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Sternal cleft is a rare congenital malformation. We present the case of a new-born with complete sternal cleft and skin defect, without any other associated anomaly. Operated on in the neo-natal period, the post-operative recovery was good. She developed a transitory right lateral cervical lymphangioma, which resolved spontaneously. The follow up a...
Citations
... Several techniques have been described for SC treatment, also including cardiac repair (7,8). Zamfir et al. (3) described one of the primary closures in newborns after testing increased intra-thoracic (9) pressure for 10 min. Jabbad et al. (10) published maneuvers that saw the left pleura opened wide, slowly bringing the sternal bars together, making it possible to use titanium plates and screws. ...
... The exact etiology is unknown, but nutritional deficiency (methylcobalamin or riboflavin deficiencies) in mothers and alcohol intake may be associated with congenital skeletal deformities like sternal cleft. 1,2 The incidence is 1:100,000 cases per live births, and represents less than 1% of all chest wall deformities. 1 Sternal cleft can be isolated or associated with syndromes that involve various systems of the body, such as brain, cardiac, abdominal, renal, eye, and skeletally related malformations. It can also be either complete or partial. ...
... When the space between the costal ridges reaches the xiphoid process, the defect is V-shaped, whereas with the bony bridge joining the two edges ending at the third or fourth costal cartilage, it is U-shaped. [2][3][4] Fetal sonographic diagnosis of this defect is possible, but less practiced. It can be easily diagnosed clinically because of the presence of paradoxical chest wall movement during respiration in early infancy and due to the cosmetic deformity on the anterior chest wall. ...
... 1,2 When the defect is associated with cardiac anomaly, it seems to be more easily identifiable due to the unusual movements of the heart under the skin. [1][2][3][4]8 The decision for surgery depends on the age of the patient, rigidity of the chest wall, the size of the defect, and presence of other associated abnormalities in the chest wall and other systems. Maintaining and improving respiratory functions and cosmetic reasons are some of the indications for surgical closure. ...
Dawit Seyoum Gebremariam,1 Asmamaw Miruts,2 Kibrom Gebreselassie Desta3 1Department of Pediatrics and Child Health, College of Health Sciences, Mekelle University, Mekelle, Tigray, Ethiopia; 2Department Surgery, Pediatric Surgery Unit, College of Health Sciences, Mekelle University, Mekelle, Tigray, Ethiopia; 3Department of Surgery, Cardiothoracic Unit, College of Health Sciences, Mekelle University, Mekelle, Tigray, EthiopiaCorrespondence: Dawit Seyoum Gebremariam, Department of Pediatrics and Child Health, College of Health Sciences, Mekelle University, P.O. Box: 1871, Mekelle, Tigray, Ethiopia, Tel +251911731454, Email dgseyoum@yahoo.comAbstract: Sternal cleft is a rare chest wall anomaly resulting from a failure of the lateral mesodermal folds to migrate to the midline, causing a cleft in the early stage of embryological development. This can be a complete or partial defect. It can also occur as an isolated anomaly or in association with other syndromes. Fetal sonographic diagnosis of this defect is possible, but less practiced. After birth, this defect can be easily diagnosed clinically because of the presence of paradoxical chest wall movement. The flexibility of the thorax is maximal and compression of the underlying structures is minimal during the neonatal and early infancy period, and this period is the preferred time for surgical repair. We report a 39-day-old infant who presented with an isolated V-shaped inferior sternal cleft, its surgical primary closure, and postoperative course.Keywords: sternal cleft, surgery, congenital anomaly, complication
... Several techniques have been described for SC treatment, also including cardiac repair (7,8). Zamfir et al. (3) described one of the primary closures in newborns after testing increased intra-thoracic (9) pressure for 10 min. Jabbad et al. (10) published maneuvers that saw the left pleura opened wide, slowly bringing the sternal bars together, making it possible to use titanium plates and screws. ...
... These two bars form the sternum and fuse between 7 th to 10 th weeks of gestation. The fusion starts at the manubrium and ends at the xiphoid process caudally; the malformation is therefore classified as superior, inferior or complete depending on the when the fusion process was interrupted [3]. A superior partial cleft is the most common type accounting for 67% of all patients, followed by the complete form seen in approximately 20%, partial inferior in 11% and sternal foramen in 2.5% [4]. ...
... The surgery aims to protect the heart from direct injury and respiratory tract infection, and ensure a good cosmetic outcome [1]. Varying surgical techniques for correcting a partial sternal cleft, such as using periosteum with a chondral graft [5], iliac bone grafts [6], wedge excision of the inferior part [7], alloplastic reconstruction [1] and muscle flap [8] have been reported in the literature [9]. ...
