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15 Prevention andTreatment ofDeformational Cephaly
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209
Diagnostics
Various methods are available for measuring the
shape of the head. These include the visual
assessment, optical acquisition using 3D scanners or digital photography, manual measurement using curve rulers or calipers, computed
tomography (CT), magnetic resonance imaging
(MRI), or cone beam computed tomography
(CBCT) [14–16]. Obtaining a CT or MRI is not
part of the routine diagnostic workup for head
measuring [17]. The indication for, for example,
computed tomography exists only when ultrasonography or radiography does not allow a denite exclusion of craniosynostosis [18], for
neither brachycephaly nor plagiocephaly is a
standard for diagnosis and classication [16].
Argenta described diagnostic criteria for the
visual diagnosis of plagio- or brachycephaly
(Tables 15.3 and 15.4).
Based on the imaging or measurement, for
plagiocephaly, the Cranial Vault Asymmetry
Index (CVAI) can be determined to conrm the
diagnosis. Various other measurement methods
are also available, but the CVAI appears to be
the most accurate [20]. (Information on the calculation of the Cranial Vault Asymmetry Index
can be found in “Fundamentals of Craniofacial
Malformations” Vol. 1, Chap. 5 [21].) For
brachycephaly, the cranial ratio (CR) is regarded
appropriate to classify the severity of the deformity. It relates the length of the skull to its width
(cranial width/cranial length × 100). Higher
CR scores indicate more severe brachycephaly
states [22].
Table 15.3 Clinical classication of posterior brachycephaly after Argenta [19]
Type Characteristics
I A depression of the central skull at the
conuence of the lambdoid with the sagittal
suture. The position of the ears, forehead, and
face is otherwise normal
II Type I and widening of the skull in its posterior
half as the brain attempts to decompress
III Type II and vertical growth of the posterior skull
and/or temporal bulging
Table 15.4 Clinical classication of positional plagiocephaly after Argenta [19]
Type Characteristics
I Cranial asymmetry is limited to the back of the
skull
II Variable degrees of posterior cranial asymmetry,
involving central cranium and skull base.
Displacement of the ear forwards and/or
downwards. The middle temporal fossa is also
affected
III Posterior cranial asymmetry, malposition of the
ipsilateral ear, and ipsilateral frontal bone
protrusion
IV Type III and ipsilateral cranial asymmetry. Facial
asymmetry originating from soft tissue. In severe
cases, bony asymmetry may develop
V Type IV and temporal bulging or abnormal
vertical growth
Prevention
Several preventive measures are recommended to
avoid the development of positional head deformity. The rst important measure here is the education of parents and other nursing staff about the
clinical picture by pediatricians and staff in infant
wards [23, 24].
The so-called tummy time—specic times
when infants should lie on their stomachs—is
recommended as the easiest and most effective
preventive measure. In addition, it is proposed to
regularly reposition the head during the night. On
the other hand, one-sided positions, such as in car
seats, should be avoided. Furthermore, the child
should be provided with stimuli from different
directions so that it is encouraged to move the
head [25]. It is also recommended to place a pillow under the child during sleep [26]. In order to
prevent the head from bending at the neck, care
should be taken to ensure that both the baby’s
head and shoulders rest on the pillow. These measures should be followed for the rst 3–4months
until the child can turn independently.
Moreover, carrying the child in a baby carrier
to reduce pressure on the head and regularly
changing sides when breastfeeding or giving bottles would also counteract one-sided pressure and
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lower the risk of a deformity. Additionally, the
baby should be regularly encouraged to look in
different directions. To do so, various stimuli in
the form of toys or other interesting objects can
be placed opposite the preferred head position. In
parts, preventive measures overlap with treatment measures.
Treatment
The treatment of deforming cephalic differs from
synostotic cephalic in that it does not require surgical intervention. This is mandatory in craniosynostosis. Positional cephaly demands an
intervention according to the severity and differs
only insignicantly in a plagiocephaly or brachycephaly [18].
