biomedicines
Case Report
Surgical Management of Pterygium Colli with Significant Skin
Laxity: A Case Report
Charline Huttin 1 , Patrick Ringenbach 2 , Anastasia Durry 2 , Mihai Hogas 3, *, Ionut Raducu Popescu 3, *,
Alin Ciobica 4,5,6 and Mihaela Elena Nastasa 2
Citation: Huttin, C.; Ringenbach, P.;
Durry, A.; Hogas, M.; Popescu, I.R.;
Ciobica, A.; Nastasa, M.E. Surgical
Management of Pterygium Colli with
Significant Skin Laxity: A Case
Report. Biomedicines 2023, 11, 984.
https://doi.org/10.3390/
biomedicines11030984
Academic Editors: Taishi Hata and
Michele Ammendola
Received: 5 December 2022
Revised: 21 February 2023
Accepted: 21 March 2023
Published: 22 March 2023
Copyright: © 2023 by the authors.
Licensee MDPI, Basel, Switzerland.
This article is an open access article
distributed under the terms and
conditions of the Creative Commons
Attribution (CC BY) license (https://
creativecommons.org/licenses/by/
4.0/).
Biomedicines 2023, 11, 984. https://doi.org/10.3390/biomedicines11030984
1 Service de Chirurgie Plastique, Reconstructive et Esthétique, CHU Hautepierre, 1 Avenue Molière,
67200 Strasbourg, France
2 Service de Chirurgie Plastique, Reconstructive et Esthétique, GHR Mulhouse, 20 Avenue du Dr René Laennec,
68100 Mulhouse, France
3 Physiology Department, “Grigore T. Popa” University of Medicine and Pharmacy, Universitatii 16,
700115 Iasi, Romania
4 Department of Biology, Faculty of Biology, Alexandru Ioan Cuza University, B dul Carol I, No 11,
700115 Iasi, Romania
5 Academy of Romanian Scientists, Splaiul Independentei Nr. 54, Sector 5, 050094 Bucuresti, Romania
6 Center of Biomedical Research, Romanian Academy, B dul Carol I, No. 8, 700115 Iasi, Romania
* Correspondence: mihaimmh@yahoo.com (M.H.); ionutraducu@gmail.com (I.R.P.)
Abstract: Pterygium Colli or “palmate neck” is a congenital malformation that is most often part of a
polimalformative syndrome. This deformity is a source of aesthetic and social embarrassment. Its
correction is surgical. We present the case of a pterygium colli in a patient with Noonan syndrome.
He had a significant excess of skin with posterior skin laxity, causing significant social discomfort
and imposing a vicious attitude, the head bent forward. We performed a posterolateral resection of
this excess by resecting two posterior triangular flaps with a resulting t-shaped scar. The results were
satisfactory; the excess skin was almost completely resorbed with minimal scarring. However, this
technique did not correct the low lateral hairline implantation, and there were still two lateral flaps
for which the patient did not wish to have a repeat surgery.
Keywords: pterygium colli; Noonan syndrome; plastic surgery
1. Introduction
Pterygium colli or “webbed neck” is a congenital malformation corresponding to a
“web” of skin that may extend from the mastoid to the acromion. It was first described in
1883 by Kobilinsky [1], and the term was defined by Funke [2] in 1902.
The etiology remains poorly understood. The excess skin could be the result of a
cystic hygroma during embryonic life which, when regressing, would leave bilateral excess
skin [3], or a growth differential leading to a skin defect in vertical dimension and a skin
excess in horizontal dimension [4]. It could also sometimes be a fusion of the cervical
vertebrae resulting in a neck that is too short, as in Klippel–Feil syndrome.
This malformation is often described in Turner syndrome where it is frequently accom-
panied by a lateral fibrous band corresponding to a thickening of the cervical fascia and a
very low lateral line of capillary implantation [5]. It is also found in Klippel–Feil syndrome,
Noonan syndrome, and some trisomies.
We present here our surgical management of a patient with Noonan syndrome affected
by a pterygium colli with significant skin excess.
2. Case
Our patient was a 23-year-old male with several congenital malformations in the con-
text of Noonan syndrome (Figure 1). At birth he presented with cystic hygroma and severe
encephalopathy, coarctation of the aorta, pterygium colli, hypertelorism with downward
https://www.mdpi.com/journal/biomedicines
 context of Noonan syndrome (Figure 1). At birth he presented with
context of Noonan syndrome (Figure 1). At birth he presented w
severe encephalopathy, coarctation of the aorta, pterygium colli,
severe encephalopathy, coarctation of the aorta, pterygium c
downward and outward directed palpebral slits, bilateral cryptorch
downward
abnormalities, and and outward
mild mentaldirected palpebral
retardation. He slits, bilateral
presented with crypt
Biomedicines 2023, 11, 984 2 of 6 a pal
abnormalities,
nificant posterior and mild skin
nuqual mental retardation.
excess, with skin He presented
loosening, withoutwithl
nificant
mobility,posterior
but with anuqual curved skin
attitude,excess,
headwith bentskin loosening,
forward. The hairlinewitho
and outward directed palpebral slits, bilateral cryptorchidism and inner ear abnormalities,
mobility,
After
and
but with
mild signing
aHecurved
the informed
mental retardation.
attitude,
consent,
presented hishead
with a palmate requestbentwasforward.
essentially
neck with significant
The hair
posterior aesth
After
mation
nuqual signing
skin caused
excess, the
with skininformed
significant consent,
loosening,social
without his
embarrassment.
limitation request
of cervical wasimportance
The
mobility, essentially
but with a o
curved attitude, head bent forward. The hairline was low posteriorly. After signing the
mation
informed caused
excessconsent,
led significant
ushistorequest
consider social
a posterolateral
was essentially embarrassment.
aesthetic, resection
as this Thesignifi-
technique.
malformation caused importan
This w
thesocial
cant Ethical
excess led Committee
us to consider
embarrassment. of Mulhouse Hospital
a posterolateral
The importance of the posterior (1/24ledNovember
skin resection
excess consider a2022).
us totechnique. Th
posterolateral resection technique. This was also approved by the Ethical Committee of
the Ethical
Mulhouse Committee
Hospital (No. 1 Approvalof of Mulhouse
24 November 2022).Hospital (1/24 November 20

