Sternal Cleft Research Articles

Adult Presentation of PHACES Syndrome

A significant percentage of children with hemangiomas may have PHACES syndrome which refers to the association of posterior fossa malformations, facial hemangiomas, arterial cerebrovascular abnormalities, cardiovascular anomalies, eye abnormalities and ventral defects like sternal clefting or supraumbilical raphe. A variety of factors have led to under diagnosis of PHACES syndrome in the past including lack of awareness and limited imaging modalities. Also, patients with PHACES syndrome with arterial cerebrovascular abnormalities can present with acute ischemic stroke. However, these patients usually present before one year of age. We describe a 29-year-old woman with no history of cerebrovascular disease who initially presented with symptoms of a stroke and was subsequently diagnosed to have PHACES syndrome exhibiting an array of multiple unusual imaging findings. We also discuss the current literature and recommendations about PHACES syndrome.

Sternal Cleft in a Neonate- Repaired with Silastic Block and Pectoralis Major Flaps

Sternal Cleft is a rare variety of congenital malformation seen in neonates. It is a potentially life threatening condition that may be associated with developmental anomalies of other visceral structures. Very few cases have been reported of this condition and experience with regard to time for intervention and management is limited. The authors report a case of partial sternal cleft, without any associated anomaly that had successful outcome after surgical reconstruction.

Phenotypic spectrum and management of sternal cleft: literature review and presentation of a new series

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.

Diagnosis of Pentalogy of Cantrell Using 2- and 3-Dimensional Sonography

Pentalogy of Cantrell is a very rare congenital syndrome with major anomalies and a very poor prognosis. It is described as a defect in midline closure and includes 5 characteristics: (1) a supraumbilical omphalocele, (2) a cleft sternum, (3) diaphragmatic defects, (4) pericardial defects, and (5) other associated cardiac anomalies. 1 The prevalence of this syndrome has been quoted to be between 1 per 65,000 to 1 per 200,000 births. 2 We report the second-trimester diagnosis of a case of pentalogy of Cantrell made by traditional 2-dimensional (2D) sonography combined with newer 3-dimensional (3D) methods.

A rare case of isolated complete congenital sternal cleft

A rare case of isolated complete congenital sternal cleft

PP-299. An unusual entity: Asymptomatic isolated superior sternal cleft

PP-299. An unusual entity: Asymptomatic isolated superior sternal cleft

Fente sternale : intérêt d’une prise en charge chirurgicale néonatale

Sternal cleft is a rare congenital abnormality. We report on a neonatal case of sternal cleft with median raphe with a follow-up of 3 years. We review the advantages of surgical repair in the neonatal period because the flexibility of the chest wall is maximal and compression of underlying structures is minimal.

Primitive mesodermal cells with a neural crest stem cell phenotype predominate proliferating infantile haemangioma

AimsInfantile haemangioma is a tumour of the microvasculature characterised by aggressive angiogenesis during infancy and spontaneously gradual involution, often leaving a fibro-fatty residuum. The segmental distribution of a subgroup of...

PHACE/S syndrome: A syndromic infantile segmental hemangioma

PHACES syndrome is a neurocutaneous disorder characterized by posterior fossa brain malformations, hemangiomas, cardiac anomalies and coarctation of aorta, eye anomalies ± sternal clefts. All reported cases are sporadic and notably common in females. The underlying cause is unknown. Here is described, one of the twin baby with characteristic features of PHACE syndrome. The presence of large segmental hemangioma, especially on face should prompt the primary care provider to act early, to prevent complications related to facial hemangiomas and other associated anomalies.

Successful Surgical Repair of Complete Sternal Cleft in an Adult

We describe direct repair of congenital complete sternal cleft in a 35-year-old woman, using titanium plates fixed by pre-drilled screws to the manubrium and costal cartilages. This provided excellent protective and cosmetic results.

Neurocutaneous vascular syndromes

There have been significant recent advances in the past several years in the field of neurocutaneous vascular syndromes, including the development of more stringent diagnostic criteria for PHACE syndrome, the renaming of macrocephaly-cutis marmorata telangiectatica congenita to macrocephaly-capillary malformation to accurately reflect the true nature of the syndrome, and discovery of new genetic mutations such as RASA-1. There have also been advances in the understanding and management of Sturge-Weber syndrome.PHACE syndrome is a constellation of neurologic, arterial, cardiac, ophthalmologic, and sternal abnormalities associated with infantile hemangiomas. PHACE is an acronym for Posterior fossa malformation, Hemangioma, Arterial anomalies, Coarctation of the aorta, Eye abnormalities. Some authors include an "S" for PHACE(S) to denote the association of ventral defects including Sternal clefting and Supraumbilical raphe.The accurate diagnosis and work-up of these patients require coordination of care across multiple disciplines, including neuroradiology, radiology, dermatology, neurology, surgery, and interventional radiology, among others.This paper is meant to update clinicians and researchers about important advances in these rare, important vascular syndromes, to improve care, and lay the foundation for future directions for research.

