Modified surgical reconstruction technique for a rare isolated congenital sternal cleft: In a six -year-old child

Abstract

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. IntroductionSternal 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 presentationSince 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. DiscussionThe 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. Conclusionin 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.