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Case report. Isolated complete congenital sternal cleft in an adult: MDCT imaging findings

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B Battal
B Battal
B Battal
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Ibrahim Karademir
Ibrahim Karademir
U Bozlar
U Bozlar
U Bozlar
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Mutlu Sağlam at Gulhane Military Medical Academy
Mutlu Sağlam
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Abstract and Figures

Sternal cleft is a very rare midline defect. Incomplete forms are more common than complete ones. In this challenging anomaly, the underlying mediastinal structures (especially the heart and great vessels) can easily be injured by external trauma. In addition, the deformity is cosmetically unpleasant and quite alarming to the young patient and the patient's family. In this report, we describe the multidetector CT (MDCT) images that demonstrate the complete sternal cleft.
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CASE REPORT
Isolated complete congenital sternal cleft in an adult: MDCT
imaging findings
1
B BATTAL, MD,
2
I KARADEMIR, MD,
3
U BOZLAR, MD,
3
M SAGLAM, MD,
3
N BULAKBASI, MD and
3
M TASAR, MD
1
Sarikamis Military Hospital, Department of Radiology, 36500, Sarikamis, Kars,
2
I
˙zmir Military Hospital, Department of
Radiology, 35362, Hatay, Izmir and
3
Gulhane Military Medical School, Department of Radiology, 06018, Etlik, Ankara,
Turkey
ABSTRACT. Sternal cleft is a very rare midline defect. Incomplete forms are more
common than complete ones. In this challenging anomaly, the underlying mediastinal
structures (especially the heart and great vessels) can easily be injured by external
trauma. In addition, the deformity is cosmetically unpleasant and quite alarming to the
young patient and the patient’s family. In this report, we describe the multidetector CT
(MDCT) images that demonstrate the complete sternal cleft.
Received 24 August 2008
Revised 27 October 2008
Accepted 3 November
2008
DOI: 10.1259/bjr/76694119
’2009 The British Institute of
Radiology
An isolated complete congenital sternal fissure or cleft
in an adult is a rare anomaly, and very few cases have
been reported [1, 2]. Although the embryology of the
thoracic wall is well known, the aetiology of cleft
sternum remains unclear. Failure of fusion of the lateral
sternal bands by an early disturbance affecting the
midline mesodermal structures between the sixth and
ninth gestational week is a possible mechanism but no
familial, teratogenic or nutritional factors have been
identified as a potential cause [3, 4]. It is difficult to
visualise sternal variations and anomalies on radio-
graphy. Multiplanar and three-dimensional recon-
structed multidetector CT (MDCT) images are useful in
the evaluation of the human skeleton, especially of
complex parts such as the sternum [5].
Case report
A 20-year-old male patient presented with a complete
sternal cleft associated with pectus excavatum since
birth, causing a cosmetic concern and constant fear of
trauma. His family history was unrevealing. Physical
examination revealed a gap at the site of the sternum and
pectus excavatum, without any other associated abnorm-
alities. The wide gap measured 3 cm at the proximal and
0.8 cm at the distal sternum. The defect was covered by a
thin layer of skin; in addition, pulsations of the heart
could be seen through the defect. A MDCT scan was
performed in our radiology department. It showed a
complete sternal cleft (Figure 1a,b). No other cardiothor-
acic abnormalities were present. An electrocardiogram
and transthoracic echocardiographic examination of the
patient were normal. There were no other abnormalities
on thorax CT. His abdominal ultrasonograph also did
not show any abnormalities.Repair surgery with
prosthetic graft material was planned, but the patient
refused surgery.
Discussion
Cleft sternum is usually not associated with cardiac
defects, is usually not fatal, and comprises a rare
spectrum of ventral fusion abnormalities, which may
be classified as complete or incomplete. Complete cleft
sternum is the less common form [6, 7]. Its clinical
significance is that it leaves the heart and great vessels
unprotected [8].
In embryonic life, the sternum has its origin in the
lateral plate mesoderm. Cells from two bands of
mesoderm on either side of the anterior chest wall
migrate toward the midline and become fused by the
tenth week, thus forming the sternum. The manubrium
is formed by primordia between the ventral ends of the
developing clavicles. Very rarely, the sternal bars fail to
join in the midline, which results in a complete sternal
cleft [9].
