Şule Selin AKYAN SOYDAŞ1, Ece
OCAK1, Murat Yasin GENÇOĞLU1, Sanem
ERYILMAZ POLAT1, Gökçen Dilşa
TUĞCU1, Güzin CİNEL1
1 Department of Pediatric Pulmonology, Ankara City
Hospital, Ankara Turkey
To the editor,
Isolated sternal cleft is rare a congenital malformation. It can be
isoleted or associated with various cardiac, abdominal, cranial and eye
malformations. Diagnosis with radiology. Management is only surgery as
soon as possible. Here in case we report a neonate with isoleted
incomplet sternal cleft.
CASE:
A twenty-five days old girl, admitted to pediatric pulmonology clinic,
with collapse on the anterior chest wall while breathing. Physical
examination revealed total retraction of the chest wall especially on
the upper part, paradoxical chest wall movements with respiration and a
raphe extending from the umbilicus to the lower end of the sternum
(Video-1). Other system findings were normal. Evaluation of sternum
related bone pathologies, thorax computed tomography (CT) and
3-dimensional (3D) reconstruction imaging was planned. On the thorax CT
hypoplasia of the inferior and non-development of the superior segment
of the sternum was found (Figure-1). Lung parenchyma was normal.
Abnormal findings were not detected in abdominal, cranial
ultrasonography, echocardiography and eye examination which were
evaluated in terms of pathologies that could accompany with strenal
cleft. At the age of fifty-six days, the patient had been operated. Due
to the large defect, primary closure couldn’t be performed as a surgical
procedure and the sternum was reconstructed with the cartilage obtained
from the tissue around the ribs.
DISCUSSION:
Sternal cleft is a rare congenital malformation, incidence of 2/100,000
[4], caused by failure in the fusion of sternal rods, on the
cranio-caudal direction, in the intrauterine 8-10th weeks [1]. It
was first described by De Torres in 1739 [2]. Malformation may be
total/parcel of the lower and/or upper part of the sternum, seen in 67%
in parsial form [3,4]. It is often sporadic, but similar cases have
been reported within the same family [5]. Sternal cleft can be
isolated or associated with syndromes such as PHACE, Cantrell Pentalogy,
accompanied by various cardiac, abdominal, cranial and eye related
malformations [7, 8]. Clinically, paradoxical chest wall movements
marked by respiration are pathagnomonic [4]. In adulthood, many
cases are asymptomatic, patients may also be diagnosed with symptoms of
accompanying malformations or various lung related symptoms [4].
Radiological imaging plays a key role in diagnosis, classification and
the search for associated malformations. Computed tomography is the gold
standard in diagnosis [4]. Surgical correction should be performed
as soon as possible (1-4 weeks in the neonatal period) to prevent
possible complications in all cases with or without any symptom [6, 9,
10]. Surgical correction is required in all cases to provide bone
protection to mediastinal tissues and organs, to create normal
intrathoracical pressure and to eliminate chest deformity regardless of
age at diagnosis [9]. In early intervention, primary repair can be
applied, in this procedure the sternal bands are simply approached
together [3]. In late intervention, complex reconstruction surgeries
are required due to the ossification of the cartilage tissue. Surgical
procedures such as oblique division of the cartilage, chondrotomy or
filling the defect with auto graft or some different materials can be
performed [3, 4].
The sternal cleft is a rare malformation; complete assessment, based
primarily on thoracic imaging is required for diagnosis. Also cardiac,
abdominal and cranial imaging and eye examination should be performed
for associated malformations. Regardless of the age at diagnosis,
patients should be managed by immediate surgery which is essential to
avoid possible complications.
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in a newborn of 20 days: Rare case. Int J Pediatr Otorhinolaryngol,
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2. JI., D.T., Extract of a letter from Jos. Ignat. De Torres, MD
to the Royal Society, containing an extraordinary case of the heart of a
child turned upside down. LondonPhilosophical Transactions, 1739.41 : p. 776–778.
3. Acastello, E., et al., Sternal cleft: a surgical opportunity.J Pediatr Surg, 2003. 38 (2): p. 178-83.
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sternal cleft, and other midline abnormalities: a new dominant
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in an Infant. European J Pediatr Surg Rep, 2015. 3 (2): p.
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sternal cleft repair. Eur J Cardiothorac Surg, 2009. 35 (3): p.
539-41.
10. Singh, S., et al., Sternal cleft repair: a report of two cases
and review of literature. Afr J Paediatr Surg, 2010. 7 (3): p.
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