The earliest therapy concept, beginning
around the fourth month of life, is “counter positioning,” in which the infant is actively positioned
against his or her preferred posture. This can be
supported by placing a special pillow or towel
underneath the head. In addition, stimuli should
be offered in such a way that they are increasingly directed against the side or the position of
the occipital attening. Tummy time is both a
preventive and therapeutic measure. Prone positioning of the awake infant or during sleep under
supervision is recommended for at least 3h a day.
These measures can be supported by manual
physiotherapy or osteopathy [27–29].
If these measures have already led to an
improvement in the deformity, they should be
continued. If no signicant improvement has
occurred, further measures should be initiated. A
frequently performed treatment of plagiocephaly
or brachycephaly to adjust the shape of the skull
to the norm is molding helmet therapy [30]. The
therapy is based on wearing a helmet that is individually adapted to the infant’s head shape
(Fig. 15.5) [31]. It provides the desired (symmetrical) shape to which the infant’s head adapts
in the course of the growth process (Fig.15.6).
The recommended wearing time is 23 h/day,
while the duration of treatment depends on the
speed of improvement [17].
H. S. Kriege et al.
Fig. 15.5 Infant wearing a modulating head orthosis.
(Photo courtesy of Dr. Holger Maas)
Fig. 15.6 Plagiocephaly with modulating head orthosis
in cross section from axial. The areas highlighted in blue
represent the cavities inside the helmet. The arrows mark
the expected growth direction of the skull
Asymmetry is conspicuous when the length of
the cranial diagonals deviates by more than 1cm.
Above 2cm, a treatment indication is also given
for functional reasons [32]. Thus, if spontaneous
correction of the head deformity does not occur
15 Prevention andTreatment ofDeformational Cephaly
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by the fourth to fth months of life, therapy with
a head orthosis is indicated.
The Congress of Neurological Surgeons published a guideline in 2016 stating that early helmet therapy is indicated for all children with
moderate-to-severe plagiocephaly whose ndings have not improved with conservative therapy
(positioning and/or physical therapy) and those
who present at a more advanced age [18, 25].
According to a statement of the Society for
Neuropediatrics and the German Society for
Social Pediatrics and Youth Medicine, the success of therapy is particularly high if therapy is
started between the fourth and sixth months or at
the latest by the twelfth month of life [17],
because the success of therapy decreases with
increasing age of the children [33] and prolongs
the duration of treatment [34]. In general, the
younger the patients are at the beginning of the
treatment, the more effective the therapy [35].
Between the fourth and sixth months of life, the
infant skull also shows the greatest dynamic
growth, increasing in volume by an average of
13% [36]. So, it would make the most sense to
treat during this period.
Early childhood helmet therapy improves cranial asymmetry more rapidly; the more severe the
head deformity, the greater the improvement with
helmet therapy [33, 37, 38]. Patients who appear
to have the greatest benet from this therapy are
those who start treatment early, have a moderateto- severe form of head deformity, and show good
compliance [39].
The therapy has only few complications. Most
of them, such as pressure sores, can be reliably
avoided or entirely eliminated by instructing the
parents [40].
In addition to the available therapeutic options,
the decisive factors for a spontaneous improvement of the head deformity appear to be an elimination of the risk factors [32]. A spontaneous
correction of the head shape is more frequent in
brachycephaly than plagiocephaly [36]. The
change in skull shape after helmet therapy is
shown in Fig.15.7.
211
Fig. 15.7 Skull shape of a plagiocephaly before (orange)
and after helmet therapy (green). The decit on the right
side has been corrected, and the skull has become clinically visibly more symmetrical
Discussion
First, it has to be noticed that the focus in the literature on acquired head deformities is clearly on
plagiocephaly and not brachycephaly [16]. This
is also the case here.
No association between the severity of cranial
asymmetry and facial symmetry was found
before treatment [41]. Argenta’s clinical classication of positional plagiocephaly includes facial
involvement in categories IV and V, which are the
most severe (Table15.4). However, several studies revealed that early childhood helmet therapy
on average also resulted in more symmetrical
faces [41–43].
The question inevitably arises as to the sense
of therapy, apart from the elimination of the aesthetic losses of a deformed head, if the deformity
“outgrows” with adolescence in most cases any-
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H. S. Kriege et al.
way. However, various studies suggest that there
are other impairments besides aesthetics.