Besides the isolated forms with good prognosis, cleft
sternum can present in association with other life-
threatening disorders, e.g. ectopia cordis, or as a part of
Cantrell’s pentalogy, which comprises a lower sternal
defect, a deficiency of the anterior diaphragm and
pericardium, a midline supraumbilical abdominal wall
defect and an intracardiac disorder [10, 11].
CT with multiplanar reconstruction may show rele-
vant anatomic detail of sternal variations and anomalies.
Address correspondence to: Bilal Battal, Sarikamis Military
Hospital, Department of Radiology, 36500, Sarikamis, Kars,
Turkey. E-mail: bilbat_23@yahoo.com
The British Journal of Radiology, 82 (2009), e202–e203
e202 The British Journal of Radiology, October 2009
Multiplanar reconstructed MDCT images are helpful in
wholly revealing the sternal anatomy. Curved–planar
coronal reconstruction is useful for showing manubrios-
ternal and sternoxyphoidal fusions, sternal bands and
clefts [5].
A number of methods for the repair of cleft sternum
have been reported, including (i) primary approxima-
tion, (ii) sliding or rotating chondrotomies, and (iii) the
use of prosthetic grafts or flaps of bone, cartilage,
autogenous tissue or pectoralis major muscle [2, 12].
In conclusion, it is difficult to visualise sternal
variations and anomalies by radiography. Therefore,
cross-sectional imaging such as CT and MRI is normally
required to describe sternal (and associated cardiovas-
cular) anomalies.
References
1. Suri KR, Sharma KR, Jha NK, Sharma BK. Complete
congenital sternal cleft in an adult: repair by autogenous
tissues. Ann Thorac Surg 1996;62:573–5.
2. Sarper A, Oz N, Arslan G, Demircan A. Complete
congenital sternal cleft associated with pectus excavatum.
Tex Heart Inst J 2002;29:206–9.
3. Hersh JH, Waterfill D, Rudletge J, Harrod MJ, O’Sheal SF,
Verdi G, et al. Sternal malformation/vascular dysplasia
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10. Cantrell JR, Haller JA, Ravitch M. A syndrome of congenital
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11. Cottrill CM, Tamaren J, Hall B. Sternal defects associated
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12. Snyder BJ, Robbins RC, Ramos D. Primary repair of
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Ann Thorac Surg 1996;61:983–4.
(a) (b)
Figure 1. Complete congenital sternal cleft associated with pectus excavatum in a 20-year-old man is well demonstrated on (a)
axial and (b) coronal maximum intensity projection multidetector CT (MDCT) images. MDCT images show the complete sternal
cleft (arrows).
Case report: Complete congenital sternal cleft: MDCT imaging findings
The British Journal of Radiology, October 2009 e203
... Congenital abnormalities of the anterior thoracic wall comprise a spectrum of deformities such as thoracic ectopia cordis, cervical ectopia cordis, thoracoabdominal ectopia cordis, pectus excavatum, pectus carinatum, cleft sternum, and short sternum [1]. Although pectus excavatum, pectus carinatum, and cleft sternum can present as isolated deformity (less than 30 cases of isolated cleft sternum are reported in literature) [2][3][4][5][6], in most cases they are associated with heart and inner organs anomalies and described as symptoms of syndromes like Marfan syndrome, Noonan syndrome, Poland anomaly, and Cantrell pentalogy [1][2][3][4][5][6][7]. ...
... Congenital abnormalities of the anterior thoracic wall comprise a spectrum of deformities such as thoracic ectopia cordis, cervical ectopia cordis, thoracoabdominal ectopia cordis, pectus excavatum, pectus carinatum, cleft sternum, and short sternum [1]. Although pectus excavatum, pectus carinatum, and cleft sternum can present as isolated deformity (less than 30 cases of isolated cleft sternum are reported in literature) [2][3][4][5][6], in most cases they are associated with heart and inner organs anomalies and described as symptoms of syndromes like Marfan syndrome, Noonan syndrome, Poland anomaly, and Cantrell pentalogy [1][2][3][4][5][6][7]. ...
... In contrast, the etiology of an isolated defect is not well understood. Many factors have been associated with murine models, like alcohol, riboflavin and methylcobalamin deficiency, and HOX4 gene disruption, but no significant associations have been reported in humans [2][3][4][5][6][7]. ...
