Congenital Multiple Metatarsal Synostoses with Proximal Phalangeal Deformities of the Foot

Abstract

Congenital foot abnormalities such as syndactyly, polydactyly, and brachymetatarsia are commonly seen by plastic surgeons. There have been reports of cases of congenital synostosis of the foot, with the majority of cases involving the rear foot and midfoot (Boccio et al. [1]). However, to the best of our knowledge, multiple metatarsal synostoses with proximal phalangeal deformities is extremely rare and independent from any syndromes and congenital anomaly patterns.

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619
References
1. Yu RC, Pryce DW, Macfarlane AW, et al. A
histopathological study of 643 cutaneous horns. Br J
Dermatol 1991;124:449-52.
2. Mantese SA, Diogo PM, Rocha A, et al. Cutaneous
horn: a retrospective histopathological study of 222
cases. An Bras Dermatol 2010;85:157-63.
Congenital Multiple
Metatarsal Synostoses
with Proximal Phalangeal
Deformities of the Foot
Ai Yokoyama, Norio Fukuda, Hirotaka Asato
Department of Plastic and Reconstructive Surgery, Dokkyo
Medical University School of Medicine, Shimotsuga, Japan
Correspondence: Ai Yokoyama
Department of Plastic and Reconstructive Surgery, Dokkyo Medical University
School of Medicine, 880 Kitakobayashi, Mibu, Shimotsuga, Tochigi 321-0293,
Japan
Tel: +81-282-87-2485, Fax: +81-282-86-1806
E-mail: aiyoko@dokkyomed.ac.jp
No potential conflict of interest relevant to this article was reported.
Received: 24 Feb 2016 • Revised: 11 May 2016 • Accepted: 24 May 2016
pISSN: 2234-6163
•
eISSN: 2234-6171
https://doi.org/
10.5999/aps.2016.43.6.619
Arch Plast Surg 2016;43:619-621
Copyright 2016 The Korean Society of Plastic and Reconstructive Surgeons
This is an Open Access article distributed under the terms of the Creative Commons
Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0/)
which permits unrestricted non-commercial use, distribution, and reproduction in any
medium, provided the original work is properly cited.
Congenital foot abnormalities such as syndactyly,
polydactyly, and brachymetatarsia are commonly
seen by plastic surgeons. ere have been reports of
cases of congenital synostosis of the foot, with the
majority of cases involving the rear foot and midfoot
Fig. 2.
(A) Cross-section of the
giant cutaneous horns (GCH)
showing hollowing of conical
component with surrounding
parakeratosis, calcific debris
and keratin. Ruler markings
in centimeters. (B) H&E
stained section ( × 100) of the
GCH base with moderately
differentiated squamous cell
carcinoma.
A B
(Boccio et al. [1]). However, to the best of our
knowledge, multiple metatarsal synostoses with
proximal phalangeal deformities is extremely rare and
independent from any syndromes and congenital
anomaly paerns.
A 28-year-old woman was referred to our plastic
surgery outpatient clinic with a chief complaint of
pain and deformity in her right forefoot. ere were
deformities of the right fourth and h toes, making
it dicult for her to purchase ready-made heeled
shoes. e patient had a history of depression, but it
was controlled with no adverse eects on her daily
life. No other relevant past history or family history
was present. A clinical examination demonstrated
abducted fourth and h toes, and the interdigital
side of the rst toe and the dorsal sides of the fourth
and h toes had callosities (Fig. 1). e le foot was
normal in all respects.
Radiographs showed metatarsal synostoses from
the third to the h metatarsal bones, and the third
metatarsophalangeal joint was comprised of the third
and fourth proximal phalanges and the third
metatarsal bone (Fig. 2). In this case, dysfunction was
not severe, and a minimally invasive operation was
planned.
e operation was performed using an open
method. First, the extensor tendons of the fourth toe
were kept; then a wedge osteotomy at an angle of 30
degrees was performed, followed by abduction and
reduction of the sha of the fourth proximal phalanx.
Finally, the lateral surfaces of the bones were
perforated and xed by crossing so wire using the
two-dimensional intraosseous wiring technique
(Moriya et al. [2]). is technique provides stronger
xation between the bones with its tension band
function.
Titanium plate xation was added on the lateral side
of the proximal phalanx to ensure more reliable
Images