Regarding motor and neurological development, affected children tend to have abnormally
altered muscle tone in infancy compared to unaffected children [44]. Also, a signicant delay in
mental and psychomotor development before
therapeutic intervention was described. Boys
appear to be more commonly affected by these
motor developmental delays than girls. There is
reason to assume that positional plagiocephaly
may be a high-risk factor for developmental
delays [45, 46].
Children’s motor skills in infancy are also
related to their cognitive aptitude and language
development [47]. When these are impaired,
learning decits in other areas are also observed.
It is also believed that children affected by plagiocephaly in primary school age are a high-risk
group for developmental disorders. It also made
no difference whether the children received helmet therapy at a younger age or not. Nor would
helmet therapy have any effect on the extent of
developmental delay [48].
The question remains whether these decits
result from the cranial asymmetry or whether the
head deformity is a consequence of the listed
limitations: Motor developmental delays cause
positional plagiocephaly. Such delays could
therefore increase the likelihood of plagiocephaly
as these children are less able to move their heads
due to their limited motor skills. Therefore, plagiocephaly might rather be seen as a marker for a
motor development risk [47].
Positional plagiocephaly is also described to
be associated with anatomical features. Affected
individuals also show orthodontic alterations
more frequently than other children. More than
twice as many have class II dentition in the rst
dentition, suffer from a head bite, or have a displaced midline. Most mandibular asymmetries
present on the contralateral side to the occipital
attening [49]. It was assumed that these abnormalities are not the result of a primary mandibular deformity but are caused by plagiocephaly
[50]. The risk of asymmetric occlusion seems to
be more signicant in frontal than in occipital
plagiocephaly [51].
Strabismus that occurs in craniosynostosis
and associated syndromes does not seem to play
a role in positional plagiocephaly [52].
Although, as shown by several studies, the
incidence of positional plagiocephaly decreases
from advanced infancy [53, 54], a recent study
nds that no spontaneous improvement occurs
without therapy within 5 years [55]. However,
this refers purely to phenotypic appearance of
positional plagiocephaly and to no other developmental factors. Are those who are still affected by
a head deformity at an advanced age also limited
in motor or cognitive abilities compared to peers
of the same age?
The effectiveness of the interventional measures in terms of cranial symmetry cannot be
doubted on the basis of the current studies.
However, it must be critically considered and further investigated whether a spontaneous course
does not have a similar or equal effect in the long
term as a therapeutic intervention. Current studies focus on the changes of the skull in the course
of therapy. There are few results comparing therapy versus non-therapy, and even if they do so, it
is limited to the conclusion that the helmet
achieves a faster and better result related to plagiocephaly [37], but not whether children receiving therapy show fewer developmental decits as
they get older.
Conclusion
The unclear benet of therapeutic measures such
as modied positioning, physiotherapy, and even
helmet therapy regarding developmental delays
that may occur certainly requires further investigation, especially with a focus on whether a spontaneous improvement of symmetry also leads to a
“spontaneous catching up” in case of possibly previously observed developmental limitations.
However, the relatively low cost of therapeutic
intervention must be weighed against the possible risks of developmental impairment. Therefore,
each child should be subjected to an individual
risk analysis. What is certain is that no infant
should be exposed to the possibility of being
disadvantaged.
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References
1. Delashaw JB, Persing J, Broaddus WC, Jane
JA. Cranial vault growth in craniosynostosis. J
Neurosurg. 1989;70:159–65. https://doi.org/10.3171/
jns.1989.70.2.0159.
2. Rogers GF. Deformational plagiocephaly, brachycephaly, and scaphocephaly. Part I: terminology, diagnosis, and etiopathogenesis. J Craniofac
Surg. 2011;22:9–16. https://doi.org/10.1097/
SCS.0b013e3181f6c313.
3. American Academy of Pediatrics. Positioning and
Sudden Infant Death Syndrome (SIDS). Pediatrics.
1992;98:1216–8.