Isolated Asymptomatic Short Sternum in a Healthy Young Girl
Article
Full-text available
  • Jul 2014
Congenital sternal defects are rare deformities frequently associated with other anomalies of the chest wall and other organ systems. Although pectus excavatum, pectus carinatum, and cleft sternum can present as isolated deformity, in most cases they are associated with heart and inner organs anomalies and described as symptoms of syndromes like Marfan syndrome, Noonan syndrome, Poland anomaly, and Cantrell pentalogy. In contrast, the etiology of an isolated defect is not well understood. We observed a short sternum (dysmorphic manubrium, hypoplastic body, and complete absence of the xiphoid process) in a completely asymptomatic 13-year-old woman. A comprehensive instrumental exams panel was performed to exclude associated anomalies of the heart and of the other organ systems. The patient was completely asymptomatic and she did not need any medical or surgical treatment. To our knowledge, this is the first case of isolated short sternum reported in literature.
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... Sternum anomalies have been evaluated in a number of studies based either on autopsy population [1][2][3], or on living individuals mainly with the use of MDCT [4][5][6][7][8][9][10][11][12][13][14][15][16]. ...
Frequency of sternal variations and anomalies in living individuals evaluated by MDCT
Article
  • Jun 2021
  • EUR J RADIOL
Purpose The aim of this retrospective study was to present the frequency and MDCT appearances of sternal variations and anomalies, as well as to highlight their clinical significance. Methods This retrospective study was carried out on 1150 patients, who underwent chest MDCT. Axial planes, multiplanar and curved-planar reconstructed images were studied. Age and sex distribution of the variations was evaluated. Results Anatomical variations of the sternum were found in 74.1%. The most frequent variation was the double-ended xiphoid process (36.9%), followed by the single xiphoidal foramen (25.8%) and the sternal sclerotic band (12.8%). Other variations observed were: sternal notch (10.1%), sternal foramen (4.9%), complete manubriosternal fusion (4.1%) and sternoxiphoidal fusion (4.1%), triple-ended xiphoid process (3.7%), sternal cleft (1.5%), whereas the rest of the variations including sternoxiphoidal junction pseudoforamen, suprasternal bone, pseudocleft, suprasternal tubercle and absence of xiphoid process were in less than 1%. In our subjects, sternal and xiphoidal foramina were adjacent to: the pericardium (37.14%), the diaphragm (22.9%), the mediastinal fat (17.1 %), the liver (11.4%), the lung (8.5%) and to the stomach (2.9%). Conclusions Sternal variations are frequent, asymptomatic, detected incidentally at cross-sectional imaging and may be confused with pathologic conditions. Radiologists should be familiar with these variations in order to discriminate them from pathologies and avoid complications during interventional procedures. Advances in knowledge This study presents thoroughly the sternal variations’ MDCT appearance, detected in a Greek population, correlates them with age and gender and discuss their clinical significance in detail.
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... If diagnosed early, these isolated anomalies are potentially correctable and have a better prognosis than if they are associated with other deformities. 2,4 In such isolated cases however, the etiology is not well understood. Various possible causes have been proposed based on animal models such as alcohol exposure, riboflavin and methyl cobalamin deficiency as well as genetic factors such as HOX4 gene disruption. ...
Isolated sternal hypoplasia: a rare cause of kyphoscoliosis
Article
Full-text available
  • Feb 2019
In most cases, kyphoscoliosis is idiopathic. However, this is a diagnosis of exclusion and can be made only if no cause can be identified. Kyphoscoliosis can occur due to various causes. Isolated sternal anomalies may also cause kyphoscoliosis secondary to the bony deformity though this has not previously been reported in literature. We have reported a case of kyphoscoliosis secondary to isolated sternal hypoplasia with complete absence of bony and cartilaginous elements of the body and xiphoid process of the sternum without any associated deformities of mediastinal structures, lung parenchyma or soft tissues in a young male patient. Careful evaluation of patients with kyphoscoliosis can ensure timely diagnosis of unusual and potentially treatable causes for the same such as sternal anomalies. Addition of lateral chest radiographs to the imaging protocol for evaluation of kyphoscoliosis can play a major role in timely diagnosis of such cases.
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Isolated Complete Cleft
Article
  • Nov 2012
Cleft sternum is a rare congenital anomaly due to failure of fusion of the sternal bone. The etiology of this defect is not well understood. The anomaly is more common in females and may be associated with Cantrell's pentalogy. It can be partial or complete. An 8-year-old female child presented with complete defect in the sternum which was confirmed with a chest radiograph and computed tomography. Only 24 cases of isolated complete cleft sternum have been reported so far. The treatment ranges from primary closure of the defect to the use of various autologous and prosthetic grafts. Our patient was treated with primary closure of the defect.