Figure 1. A 23-year-old man with pterygium colli associated with Noonan sy

Figure
Figure 1. A
1.The 23-year-old
drawings
A 23-year-old were
man with man with
made
pterygium pterygium
pre-operatively,
colli associated collisyndrome.
with Noonan associated with
on a seated Noon
and aw
zontal mid-auricular
The drawings line was drawn
were made pre-operatively, inand
on a seated the hairline
awake patient. area, as well a
A horizontal
ThenThe
mid-auricular drawings
line
a triangle with were
was drawn in made
antheupper
hairline pre-operatively,
area,
base as well drawn
was as a vertical ona apinch
midline.
with seated and
Then atest, re
triangle with an upper base was drawn with a pinch test, representing the skin resection.
resection.
zontal
The Thetheresult
result ismid-auricular
in fact drawingisofin
anfact
line was
“A-T”the drawing
(Figure 2).inofthe
flapdrawn an “A-T”
hairline flaparea,
(Figure
as w2)
Then a triangle with an upper base was drawn with a pinch te
resection. The result is in fact the drawing of an “A-T” flap (Figu

Figure
Figure 2. Preoperative
2. Preoperative drawing wasdrawing wasanaupper
a triangle with triangle with
base made ana pinch
with uppertest. base made
The result wit
issult
in factisthe drawing of an “A-T” flap.
in fact the drawing of an “A-T” flap.
Furthermore, a schematic view on the A-T plasty is presented in Figure 3.
Furthermore, a schematic view on the A-T plasty is presented in
Figure 2. Preoperative drawing was a triangle with an upper base mad
sult is in fact the drawing of an “A-T” flap.

