Sternal cleft is a rare congenital anomaly and very few cases have been described especially in Africa. This is a case of an isolated superior sternal cleft in a term neonate in Western Kenya

Koech, H

Ogalo, E

Okwako, F

Simba, J

English

AJOL - African Journals Online

286 East african MEdical Journal August 2014 East African Medical Journal Vol. 91 No. 8 August 2014AN ISOLATED SUPERIOR STERNAL CLEFT IN A NEONATE IN WESTERN KENYA: CASE REPORTF. O. Okwako, MBChB, MMed, Ministry of Health, E. A. Ogalo, MBChB, MMed, Paediatrician, Department of Child Health and Paediatrics, Moi Teaching and Referral Hospital, J. M. Simba, MBChB, MMed, Assistant Lecturer, Department of Child Health and Paediatrics, Moi University, P. O. Box 4606, Eldoret and H. Koech, MBChB, MMed, Paediatrician, Ministry of Health, Kapsabet County Hospital, P. O. Box 5-30300, Kapsabet, KenyaRequest for reprints to: Dr. E. A. Ogalo, P.O.Box 8402-30100, Eldoret, Kenya. eapondi@yahoo.comAN ISOLATED SUPERIOR STERNAL CLEFT IN A NEONATE IN WESTERNKENYA: CASE REPORTF. OKWAKO , E. OGALO, J. SIMBA and H. KOECHSUMMARYSternal cleft is a rare congenital anomaly and very few cases have been describedespecially in Africa. This is a case of an isolated superior sternal cleft in a term neonatein Western Kenya.INTRODUCTIONAlthough the first case of sternal cleft was reportedmore than three centuries ago(1), and severalsuccessful repairs have been done in the developedworld, very little reporting has been done in thedeveloping world. Almost no surgeries have beenreported in East Africa. Although this condition israre, attention needs to be given to those affected byit to improve their survival and quality of life. Thisis a case report of an isolated superior sternal cleft inEast Africa, in a context where there is limited accessto surgical repair.CASE REPORTA one day old neonate was referred from a peripheral health centre after having been noted to have a midline chest wall abnormality at birth. The neonate was born to a 28 year old para 3+0 mother via spontaneous vertex delivery at 39 weeks gestation by dates ,cried immediately and had a birth weight of 2.5kgs. The baby had no respiratory distress or difficulties in breastfeeding after birth. There was no family history of similar or other congenital anomalies. She passed meconium within one hour of birth and was passing urine well. Antenatal period was uneventful and the mother had received the routine antenatal care in the same health centre. On examination we found a stable female baby with an anterior sternal defect measuring 2.5 cm covered with thin skin and visible pulsations of the great vessels. There was an anterior abdominal wall median raphe measuring seven centimeters. There was no tachypnea or respiratory distress. The breath sounds were normal with no added sounds. There was no murmur. The head circumference was 32.5cm, the anterior fontanel measured 6x4 cm with normal suture lines. There were no dysmorphic features or other clinically evident congenital anomalies. A chest X-ray showed absence of sternal ossification centres. A 2D echocardiogram done on the third day of life showed no structural heart defects but had a moderate pulmonary hypertension (63mmHg) and mild Right Atrial and Right Ventricular dilatation.A repeat echocardiogram at day eight of life showeda decline in the pulmonary pressures to 54 mmHg. Abdominal Ultrasound was normal. Renal functiontests and the complete blood count were normal. The baby was dischargedwith advice on conservative management from the cardiothoracic team. The babywas reviewed at two weeks of age and was weighing3.3kg (average weight gain of 20g/kg/day), stable and was reported to be breastfeeding well without exhaustion. Further follow up was to be done at theage of three months.Figure 1A Photograph of the child showing the sternal cleft andabdominal rapheAugust 2014 E AST  A FRICAN  M E DICAL  JOURNAL     1East African Medical Journal Vol. 91 No. 8 August 2014AN ISOLATED SUPERIOR STERNAL CLEFT IN A NEONATE IN WESTERN KENYA: CASE REPORTF. Okwako1 Registrar, Department of Child Health and Paediatrics, Moi University, E. Ogalo2, Paediatrician, Department of Child Health and Paediatrics, Moi Teaching and Referral Hospital, J. Simba 3 Assistant Lecturer, Department of Child Health and Paediatrics, Moi University, Koech Hellen4 Paediatrician, Ministry of Health, KenyaRequest for reprints to: Ogalo, Edith, P.O.Box 8402-30100, Eldoret, Kenya. eapondi@yahoo.comAN ISOLATED SUPERIOR STERNAL CLEFT IN A NEONATE IN WESTERN KENYA: CASE REPORTF. OKWAKO, E. OGALO, J. SIMBA and H. KOECHSUMMARYSternal cleft is a rare congenital anomaly and very few cases have been described especially in Africa. This is a case of an isolated superior sternal cleft in a term neonate in Western Kenya.INTRODUCTIONAlthough the first case of sternal cleft was reported more than three centuries ago(1), and several successful repairs have been done in the developed world, very little reporting has been done in the developing world. Almost