Isolated congenital left ventricular diverticulum

Two cases of isolated left ventricular diverticulum are presented. A 12-year-old boy and a 7-year-old girl, both asymptomatic, were admitted to our Department for evaluation of an abnormal electrocardiogram and an abnormal cardiac silhouette, respectively. Both patients had the diagnosis confirmed by cardiac catheterization and angiography and underwent successful surgical correction of the abnormality. We discuss the diagnosis, prognosis, and surgical treatment of this entity and we advocate early surgical treatment for all diagnosed ventricular diverticula, even if asymptomatic.     

Ectopia cordis and other midline defects

BACKGROUND: Thoracic ectopia cordis and other midline defects are rare congenital anomalies that often occur with other intracardiac defects. Despite significant improvements in neonatal and infant cardiac surgeries, operations for thoracoabdominal ectopia cordis carry an extremely high mortality with only a few reported survivors of thoracic ectopia cordis. METHODS: The clinical charts of 4 patients with ectopia cordis over a 6-year period were reviewed. Three of the patients showed varying degrees of Cantrell's Pentalogy; thoracic ectopia cordis was found in 1. We have reviewed our surgical strategies and reported the patients' clinical outcomes. RESULTS: All 4 patients are alive at follow-up. Two infants with double-outlet right ventricle have been fully corrected, and extracorporeal membrane oxygenation was necessary in 1 infant for cardiac failure following the cardiac repair. A newborn with thoracoabdominal ectopia cordis underwent primary repair of his diaphragmatic defect, and a silo was used to progressively reduce the omphalocele. He is currently awaiting elective repair of tetralogy of Fallot. Lastly, the patient with thoracic ectopia cordis underwent successful soft tissue coverage, and she is being followed in the clinic with restrictive muscular ventricular septal defects and a left ventricular diverticulum. CONCLUSIONS: Our experience along with other reports in the literature demonstrates that patients with thoracic and thoracoabdominal ectopia cordis can undergo and survive full cardiac, neurologic, and abdominal repair during infancy. Furthermore we advocate different approaches determined by the severity of the presentation and the presence of other complicating factors.     

Left ventricular diverticulum with hypertrophy of the left ventricular apex.

A surgical case of diverticulum in the left ventricular apex is presented. A two-dimensional echocardiogram and magnetic resonance image showed a calcified tumor buried in the marked hypertrophied apex of the left ventricle. Enucleation of the oval and hard tumor (4 x 2.5 x 2.5 cm) was performed through the apex, and the defect was anastomosed by buttress sutures. Histologic examination demonstrated that the tumor cavity was filled with a thrombus encapsulated by thickened and calcified endocardium that extended to the left ventricular cavity. In this report, the etiology of the diverticulum with a hypertrophied myocardium is discussed.     

Stereotactic management of congenital midline cysts

Summary The treatment of congenital midline cysts remains a controversial issue. The Stereotactic management of 27 patients (6 symptomatic cavum septi pellucidi/cavum Vergae, 6 suprasellar cysts, 5 intraventricular cysts, 4 parasagittal cysts, and 6 supracollicular cysts) is reviewed. In 23 patients Stereotactic ventriculo-cystostomy by catheter implantation (internal drainage) led to clinical recovery or improvement accompanied by decreased cyst size. In four patients the internal drainage was not sufficient and was therefore completed as a ventriculo-atrial shunt system. Three minor complications (bleeding, aseptic meningitis, catheter infection) led to no sequelae. The results suggest that Stereotactic internal drainage of these benign lesions is a safe, minimally invasive and efficient procedure.     

Case report: congenital ureteral diverticulum and solitary kidney.

A case of true congenital diverticulum of the ureter associated with a solitary kidney is reported. The diverticulum was producing hydroureteronephrosis. Treatment consisted of excision of the diverticulum and repair of the narrowed area in the ureter. At 4-year followup there was no deterioration on excretory urography and renal function tests have remained normal.     

The congenital cervical midline cleft. Case report and review of literature.

Congenital midline cervical clefts (CMCC) are rare. There have been approximately 50 reports in the international literature with the majority found in Caucasian females. They consist of a skin tag, mucosal surface and a caudal sinus in the ventral midline of the neck. The embryological cause is disputed, as is the best course of treatment. A case of a 4-month-old Indian boy with a congenital midline cervical cleft is reported. The appropriate investigations are described as well as a new method of correcting the lesion. In addition, the most likely aetiological basis for the condition are discussed.     

Echocardiographic analysis of ventricular geometry and function during repair of congenital septal defects

BACKGROUND: This study investigated changes in left ventricular (LV) geometry and systolic function after corrective surgery for atrial (ASD) and ventricular septal defects (VSD). METHODS: Transesophageal LV short-axis echocardiograms were recorded before and after operative repair of ASD (n = 11) and VSD (n = 7). Preload was measured using LV end-diastolic area indexed for body surface area. Measurements of septal-freewall (D1) and anterior-posterior (D2) endocardial diameters were used to assess LV symmetry from D1/D2. Systolic indices included stroke area, area ejection fraction, and fractional shortening. RESULTS: Preload, stroke area, area ejection fraction, and fractional shortening of D1 increased after ASD repair but decreased after VSD repair (p < 0.05). End-diastolic symmetry increased after ASD closure and decreased after VSD closure (p < 0.05). Increases in stroke area and ejection fraction after ASD correction primarily reflected increased shortening of D1. A positive correlation was found overall between percent change in end-diastolic area (EDA) and percent change in area ejection fraction (r(2) = 0.80, p < 0.0001, n = 18). CONCLUSIONS: Preload was the primary determinant of changes in LV function in this series of ASD and VSD repairs. Intraoperative changes in position of the interventricular septum affected systolic and diastolic LV symmetry and septal free wall shortening. Additional studies are needed to define changes in afterload and contractility as well as diastolic compliance and systolic mechanics.     