Extrathoracic heart in northern Cameroon: a case report

Sternal clefts, ectopia cordis, and Cantrell's pentalogy continue to be very rare congenital anomalies in pediatric surgery. The prenatal diagnosis is easily made with ultrasound by visualizing the heart outside the thoracic cavity. Ectopia cordis is frequently associated with other congenital defects involving multiple organ systems. We report a case of ectopia cordis with successful surgical correction on a 7 months old child from northern Cameroon.

Sternal cleft repair: A report of two cases and review of literature

A cleft sternum is a rare congenital anomaly often diagnosed as asymptomatic at birth. Clinical outcome may be unfavourable when an associated anomaly, particularly, an intra cardiac anomaly coexists with the defect. Primary repair should be employed in the neonatal period because the flexibility of the chest wall is maximal and thus the compression of underlying structures is minimal. However, patients with sternal cleft may even present late in the childhood or adolescence period. We herein report two cases of successful repair of sternal clefts with review of the available literature.

Primary Repair is Possible in Complete Sternal Cleft: The First Saudi Experience

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Complete Sternal Cleft in a Filipino Newborn

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Autologous repair of isolated complete sternal cleft in an adolescent

Isolated sternal clefts are rare congenital anomalies. Complete clefts are rarer still with only 23 cases being reported. We present an adolescent girl with a complete sternal cleft. The cleft was treated with autologous anterior perichondrial flaps and pectoralis advancement flaps.

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

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.

Congenital superior sternal cleft—An unusual age of diagnosis with review of the literature

Sternal cleft is a rare congenital anomaly which is generally observed at birth and stays asymptomatic in most cases. The aetiology remains obscure. Surgery is recommended not only to protect the heart and other mediastinal contents from trauma but also to improve respiratory dynamics. We report the case of a 23-year-old young woman with a failure of the upper two thirds of the sternum, causing pain in lateral decubitus position and an aesthetic appearance problem. Through this case report we will show the chest computed tomography (CT) aspects regarding this malformation and we will give a review of the literature.

Low cervical skin crease approach for superior sternal cleft repair

Superior sternal cleft repair aims to restore bony protection to mediastinal structures and to eliminate visible deformity. We describe a 4-day-old neonate with an isolated defect who successfully underwent primary repair using a low cervical skin crease incision. Such a novel approach combined optimal surgical exposure and excellent cosmesis.

An asymptomatic young woman with abnormal manubrium sterni: diagnosis and discussion

First developing from a pair of longitudinal mesenchymal condensations in utero, the right and left sternal bars meet along the midline and fuse to form a single cartilaginous rod. Fusion commences at the manubrium and proceeds caudad [1]. Incomplete fusion of lower sternebrae results in well-known bony defects variously named sternal foramen, cleft or fissure [2]. Although clefts and foramina are commonly found in the corpus sterni, description of manubrial fissure or foramen had been sparse [3–5] before the study by Yekeler et al., who reported a manubrial cleft in the sterna of six of 1,000 adults [2]. In all of the six patients the cleft was confined to the superior portion of the bone, associated with a sclerotic band just inferior to it in five of them. To our knowledge, a complete manubrial cleft is extremely rare, with only two cases having been reported in the non-English literature [3, 4]. In one of the two cases, reported in 1979, Botsch et al. used conventional tomography to diagnose complete fissure of the manubrium [4], which showed slightly jagged and sclerotic medial margins of the right and left hemimanubria and an inverted-Y shape, almost identical to that in our patient, formed by the interhemimanubrial and hemimanubriosternal joints (Fig. 1b and c in the case presentation). It is difficult to postulate the developmental origin of a manubrial fissure. However, we suppose that there might be two possible mechanisms: first, on rare occasions, the cephalo-most part of the sternal bar might have been left unfused in utero. Second, there might have been some muscular dynamics facilitating horizontal segmentation of the manubrium at a later stage, similar to diversified movements of the forelimbs facilitating longitudinal segmentation of the sternum [6]. Now that thin-section CT scanning of the bony thorax is becoming routine in clinical practice, we believe that more cases of this condition will be found in the future.