no surgeries have been reported in East Africa. Although this condition is rare, attention needs to be given to those affected by it to improve their survival and quality of life. This is a case report of an isolated superior sternal cleft in East Africa, in a context where there is limited access to surgical repair. CASE REPORT: A 1 day old neonate was referred to us from a peripheral health centre after having been noted to have a midline chest wall abnormality at  0+3 arap dlo raey 82 a ot nrob saw etanoen ehT .htribmother via spontaneous vertex delivery at 39 weeks gestation by dates ,cried immediately and had a birth weight of 2.5kgs. The baby had no respiratory distress or difficulties in breastfeeding after birth. There was no family history of similar or other congenital anomalies. She passed meconium within 1 hour of birth and was passing urine well.Antenatal period was uneventful and the mother had received the routine antenatal care in the same health centre.On examination we found a stable female baby with an anterior sternal defect measuring 2.5 cm covered with thin skin and visible pulsations of the great vessels. There was an anterior abdominal wall median raphe measuring 7cm. There was no tachypnea or respiratory distress. The breath sounds were normal with no added sounds. There was no murmur. The head circumference was 32.5cm, the anterior fontanel measured 6x4 cm with normal suture lines. There were no dysmorphic features or other clinically evident congenital anomalies. A chest X-ray showed absence of sterna ossification centres. A 2D echocardiogram done on the 3rd day of life showed no structural heart defects but had a moderate pulmonary hypertension (63mmHg) and mild Right Atrial and Right Ventricular dilatation. A repeat echocardiogram at day 8 of life showed a decline in the pulmonary pressures to 54mmHg. Abdominal Ultrasound was normal. Renal function tests and the complete blood count were normal. The baby wasdischargedwith advice on conservative management from the cardiothoracic team. The baby was reviewed at 2 weeks of age and was weighing 3.3kg (average weight gain of 20g/kg/day), stable and was reported to be breastfeeding well without exhaustion. Further follow up was to be done at the age of 3 months.Figure 1A Photograph of the child showing the sternal cleft and abdominal rapheAugust 2014 East african MEdical Journal    287Figure 2Chest X-ray with absence of sternal ossification centres2 E AST  A FRICAN  M E DICAL  JOURNAL  August 2014 Figure 2Chest X-ray with absence of sternal ossification centresDISCUSSIONThe sternum has its origin form the lateral plate mesoderm. These two lateral bands move anteriorly and superiorly and fuse in the ventral midline in the 3rd month of life to form the sternum(2). The midline fusion is craniocaudal, and a concurrent lateral fusion occurs with the costal cartilages.  On rare occasions, the midline fusion fails to occur (complete sternal cleft) or occurs partially (incomplete sternal cleft)(2,3). Complete sternal clefts are very rare and may  .stcefed noisuf enildim rehto htiw detaicossa ebComplete sternal cleft also occurs in association with pentalogy of Cantrell, a syndrome consisting of a midline supraumbilical abdominal wall defect, a defect of the lower sternum, a deficiency of the diaphragmatic pericardium, a deficiency of the anterior diaphragm, and intracardiac anomalies4. Sarper et.al also described a case of a 13 year old girl who had complete sternal cleft with pectum excavatum(5). Incomplete sternal fusion occurs either inferiorly or superiorly. Inferior sternal clefts are often associated with other anterior chest wall defects such as ectopiacordis. It has also been described as part of pentalogy of Cantrell and in association with other midline defects such as disorders of the oesophagus and anterior abdominal wall(6).Superior sternal cleft, such as is in our case, may be isolated or occur with other congenital malformations. Singh et.al (India) reported a cast of a 6 month old male infant who presented with recurrent pneumonia and a midline thoracic bulge. He was found to have a 4.5 cm isolated u shaped sternal cleft and underwent a successful primary repair.  This team also described a successful repair of an isolated u shaped sternal cleft in an 18 day old male neonate(7). Superior sternal cleft has been described in association with PHACES syndrome(8, 9). PHACES is a neurocutaneous syndrome consisting of posterior fossa malformations, haemangioma, arterial anomalies, coarctation of the aorta, cardiac defects, eye abnormalities, and sternal cleft with or without abdominal raphe. Mazzie et.al. described a term female neonate with PHACES syndrome who was successfully managed at Winthrop University Hospital-USA(10).In this case, the neonate was diagnosed with multiple congenital heart defects on echocardiogram at birth. The sternal cleft was successfully repaired. An aberrant subclavian artery was diagnosed at 2 weeks of age. At 1 month of age she was diagnosed with a proximal tracheal haemangioma causing subglottic stenosis together with haemangioma on the lips and gums.  