Furthermore, a schematic view on the A-T plasty is presente

 Biomedicines 2023, 11, x FOR PEER REVIEW 3 of 6
Biomedicines 2023, 11, 984 Biomedicines 2023, 11, x FOR PEER REVIEW 33ofof6 6
Also, a schematic view on the A-T plasty is presented in the Figure 3.
Also, a schematic view on the A-T plasty is presented in the Figure 3.

Figure 3. A schematic view on the 3.

Figure A-TA plasty.
schematic view on the A-T plasty.
Under general anesthesia, the
Figure patient
3. A
Figure 3.schematic view
A schematic on the
Figure A-T
view
3. A plasty.
on the A-T
schematic viewplasty.
on the A-T plasty.
UnderUnder
general general
anesthesia, the patient
anesthesia, the patient was placed in the prone position. We first in-
cisedUnder
the upper horizontal
general line, general
Under
anesthesia, then the vertical
patientalong
theanesthesia, the the
was midline
patient
placed into
was theprone
placed
the tip
in of
thethe trian-position.
prone
position. We first
We first in-
incised
gle. The two lateralcised
flapsthewere thenhorizontal
peeled offline,in the subcutaneous planethe
the upper horizontal line, upper
then the vertical then
along the vertical along
the midline totothe
the lateral
midline to the tip of the trian-
tip of the triangle. The
border of the excessgle.
skinThe(Figure 4). Each
two lateral flapwere
flaps was pulled to theoff
then peeled contralateral side on ei- plane to the lateral
in the subcutaneous
two
ther lateral flaps
side of the were
midline then
of thepeeled
to determine
border off (Figure
the skin
excess in the
resection. Thensubcutaneous
4). they
Eachwere was plane
flap incised to
at this
pulled thecontralateral
lateral border
to midline.
the of ei-
side on the
excess
The skinskin
was (Figure
sutured 4).
sideEach
therwith skin flap
staples
of the was
midlinewith pulled
a resulting
to determine tothe the
“T” contralateral
shaped
resection. scar. theyside
ThenDrainage on
werewas either
incised side
at this of the
midline.
performedtowith
midline twoThe
Blake
determine skin10was
the redons, removed
resection.
sutured Then
with at D1
skin postoperatively.
they
stapleswerewithincised at this
a resulting midline.
“T” shaped scar.The skinwas
Drainage was
Anatomopathological
sutured with skin performed
staples analysis
with
withtwoshowed
Blakean
a resulting 10epidermis
redons, and dermis
removed
“T” shaped at D1of
scar. normal struc-
postoperatively.
Drainage was performed with
tures; some fibrotic areasAnatomopathological
were found in the hypodermis without obvious
analysis showed dystrophy,
an epidermis with of normal struc-
and dermis
two Blake 10 redons,
ectatic vessels.
removed at D1 postoperatively.
tures; some fibrotic areas were found in the hypodermis without obvious dystrophy, with
ectatic vessels.