4. Obladen M.In God’s image? The tradition of infant
head shaping. J Child Neurol. 2012;27:672–80.
https://doi.org/10.1177/0883073811432749.
5. Trinkaus E. Articial cranial deformation in the
Shanidar 1 and 5 Neandertals. Curr Anthropol.
1982;23:198–9. https://doi.org/10.1086/202808.
6. Schijman E. Articial cranial deformation in newborns in the pre-Columbian Andes. Childs Nerv
Syst. 2005;21:945–50. https://doi.org/10.1007/
s00381- 004- 1127- 8.
7. Schildkrout E.Gender and sexuality in Mangbetu art.
In: Unpacking culture: art and commodity in colonial
and postcolonial worlds. p.197–213.
8. Ayer A, Campbell A, Appelboom G, Hwang BY,
McDowell M, Piazza M, etal. The sociopolitical history and physiological underpinnings of skull deformation. Neurosurg Focus. 2010;6:1–6.
9. Ballardini E, Sisti M, Basaglia N, Benedetto M,
Baldan A, Borgna-Pignatti C, Garani G.Prevalence
and characteristics of positional plagiocephaly in
healthy full-term infants at 8-12 weeks of life. Eur J
Pediatr. 2018;177:1547–54. https://doi.org/10.1007/
s00431- 018- 3212- 0.
10. Roby BB, Finkelstein M, Tibesar RJ, Sidman
JD. Prevalence of positional plagiocephaly in teens
born after the “back to sleep” campaign. Otolaryngol
Head Neck Surg. 2012;146:823–8. https://doi.
org/10.1177/0194599811434261.
11. de Bock F, Braun V, Renz-Polster H.Deformational
plagiocephaly in normal infants: a systematic review of causes and hypotheses. Arch Dis
Child. 2017;102:535–42. https://doi.org/10.1136/
archdischild- 2016- 312018.
12. Persing J. MOC-PS(SM) CME article: management
considerations in the treatment of Craniosynostosis.
Plast Reconstr Surg. 2008;121:1–11. https://doi.
org/10.1097/01.prs.0000305929.40363.bf.
13. World Health Organization. Child growth standards:
head circumference-for-age boys, birth to 2 years
(percentile). Geneva: World Health Organization;
2007.
14. Schaaf H, Wilbrand J-F, Boedeker R-H, Howaldt
H-P.Accuracy of photographic assessment compared
with standard anthropometric measurements in nonsynostotic cranial deformities. Cleft Palate Craniofac
J. 2010;47:447–53. https://doi.org/10.1597/09- 026.
15. Dörhage K. Ursache und Diagnostik der lagebedingten Plagiozephalie. Man Med. 2010;48:125–34.
https://doi.org/10.1007/s00337- 010- 0743- 5.
16. Siegenthaler MH. Methods to diagnose, classify,
and monitor infantile deformational Plagiocephaly
and Brachycephaly: a narrative review. J Chiropr
Med. 2015;14:191–204. https://doi.org/10.1016/j.
jcm.2015.05.003.
17. Rosenbaum T, Borusiak P, Schweitzer T, Berweck S,
Sprinz A, Straßburg H, Klepper J.Stellungnahme der
gemeinsamen Therapiekommission der Gesellschaft
für Neuropädiatrie und der Deutschen Gesellschaft
für Sozialpädiatrie und Jugendmedizin. 2012.
18. Flannery AM, Tamber MS, Mazzola C, Klimo P,
Baird LC, Tyagi R, et al. Congress of Neurological
Surgeons systematic review and evidencebased guidelines for the management of patients
with positional plagiocephaly: executive summary. Neurosurgery. 2016;79:623–4. https://doi.
org/10.1227/NEU.0000000000001426.
19. Argenta L.Clinical classication of positional plagiocephaly. J Craniofac Surg. 2004;15:368–72. https://
doi.org/10.1097/SCS.0000000000001511.
20. Aarnivala H, Vuollo V, Heikkinen T, Harila V,
Holmström L, Pirttiniemi P, Valkama AM.Accuracy
of measurements used to quantify cranial asymmetry
in deformational plagiocephaly. J Craniomaxillofac
Surg. 2017;45:1349–56. https://doi.org/10.1016/j.
jcms.2017.05.014.
