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Pentalogy of Cantrell

Cantrell Syndrome


Presentation

  • Sternal and pericardial defects were each present in 40% of patients. Additional anomalies present included pulmonary hypoplasia, pulmonary artery stenosis, and chromosomal abnormalities.[ncbi.nlm.nih.gov]
  • Neonates usually present with severe respiratory distress and cyanosis.[orpha.net]
  • We present this case because of its rarity and discuss the pathologic findings.[ncbi.nlm.nih.gov]
  • Understanding the molecular-genetic basis of diaphragmatic hernias may shed light on this unusual presentation and explain why other cases show no fusion.[ncbi.nlm.nih.gov]
Swelling
  • Here a six-year-old girl who underwent surgery was presented with a large abdominal swelling. She had a large supra-umbilical abdominal wall defect covered with coarse tissue through which the abdominal contents were herniating.[ncbi.nlm.nih.gov]
  • On colour Doppler ultrasonogram at MMCH showed there were VSD, ASD, and a small epigastric swelling which was vascular and appears to be attached to the apex of the heart.[ncbi.nlm.nih.gov]
  • His chest X-ray showed a midline heart with inferior displacement [Figure 3], abdominal ultrasound showed the swelling to contain loops of bowl and echocardiography revealed a 6 mm secundum atrial septal defect.[nigeriamedj.com]
Large Pericardial Effusion
  • A large pericardial effusion compressed the lungs and had led to a reduced lung growth with an observed-to-expected total lung volume of 28% as measured by MRI.[ncbi.nlm.nih.gov]
Multiple Congenital Anomalies
  • She gave birth to a preterm female infant who had multiple congenital anomalies. The infant was allowed to die in peace because she would not have survived eventually and corrective surgeries were impossible.[annsaudimed.net]
Regurgitation
  • Findings were situs solitus, mesocardia to dextrocardia, DORV, large VSD, mild mitral inflow, mild TR (triuspid regurgitation), good LV (left ventricle) and RV (right ventricle) systolic function.[ncbi.nlm.nih.gov]
Failure to Thrive
  • By taking medical treatment patient condition is well except failure to thrive and cyanosis develops during feeding and crying according to the statement of guardian of the patient.[ncbi.nlm.nih.gov]
Systolic Murmur
  • On physical examination her vital signs were within normal limit, she had a systolic murmur on heart at lower left sternal area and there was a vascular structure present on the upper part of abdomen which was pulsatile and pulsation was synchronized[ncbi.nlm.nih.gov]
  • He was not tachypneic or tachycardic and had first and second heart sounds with a systolic murmur. Bowel sounds were audible and normal.[nigeriamedj.com]
Thrombosis
  • […] and major chromosomal abnormalities Hydropic placenta Pathology of the umbilical cord Abnormal length Short cord Long cord Insertion abnormalities Abnormal coiling Umbilical vessels pathology Umbilical cord accidents which compromise the blood flow Thrombosis[atlases.muni.cz]
Foot Deformity
  • Although left unilateral club foot deformity was also detected as an associated anomaly in the same examination, severe lumbar lordoscoliosis was only detected by using three-dimensional sonography because of the spatial configuration of the deformity[ncbi.nlm.nih.gov]
Lordosis
  • […] multiple fetal anomalies suggesting the diagnosis of CP (a large thoraco-abdominal defect with herniating liver and bowel, heart deviated anteriorly with concomitant ventricular septal defect), and the 'S' shaped fetal spine due to increased lumbar lordosis[ncbi.nlm.nih.gov]
Cryptorchidism
  • […] malformations of female genitalia Congenital malformations of the internal genitalia Congenital malformations associated with congenital malformations of urinary system Congenital malformations of the vulva Congenital malformations of the male genital organs Cryptorchidism[atlases.muni.cz]

Workup

Candida
  • […] anomalies Club foot Constriction (amniotic) band syndrome Placental inflammation Ascending infections Placental inflammation, acute Hematogenous infections Fetomaternal listeriosis Pathology of the fetal membranes Amniotic band syndrome Meconium staining Candida[atlases.muni.cz]

