A complete case of Cantrell's Pentalogy with isolated left ventricular diverticulum

The congenital left ventricular diverticulum is a rare cardiac malformation, and it may associate with Cantrell’s Pentalogy with other cardiac defects. However, isolated ventricular diverticulum without any other cardiac defect in complete Cantrell’s syndrome is very rare. We describe a 6-year-old male patient with a complete Cantrell’s syndrome with isolated left ventricular diverticulum.

Abdominal ectopia cordis in an aborted calf without chromosomal aberrations

Ectopia cordis is a rare congenital heart disease characterized by partial or complete displacement of the heart out of the thoracic cavity. Apart from cattle, the condition has also been described in humans and is frequently associated with Cantrell’s pentalogy. It is classified into five types: cervical, cervicothoracic, thoracic, abdominal and thoracoabdominal. The prognosis is poor and ectopia cordis may be linked to the presence of unbalanced chromosome alterations. In this report, a case of abdominal ectopia cordis is described in an aborted calf, in which no unbalanced structural chromosomal variants could be identified.

What the neonatologists need to know about the Cantrell’s pentalogy

Cantrell’s pentalogy is a congenital syndrome that includes multiple defects which predispose the fetus to a high mortality rate. Over time, the association of malformations defining this syndrome has changed accepting incomplete forms, or forms that associate other anomalies. The therapeutic strategy in Cantrell's pentalogy is an intensely debated one as it deals with newborns having a low survival rate and multiple malformations. Once treatment is started for certain malformations it can lead to the aggravation of others. In conclusion, a conservative approach is recommended, with the initiation of surgical treatment after the stabilization of the newborn. A multidisciplinary approach is needed to achieve optimal results in these difficult cases.

A propósito de un caso: Resolución quirúrgica en dos tiempos de Pentalogía de Cantrell

BACKGROUND: Cantrell’s pentalogy includes the presence of five birth defects that represent a great challenge for surgeons. Abnormalities of the heart, pericardium, diaphragm, sternumand anterior abdominal wall are the main findings. Its incidence is low, however, it is essential to identify Cantrell´s pentalogy timely to adopt an adequate therapy for all specific defects, since it has high mortality. CASE REPORT: The patient was a full- term male newborn, with a history of abdominal wall defect compatible with an omphalocele detected by prenatal ultrasound. After the caesarean section, the abdominal wall defect was notable, the left liver lobe, intestines and heart emerged through it, the sternum also had a low fissure. The echocardiogram revealed a permeable oval foramen, mild tricuspid regurgitation, and severe pulmonary hypertension. EVOLUTION: Immediate surgical management was decided. Silo was placed, with progressive closure of the midline in 7 days. During the second surgical procedure, the diaphragmatic and pericardial defect was corrected with a bovine pericardial prosthesis. Despite the adequate evolution after surgery, at day 28 he presented with sudden cyanosis and didn’t respond to cardiopulmonary resuscitation and died. CONCLUSIONS: Cantrell’s Pentalogy is a rare disease, with peculiar clinical, anatomical and embryological characteristics, it represents a unique challenge for surgeons. Early diagnosis, as well as follow-up during pregnancy, planning a cesarean section in a high-level center and immediate surgical approach with a multidisciplinary team, are the key components in the management of patients with Cantrell’s Pentalogy. KEY WORDS: PENTALOGY OF CANTRELL, ECTOPIA CORDIS, UMBILICAL HERNIA. CONGENITAL.

Early diagnosis of Cantrell’s Pentalogy in a newborn baby

A clinical case of early diagnosis of Pentalogy of Cantrell (POC) in a newborn infant is presented. The features of the formation of the defect and its classification are described. A crucial role in verifying the diagnosis was performed by CT scan with intravenous contrast, which revealed a narrowing of the pulmonary artery trunk; located subcutaneously above the umbilical ring, the diverticulum of the left ventricle of the heart, originating from the apical section of the left ventricle and connected to its cavity by a linear isthmus located along the middle line, as well as a local defect of the diaphragm in the anterior section. Taking into account the accumulated experience, it is generally recognized that when establishing this diagnosis in the first trimester, termination of pregnancy is more preferable. Therefore, parents should be notified of a possible adverse outcome in a timely manner. The presented observation reflects the high importance of early diagnosis of congenital forms of combined pathology in children, requiring the use of modern research methods to determine the tactics and conduct timely effective treatment.

Novel peritoneopericardial diaphragmatic hernia in a dog

A 23-month-old German shepherd dog (GSD) with chronic intermittent gastrointestinal signs presented following acute deterioration compatible with gastrointestinal obstruction and suspected peritoneopericardial diaphragmatic hernia (PPDH). Physical examination revealed depression, abdominal pain, decreased pulse quality, reduced heart sounds and tachypnoea with a shallow breathing pattern. Radiography confirmed PPDH and a granular, 1.3 cm mineral opacity cranial to the cardiac silhouette within the cranioventral thorax. Coeliotomy and median sternotomy revealed strangulated jejunum within the cranial mediastinum, cranial to the pericardium. This was resected and herniorrhaphy was performed. Postoperatively the dog became normal. Midline fusion defects, including the pericardium, can be associated with PPDH and in such cases sternotomy may be required. This is the first report of surgical management of strangulated intestine secondary to an initially conservatively managed PPDH. Mineral opacity on radiography associated with PPDH may represent chronic partial obstruction. This possibly represents a second GSD with Cantrell’s pentalogy.

Gravitational Autoreposition for Staged Closure of Omphaloceles

Introduction Management strategies for large omphaloceles remain controversial. In this study, we discuss the use of GRAVITAS (gravitational autoreposition sutures), the method used at our institution when successful primary closure is deemed questionable. Patient's primary clinical course and long-term outcomes were analyzed. Materials and Methods This is a single-center retrospective analysis of all consecutive patients with omphaloceles treated between 1997 and 2018. Decision for GRAVITAS was made when the defect was estimated too large for primary closure. Traction sutures were placed in the fascia surrounding the defect and then suspended from the top of the incubator to allow gravitational autoreposition of the herniated organs. Ventilation and muscle relaxation were maintained until secondary closure, which was performed after the obtruding viscera had been reduced by repeated adjustment of the suture's tension. Data are presented as mean ± standard deviation. Results Out of 49 patients with omphaloceles, 12 were treated with GRAVITAS, 33 underwent primary closure, and 4 were treated using Schuster's technique. Mean time to secondary closure after GRAVITAS was 7 ± 10 days. In nine of the patients who had isolated omphalocele, secondary closure was achieved after 4 ± 2 days. Ventilation time was 5 ± 2 days, and time to full feeds was 18 ± 16 days. In three patients (one with Fallot's tetralogy, one with Cantrell's pentalogy, and one with lung hypoplasia), abdominal closure was achieved after 17 ± 15 days. Due to cardiorespiratory comorbidity, ventilation time was >30 days. Five patients received initial closure of the skin and secondary fascial closure after 18 ± 15 months. One patient with prior fascial closure underwent later repair of an abdominal wall hernia. During follow-up (30 ± 35 months), one patient with gastrointestinal obstruction due to adhesions required laparotomy, and one patient with gastroesophageal reflux disease underwent fundoplication. Conclusion GRAVITAS is a feasible method for staged closure of large omphaloceles when successful primary closure is deemed questionable.