|Year : 2017 | Volume
| Issue : 1 | Page : 60-63
Isolated sternal cleft in a patient with atrial septal defect: A rare sole association
Olugbenga O Olusoji, Saheed B Sanni, Omotayo O Omodara, Oluwaseye F Akerele
Cardiothoracic Surgery Unit, Department of Surgery, Lagos University Teaching Hospital, Idi-Araba, Lagos, Nigeria
|Date of Web Publication||9-May-2017|
Olugbenga O Olusoji
P.M.B. 12003, Lagos
Source of Support: None, Conflict of Interest: None
Sternal cleft (SC) is a rare form of chest wall deformity with an incidence of <0.15%. It occurs because of failure of fusion of the sternal bars about the 7–9th week of intrauterine life. It could occur in isolation or as a part of a syndromic complex. This condition exposes the vital mediastinal structures to a huge risk of injury, aside from cosmetic issues. Treatment in the neonatal period is most favourable, though other modalities are available in the later stages of life, but it can be challenging. We present the case of a 2-month-old female infant with a superior SC with a single association of secundum atrial septal defect. She was asymptomatic. She was treated by primary repair to protect the underlying mediastinal structures as well as for cosmetic reasons. Such a repair resulted in good clinical outcome.
Keywords: Orthotopic, primary repair, sternal clefts, syndromes
|How to cite this article:|
Olusoji OO, Sanni SB, Omodara OO, Akerele OF. Isolated sternal cleft in a patient with atrial septal defect: A rare sole association. Niger Postgrad Med J 2017;24:60-3
|How to cite this URL:|
Olusoji OO, Sanni SB, Omodara OO, Akerele OF. Isolated sternal cleft in a patient with atrial septal defect: A rare sole association. Niger Postgrad Med J [serial online] 2017 [cited 2020 Nov 24];24:60-3. Available from: https://www.npmj.org/text.asp?2017/24/1/60/205968
| Introduction|| |
Sternal cleft (SC) is the failure of ventral fusion of the sternum in utero that persists till delivery. Globally, it is a very rare chest wall deformity, particularly in Africa. Most of the reported cases in literature were from other continents. It accounts for <1% of all chest wall deformities. It is commonly associated with different types of intracardiac anomalies and can be a complete or partial defect − the former being rarer. The partial or incomplete defect can be sub-classified further into superior and inferior SCs. Various approaches to repair the defect have been devised based on the presentation of the patient. However, the most favoured is a primary repair, whenever and wherever it is feasible.
| Case Report|| |
We report the case of a 2-month-old female infant, who presented to the surgical outpatient department of Lagos University Teaching Hospital in December 2015 with anterior chest wall defect noted since birth. The defect was surrounded by normal skin; however, a central, oval-shaped, whitish necrotic skin was noted overlying the defect. The defect was limited to the upper part of the chest with visible heart pulsations. There was no associated history of cough, difficulty in breathing or upper respiratory tract infection. Reddish skin discoloration and other extrinsic structural anomaly were absent. Pregnancy was supervised from gestational age 5 months and was largely uneventful. The mother neither used self-prescribed medications nor traditional medication. Delivery was by emergency Caesarian section due to cervical dystocia. Apgar score was largely within normal range. Developmental milestones were delayed − sat at 7 months, did not crawl and currently was standing with support at 18 months of age. Immunization was up to date according to the National Expanded Programme on Immunisation. No family history of similar deformity was reported. The patient is currently the only child of the parents.
An examination revealed a healthy-looking female infant without dysmorphic features. There was a 4 cm × 6 cm anterior chest wall defect in the superior portion of the sternum, with paradoxical movement with respiration [Figure 1]a. There was a wide separation of the strap muscles of the neck; however, no rectus abdominis divarication was seen, and a supraumbilical midline raphe was not present. Sensations and motor functions of all the limbs were intact, with no evidence of spinal lesion.
Her chest radiograph was essentially normal except for an increased interclavicular distance [Figure 2]a. An echocardiography revealed a normally located heart in its pericardium. There was a 3.8-mm secundum atrial septal defect (ASD) shunting from the left to the right. No other intracardiac defect or disease was seen, and no patent ductus arteriosus. The haematological and biochemical parameters were all normal. A diagnosis of an isolated congenital SC with an associated secundum ASD was made, and she was immediately scheduled for primary repair because of her age at presentation, the size of the defect and the absence of other associated major anomalies.