Treatment

  • Other treatment of pentalogy of Cantrell is symptomatic and supportive.[en.wikipedia.org]
  • One case with double outlet right ventricle suffered from low cardiac output syndrome during the postoperative period and recovered after medical treatment.[ncbi.nlm.nih.gov]
  • They advised medical treatment and requested to review after one year.[ncbi.nlm.nih.gov]
  • Collaboration between cardiac surgeons and plastic surgeons is essential for optimal treatment.[ncbi.nlm.nih.gov]
  • We present a case and its treatment of PoC with complete ectopia cordis and congenital heart disease. Postnatally the congenital heart defect was surgically corrected and the ectopic heart was covered by musculous mobilized flap.[ncbi.nlm.nih.gov]

Prognosis

  • The syndrome is said to affect males and females in equal numbers and the prognosis is generally poor. We report two cases of Cantrell's Pentalogy diagnosed antenatally at the Korle Bu teaching Hospital.[ncbi.nlm.nih.gov]
  • The long-term prognosis for children with this anomaly depends to a great extent on the complexity of the associated congenital heart defect.[ncbi.nlm.nih.gov]
  • The prognosis of PC relies on the presence and severity of cardiac anomalies but in most cases outcome is fatal. To the best of our knowledge, the presence of kidneys into the omphalocele in Cantrells' pentalogy has not been reported yet.[ncbi.nlm.nih.gov]
  • In conclusion the prognosis seems to be poorer in patients with the complete form of pentalogy of Cantrell, EC, and patients with associated anomalies. Intracardial defects do not seem to be a prognostic factor.[ncbi.nlm.nih.gov]
  • Prognosis The prognosis of POC is poor and only few patients survive through early childhood. The main causes of death are tachyarrhythmias, bradycardia, low blood pressure, rupture of the diverticulum, and heart failure.[orpha.net]

Etiology

  • This morphologic constellation prompts us to emphasize the consideration of this etiological influence and provides further evidence.[ncbi.nlm.nih.gov]
  • A common etiology for these defects, an alteration in vascular development, is proposed.[ncbi.nlm.nih.gov]
  • We discuss here the etiology, prenatal diagnosis, and severity of and the mortality associated with this condition. To our knowledge, this was the first reported case of PC in Saudi Arabia.[ncbi.nlm.nih.gov]
  • Etiology The etiology of POC is still unknown.[orpha.net]

Epidemiology

  • Epidemiology [ edit ] Pentalogy of Cantrell occurs in 1/65,000 to 1/200,000 live births. [2] History [ edit ] It was first characterized in 1958. [4] References [ edit ] External links [ edit ][en.wikipedia.org]
  • Summary Epidemiology The prevalence of POC is estimated to be 1/65,000-1/200,000 live births.[orpha.net]
Sex distribution
Age distribution

Pathophysiology

  • This article presents a case of pentalogy of Cantrell and examines the literature to report the most current evidence relative to the embryology and pathophysiology.[ncbi.nlm.nih.gov]
  • Purpose: This article presents a case of pentalogy of Cantrell and examines the literature to report the most current evidence relative to the embryology and pathophysiology.[ingentaconnect.com]

Prevention

  • Antenatal ultrasound can also detect the anomaly, and early postnatal diagnosis of the syndrome, followed by immediate surgical repair, can prevent lethal complications.[ncbi.nlm.nih.gov]
  • Prevention - Pentalogy of Cantrell Not supplied. Diagnosis - Pentalogy of Cantrell Not supplied. Prognosis - Pentalogy of Cantrell Not supplied. Treatment - Pentalogy of Cantrell Not supplied. Resources - Pentalogy of Cantrell Not supplied.[checkorphan.org]
  • Early diagnosis and ligation/resection of the LVD is important to prevent complications. We report on a case of pentalogy of Cantrell, in which a LVD was diagnosed only at 2 months of age despite preceding pre- and postnatal echocardiography.[thieme-connect.com]
  • Although the early gestational week of our patient prevented the detailed evaluation of intra-cardiac defects, the presence of ectopia cordis and omphalocele provided us to diagnose the Cantrell pentalogy in the first trimester.[ijcasereportsandimages.com]
  • […] accomplished despite its high mortality rate, but the surviving cases did not have, or had only minor degrees of, ectopia cordis with small omphaloceles and simple intracardiac anomalies. 4 Nevertheless, surgical intervention is the treatment of choice to prevent[circ.ahajournals.org]

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