21. Visse HS, Runte C, Meyer U, Dirksen
D.Morphometrics, optical 3D imaging, and monitoring of craniofacial development and malformations.
In: Meyer U, editor. Fundamentals of craniofacial
malformations. 1st ed. Cham, Switzerland: Springer
International Publishing; 2021. p.53–64.
22. Wilbrand J-F, Schmidtberg K, Bierther U, Streckbein
P, Pons-Kuehnemann J, Christophis P, etal. Clinical
classication of infant nonsynostotic cranial deformity. J Pediatr. 2012;161:1120–5. https://doi.
org/10.1016/j.jpeds.2012.05.023.
23. Laughlin J, Luerssen TG, Dias MS. Prevention
and management of positional skull deformities in
infants. Pediatrics. 2011;128:1236–41. https://doi.
org/10.1542/peds.2011- 2220.
24. Persing J, James H, Swanson J, Kattwinkel
J. Prevention and management of positional skull
deformities in infants. Pediatrics. 2003;112:199–202.
https://doi.org/10.1542/peds.112.1.199.
25. Linz C, Kunz F, Böhm H, Schweitzer T. Positional
skull deformities. Dtsch Arztebl Int. 2017;114:535–
42. https://doi.org/10.3238/arztebl.2017.0535.
26. Lennartsson F. Developing guidelines for child
health care nurses to prevent nonsynostotic plagiocephaly: searching for the evidence. J Pediatr
Nurs. 2011;26:348–58. https://doi.org/10.1016/j.
pedn.2010.07.003.
27. Binkiewicz-Glinskka A, Mianowska A, Sokolow M,
Renska A, Ruckeman-Dziurdzinska K, Bakula S,
Kozlowska E.Early diagnosis and treatment of children with skull deformations: the challenge of modern
medicine. Dev Period Med. 2016;20:289–95.
214
https://t.me/medicina_free
H. S. Kriege et al.
28. Cabrera-Martos I, Valenza MC, Valenza-Demet
G, Benítez-Feliponi A, Robles-Vizcaíno C, RuizExtremera A. Effects of manual therapy on treatment duration and motor development in infants
with severe nonsynostotic plagiocephaly: a randomised controlled pilot study. Childs Nerv
Syst. 2016;32:2211–7. https://doi.org/10.1007/
s00381- 016- 3200- 5.
29. Lessard S, Gagnon I, Trottier N.Exploring the impact
of osteopathic treatment on cranial asymmetries associated with nonsynostotic plagiocephaly in infants.
Complement Ther Clin Pract. 2011;17:193–8. https://
doi.org/10.1016/j.ctcp.2011.02.001.
30. Steinberg JP, Rawlani R, Humphries LS, Rawlani V,
Vicari FA.Effectiveness of conservative therapy and
helmet therapy for positional cranial deformation.
Plast Reconstr Surg. 2015;135:833–42. https://doi.
org/10.1097/PRS.0000000000000955.
31. Clarren SK, Smith DW, Hanson JW.Helmet treatment
for plagiocephaly and congenital muscular torticollis. J Pediatr. 1979;94:43–6. https://doi.org/10.1016/
S0022- 3476(79)80347- 9.
32. Blecher C, Kunz C, Mayr J, Schwenzer-Zimmerer
K. Helmtherapie: konservative Kopfmodellierung.
MKG-Chirurg. 2012;5:289–96. https://doi.
org/10.1007/s12285- 012- 0306- y.
33. Kunz F, Schweitzer T, Kunz J, Waßmuth N, StellzigEisenhauer A, Böhm H, etal. Head orthosis therapy in
positional plagiocephaly: inuence of age and severity of asymmetry on effect and duration of therapy.
Plast Reconstr Surg. 2017;140:349–58. https://doi.
org/10.1097/PRS.0000000000003517.
34. Kluba S, Kraut W, Reinert S, Krimmel M. What is
the optimal time to start helmet therapy in positional
plagiocephaly? Plast Reconstr Surg. 2011;128:492–8.
https://doi.org/10.1097/PRS.0b013e31821b62d6.