Infected congenital urethral diverticulum in an adult male.

Congenital anterior urethral diverticulum is a rare anomaly. The majority present in infancy with urinary obstruction. Those who present beyond infancy do so on account of recurrent urinary tract infection or dribbling. We present a case of congenital anterior urethral diverticulum manifesting in adulthood with suppuration in the diverticulum.     

Spontaneous perforation of congenital bladder diverticulum

A traumatic rupture of the bladder in a healthy child is extremely rare and is usually not considered during evaluation of abdominal pain or sepsis. However, a delay in the diagnosis of bladder perforation can result in morbid and sometimes catastrophic outcomes. We report a delayed diagnosis of spontaneous rupture of a congenital bladder diverticulum in a previously healthy child. This case demonstrates that spontaneous bladder rupture may be a rare cause of abdominal complaints and sepsis when the etiology at first seems unclear.     

Reconstruction of complex thoraco-abdominal defects with extended anterolateral thigh flap

Background:

The reconstruction of complex thoraco-abdominal defects following tumour ablative procedures has evolved over the years from the use of pedicle flaps to free flaps. The free extended anterolateral thigh flap is a good choice to cover large defects in one stage.

Materials and Methods:

From 2004 to 2009, five patients with complex defects of the thoracic and abdominal wall following tumour ablation were reconstructed in one stage and were studied. The commonest tumour was chondrosarcoma. The skeletal component was reconstructed with methylmethacrylate bone cement and polypropylene mesh and the soft tissue with free extended anterolateral thigh flap. The flaps were anastomosed with internal mammary vessels. The donor sites of the flaps were covered with split-skin graft.

Result:

All the flaps survived well. One flap required re-exploration for venous congestion and was successfully salvaged. Two flaps had post operative wound infection and were managed conservatively. All flap donor sites developed hyper-pigmentation, contour deformity and cobble stone appearance.

Conclusion:

Single-stage reconstruction of the complex defects of the thoraco-abdominal region is feasible with extended anterolateral thigh flap and can be adopted as the first procedure of choice.     

Expanded midline forehead flap for coverage of nonnasal facial defects.

A 13-month-old infant with a giant hairy nevus of the superior portion of the right cheek, which measured 3.5 x 5.5 cm, was treated by excision and coverage using expanded midline forehead flap. This approach stands in contrast both to the standard technique of excision and full-thickness grafting for large facial defects as well as to the use of the midline forehead flap for nonnasal reconstruction. Although this approach did necessitate the placement of a midline forehead scar, the overall result was sufficiently superior to justify its use. The child has good, stable, soft coverage with no contour or landmark distortion. The child is presented in an 18-month follow-up with photographic documentation.     

A case of congenital midline cervical cleft with congenital heart disease

A congenital midline cervical cleft (CMCC) is a rare developmental anomaly. It may represent failure of the branchial arches to fuse in the midline and presents at birth with a ventral midline defect of the skin of the neck. Congenital heart disease along with CMCC is rarer, and most of the cases reported are associated with chest wall defects or thoracic ectopia cordis. The authors report a case of a 5-month-old girl with CMCC and an atrial septal defect (ASD) and discuss the clinical presentation, embryologic development, and treatment.     

Congenital fourth ventricular midline outlet obstruction. Report of two cases

Obstruction of the foramen of Magendie unrelated to illness or trauma is rare in adults. Two cases of congenital membranous occlusion of the foramen of Magendie in adults are presented. Analysis of the reported cases of fourth ventricular outlet obstruction disclosed only three similar cases.     

The association of scoliosis and congenital heart defects.

The effects of cardiac surgery on patients with congenital heart defects and the subsequent development of scoliosis were studied. A group of 998 patients with congenital heart defects who were less than sixteen years old were operated on at the Mayo Clinic during the ten-year period 1950 through 1959. Standing roentgenograms of the spine were made of 377 of the patients ten years or more after surgery. The ages of the patients at follow-up ranged from ten years and seven months to thirty-five years and three months, with a mean age of twenty-one years and four months. The average length of follow-up was fourteen years and eleven months. Of the 377 patients, thirty-two (8.5 per cent) had curves greater than 20 degrees. The female:male ratio of patients with congenital heart defects was 1:1, whereas of those who developed scoliosis it was 5:3. There was no correlation between scoliosis and the following: patient's sex, cardiac abnormality, size or side of the heart, side of the aortic arch, presence of cyanosis, age at surgery, number and type of surgical incisions, number and side of ribs removed, or number and type of surgical procedures.