Sternal clefts are rare congenital chest-wall deformities, which can be complete or incomplete; therefore, reconstruction with autologous tissue is essential to protect the heart and prevent respiratory infection. In this report, we present the case of a 16-month-old baby girl from a preterm, twin pregnancy with a partial superior sternal cleft. A moist dressing for promoting wound healing was used until cutaneous layer complete epithelialization at which time we performed reconstruction with autologous tissue. After surgery the patient recovered with close-to-natural chest wall contour and adequate heart and lung function.
... Sternal cleft (SC) is a rare congenital malformation that results from the defective embryologic fusion of mesenchymal cells [1].Sternal clefts can be classified into 3 separate categories: superior, inferior, and complete [2]. Different surgical techniques such as primary closure, bone graft interposition, muscle flap interposition, prosthetic closure, muscle flap interposition, and others have been described [1].Most patients with SC show associated malformations, but an isolated sternal cleft without any associated anomaly is very uncommon. ...
... Sternal cleft (SC) is a rare thoracic malformation with very few cases have been reported in Engliah Literature [1]. The sternal cleft may be partial or total,and a partial defect could be superior or inferior [2]. The reason for fusion failure in SC is not yet known. ...
Sternal cleft is a rare congenital malformation resulting from a fusion failure of the sternum and often patients have no clinical signs at birth. The defect is usually divided into partial or complete forms and is often accompanied by other anomalies. We herein report a case of upper sternal cleft that was repaired in the neonatal period by primary closure after partial thymectomy. The patient was discharged 9 days after the operation with good condition and normal respiratory movements. Sternal cleft is a rare case, with little more than 100 cases published worldwide.
... Isolated sternal cleft without any other associated abnormalities is very rare. It has been reported as apart of defined syndromes like PENTALOGY OF CANTRELL, VACTREL, DANDY WALKER, and PHASE (Posterior fossa brain malformation, hemangioma, arterial lesions, cardiac abnormalities, and eye abnormality [7]. Embryologically sternum originate from the somatic layer of lateral mesodermal plates as bilateral bands. ...
We report the modified surgical reconstruction technique for correction for a large isolated congenital sternal cleft in 6 years old girl using a methyl methacrylate marlex mesh sandwich plate (MMS). The patient was referred to our tertiary care institution with a sizeable anterior chest wall bony defect. There was a large bulging under the skin due to protrusion of mediastinal viscera and visible cardiac pulsations with breathing. A chest x-ray and computed tomographic scan (CT) of the thorax was done to evaluate the bony defect. We reconstructed the sternal bony defect by our innovative technique using methyl methacrylate. The patient was discharged after three days for further follow up in outpatient. One year follow up patient is doing well with excellent results. Our technique is simple, cost-effective, and provides a perfect cosmetic effect for children's sternal large defects.
Introduction
Sternal clefts are rare congenital malformations that result from the defective embryologic fusion of paired mesodermal bands in the midline. This rare anomaly incidence is 1:100,000 cases per live births, which constitute 1% of all congenital chest wall deformities. The first Case of the sternal cleft was reported in 1740. The hereditary sternal gap is rare, and hence sporadic cases have been reported in the medical literature. Ravitch described that the first surgical correction was reported by Lannelongue et al., in 1988., But Burton published the first successful repair in 19474. An isolated sternal cleft is a rare entity and is classified into two categories' complete and incomplete sternal gap. Early repair is recommended; otherwise, surgical correction is challenging in children as the hypo plastic sternal edges cannot be approximated primarily, and this requires prosthetic, autologous grafts or some kind of parasternal chondroplasties.
Case presentation
Since birth, a six-year-old girl was referred to our tertiary care center with a large central chest wall defect. She was a full-term normal delivery with no other congenital malformations. The defect was noticed at birth and became more evident as she grew up. In addition to cosmetic concern on coughing, there was bulging under the skin and visible cardiac pulsation. On examination, there was a large gap (7cm) in the midline of the chest with sternal adages well apart, moving independently.
Discussion
The sternal cleft is a congenital anomaly with less than 0.15% and is more common in the female gender. Isolated sternal cleft without any other associated abnormalities is very rare. It has been reported as a part of defined syndromes like PENTALOGY OF CANTRELL, VACTREL, DANDY WALKER, and PHASE (Posterior fossa brain malformation, hemangioma, arterial lesions, cardiac abnormalities, and eye abnormality).6 Embryo logically sternum originate from the somatic layer of lateral mesodermal plates as bilateral bands. They fuse in the midline by the 10th week to constitute a cartilaginous framework of manubrium, sternum, and xiphoid process. Failure of this fusion can lead to a partial or complete sternal cleft.7 Etiology of this disease is unknown; however, it has been linked with riboflavin or methyl-cobalamin deficiency, high alcohol intake during pregnancy.