The patient was positioned supine and both upper upper limbs abducted to 90°. Repair was performed under general anaesthesia with endotracheal intubation. After standard skin preparation and draping, a vertical elliptical skin incision was made to excise the necrotic skin patch [Figure 1]b. This was extended inferiorly to just below the lower margin of the defect. Dissection was performed down to the endothoracic fascia without breaching the pericardium or the pleurae. The thymus gland was left intact. The edges of the sternal bars were freshened, and the ‘U’-shaped defect converted to a ‘V’ by a careful wedge osteotomy at the lower end of the defect. Strong non-absorbable sutures (Silk 1) were laid on either side of the sternal bars through the intercostal spaces [Figure 1]c. These were carefully tied to appose the sternal bars, hence primarily closing the defect [Figure 1]d. Haemodynamic parameters were monitored for 5 min and were noted to be normal. This confirmed a successful closure without undue pressure on the mediastinal contents. A chest tube drain connected to an underwater seal drainage system was placed in the anterior mediastinum prior to final knotting of the sternal sutures. The strap muscles were sutured together inferiorly in the midline; subsequently, the anterior chest wall muscles, the subcutaneous tissues and the skin were finally closed [Figure 1]e.
Recovery from anaesthesia was excellent. She was extubated in the suite before transfer to the recovery room, from where she was moved to the wards. The post-operative period was uneventful. She was managed with intravenous fluids, antibiotics (cephalosporin) and analgesics. Oral feeding was commenced about 18 h post-op. Haematologic and biochemical parameters after the surgery were normal. Wound site at 5th day post-op was satisfactory, and she was discharged home on the 7th day post-op for follow-up in our outpatient clinic. The patient was commenced on prophylactic neck physiotherapy to prevent mento-sternal contracture, the result of which has been commendable. She is now 1-year post-repair, with good cosmetic appearance [Figure 3], regular on follow-up visits and has been gaining weight considerably.
| Discussion|| |
There are few reported cases of SCs in Africa. In a reported series by Acastello et al., it is <0.15% of chest wall deformity, confirming its rarity. The partial or complete non-fusion of the sternal bars around the 7–9th week of intrauterine life is embryologically responsible for this congenital anomaly. The cause is largely unknown, although some genetic abnormalities such as Hox B gene have been suggested as possible aetiology in mice. Other speculations are intake of alcohol in early pregnancy and methylcobalamin or riboflavin deficiency. It is more common in females, but no ethnic or racial preponderance has been reported.
In almost three-quarter of the cases, the patients are asymptomatic, especially when it is an isolated SC such as in the index patient. Some have been discovered incidentally in adults during investigations for other disease conditions. However, in others, clinical presentations range from paradoxical chest wall movement in the region of the defect as seen in our patient, dyspnoea, tachypnoea and frequent respiratory tract infections in relation to the sub-optimal negative intrathoracic pressure as a result of the chest wall malformation. SC can also be associated with syndromes such as PHACES syndrome (Posterior fossa brain malformations, facial Haemangiomas, Arterial anomalies, Cardiac defects, Eyes abnormalities, Sternal clefts and Supraumbilical raphe) PTFE (PolyTetra Fluoro Ethylene), pentalogy of Cantrell, vascular dysplasias and midline defects. Syndromic forms such as those enumerated above present with different associated clinical features, namely, intracardiac defects (tetralogy of Fallot and ventricular septal defect), hamartomas and haemangiomas − facial, neck and/or pharyngeal, supraumblical midline defects, ectopia cordis, vascular disorders, etc.
Diagnosis is largely clinical. Imaging modalities help in confirming the defects’ location, types, sizes and other associated disorders. For this, a computerized tomography scan of the chest is the investigation of choice. It gives detailed information regarding the defect that needs to be repaired. However, in resource-challenged environments or in situations where caregivers are low-income earners, such as in the index patient, this may not be obtainable. Hence, a chest radiograph will be necessary. A widened interclavicular distance on chest radiograph with other clinical findings on examination confirms the diagnosis. Echocardiography is needed to identify the intracardiac defects and shunts. This revealed an orthotopic heart with isolated ASD in this patient, a sole association of which was only reported by la Tour et al. in a male infant of a similar age group as ours in their reported series.
The main aim of surgical repair is to protect the mediastinal structures, allow optimal growth of the chest wall and for cosmesis. Primary repair is the treatment with most rewarding outcome and is used when feasible; in addition, it is the most preferred treatment choice. Shamberger and Welch opined that this is preferable in the neonatal period and during early infancy when the chest wall is still relatively malleable, and the intrathoracic organs can tolerate external pressures without compromising the haemodynamic status. This was what our patient had. Other approaches of repair include chondrotomies, muscle flap interpositions, use of biological non-absorbable synthetic grafts or materials − PTFE (Gore-Tex® biomaterials and calcium phosphate cements) and absorbable prosthesis (Lactosorb®). These approaches are deployed in much older patients with stiff chest wall, in whom primary repair cannot be achieved successfully, or as a bridge to primary repair in some patient with associated syndrome, for example, Cantrell pentalogy.
In conclusion, SC is a rare congenital chest wall malformation, which can occur as an isolated condition or as a part of a syndrome. Careful and thorough clinical and laboratory evaluations of the patient at presentation are necessary to identify all associated diseases and plan for intervention. However, clinical assessments alone may suffice in resource-challenged environments to give such patients the best care comparable to other centres in the world.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3]