We report a case of sternal reconstruction using bilateral sternal bar turnover flaps in a 4-year-old boy with an inferior sternal cleft, as part of Cantrell's pentad. When the patient was 10 months old, he underwent sternal reconstruction using a resorbable poly-L-lactic-polyglycolic acid plate in the first stage when there was insufficient autogenous tissue to provide a reliable reconstruction. Bilateral sternal bar turnover was performed in the second stage at 4 years of age. This operative technique is described in this report. This novel technique provides a robust, dynamic, and reliable reconstruction for inferior sternal defects.
Child, Preschool ; Ectopia Cordis ; Humans ; Male ; Pentalogy of Cantrell*

Double Outlet Right Ventricle ; complications ; Female ; Humans ; Infant, Newborn ; Pentalogy of Cantrell ; diagnosis ; etiology

Cantrell's pentalogy may be defined as a failure of fusion of the midline from the sternum to the umbilicus. Thus, this malady consists of multiple anomalies of the sternum, heart, pericardium, diaphragm and umbilicus or anterior abdominal wall. According to the degrees of each anomaly, various operations can be planned as a one-stage operation or as a multi-stage operation and then palliative or corrective operations. The authors experienced a case of Cantrell's pentalogy that consisted of a bifid sternum, ventricular septal defect, atrial septal defect, ventricular diverticulum, dextrocardia, pericardial defect, anterior diaphragmatic defect and diastasis recti; all of these problems were corrected by a one-stage operation.
Abdominal Wall ; Dextrocardia ; Diaphragm ; Diverticulum ; Heart ; Heart Septal Defects, Atrial ; Heart Septal Defects, Ventricular ; Pentalogy of Cantrell ; Pericardium ; Sternum ; Umbilicus

OBJECTIVETo analyze the diagnostic feature, treatment and prognosis of patients with Cantrell syndrome.

METHODSClinical manifestation, diagnosis, operation and follow-up data of 5 patients with Cantrell syndrome were summarized in this retrospective analysis.

RESULTSThe age of the 5 patients was 7 days-76 years, definite diagnosis was made in 3 cases and 2 cases presented feature of incomplete Cantrell syndrome. Three patients with full Cantrell syndrome were correctly diagnosed before operation and confirmed by operation. One patient with incomplete Cantrell syndrome (two-vessel stenosis) received bypass surgery. Another asymptomatic patient with incomplete Cantrell syndrome (apical diverticulum of the left ventricle) does not need operation and is under observation. During follow-up, 1 patient died at 60 months after operation and the remaining 4 patients are alive and well.

CONCLUSIONSWith the development of modern imaging technology, it becomes easy to make correct diagnose Cantrell syndrome before operation. Prognosis is fine post timely operation and related intervention.

Adolescent ; Adult ; Aged ; Child ; Child, Preschool ; Female ; Follow-Up Studies ; Humans ; Infant ; Infant, Newborn ; Male ; Middle Aged ; Pentalogy of Cantrell ; diagnosis ; therapy ; Prognosis ; Retrospective Studies ; Young Adult

Pentalogy of Cantrell was very rare congenital anomaly and represent a regional prevalence of 5.5/1 million live births. The syndrome, Cantrell's pentalogy is characterized by defects in the lower sternum with ectopia cordis, anterior diaphragm defects, midline supraumbilical abdominal wall defects, defects in the diaphragmatic pericardium, and various intracardiac defects. Anencephaly involves acrania with a degenerated mass of brain tissue. Combination of both conditions are extremely rare. We experienced a case of Cantrell's pentalogy with anencephaly detected by ultrasonography at 15 weeks 1 day menstural age and confirmed at autopsy. Thus we report a case with brief review of the literature.
Abdominal Wall ; Anencephaly* ; Autopsy ; Brain ; Diaphragm ; Ectopia Cordis ; Hernia, Umbilical ; Live Birth ; Neural Tube Defects ; Pentalogy of Cantrell* ; Pericardium ; Prevalence ; Sternum ; Ultrasonography ; Ultrasonography, Prenatal*

Identification of certain abnormalities of the chest wall can be extremely helpful in correctly diagnosing a number of syndromic conditions and systemic diseases. Additionally, chest wall abnormalities may sometimes constitute diagnoses by themselves. In the present pictorial essay, we review a number of such conditions and provide illustrative cases that were retrospectively identified from our clinical imaging database. These include pentalogy of Cantrell, Klippel-Feil syndrome, cleidocranial dysplasia, Poland syndrome, osteopetrosis, neurofibromatosis type 1, Marfan syndrome, Gardner syndrome, systemic sclerosis, relapsing polychondritis, polymyositis/dermatomyositis, ankylosing spondylitis, hyperparathyroidism, rickets, sickle cell anemia, thalassemia, tuberculosis, septic arthritis of the sternoclavicular joint, elastofibroma dorsi, and sternal dehiscence.
Anemia, Sickle Cell ; Arthritis, Infectious ; Cleidocranial Dysplasia ; Diagnosis ; Gardner Syndrome ; Hyperparathyroidism ; Klippel-Feil Syndrome ; Marfan Syndrome ; Neurofibromatosis 1 ; Osteopetrosis ; Pentalogy of Cantrell ; Poland Syndrome ; Polychondritis, Relapsing ; Retrospective Studies ; Rickets ; Scleroderma, Systemic ; Spondylitis, Ankylosing ; Sternoclavicular Joint ; Thalassemia ; Thoracic Wall ; Tuberculosis