Conclusion
in conclusion, our improvised reconstruction technique for large sternal cleft in children has several advantages. There is no need to do extensive chordotomies or using bone grafts. Less complicated procedure Provides more rigid frame for protection of thoracic structures and better chest wall stability. Hospital stay is minimal and is very cost-effective. The child's future growth is not affected as ribs and costal cartilages are left intact in this technique. There is no chance of displacement or excursion of the MMS plate. In female patients, this provides better cosmoses as there is no need to mobilize the pectoralis significant muscles flaps for coverage. The geometry of the rib cage is well preserved.
... nacidos vivos, que representa alrededor del 0,15 % de todas las malformaciones de la pared torácica [1][2][3] . Se presenta por una alteración en la fusión de las dos bandas mesenquimales que forman el esternón hacia las semanas 6 a10 del desarrollo intrauterino 1 y su etiología no está claramente establecida 1,4 . Es mas frecuente en mujeres y se diagnostica en el período neonatal 3,5 . ...
... La etiología no se conoce muy bien, pero se ha relacionado con el consumo de alcohol durante el embarazo, la deficiencia de metilcobalamina, rivoflavina y con la alteración en la expresión del gen HoxB, sin embargo, no se ha descrito predisposición familiar 1,4 . ...
... El diagnóstico se hace de manera clínica, en la etapa neonatal, mediante la inspección y la palpación, cuando el defecto es superior o aislado, debido a que estos pacientes son asintomáticos. En defectos más graves, como la ectopia cordis, y en los pacientes con síndromes asociados, generalmente el diagnóstico se hace de forma prenatal, mediante el ultrasonido 3,4 . ...
La agenesia esternal es una rara malformación del desarrollo embrionario del esternón, que puede estar o no relacionado con otras anomalías congénitas. Se presenta el caso de un paciente de 4 años de edad con agenesia esternal total, que fue llevado a reconstrucción de la pared torácica anterior con material protésico absorbible y pericardio bovino, logrando la adecuada estabilidad de la pared torácica y la corrección de la función de la misma, con una evolución satisfactoria después de dos años de seguimiento.
... In elderly patients, the rib cage is stiff and repair is difficult. Increase in thoracic wall compliance to allow primary closure can be attained through sliding chondrotomies, osteotomies, or clavicular dislocation [13,14]. ...
Congenital absence of the sternum is a rare chest wall malformation resulting from the failure of midline fusion during embryonic development. It is a potentially life-threatening congenital midline defect. Only sporadic cases have been reported in literature. The abnormality can cause significant morbidity, and like other congenital anomalies can have associated defects. Repair of congenital absence of the sternum should ideally be undertaken in the neonatal period when the chest wall is highly compliant, and hence, primary closure can thus be achieved without significant cardiopulmonary compression. As the patient ages, chest wall compliance decreases and closure will become progressively difficult as venous return and lung compliance are increasingly compromised. We report a case of congenital absence of the sternum as it is very rare and because it was successfully operated in a neonate period.
... Normally, the sternum is developed by fusion of two mesenchymal bars, between the 7th and 10th week of gestation, extending from the manubrium to the xiphoid process [1]. Sternal cleft (SC) is a rare chest wall anomaly occurs after failure of the sternal bars to fuse. ...
... Prenatal diagnosis of SC by fetal ultrasound is well done between the 18 th and 26 th week of gestation when the number and size of ossification centres are small or absent [4]. Also, prenatal identification of SC is easier when cardiac anomalies exist as these anomalies indicate heedful research of other malformations [1]. At birth, the clinical inspection and palpation of the chest wall can help diagnosis of SC, when cardiac pulsations under the skin are inspected, with V-shaped cleft to xiphoid or U-shaped space between the costal ridges up to 4th costal cartilage ( Figure 1) [5]. ...
Sternal cleft (SC) is a rare congenital anomaly of the chest wall, isolated or combined with other malformations. We try to provide an appropriate stepwise approach for the diagnosis and treatment of SC. Prenatal ultrasound diagnosis can be obtained after the 18th week of gestation. At birth, diagnosis is based on the findings of clinical examination, chest x-ray, computed tomography and additional investigations for the associated anomalies. Primary approximation of the sternal bars is preferred before the age of 3 months. For older cases and rigid chest wall, the surgical correction can be achieved using prosthetic materials, autologous grafts, or biologic implants. The surgical decision depends on the age of patient, rigidity of the chest wall, and presence of other abnormalities in the chest wall.