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Phenotypic spectrum and management of sternal cleft: Literature review and presentation of a new series
Article
  • Jul 2011
  • EUR J CARDIO-THORAC
Sternal cleft is a chest wall malformation that can expose mediastinal viscera and vessels to injuries. It can be classified into two forms, complete and partial. Its etiology and incidence are unknown and it is often associated with other defects. The aim of this article is to review the literature and report our experience with this rare anomaly, focusing on clinical presentation and management. We reviewed the English written literature about sternal cleft and collected the clinical data of all the published series. We present seven new cases that we have observed and treated since 1999. Literature reports 51 series including 86 patients, more frequently female (62%) and affected with partial superior form (67%). Sternal cleft is often asymptomatic (74%) and associated with other defects (72%). Surgical treatments include primary closure (73%), bone graft interposition (10%), prosthetic closure (7%), and muscle flap interposition (3%). In our series, primary closure was possible in four cases, while in three cases we placed a prosthesis. Five patients had associated defects and two were affected with PHACES (posterior fossa abnormalities, hemangiomas, arterial lesions, cardiac abnormalities/aortic coarctation, abnormalities of the eye, and sternum defects) syndrome. We report for the first time the association of sternal cleft with connectival nevi in three of our patients. At follow-up, we observed no major complication or recurrences. Although primary closure is the preferred option and should be performed in the neonatal period, the use of prostheses warrants good results as well. Prior to treatment, associated defects and syndromes should be excluded.
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Repair of congenital sternal cleft in infants and adolescents
Article
Full-text available
  • Nov 1998
  • ANN THORAC SURG
Clinical and surgical aspects of sternal cleft repair are presented. Primary repair in the neonatal period is the best management for this rare condition, but none of the patients in this report were referred to us during that period. Autologous repair is suitable for older patients because it avoids problems related to the implant of prosthetic materials. This article reviews 8 cases of sternal cleft not associated with ectopia cordis in patients presenting between October 1979 and November 1997. Surgical repair consisted of three sliding chondrotomies, three posterior sternal wall repairs, one combination with the Ravitch technique for pectus excavatum repair, and one posterior sternal wall repair associated with total repair of Cantrell's pentalogy. All patients who submitted to surgical correction had good aesthetic and structural results. The postoperative period was uneventful except that a subcutaneous fluid collection developed in 1 patient. The mean hospital stay was 5.8 days. The patients were followed up from 4 months to 18 years. Whether dealing with older children or young adults, the technique of reconstructing a new sternum with a posterior periosteal flap from sternal bars and chondral grafts is a simple, quick, inexpensive, and effective option.
View
Complete Congenital Sternal Cleft Associated with Pectus Excavatum
Article
Full-text available
  • Feb 2002
We report herein a rare case of complete congenital sternal cleft (absent sternum) and anterior pericardial defect in association with pectus excavatum. In neonates with absent sternum, the sternal bars can be easily approximated by simple suture, due to the flexibility of the cartilaginous thorax. There is also little danger of cardiac compression when the repair is performed early in life. If reconstruction is delayed, the increased rigidity of the chest wall and the physiologic accommodation of the thoracic organs to the circumference of the chest render simple approximation impossible, without serious compromise of the heart and lungs. Our patient was a 13-year-old girl, whose case was particularly unusual because of the association of sternal cleft with pectus excavatum. After surgical correction of the pectus excavatum, we were able to construct a sternum by incising the lateral border of each sternal bar, thereby creating flaps that we sutured together at midline. The sternal bars were then approximated by loops of nonabsorbable suture around their circumference. The patient had an uncomplicated course, and at the 12-month follow-up visit, her sternal appearance was normal.
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Sternal malformation/vascular dysplasia association
Article
  • May 1985
Sternal defects associated with superficial craniofacial vascular lesions are rare. We report on two additional patients with a sternal cleft and cutaneous, craniofacial hemangiomata to emphasize that this unusual combination of findings represents a recognizable sternal malformation/vascular dysplasia association. In addition, internal vascular lesions were also identified in these individuals, in one instance involving the upper respiratory tract and in the other the viscera. Although the pathogenesis of these findings is unclear, an early disturbance affecting midline mesodermal structures leading to lack of complete fusion of lateral sternal bands and overlying cutaneous tissue, or deficient formation of a proposed medioventral unpaired structure which may be involved in the formation of the sternum, and persistence and proliferation of midline angioblastic tissue may be possible mechanisms during the sixth to ninth gestational weeks. To date, all but one of the 15 known cases have been sporadic and no teratogen has been identified as a cause for these clinical manifestations. The presence of this association should signal the need to search for potentially life-threatening internal hemangiomata.