620
stability. e h toe was reshaped to be the same as
the fourth toe (Figs. 3, 4). Non-weight-bearing
rehabilitation continued for one month aer the
operation. e foot decreased in width, and pressure
on the interdigital side of the rst toe was relieved.
Consequently, the callosities on the foot disappeared
completely.
e patient has good foot alignment that has been
maintained for more than one year postoperatively
(Fig. 5). e patient can now wear heeled shoes
without any pain.
Metatarsal synostosis occurs as one of the
components of some congenital malformation
syndromes: Pfeier syndrome, Apert syndrome, and
Townes-Brocks syndrome. e responsible genes and
mode of inheritance have been identied in these
syndromes, but isolated cases are rare, and very few
cases have been reported; thus, their epidemiology
and pathogenesis are unknown. A case of non-
syndromic distal metatarsal synostosis of the fourth
and h toe was described in 2014 (Aspros et al.
[3]), but a report of multiple metatarsal synostoses as
in this case could not be found.
e hypoplasia of the fourth metatarsal bone and
the deformities of the fourth and h toes suggest the
possibility of a variant of cle foot with a normal
number of toes.
Blauth and Borisch [4] described metatarsal
hypoplasty or synostoses in a study on the
classication of cle feet. Partial synostoses usually
Fig. 1.
Preoperative photograph
illustrating the fourth and fifth
toe deformities of the right
foot.
Fig. 2.
Preoperative radiograph of
the right foot showing the
synostoses and the third
metatarsophalangeal joint
malformation.
Fig. 3.
The schema of the osteotomy and the two-dimensional
intraosseous wiring technique fixation for the fourth
and fifth proximal phalanges.
Fig. 4.
Postoperative dorsal radiograph.

Vol. 43 / No. 6 / November 2016
621
occurred at the distal parts of the phalanges or
metatarsal bones; most frequently, the rst and
second metatarsals. e third, fourth, and h toes
were partially or completely synostotic (Blauth and
Borisch [4]).
Ogino and Kato [5] described six cases of cle hand
with a normal number of ngers as well. Some
anomalies of the adjacent ngers were recognized in
several cases, including cutaneous syndactyly,
brachymesophalangy, and central polydactyly. ey
considered the cause of cle hand without absence of
the nger as a failure of separation of the nger rays.
is assumption is now accepted as “cle of the
palm”, which is classied as a type of abnormal
induction of the digital rays in the modied
International Federation of Societies for Surgery of
the Hand classication.
Ogino and Kato found another trend, in that the
cle in cases of cle hand without absence of the
nger was not much more severe than with absence
of the nger (Ogino and Kato [5]). Given the
literature, it is possible that the present case is “mild
abnormal induction of digital rays,” though we were
not able to nd a cle involving the foot. However, it
is very dicult to make a fair judgment on the
classication of this case. Further studies of the cause
of this congenital foot abnormality are needed.
References
1. Boccio JR, Dockery GL, LeBaron S. Congenital
Fig. 5.
Photograph of the right foot at one year
postoperatively.
metatarsal synostosis. J Foot Surg 1984;23:41-5.
2. Moriya K, Yoshizu T, Maki Y, et al. New internal xation
technique of the digital skeleton in the hand: clinical
results of two-dimensional intraosseous wiring. Seikei
Geka 2012;63:9-13.
3. Aspros D, Ananda-Rajan E, Jnr ZK, et al. Distal
metatarsal synostosis: a case report. Foot (Edinb)
2014;24:153-6.
4. Blauth W, Borisch NC. Cle feet. Proposals for a new
classication based on roentgenographic morphology.
Clin Orthop Relat Res 1990;(258):41-8.
5. Ogino T, Kato H. Cle hand without absence of a nger.
Handchir Mikrochir Plast Chir 1988;20:184-8.
Early Vascularized Fibular
Gras in Infants with
Congenital Pseudarthrosis
Ki Ho Kim1, Seungki Youn1, Tai Seung Kim2,
Heechang Ahn1
Departments of 1Plastic and Reconstructive Surgery and
2Orthopedic Surgery, Hanyang University College of Medicine,
Seoul, Korea
Correspondence: Heechang Ahn
Department of Plastic and Reconstructive Surgery, Hanyang University College
of Medicine, 222-1 Wangsimni-ro, Seongdong-gu, Seoul 04763, Korea
Tel: +82-2-2290-8560, Fax: 02-2295-7671
E-mail: ahnhc@hanyang.ac.kr
No potential conflict of interest relevant to this article was reported.
Received: 22 Jun 2016 • Revised: 30 Aug 2016 • Accepted: 20 Sep 2016
pISSN: 2234-6163
•
eISSN: 2234-6171
https://doi.org/
10.5999/aps.2016.43.6.621
Arch Plast Surg 2016;43:621-623
Copyright 2016 The Korean Society of Plastic and Reconstructive Surgeons
This is an Open Access article distributed under the terms of the Creative Commons
Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0/)
which permits unrestricted non-commercial use, distribution, and reproduction in any
medium, provided the original work is properly cited.
Neurobromatosis type I, also known as Von
Recklinghausen disease, is one of the most common
congenital disorders, with an incidence of 1 out of
3,000–4,000. It can be diagnosed on the basis of
characteristic phenotypic manifestations, such as
café-au-lait spots, axillary and inguinal freckling,
neurobromas, optic gliomas, Lisch nodules and
distinctive osseous lesions. Osseous lesions occur in
2%–3% of neurobromatosis type I cases, and are
known to be caused by a lack of osteoblast function
and the consolidation of osteoclast activity [1]. e
most commonly aected region is the diaphysis of
the tibia. Due to segmental disturbances in periosteal
bone formation, anterior bowing occurs, and
Images