Figure 4. Surgical technique with the two laterals flaps peeled off in the sous cutaneous plane to the
Figure 4. Surgical technique with the two laterals flaps peeled off in the sous cutaneous plane to the
lateral border of the excess
Figureskin (intraoperative
4. Surgical view).
technique with the two laterals flaps peeled off in the sous cutaneous plane to the
lateral border of the excess
lateral skin
border (intraoperative
of the view). view).
excess skin (intraoperative
In the aftermath, a small disunion occurred on the vertical scar, with a favorable
Anatomopathological
evolution. Most of the excess analysis
skin showed
was resorbed.
In the aftermath, a small The an epidermis
capillary
disunion occurredline on and
wasthe only dermis scar,ofwith
slightly
vertical normal struc-
a favorable
raised. The scar is discreet,
evolution.hidden
Most in
ofthe hair.
the The
excess patient
skin waswas reviewed
resorbed. The
tures; some fibrotic areas were found in the hypodermis without obvious dystrophy, with at 1 year,
capillarywith-
line was only slightly
out any sign of recurrence. However, the persistence of a too low hairline and of lateral
ectatic vessels. raised. The scar is discreet, hidden in the hair. The patient was reviewed at 1 year, with-
flanges were noted.out Forany
thesign
latter, we proposed
of recurrence. to the patient
However, a surgicalofrevision
the persistence a too low with
hairline and of lateral
InZ-plasties,
lateral the aftermath,
but
flanges a small
he refused.
were noted.disunion
In fact, thethe
For occurred
patient is we
latter, very on the
satisfied
proposed to vertical
with
the the scar,
result:
patient he with revision
a surgical a favorable
with
evolution.
can hold hisMost of the Z-plasties,
head upright
lateral excess
and has skin
nobut was resorbed.
functional discomfort
he refused. The
In fact, in capillary
themobilizing lineneck
patient is his
very was only
(Fig-
satisfied slightly
with raised.
the result: he
ure 5).
The scar is discreet,can hidden
hold his in
headtheupright and has
hair. The no functional
patient discomfort
was reviewed at in mobilizing
1 year, his neck
without any(Fig-
sign
of recurrence. However,ure 5). the persistence of a too low hairline and of lateral flanges were
noted. For the latter, we proposed to the patient a surgical revision with lateral Z-plasties,
but he refused. In fact, the patient is very satisfied with the result: he can hold 4his
Biomedicines 2023, 11, x FOR PEER REVIEW of 6
head
upright and has no functional discomfort in mobilizing his neck (Figure 5).

Figure 5. Results at 1 year postoperatively with a low hairline and lateral flanges.
Figure 5. Results at 1 year postoperatively with a low hairline and lateral flanges.
3. Discussion
Noonan syndrome concerns 1/2500 births. It is due to a mutation of the PTPN11
gene, with autosomal dominant transmission. It generally includes cardiac malfor-
mations, particular facial features (poorly developed cheekbone, short nose, backward
tilted ears, hypertelorism, ptosis, downward and outward eyelid slits, ogival palate,
pterygium colli), short stature in adulthood, thoracic deformity, cryptorchidism, deaf-
Biomedicines 2023, 11, 984 4 of 6