She underwent a successful tracheal laser surgery. Our patient appeared to have isolated type. We could not find any other anomalies from the clinical examination and the investigations done. Prenatal ultrasonography diagnosis has been described in a case of an upper sternal cleft(11). At birth the diagnosis of sternal cleft is easily done by inspection and palpation. Diagnostic investigations are thus directed to exclude the infrequent associated anomalies.Prognosis of isolated sternal cleft is good although early surgical repair is advised even in asymptomatic cases. The repair improves respiratory dynamics, protects the heart form external trauma and is aesthetic. Early repair in the neonatal period is advised due to flexibility of the cartilaginous thorax and ability of the chest viscera to accommodate closure of the chest wall(12,13). Delayed closure increases the risk of serious compromise of the heart and lungs especially so with complete sternal cleft. Although surgical repair performed in the neonatal period in patients with sternal cleft is the most advocated, a safe and favourable operation may also be performed with the use of autogenous tissues even in late infancy.Yavuzer and Kara reported a case of a 4-year-old girl with sternal cleft who showed a favourable clinical outcome following successful primary surgical repair with the use of autogenous tissues (14). The case presented here was to be scheduled forsurgery at a later date. This plan was agreed upon bearing in mind the limitations in neonatal critical care in our set up and the potential for a later repair though with likely increased surgical risks. This approach, however is not unusual as Chakkarapani et.al in United Kingdom, have reported previously of a case of a term baby diagnosed at birth but was on follow up as at 3 months awaiting repair later(15). It has been a common practice in developing countries to delay surgery if possible to avoid complications which may relate to critical care in the neonatal period, a service which is normally not available in many facilities.ACKNOWLEDGEMENTSWe wish to acknowledge the Moi Teaching and Referral Hospital, Eldoret, hospital management for allowing us to publish this case report. We are also thankful to the parents of the child for their consent to have this case presented.DISCUSSIONThe sternum has its origin form the lateral platemesoderm. These two lateral bands move anteriorlyand superiorly and fuse in the ventral midline in thethird month of life to form the sternum(2). The midlinefusion is craniocaudal, and a concurrent lateral fusionoccurs with the costal cartilages. On rare occasions,the midline fusion fails to occur (complete sternal cleft)or occurs partially (incomplete sternal cleft)(2,3).Complete sternal clefts are very rare and maybe associated with other midline fusion defects.Complete sternal cleft also occurs in associationwith pentalogy of Cantrell, a syndrome consistingof a midline supraumbilical abdominal wall defect,a defect of the lower sternum, a deficiency of thediaphragmatic pericardium, a deficiency of theanterior diaphragm, and intracardiac anomalies4.Sarper et.al also described a case of a 13 year oldgirl who had complete sternal cleft with pectumexcavatum(5). Incomplete sternal fusion occurs either inferiorlyor superiorly. Inferior sternal clefts are often associatedwith other anterior chest wall defects such asectopiacordis. It has also been described as part ofpentalogy of Cantrell and in association with othermidline defects such as disorders of the oesophagusand anterior abdominal wall(6). Superior sternal cleft, such as is in our case,may be isolated or occur with other congenitalmalformations. Singh et.al (India) reported a casteof a six month old male infant who presented withrecurrent pneumonia and a midline thoracic bulge.He was found to have a 4.5 cm isolated u shapedsternal cleft and underwent a successful primaryrepair. This team also described a successful repairof an isolated u shaped sternal cleft in an 18 dayold male neonate (7). Superior sternal cleft has beendescribed in association with PHACES syndrome(8,9). PHACES is a neurocutaneous syndrome consistingof posterior fossa malformations, haemangioma,arterial anomalies, coarctation of the aorta, cardiacdefects, eye abnormalities, and sternal cleft with orwithout abdominal raphe. Mazzie et.al. described aterm female neonate with PHACES syndrome whowas successfully managed at Winthrop UniversityHospital-USA(10).In this case, the neonate wasdiagnosed with multiple congenital heart defectson echocardiogram at birth. The ster al cleft successfully repaired. An aberrant subclavian arterywas diagnosed at two weeks of age. At one month ofage she was di gnosed with a proximal tr chealhaeman ioma c using subglottic stenosis t getherwith haemangioma on the lips and gums. Sheunderwent a successful tracheal las r surgery. Oupatient