35. Dörhage KWW, Wiltfang J, von Grabe V, Sonntag
A, Becker ST, Beck-Broichsitter BE.Effect of head
orthoses on skull deformities in positional plagiocephaly: evaluation of a 3-dimensional approach. J
Craniomaxillofac Surg. 2018;46:953–7. https://doi.
org/10.1016/j.jcms.2018.03.013.
36. Meyer-Marcotty P, Kunz F, Schweitzer T, Wachter
B, Bohm H, Wassmuth N, Linz C. Cranial growth
in infants horizontal line a longitudinal threedimensional analysis of the rst months of life. J
Craniomaxillofac Surg. 2018;46:987–93. https://doi.
org/10.1016/j.jcms.2018.04.009.
37. Vles JSH, Colla C, Weber JW, Beuls E, Wilmink
J, Kingma H. Helmet versus non helmet treatment
in nonsynostotic positional posterior plagiocephaly. J Craniofac Surg. 2000;11:572–4. https://doi.
org/10.1097/00001665- 200011060- 00010.
38. Tamber MS, Nikas D, Beier A, Baird LC, Bauer
DF, Durham S, et al. Congress of Neurological
Surgeons systematic review and evidence-based
guideline on the role of cranial molding orthosis
(helmet) therapy for patients with positional plagiocephaly. Neurosurgery. 2016;79:E632–3. https://doi.
org/10.1227/NEU.0000000000001430.
39. Thompson JT, David LR, Wood B, Argenta A,
Simpson J, Argenta LC. Outcome analysis of helmet therapy for positional plagiocephaly using a
three-dimensional surface scanning laser. J Craniofac
Surg. 2009;20:362–5. https://doi.org/10.1097/
SCS.0b013e3181992382.
40. Wilbrand J-F, Wilbrand M, Malik CY, Howaldt H-P,
Streckbein P, Schaaf H, Kerkmann H.Complications
in helmet therapy. J Craniomaxillofac Surg.
2012;40:341–6. https://doi.org/10.1016/j.
jcms.2011.05.007.
41. Visse HS, Meyer U, Runte C, Maas H, Dirksen
D. Assessment of facial and cranial symmetry in
infants with deformational plagiocephaly undergoing molding helmet therapy. J Craniomaxillofac
Surg. 2020;48:548–54. https://doi.org/10.1016/j.
jcms.2020.04.003.
42. Kreutz M, Fitze B, Blecher C, Marcello A, Simon R,
Cremer R, et al. Facial asymmetry correction with
moulded helmet therapy in infants with deformational skull base plagiocephaly. J Craniomaxillofac
Surg. 2018;46:28–34. https://doi.org/10.1016/j.
jcms.2017.10.013.
43. Lee MC, Hwang J, Kim YO, Shim KW, Park
EK, Lew DH, Yun IS. Three-dimensional analysis of cranial and facial asymmetry after helmet
therapy for positional plagiocephaly. Childs Nerv
Syst. 2015;31:1113–20. https://doi.org/10.1007/
s00381- 015- 2677- 7.
44. Fowler EA, Becker DB, Pilgram TK, Noetzel
M, Epstein J, Kane AA. Neurologic ndings in infants with deformational plagiocephaly. J Child Neurol. 2008;23:742–7. https://doi.
org/10.1177/0883073808314362.
45. Knight SJ, Anderson VA, Meara JG, Da Costa
AC.Early neurodevelopment in infants with deformational plagiocephaly. J Craniofac Surg. 2013;24:1225–
8. https://doi.org/10.1097/SCS.0b013e318299777e.
46. Martiniuk ALC, Vujovich-Dunn C, Park M, Yu W,
Lucas BR.Plagiocephaly and developmental delay: a
systematic review. J Dev Behav Pediatr. 2017;38:67–
78. https://doi.org/10.1097/DBP.0000000000000376.
47. Collett BR, Wallace ER, Kartin D, Speltz ML.Infant/
toddler motor skills as predictors of cognition and language in children with and without positional skull
deformation. Childs Nerv Syst. 2018;35:157. https://
doi.org/10.1007/s00381- 018- 3986- 4.