3. Discussion
Noonan syndrome concerns 1/2500 births. It is due to a mutation of the PTPN11
gene, with autosomal dominant transmission. It generally includes cardiac malformations,
particular facial features (poorly developed cheekbone, short nose, backward tilted ears,
hypertelorism, ptosis, downward and outward eyelid slits, ogival palate, pterygium colli),
short stature in adulthood, thoracic deformity, cryptorchidism, deafness, and learning diffi-
culties. Coagulation disorders may also be present, making it necessary to systematically
search for them in the preoperative workup.
Although pterygium colli is the subject of numerous publications, its surgical correc-
tion is less often discussed in the literature. Overall, posterior techniques are opposed to
lateral techniques.
Initially Chandler [6] described a technique of lateral resection of the excess by Z-plasty.
However, this type of scar was often hypertrophic and very visible, and did not allow
correction of the line of implantation. Several authors then presented different techniques
of modified lateral Z-plasties [3,7] allowing the raising of the hairline laterally, to limit the
risks of scarring or recurrence by resecting the fibrous thickening of the cervical fascia.
Thompson [8] then described a technique of lateral resection in Ellipse, which could
combine a resection of the upper ear according to a horizontal incision in the hair allowing
to correct the low hairline implantation. Reichenberger [9] associates lateral resection in
ellipse with an advancement flap for significant skin excess.
Chaput [10] more recently describes a new technique for posterior cervical lifting, by
resecting the lateral skin excesses in an ellipse, and by fixing the cervical fascia posteriorly
on the nuchal ligament.
Furthermore, Quian [11] performs simple lateral spindle resections for moderate skin
excess, but this technique again presents a risk of scarring and does not allow correction of
hair implantation.
The group of Murthy [12] presents a modified trident plasty (“M to T” plasty), where
two triangular flaps are resected, with resection of the fibrous fascia. This technique allows
resection of a possible fibrous flange and limits the forward displacement of the hairline,
but the scars are very visible.
Posterior surgical correction techniques are less represented in the literature. Fou-
car [13] was the first to describe a posterior resection technique with a resulting Y-shaped
scar. Schearin [14] performed a butterfly flap correction, but this technique presents numer-
ous recurrences due to retractions.
In addition, Menick [15] provided a correction technique using lateral advancement
flaps. Rossilon [16] in 1989 noted a technique with four transposition flaps, allowing
correction of low and lateral hair implantation in Turner Syndrome: two lower medial flaps,
transposed laterally, and two lateral and upper flaps, transposed medially. Thompson [8]
in 1990 described a vertical spindle resection technique, combining, if necessary to correct
hair implantation, a resection in the hair of two oblique spindles with a resulting “Y”
shaped scar.
In relation to this, we can also describe here the work of Zieliński et al., which demon-
strated in 2015 that the lateral approach with a shift of glabrous skin flap to the back results
in an effective reduction of the webbed neck and correction of the low hairline on the side
of the neck [17].
Finally, Komatsu [18] shows us a posterolateral resection technique according to a
vertical spindle, associated with a horizontal resection of the hairy area, by detaching and
bringing together the two lateral flaps, with a resulting “Y” shaped scar.
It is also possible to perform lateral skin expansions, in particular to expand the
glabrous skin, but the morbidity of this technique and the recurrence rate explain why it
has not been further developed.
The choice of these techniques depends above all on the type of pterygium colli, the
excess skin which may be significant or more moderate, the need to correct the hairline,
as in Turner’s syndrome, for example, where it is very often low and lateral, the need to
Biomedicines 2023, 11, 984 5 of 6

restore neck movement if this is limited, and finally the presence of lateral fibrous bands
which will have to be considered for excision or incision in order to avoid recurrence in the
form of scarring. One must also take into account the high risk of keloid scars in the lateral
cervical scars.
Our patient presented with a pterygium colli associated with a large posterior skin
excess with a large skin slackening and a low hairline, without fibrous thickening of the
cervical fascia, nor limitation of neck mobility. The request was essentially aesthetic. Our
postero-lateral technique allowed us to resorb a large part of the excess, with moderate
scarring, since the horizontal part of the scar was hidden in the hair.
However, this technique did not allow the removal of all the lateral excess, which was
very important, nor did it correct the hairline.
As post operatory recommendations we can describe the massage of the scars at 3
weeks post surgery with a healing cream until the scars will be smooth, the application of
silicone sheets or silicone cream for 2 months, as well as sun protection of the scars for 1
year.

4. Conclusions
The technique of posterolateral correction in TA seems to us to be adapted to a very
important skin excess without lateral fibrous flanges. The resulting scars are discreet, with
little risk of keloid scarring. However, this technique does not allow for correction of the
hairline, nor does it allow for resection or breaking of any lateral fibrous flange.

Author Contributions: Substantial contributions to conception and design by C.H., P.R., A.D. and
M.E.N. Acquisition of data: M.E.N. Contributed to analysis and interpretation of data: M.H., I.R.P.
and A.C. Drafted the article: M.H., I.R.P., A.C. and M.E.N. All authors have read and agreed to the
published version of the manuscript.
Funding: This research received no external funding.
Institutional Review Board Statement: This was also approved by the Ethical Committee of Mul-
house Hospital (Approval number: No. 1 Approval of 24 November 2022).
Informed Consent Statement: Informed consent was obtained from all subjects involved in the study.
Data Availability Statement: All data is available on request.
Conflicts of Interest: The authors declare no conflict of interest.

