D. Mendis 1; Moss A. L.h. 2
Department of ENT Surgery, University Hospital Coventry, Coventry, and
1; Department of Plastic and Reconstructive Surgery, St George’s NHS Trust, London, United Kingdom
Vyšlo v časopise:
ACTA CHIRURGIAE PLASTICAE, 49, 3, 2007, pp. 71-74
Caucasian male infant presented to the department with a midline
cervical cleft, which had been present since birth. The infant was
born full-term by a normal vaginal delivery. The pregnancy was
uncomplicated and there was no relevant maternal history, antenatal
investigations were also normal. There was a family history of
a thyroglossal cyst, present in a fellow sibling.
cleft was situated in the midline between the symphysis menti and
suprasternal notch. It consisted of a skin tag, an area of
friable epithelium and a sinus tract extending to the
suprasternal notch. The friable area had previously bled
intermittently and the midline of the mandible was noted to be
notched. There was no contracture of the neck and the infant did not
appear to be troubled by the cleft.
were no other dysmorphic features and a full examination was
otherwise unremarkable apart from a slightly low weight, which
had been a problem since birth. Ultrasound of the neck
demonstrated a normal thyroid gland.
surgery consisted of an excision of the midline cleft and primary
closure with a local flap. The area of friable epithelium and
the sinus tract was excised with an elliptical incision. The midline
skin tag was divided in half in the sagittal plane and excised
creating two lateral flaps for closure. There were no significant
female Caucasian infant presented to the department with a midline
ventral cervical cleft. The baby was born prematurely at 33 weeks and
delivered by a normal vaginal delivery. At birth the baby was in
respiratory distress and was ventilated for 10 days, with parenteral
nutrition being given for 7 days. Other noted conditions were
a patent ductus arteriosus. The thyroid function tests were
noted to be normal at birth. There was no family history of
note. Surgery consisted of excision of the mucosal track, using
part of the accessory ‘tag’ as two transposition flaps.
Z-plasties were also used to break up the vertical linear scar
specimens were fixed, sectioned and stained with haematoxylin and
from the first case showed epidermis and dermis with some attached
subcutaneous tissue. The sinus tract was lined by ciliated
pseudostratified columnar epithelium (Fig. 6, 7). This was also
concluded to be bronchogenic in origin with no
features of atypia.
specimen from the second case contained a cutaneous tag devoid
of adnexal structures, the distal part of the specimen consisted of
a non-ciliated columnar epithelium opening into a more
dilated cystic structure lined by pseudo-stratified columnar
epithelium (Fig. 8).
is a rare anomaly of the ventral neck that may present at any
level between the mandible and manubrium. They appear to be sporadic
in the patient’s families and more commonly affect Caucasian
the cephalic skin tag consists of epidermis with occasional bundles
of skeletal muscle in the core. The mucosal surface consists of
a stratified squamous epithelium surface with an absence of skin
appendages. The caudal sinus can be lined with pseudostratified
columnar epithelium or squamous epithelium (absence of ciliation)
indicating a branchial origin. The sinus can also be lined with
ciliated pseudostratified columnar or squamous epithelium, the
presence of ciliation indicates a bronchogenic origin.
cysts have a cuboidal or columnar, ciliated pseudostratified
epithelial lining, commonly squamous metaplasia can occur following
infection, as in our first case. Other structures invariably present
in the cyst wall are blood vessels, hyaline cartilage, smooth muscle,
seromucinous glands and elastic fibres (8). They can occur in the
mediastinum, along the tracheobronchial tree or peripherally in lung
parenchyma. Location in the subcutaneous tissues is rare (9). When
subcutaneous they present as a soft swelling or draining sinus
in the suprasternal notch or supraclavicular area.
extra-thoracic subcutaneous location of bronchogenic cysts is thought
to arise by the pinching off the pulmonary trunk during the embryonic
development of the sternum. The laryngotracheal groove separates the
primitive foregut into ventral (tracheal) and dorsal (oesophageal)
structures beginning in the 5th
week of gestation. The ventral portion gives rise to the trachea and
major bronchi. The sternum arises by the fusion of lateral
cartilaginous plates, during this process pulmonary parenchyma may
protrude through the gap and become pinched off as the bars fuse
completely by the third month in utero
forming a bronchogenic cyst (8).
of our reported cases have the clinical features of CMCC, with the
first case appearing to be bronchogenic in origin and the second case
appears to be solely branchial in origin due to the absence of
of midline fusion of the branchial arches are thought to be
responsible for the spectrum of branchial arch anomalies. If this
involves the second cleft it may lead to an isolated cervical cleft.
The branchial arches form part of the branchial apparatus, which
consists of embryonic ectoderm, mesoderm and endoderm and is
responsible for forming the head and neck structures. Development of
the apparatus begins during the second week of gestation and is
completed by week 6–7. The apparatus consists of five branchial
arches appearing at the lateral wall of the foregut, separated from
each other externally by ectoderm-lined branchial clefts and
internally by endoderm-lined pharyngeal pouches. The arches are
numbered 1, 2, 3, 4 from cranium to cauda; the 6tharch does not appear externally and is only
represented by a mesodermal core (Fig. 9).
for the failure of midline fusion include increased pressure of the
pericardial roof on the most distal branchial arches in the early
stages of the developing embryo (30th–35th
day) resulting in necrosis and ischaemia. At this stage separation of
the respiratory system from the digestive system is occurring and
a disturbance in this area is likely to affect both systems (6).
Another theory is the rupture of a pathological adhesion between
the epithelium of the cardiohepatic fold with that of the ventral
part of the first branchial arch (10).
real question is the whether the clinical picture of CMCC originates
from a branchial developmental abnormality in-utero
or are the previous reported cases actually bronchogenic in origin
with squamous metaplasia accounting for the squamous component. From
a review of previously reported cases, the histology has
undoubtedly had some respiratory components in the majority. We have
reported on two theories which account for the abnormality in the
second branchial arch and CMCC and one theory which accounts for
bronchogenic defects in isolation. There appears to be much
variability in CMCC, with it either being solely branchial or solely
bronchogenic or both. No satisfactory theory accounts for how they
can exist together and some of the previously reported cases do not
clearly differentiate the histology and the two entities leading to
report on two cases with the clinical diagnosis of CMCC, the
embryology of the first case suggests a bronchogenic component
with squamous metaplasia and the second case indicates a solely
branchial origin. This indicates the variability in the embryonic
origin of a clinical CMCC, implying that this is spectrum of
disorders with variations on the same theme. The recommended
treatment is complete excision for cosmetic reasons and to overcome
restricted neck extension due to fibrous midline cervical webbing. It
may be difficult to categorically prove the embryological cause for
this clinical entity but by identifying more cases the importance of
raising awareness of this condition has relevance to plastic
surgeons, otolaryngologists and maxillofacial surgeons.
woud like to thank Dr Ruth Nash and Dr Heung Chong, Consultant
Histopathologists, St. George’s Hospital, for their much
appreciated comments. We also thank Dr Anand Saggar, Consultant
Geneticist, for his valued advice.
ENT Surgery, University Hospital Coventry
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