Congenital absence of sternum in an infant

Shrivastava, Anubha; Siddiqui, Shahid Akhtar

doi:10.1136/bcr-2016-219081

Cited by 3 publications

(2 citation statements)

References 3 publications

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“…SC may be associated with other anomalies like maxillofacial hemangiomas, cleft lip/palate, gastroschisis, precordial skin tags and supraumbilical raphe which can be ruled out by a careful physical examination of the neonate. SC has also been found to be associated with pentalogy of Cantrell (ectopia cardis, sternal cleft, intracardiac defects, omphalocele and pericardial defect), VACTERL syndrome and PHACES syndrome (posterior fossa abnormalities, hemangioma, arterial abnormalities, coarctation of aorta, eye abnormalities and sternal malformations) [6,7]. Imaging studies like chest X-ray, CT scan, ECG and echocardiography are important diagnostic tools to identify associated anomalies.…”

Section: Discussionmentioning

confidence: 99%

Complete Sternal Cleft

Mehta¹,

Grover²,

Soni³

et al. 2022

JCR

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Background: Sternal cleft is a rare chest wall anomaly with a reported incidence of 1 in 100,000 live births. It results from failure of midline fusion during embryonic development. It can present as an isolated anomaly or be associated with other malformations. The condition is easily diagnosed in a neonate due to the paradoxical movements of the chest. Early surgical repair in the neonatal period is the treatment of choice to improve respiratory dynamics and prevent recurrent respiratory infection with their sequelae. Case Report: We report a case of a neonate who was diagnosed to have absence of sternum at birth due to the mediastinal bulge with paradoxical chest wall movements. The diagnosis was later confirmed on imaging studies and the neonate was successfully managed conservatively and later referred to tertiary centre for surgical repair. Conclusion: Paradoxical movements of the thorax in a newborn should alert the physician towards possibility of sternal cleft.

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Section: Discussionmentioning

confidence: 99%

Complete Sternal Cleft

Mehta¹,

Grover²,

Soni³

et al. 2022

JCR

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“…Absent sternum entails the risk of injury to the mediastinal structures, hypothermia, increased insensible fluid losses, cyanosis due to altered compliance of the breathing pattern and recurrent infections of the chest if the overlying skin is exposed or gets exposed to the external environment after birth. Early surgical correction of defect should be considered as it becomes challenging as the babies grow older due to the lesser mobility of the rib cage, widening of the defect and the grown-up mediastinal structures in the lesser accommodating mediastinum [ 3 ]. Due to the rarity of the disease and the variation in the degree of the defect, the management protocols are confined only to some case reports.…”

Section: Introductionmentioning

confidence: 99%

Congenital Absence of Sternum: A Surgical Technique for Successful Outcome

Santoki,

Negi,

Kumari

et al. 2024

Cureus

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Congenital absence of sternum is a rare malformation of the anterior chest wall that needs surgical correction to avoid life-threatening complications as a consequence of such defect. It results from either partial or complete failure of fusion of mesenchymal strip during in utero organogenesis. The absence of sternum entails the risk of trauma to the mediastinal structures and other life-threatening complications. This defect is evaluated by a thorough clinical examination and computed tomography imaging of the thoracic cage to plan the surgical procedure. Early repair of the defect when the thoracic cage is still compliant yields the best result.

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Congenital absence of sternum with tetralogy of Fallot, right aortic arch, and bilateral superior vena cava

Chavan,

Narkhede,

Weling

2023

Egypt Pediatric Association Gaz

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Background Congenital complete absence of sternal bone or cleft sternum or absent sternum is a very rare and potentially life-threatening birth defect. An association of cleft sternum with tetralogy of Fallot, right aortic arch, and bilateral superior vena cava is extremely rare, and not many cases have been reported. Case presentation A 2-year-old child was brought for routine checkup. He was a known case of congenital heart disease and midline thoracic defect at age of 3 months. Further investigations revealed absent sternum with tetralogy of Fallot, large cono-ventricular defect with bidirectional shunt, severe infundibular pulmonary stenosis, superiorly located right aortic arch, and bilateral superior vena cava. Conclusion Congenital absence of the sternum with intracardiac defects like tetralogy of Fallot with right aortic arch and bilateral superior vena cava is extremely rare malformation. Early diagnosis and surgical correction give the infant the best chance of survival.

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Congenital absence of sternum in an infant

Cited by 3 publications

References 3 publications

Complete Sternal Cleft

Complete Sternal Cleft

Congenital Absence of Sternum: A Surgical Technique for Successful Outcome

Congenital absence of sternum with tetralogy of Fallot, right aortic arch, and bilateral superior vena cava

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