References
1. Kobilinsky, O. Uber eine flughautahnliche Ausbreitung am Halse. Arch. Antropol. 1883, 14, 343.
2. Funke, F. Pterygium colli. Dtsch. Z. Chir. 1902, 63, 162. [CrossRef]
3. Posso, C.M.; Wolff, G.A.; Suarez, L.D. Pterigium colli: Secondary surgical correction of one severe case. Ann. Chir. Plast. Esthet.
2012, 57, 299–303. [CrossRef] [PubMed]
4. Miller, L.B.; Kanter, M.; Wolfort, F. Treatment of webbed neck in Turner’s syndrome with tissue expansion. Ann. Plast. Surg. 1990,
24, 447–450. [CrossRef] [PubMed]
5. Turner, H.H. A syndrome of infantilism, congenital web neck, cubitus valgus. Endocrinology 1938, 23, 566–574. [CrossRef]
6. Chandler, F.A. Webbed neck (Pterygium colli). Am. J. Dis. Child. 1937, 53, 798–803. [CrossRef]
7. Hikade, K.R.; Bitar, G.J.; Edgerton, M.T.; Morgan, R.F. Modified Z-plasty repair of webbed neck deformity seen in Turner and
Klippel-Feil syndrome. Cleft Palate Craniofac. J. 2002, 39, 261–266. [CrossRef] [PubMed]
8. Thompson, S.J.; Tanner, N.S.; Mercer, D.M. Web neck deformity; anatomical considerations and options in surgical management.
Br. J. Plast. Surg. 1990, 43, 94–100. [CrossRef] [PubMed]
9. Reichenberger, M.A.; Goertz, O.; Lehnhardt, M.; Germann, G.; Ryssel, H.; Czermak, C.; Kolbenschlag, J. Surgical correction of
pterygium colli. J. Pediatr. Surg. 2013, 48, 464–469. [CrossRef] [PubMed]
10. Chaput, B.; Chavoin, J.P.; Lopez, R.; Meresse, T.; Nadon, F.; Herlin, C.; Garrido, I.; Grolleau, J.L. The “posterior cervical lift”: A
new approach to pterygium colli management. Plast. Reconstr. Surg. Glob. Open 2013, 1, e46. [CrossRef] [PubMed]
11. Qian, J.G.; Wang, X.J. Noonan syndrome and correction of the webbed neck. J. Plast. Reconstr. Aesthet. Surg. 2007, 60, 316–319.
[CrossRef] [PubMed]
12. Murthy, A.S.; McGraw, M. M to T rearrangement: An approach to correct webbed neck deformity. Case Rep. Med. 2014, 2014, 682806.
[CrossRef] [PubMed]
Biomedicines 2023, 11, 984 6 of 6

13. Foucar, H.O. Pterygium colli and allied conditions. Can. Med. Assoc. J. 1948, 59, 251–255. [CrossRef] [PubMed]
14. Shearin, J.C.; DeFranzo, A.J. Butterfly correction of webbed-neck deformity in Turner’s syndrome. Plast. Reconstr. Surg. 1980, 66,
129–133. [CrossRef] [PubMed]
15. Menick, F.J.; Furnas, D.W.; Achauer, B.M. Lateral cervical advancement flaps for the correction of webbed-neck deformity. Plast.
Reconstr. Surg. 1984, 73, 223–229. [CrossRef] [PubMed]
16. Rossillon, D.; De Mey, A.; Lejour, M. Pterygium colli: Surgical treatment. Br. J. Plast. Surg. 1989, 42, 178–183. [CrossRef] [PubMed]
17. Zieliński, T.; Lorenc-Podgórska, K.; Antoszewski, B. Surgical Correction of a Webbed-Neck Deformity in Turner’S Syndrome. Pol.
Przegl. Chir. 2015, 87, 134–138. [CrossRef] [PubMed]
18. Komatsu, S.; Shinaoka, A.; Kimata, Y. Modified Posterolateral Approach for Pterygium Colli. J. Craniofac. Surg. 2019, 30, 214–217.
[CrossRef]

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