48. Miller RI, Clarren SK.Long-term developmental outcomes in patients with deformational plagiocephaly.
Pediatrics. 2000;105:e26. https://doi.org/10.1542/
peds.105.2.e26.
49. Kluba S, Rosskopf F, Kraut W, Peters JP, Calgeer B,
Reinert S, Krimmel M.Malocclusion in the primary
dentition in children with and without deformational
plagiocephaly. Clin Oral Investig. 2016;20:2395–401.
https://doi.org/10.1007/s00784- 016- 1716- 4.
50. St. John D, Mulliken JB, Kaban LB, Padwa
BL. Anthropometric analysis of mandibular asymmetry in infants with deformational posterior pla-
15 Prevention andTreatment ofDeformational Cephaly
https://t.me/medicina_free
215
giocephaly. J Oral Maxillofac Surg. 2002;60:873–7.
https://doi.org/10.1053/joms.2002.33855.
51. Baumler C, Leboucq N, Captier G.Mandibular asymmetry in plagiocephaly without synostosis. [Etude
de l'asymetrie mandibulaire dans les plagiocephalies sans synostose]. Rev Stomatol Chir Maxillofac.
2007;108:424–430. https://doi.org/10.1016/j.
stomax.2007.07.005.
52. Gupta PC, Foster J, Crowe S, Papay FA, Luciano
M, Traboulsi EI. Ophthalmologic ndings in
patients with nonsyndromic plagiocephaly. J
Craniofac Surg. 2003;14:529–32. https://doi.
org/10.1097/00001665- 200307000- 00026.
53. Hutchison BL, Hutchison LAD, Thompson JMD,
Mitchell EA. Plagiocephaly and brachycephaly in
the rst two years of life: a prospective cohort study.
Pediatrics. 2004;114:970–80. https://doi.org/10.1542/
peds.2003- 0668- F.
54. Bialocerkowski AE, Vladusic SL, Ng
CW. Prevalence, risk factors, and natural history of
positional plagiocephaly: a systematic review. Dev
Med Child Neurol. 2008;50:577–86. https://doi.
org/10.1111/j.1469- 8749.2008.03029.x.
55. Wilbrand J-F, Lautenbacher N, Pons-Kuhnemann
J, Streckbein P, Kahling C, Reinges MHT, et al.
Treated versus untreated positional head deformity. J Craniofac Surg. 2016;27:13–8. https://doi.
org/10.1097/SCS.0000000000002167.
Part VII
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Diseases: Soft Tissue Malformations
Treatment Principles ofSkin
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Malformations
MariaAhls andJanD.Raguse
16
Congenital Melanocytic
Nevus (CMN)
By denition, congenital nevi are accumulations
of melanocytes that either are present before birth
or appear within 0–4weeks after birth [1, 2]. The
occurrence of congenital melanocytic nevi shows
a familial accumulation and thus points to a
genetic component [3].
Based on the clinical expansion in adulthood,
CMN can be classied as small (up to 1.5cm),
moderate (1.5–20 cm), and large (20–40 cm)
(Table16.1). There are also special forms such as
the congenital melanocytic giant nevus (>40cm)
and the tardive congenital melanocytic nevus.
An NRAS mutation can be found in congenital melanocytic giant nevus. Tardive congenital
melanocytic nevi appear after the neonatal period,
often in the rst 2years of life, and their occurrence is signicantly more frequent than that of
congenital melanocytic nevi (6–20% in adolescents and adults). They reach a size of up to
1.5cm. They cannot be distinguished clinically
and histologically from true congenital melanocytic nevi [1, 4–7].
