Perioperative management of a patient with Dandy Walker malformation with tetralogy of Fallot undergoing total correction and fresh homologous pericardial pulmonary valve conduit implantation: Report of a rare case

Objectives: To detect the early outcome of total correction of Tetralogy of Fallot (TOF) in adult patients of age 16 years or above. Study Design: Retrospective descriptive case series. Setting: Paediatric Cardiac Surgery Department of Faisalabad Institute of Cardiology, Faisalabad. Period: October 2016 to June 2019. Material & Methods: All consecutive patients of age 16 years or above who underwent total correction for TOF during study period were included. Surgical procedure and early outcome measures were recorded and analyzed. Results: Sixty nine patients underwent total correction for TOF during the study period. Majority of patients (n=55, 79.7%) were between16 to 25years of age. Male dominated the study population (56.5%, n=39) with male to female ratio 1.3: 1. Nine patients (13%) had coiling of MAPCA before surgery while prior palliation with Blalock Taussig shunt (BT Shunt) was seen in 5.8 % patients (n=4). All patients were operated with trans-atrial trans-pulmonary approach (n=69, 100%). Pulmonary artery was augmented with pericardial patch in 22 cases (32%) while left pulmonary artery (LPA) augmentation was done in two patients. Pulmonary valve annulus divided in 22 % of patients (n=15), RVOT was reconstructed with trans-annular pericardial patch in 10.1% patients (n=7) while Pulmonary valve was replaced with tissue valve in 7 patients (10.1%). In one patient RV to PA continuity was established with contegra. Post operatively Inotropic support was given for initial 39 ±45 hours, mean ICU stay was 39±75 hours, mean chest drainage 1086±741 ml and mean requirement for blood transfusion was 2.2 ±2.4 units. As regard early complications, six patients had tiny to small residual VSD (8.7%) while no patient developed complete heart block. Two patients had neurological damage (2.9%), one patient developed renal failure (1.45%) while chest reopening was done in two patients due to postoperative bleeding and/ or tamponade (2.9%). There was death of two patients (2.9%), one had renal failure due to low cardiac output (LCOS) and the other had stroke. Conclusion: The early outcome of complete repair of TOF in patients 16 years and above is good with a negligible mortality and limited number of complications.

Download Full-text

A rare case of tetralogy of Fallot with absent Pulmonary valve

Modern pediatrics. Ukraine ◽

10.15574/sp.2020.106.93 ◽

2020 ◽

pp. 93-99

Author(s):

Andriana Malska ◽

◽

Olga Kurilyak ◽

Keyword(s):

Tetralogy Of Fallot ◽

Rare Case ◽

Pulmonary Valve ◽

Absent Pulmonary Valve

Download Full-text

PHACES syndrome with Ectopia cordis

International Journal of Contemporary Pediatrics ◽

10.18203/2349-3291.ijcp20181563 ◽

2018 ◽

Vol 5 (3) ◽

pp. 1153

Author(s):

Rambabu Sharma ◽

Dhan Raj Bagri ◽

Narendra Jangid

Keyword(s):

Rare Case ◽

Surgical Intervention ◽

Corneal Opacity ◽

Coarctation Of Aorta ◽

Ectopia Cordis ◽

Phaces Syndrome ◽

Dandy Walker Malformation ◽

Arterial Lesions ◽

Walker Malformation

PHACES syndrome is an acronym for, P, posterior fossa anomalies as Dandy- Walker malformation; H, hemangioma (capillary segmental faces); A, arterial lesions of the head and neck (the most common ones include aberrant origin or course, hypoplasia, dysplasia and agenesis); C, cardiac abnormalities as coarctation of aorta; E, abnormalities of the eye and S, sternal defect. The constellation of findings of PHACES syndrome may vary significantly in different patients. Complete sternal cleft with ectopia cordis is an extremely rare congenital anomaly failed ventral migration and fusion of the two-lateral mesodermal sternal bands between the sixth and ninth weeks of gestation. We are reporting a rare case of PHACES syndrome, with partial ectopia cordis as a midline defect, who is in our follow up since birth. Survival of the child without surgical intervention with epithelialization over the defect and Leucomatous corneal opacity are interesting findings.

Download Full-text

Total Correction of Tetralogy of Fallot: Effect of Transannular Patch on Early Outcome

Cardiovascular Journal ◽

10.3329/cardio.v10i2.36291 ◽

2018 ◽

Vol 10 (2) ◽

pp. 194-200 ◽

Cited By ~ 1

Author(s):

Md Azizul Islam Khan ◽

Kazi Abul Hasan ◽

ABM Abdus Salam ◽

Quazi Abul Azad ◽

Sadia Sajmin Siddiqua ◽

...