View
Repair of congenital sternal clefts in adolescence and infancy
Article
  • Jan 1995
Two cases of sternal cleft not associated with ectopia cordis are presented. An 11-year-old girl with a superior incomplete sternal cleft underwent reconstruction of the sternum with autologous rib, cartilage, and sternal periosteum. At the 1-year follow-up her sternal appearance was normal. The second patient, a full-term baby girl, had complete sternal cleft diagnosed by ultrasonography at 21 weeks' gestation. She underwent primary repair in the neonatal period and currently is asymptomatic with a normal-appearing sternum (10 months postoperatively). Primary repair in the neonatal period is the best type of management for this rare condition. For older patients, autologous repair is appropriate and avoids problems associated with the use of prosthetic materials.
View
Primary repair of complete sternal cleft with pectoralis major muscle flaps
Article
  • Apr 1996
  • ANN THORAC SURG
We report the successful coverage of a complete sternal cleft with exposed pericardium using bilateral pectoralis major muscle flaps in a newborn. The patient was transferred to our institution with nearly complete sternal aplasia, rectus diastasis, and only dessicated pericardium covering the visible heart. Complete closure without compromise of cardiac function was achieved at 2 days of age with bilateral pectoralis major muscle flaps and fasciocutaneous advancement flaps.
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Complete congenital sternal cleft in an adult: Repair by autogenous tissues
Article
  • Sep 1996
  • ANN THORAC SURG
We are reporting here a rare case of congenital complete sternal cleft in an adult of 25 years of age, with absent anterior pericardium and "diastasis recti." Successful surgical repair was achieved along with the preservation of the already existing anatomic and mechanical sanctity of thoracic cage, sternal reconstruction, and protection of the underlying heart and great vessels with a good cosmetic result using autogenous tissue i.e., iliac bone and "V-Y" myoplasty of pectoralis major muscles with their functional preservation and correction of associated anomalies.
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Sternal defects associated with congenital pericardial and cardiac defects
Article
  • Feb 1998
We describe three cases of sternal defects of varying severity associated with other congenital anomalies. In the most severe case, both anterior and posterior defects were seen, with near-absence of the sternum and pericardium continuous with a large omphalocele. This resulted in external location of organs usually confined within the thoracic and abdominal cavities. A ventricular septal defect was present, and the arterial duct was absent. The course of the ascending aorta was anomalous. The baby had anencephaly and rachischisis. In the intermediate case, a proximal sternal cleft was associated with shortening of the sternum, and absence of the manubrium. Anterior pericardial and diaphragmatic defects were seen, while a scalp defect and an encephalocele were present on the posterior aspect of the head. This baby had tricuspid atresia. The remaining case had only an anterior defect with a shortened sternum. A supra-umbilical omphalocele contained a left ventricular diverticulum without interposing pericardium or diaphragm. Ventricular and atrial septal defects were present. The first two cases can be considered as representing failure of development of both an anterior and a posterior midline field. The third case, much milder than the other two, represents failure of development of an anterior field.
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Cleft sternum and sternal foramen
Article
  • Jun 2000
  • Chest Surg Clin
Cleft sternum is a rare congenital defect of the anterior chest wall and is the result of a failed midline fusion of the sternum. Depending on the degree of separation, there are complete and incomplete forms. Its clinical significance is that it leaves the heart and great vessels unprotected. Association with craniofacial hemangiomas and omphalocele is common. Prenatal diagnosis by ultrasonography is possible. Surgical correction should be performed during the neonatal period when the direct suturing of the sternal halves is possible and the thorax can accommodate the thoracic viscera. At an older age, surgical repair is feasible, but it may require additional measures, such as sliding chondrotomies of the adjacent costal cartilages and notching of the sternal bars, to facilitate the approximation. Lung herniation at the base of the neck should be corrected by uniting the cervical muscles. The first postoperative day is the most critical because of acute reduction of the mediastinal space. Long-term results are satisfactory. Sternal foramen is a congenital oval defect at the lower third of the sternum that is asymptomatic and could be detected by CT scanning. The awareness of the anomaly is important in acupuncture practice because of the danger of heart damage.
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