP-OSS) to assess oral health [65–67].
The difficulty of these surveys is related to the young age of patients
and their potential inability to accurately answer the survey questions.
However, there is some benefit to these assessments when used as a
checklist to identify postoperative issues as opposed to determination of
patient satisfaction [28] 57. From a clinician standpoint, the optimal
postoperative goals of cleft lip and palate repair are to close the open
communication between oral and nasal cavities and provide patients with
sufficient postoperative speech ability (construction of a functional velum)
[68].
Generally, about 15% of patients will experience complications, usually
hypertrophic scarring with cleft lip repair and oronasal fistula with cleft
palate repair. Most patients achieve satisfactory postoperative function and
appearance, although the latter is much more difficult to assess [69–72].
Patients who undergo cleft lip and palate repair at a younger age (less than
18months of age) generally achieve better speech function and cosmetic
outcomes. On the other hand, later repairs tend to allow for increased
maxillary growth despite less than ideal functional and aesthetic outcomes
[72]. Figures 7 and 8 show the preoperative, intraoperative, and
postoperative images of patients with complete cleft palate.
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Fig. 7 Postoperative results of complete cleft palate repair are shown in image C, with the
preoperative appearance (a) and intraoperative view (b) included for reference [73]
Fig. 8 Similar to Fig. 7, perioperative images of a patient with complete cleft palate are shown in the
figure [73]
4 Surgical Reconstruction of Craniosynostosis
As discussed in Sect. 4.2, craniosynostosis occurs when the cranial sutures
between the skull bones fuse prematurely. The resultant perpendicular
growth of the osseous tissues causes increased intracranial pressure (ICP),
incurs damage to respiratory and neurologic systems, and produces visible
aesthetic deformities in affected patients [74, 75].
4.1 Review of Relevant Anatomy
The skull is divided structurally into the upper calvaria and lower base,
which are both composed of multiple bones attached at suture joints. The
calvaria is composed of anterior frontal bone, which is fused posteriorly to
bilateral parietal bones at the coronal suture. A sagittal suture fuses the
paired parietal bones superiorly along the midline, and temporal bones are
fused at the inferior aspect via squamosal sutures. Posteriorly, lambdoid
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sutures fix the parietal bones to the occipital bone. Cranial bones and their
connecting suture joints are labeled in Fig. 9.
Fig. 9 Cranial bone anatomy [76]
Sphenoid and ethmoid bones form the skull base, along with inferior
portions of the occipital, temporal, and frontal bones. The pterion is an
osseous landmark significant for the articulation of sphenoid, temporal,
frontal, and parietal bones, while the asterion contains bony articulations of
occipital, parietal, and temporal bones [77].
4.2 Preoperative Patient Assessment
Generally, 75% of craniosynostosis cases will present nonsyndromically,
although a thorough patient history and examination is warranted to rule out
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syndromic conditions and confirm diagnosis. Some of the vital information
to be obtained is detailed below.
Surgeons should obtain a thorough maternal gynecological history to
assess for previous in-utero exposures (teratogens) and prior complications
(abnormal fetal position, intrauterine restraints), in addition to any
difficulties encountered in the intrapartum period. This information should
mainly be obtained for the affected child, although any available
information from previous maternal gestation or birth history may be
helpful in assessing the risk of future occurrence. The developmental
history will also be discussed at the preoperative visit with significance
placed on any delayed milestones encountered in the affected child, as well
as feeding difficulty or prior diagnosis of obstructive sleep apnea (OSA).
OSA presents similarly in neonates when compared to adults and can be
observed as loud breathing or snoring followed by apneic periods (no
breathing). The obstructive symptoms may be accompanied by daytime
fatigue or failure to thrive (FTH), the former of which is somewhat difficult
to assess in newborns [75, 78].
The key aspects of the physical examination are detailed in the bullet
points below [75, 79]:
Visual examination: Assess for asymmetry, deformity, and head shape.
Calculation of cephalic index using measured head circumference.
– Cephalic index=(maximal skull circumference * 100)/(maximal skull
length).
Palpation of the skull and facial bones.
– Assess for ridges at the suture lines, large blood vessels, indentations,
or contour deformities.
Measure and examine fontanelles.
Ophthalmologic examination: Assess for papilledema (presents with ICP)
and corneal exposure.
– Abnormally elevated intracranial pressure often presents in neonates
with inconsolable crying, distress and/or headbanging, which tends to
worsen at night when lying flat.
Airway examination.
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