Keyword(s):

Right Ventricular ◽

Tetralogy Of Fallot ◽

Pulmonary Valve ◽

Total Correction ◽

Early Outcome ◽

Transannular Patch ◽

Group A ◽

Valve Annulus ◽

Z Value ◽

Group B

Background: It is arguable whether presence of transannular patch is itself a risk factor for adverse outcome at total correction(repair) of tetralogy of Fallot (TOF).This study intended to compare early outcome of intact pulmonary valve annulus with transannular patching at repair of TOF.Methods: This prospective observational study was conducted from July 2015 to January 2017.40 patients were enrolled in 2 groups- A & B.In group A,14 patients with intact pulmonary valve annulus& in group B, 26 patients with transannular patch. The diameter of pulmonary valve annulus was measured with Hegar dilator and Z value of the measured diameter were calculated from an established published nomogram. Transannular patch was placed if Z value of annular diameter < -3 or post repair operative room right ventricle/left ventricle pressure ratio (P RV/LV)> 0.7.Patients were monitored in the intensive care unit(ICU) and followed up for 3 months following discharge from hospital.Results: Patients of group B were younger and smaller body surface area. There were no significant difference of preoperative variables in terms of peripheral arterial oxygen saturation(SpO2%), haematocrit (%),NYHA functional class, right ventricular hypertrophy, and level & severity of right ventricular out flow tract obstruction. Early out come in terms of duration of ventilation time, inotrope support & ICU stay; post operative morbidity & mortality were more in group B than group A patients. Pulmonary regurgitation & right ventricular dysfunction following transannular patch at repair of TOF plays important role for adverse outcome.Conclusion: Transannular patch is associated with higher morbidity and mortality in total correction of Tetralogy of Fallot.Cardiovasc. j. 2018; 10(2): 194-200

Download Full-text

Cerebellar Agenesis: A Case to Remember

Journal of Pediatric Neurology ◽

10.1055/s-0039-1679892 ◽

2019 ◽

Vol 18 (01) ◽

pp. 045-048

Author(s):

John V. Dennison ◽

Dharmesh R. Tailor

Keyword(s):

Posterior Fossa ◽

Congenital Anomalies ◽

Rare Case ◽

Genetic Causes ◽

Cerebellar Function ◽

Dandy Walker Malformation ◽

Wide Range ◽

Walker Malformation

AbstractThere is a wide range of congenital anomalies of the posterior fossa. Of these, absence of cerebellar components seems to exist on a spectrum, much like anomalies seen with the more frequently encountered Dandy–Walker malformation. Here, we present a rare case of cerebellar agenesis, as seen at our institution, which falls on the extreme side of the spectrum. We also discuss normal and aberrant posterior fossa development, established or hypothesized genetic causes of such development, and implications on cerebellar function.

Download Full-text

Unusual Ventriculoperitoneal (VP) Shunt Tube Extrusion through Anus in a Child with Dandy Walker Malformation: A Rare Case Report

JOURNAL FOR CLINICAL AND DIAGNOSTIC RESEARCH ◽

10.7860/jcdr/2015/11354.5488 ◽

2015 ◽

Keyword(s):

Case Report ◽

Rare Case ◽

Vp Shunt ◽

Shunt Tube ◽

Tube Extrusion ◽

Dandy Walker Malformation ◽

Rare Case Report ◽

Walker Malformation

Download Full-text

Perioperative management of tetralogy of Fallot with absent pulmonary valve

Pediatric Anesthesia ◽

10.1046/j.1460-9592.2002.00919.x ◽

2002 ◽

Vol 12 (8) ◽

pp. 705-711 ◽

Cited By ~ 7

Author(s):

Stephen A. Stayer ◽

Shakunthala Shetty ◽

Dean B. Andropoulos

Keyword(s):

Tetralogy Of Fallot ◽

Perioperative Management ◽

Pulmonary Valve ◽

Absent Pulmonary Valve

Download Full-text

DANDY WALKER MALFORMATION: A RARE CASE REPORT

10.36106/ijsr/4508031 ◽

2021 ◽

pp. 71-72

Author(s):

Triza Kumar Lakshman ◽

Yeshwanthini J ◽

Ravindra S Pukale

Keyword(s):

Case Report ◽

Posterior Fossa ◽

Fourth Ventricle ◽

Rare Case ◽

Male Fetus ◽

Dandy Walker Malformation ◽

Rare Case Report ◽

Upward Displacement ◽

Walker Malformation ◽

Posterior Fossa Malformation

Dandy-Walker malformation (DWM) or syndrome is a posterior fossa anomaly characterized by agenesis or hypoplasia of the vermis and cystic enlargement of the fourth ventricle causing upward displacement of tentorium and torcula. Most patients have hydrocephalus at the time of diagnosis. DMW is the most common posterior fossa malformation, and it typically occurs sporadically. This is a case report of male fetus that was diagnosed to have Dandy Walker Malformation during the antenatal obstetric scans.

Download Full-text