ppeared to have isolated type. We could notfi  any other anomalies from the clinical examinationand the inv stigations done. Prenatal ultrasonography diagnosis has beedescribed in a c se of an upper sternal cleft (11). Atbirth the di gnosis f ste nal cleft is ea ily done byinspection and palpatio . Diagnostic investigationsare thus directed to exclude the infrequent ass ciatedanomalies. Prognosis of isolated sternal cleft is g odltho gh early surgi al repair is advised even insy ptomatic cases. The repair imp oves r spiratorydynamics, p otects the heart form external traumand is aesthetic. Early repair in the neonatal pe iod isadvised due t  flexibility of the cartilaginous thorax ability of the chest viscera to accommodate closureof the chest wall (12,13). Delayed losure i creases therisk of serious compromise of the heart nd lungsespecially so with compl te sternal cleft. Although surgical repair performed in theneonatal period in patients with sternal cleft is themost advocated, a safe and favourable operationmay also be performed with the use of autogenoustissues even in late infancy. Yavuzer and Kara reporteda case of a four year old girl with sternal cleft whoshowed a favourable clinical outcome followingsuccessful primary surgical repair with the use ofautogenous tissues (14). The case presented here wasto be scheduled for surgery at a later date. This planwas agreed upon bearing in mind the limitations inneonatal critical care in our set up and the potentialfor a later repair though with likely increased surgicalrisks. This approach, however is not unusual asChakkarapani et.al in United Kingdom, have reportedpreviously of a case of a term baby diagnosed atbirth but was on follow up as at three months awaitingrepair later (15). It has been a common practice indeveloping countries to delay surgery if possible toavoid complications which may relate to critical carein the neonatal period, a service which is normallynot available in many facilities.ACKNOWLEDGEMENTSTo the Moi Teaching and Referral Hospital, Eldoret, hospital management for allowing us to publish this case report. We are also thankful to the parents of the child for their o sent to have this case presented.288 East african MEdical Journal August 2014 REFFERENCES1.  Shamberger, R.C. Chest wall deformities. In: Ashcraft, K.W., Editor. Pediatric Surgery. Philadelphia, Pennsylvania: WB Saunders 2000; 248-251.2.  Ravitch, M. M. Disorders of the sternum and the thoracic wall. In: Sabiston, D.C. Jr, Spencer FC, eds. Gibbon’s surgery of the chest. 4th ed. Philadelphia: WB Saunders; 1983; 318–360.3.  Shamberger, R. C. and Welch, K. J. Sternal defects. Pediatric Surgery International 1990; 5: 156–164.4.  Mahone, P., Sherer, D., D’Angio, C. and Woods, J. Prenatal ultrasonographic findings associated with wide clefting of the upper two thirds of the fetal sternum. Am. J. Obstet Gynecol. 1992; 166: 1219–1221.5.  Sarper, A., Oz, N., Arslan, G., and Demircan, A. Complete Congenital Sternal Cleft Associated with PectusExcavatum. Texas Heart Institute Journal. 2002; 29: 206–209.6.  de Campos, J.R., Filomeno, L.T., Fernandez, A., et.al. Repair of congenital sternal cleft in infants and adolescents. Ann. Thorac. Surg. 1998; 66:1151–1154.7.  Singh, S., Lahoti, B.K., Garge, S., Negi, A. and Jain, V. Sternal cleft repair: A report of two cases and review of literature. African Journal of Pediatric Surgery. 2010; 7: 211-213.8.  Metry, D. W., Dowd, C. F., Barkovich, A. J. and Frieden, I. J. The many faces of PHACE syndrome. J. Pediatr. 2001; 139:117–123.9.  Frieden, I., Reese, V. and Cohen, D. PHACE syndrome: the association of posterior fossa brain malformations, hemangiomas, arterial anomalies, coarctation of the aorta and cardiac defects, and eye abnormalities. Arch Dermatol 1996; 132:307–311.10. Mazzie, J. P., Lepore, J., Price, A. P. et.al. Superior Sternal Cleft Associated With PHACES Syndrome: Postnatal Sonographic Findings. J. Ultrasound Med. 2003; 22:315–319.11.  Mahone, P. R., Sherer, D. M., D’Angio, C. and Woods, J. R. Prenatal ultrasonographic findings associated with wide clefting of the upper two thirds of the fetal sternum. Am. J. Obstet. Gynecol. 1992; 4:1219-1221.12.  Samarrai, A., Charmockly, H. and Attra, A. Complete cleft sternum: classification and surgical repair. Int. Surg. 1985; 70:71–73.13.  Domini, M., Cupaioli, M., Rossi, F., et al. Bifid sternum: neonatal surgical treatment. Ann. Thorac. Surg. 2000, 69:267–269.14.  Yavuzer, S. and Kara, M. Primary repair of a sternal cleft in an infant with autogenoustissues. Interact Cardio. Vasc. Thorac. Surg. 2003; 2: 541-543.15.  Chakkarapani, E., Barnard, I. and Couriel, J. Superior sternal cleft, cutaneous, and airway haemangiomas. Arch. Dis. Child Fetal Neonatal Ed. 2007; 92: F3.

An isolated superior sternal cleft in a neonate in western Kenya: case report

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An isolated superior sternal cleft in a neonate in western Kenya: case report

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