Often, the congenital nevus presents clinically
as a light to dark-brown area with a median size
M. Ahls · J. D. Raguse (*)
Department for Oral and Maxcillofacial Surgery,
Fachklinik Hornheide, Münster, Germany
e-mail: Maria.Ahls@fachklinik-muenster.de
Table 16.1 Summary of classication of congenital
melanocytic nevus [4–7]
Small Up to 1.5cm
Medium
sized
Size 20–40cm
Special shape Congenital melanocytic giant nevus
of over 1.5cm. Hypertrichosis and a smooth surface are common in this area. Macroscopically,
the surface appears homogeneous. As the skin
grows with age, the size of the CMN increases
proportionally [8]. The lesions can increase in
size signicantly over time and the surface can
also appear more inhomogeneous. Especially in
the larger CMN, there are differences in the clinical appearance. Larger CMN often have color
variations within the mark. While in the case of
congenital melanocytic giant nevi, satellite nevi
can increasingly occur distributed over the entire
tegument and you can nd neurocutaneous melanosis [9, 10].
Histopathologically, the congenital melanocytic nevi can be distinguished from the acquired
melanocytic nevi. While the melanocytes are
found in the upper third of the dermis in acquired
melanocytic nevi, the melanocytes in CMN can
1.5–20cm
• NRAS mutation in the neural crest
Tardive congenital nevus
• Max. 1.5cm
• Occurs in the rst 2years of life
• 6–20% incidence
© Springer Nature Switzerland AG 2023
U. Meyer (ed.), Fundamentals of Craniofacial Malformations,
https://doi.org/10.1007/978-3-031-28069-6_16
219
220
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M. Ahls and J. D. Raguse
also colonize the lower two-thirds of the dermis
and show neuroid differentiation [1, 2]. The histological features of CMN are important with
regard to a possible malignant transformation.
The deep expansion of the melanocytes in the
lower area of the dermis represents a corresponding risk, since in 2/3 of the cases, malignant processes grow in the dermis or deeper layers and
are clinically only recognized at a late stage,
which leads to a poor prognosis [11].
Unlike CMN, melanomas that develop on the
basis of nevus cell nevi show an epidermal growth
pattern and appear primarily in adulthood. The
risk of developing malignancies from a CMN
increases with its size [1, 12]. With a risk of
5–15%, large CMN in particular have an
increased risk of degeneration [1, 13, 14]. It is
described in the literature that almost 50% of
children with a large CMN develop a melanoma
in the rst 5years of life [15–17].
Since the skin is particularly elastic and
stretchable in infancy and toddlers, an excision
should already be carried out at this age. This can
reduce stigma, psychological stress for those
affected, and risk of malignant transformation
[18, 19]. In the case of smaller CMN, it is possible
to remove the mole by means of a spindle- shaped
excision and to close the defect directly, e.g., with
an expansion ap [19]. With larger CMN, it is
important that the excision does not result in any
aesthetic losses. It is possible to surgically remove
the nevus in serial excisions (Fig.16.1a, b). With
this method, the KMN is reduced in several consecutive operations until it can be completely
removed. In this way, the tissue can recover
between operations and there are no aesthetic
losses. Another option is it to achieve a plastic
reconstruction by means of skin transplantation
after the excision (Figs.16.2a–d and 16.3a, b).
Furthermore, once contraindications have
been ruled out, the skin expander technique can
be used. The expander is implanted near the
nevus and then lled with liquid so that the skin
can stretch to the required size. Once this goal
has been achieved, the CMN is surgically excised
and the previously stretched skin can be rotated
into the defect (Fig.16.4a–c) [20].
If necessary, the subsequent aesthetic and
functional result can be improved after the complete excision. For example, if hypertrophic and
uneven scars occur, dermabrasion can be performed. In the case of scars, surgical scar correction can bring relief.
Due to a lack of long-term results and a lack of
knowledge about the long-term effects on CMN,
laser therapy is only given to certain regions,
such as the lips, eyelids, ears, and genito-anal
area. Functional and aesthetic impairments can
occur here as a result of surgical methods [1, 21].
If the case arises that the CMN cannot be completely excised, aesthetically unsightly, highly
suspected of being malignant and difcult-tocontrol areas can be excised or treated with laser
therapy, curettage, or dermabrasion [1, 18, 22].
Fig. 16.1 Preoperative
ndings in a 2-year-old
patient with CMN of a
third of the cheek (a).
Now 11-year-old patient
after two serial excisions
with a small residual
nding but without
aesthetic or functional
impairment (b)
ba
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