Truncal Wall Research Articles

Management of Chronic Truncal Wall Type-I Complex Regional Pain Syndrome in a Patient with Uncontrolled Diabetes Mellitus: Case Report

Complex regional pain syndrome (CRPS) is a chronic pain syndrome associated with sensory, vasomotor, sudomotor, motor and trophic changes commonly affecting the upper and lower extremities; however, we report a case of truncal wall CRPS in an uncontrolled type 2 diabetes mellitus patient. A male in his late fifties presented with right lower chest and upper abdominal wall burning, sharp and stabbing type of pain for 5 years associated with hyperalgesia and allodynia. The extensive evaluation ruled out any underlying pathology and the symptoms were increasing with time. He failed to respond to conservative pharmacological, non-pharmacological treatment and rehabilitation measures. In our institute, he underwent alcohol neurolysis of subcostal transversus abdominis plane and rectus sheath nerves with rehabilitation measures and reported significant relief in his symptoms. It has now been 6 months since the procedure was done and he has complete relief in pain. Hence, if sympathetic block is difficult to do, peripheral nerve block with alcohol and rehabilitation measures provide desirable effect in abdominal wall CRPS.

Long-term Outcome of 181 Patients With Liposarcomas of the Extremity and Truncal Wall.

Liposarcomas (LS) are one of the most common entities within the heterogenous group of soft tissue sarcomas. The aim of this study was to identify prognostic indicators in patients with LS of the extremities and truncal wall. We analysed the influence of potential prognostic factors on local recurrence-free survival (LRFS) and overall survival (OS) in 181 patients who were suitable for surgical treatment with curative intent. The median follow-up period was 7.1 years. The 5-year LRFS and OS rates were 79.1 and 93.3%. The 5-year OS rate was 94.7% in patients with R0-resected primary tumors and 72.7% in patients with R1/R2-status (p=0.023). In multivariate analysis, only histologic grade was found to be an independent prognostic factor of OS. Negative margins were not an independent prognostic factor in our series. Tumor biology reflected by histologic grade dictated the outcome.

Somatic Leiomyosarcoma of the Soft Tissues: A Single-Institutional Analysis of Factors Predictive of Survival in 164 Patients.

Somatic leiomyosarcoma (LMS) is an aggressive soft tissue sarcoma entity with a high metastatic potential. The purpose of this study was to identify prognostic indicators of survival in patients with somatic LMS of the soft tissues. We retrospectively assessed the relationship between local recurrence-free survival (LRFS), disease-specific survival (DSS), overall survival (OS) and potential prognostic factors in 164 patients who were suitable for surgical treatment in curative intent. Patients with soft tissue LMS of the extremities, the truncal wall and the head and neck area were included. The median follow-up time was 4.9years. In the entire cohort, the 5-year estimate of the DSS, OS and LRFS rate were 74.5% (95% confidence interval [CI] 65.0-81.8), 70.6% (95% CI: 60.9-78.3) and 63.4% (95% CI 53.4-71.9), respectively. Thirty-eight patients (23.2%) developed distant metastases with a median survival time of 1.5years after diagnosis of metastasis. Surgical margins attained at the initial oncologic resection and eventual re-excisions did not influence DSS, OS and LRFS significantly. Within the R0 subgroup, close and wide negative margins led to similar outcomes. High histologic grade (P<0.001), size >5cm (P=0.002) and subfascial localisation (P=0.002) were associated with significantly diminished DSS in univariate analysis. In multivariate analysis, only histologic grade was found to be an independent prognostic factor of DSS. The data from this study could not determine a prognostic significance of surgical margins suggesting that tumour characteristics other than margin status are important. Tumour biology reflected by the histologic grade dictates the final outcome.

Results of radiation therapy for unresected soft-tissue sarcomas

Definitive radiotherapy is uncommonly used in the management of soft-tissue sarcoma (STS). The purpose of the study was to evaluate the results of radiotherapy for unresected STSs treated in a single institution. Between 1970 and 2001, 112 patients with STSs underwent radiotherapy for gross disease. Locations of the tumor were 43% in the extremities, 26% retroperitoneal, 24% in the head and neck, and 7% in the truncal wall. Histologic grades were 11% G1 and 89% G2 to G3. Median size of tumor at radiotherapy was 8 cm (range, 1-30 cm). Median radiation dose was 64 Gy (range, 25-87.5 Gy). Twenty percent of patients received chemotherapy. Local control (LC), disease-free survival (DFS), and overall survival (OS) rates were evaluated in univariate (log-rank) and then multivariate (Cox model) analysis to determine prognostic factors for STS. Median follow-up for patients is 139 months (range, 30-365 months). The 5-year actuarial LC, DFS, and OS were 45%, 24%, and 35%, respectively. Tumor size at radiotherapy and radiation dose influenced LC, DFS, and OS in univariate analysis. LC at 5 years was 51%, 45%, and 9% for tumors less than 5 cm, 5 to 10 cm, and greater than 10 cm, respectively. Patients who received doses of less than 63 Gy had 5-year LC, DFS, and OS rates of 22%, 10%, and 14%, respectively, compared with 5-year LC, DFS, and OS rates of 60%, 36%, and 52%, respectively, for patients who received doses of 63 Gy or more. AJCC stage was related to the OS and DFS without statistically significant influence on LC. Use of chemotherapy, histologic grade, age, and location did not influence results. In multivariate analysis, LC was related to total dose (p = 0.02), T size at radiotherapy (p = 0.003), and AJCC stage (p = 0.04); DFS was related to total dose (p = 0.007), T size at radiotherapy (p = 0.01), and AJCC stage (p < 0.0001); and OS was related to AJCC stage (p = 0.0001) and total dose (p = 0.002), but not to T size, at radiotherapy. Major radiotherapy complications were noted in 14% of patients; 27% of patients who received doses of 68 Gy or more had these complications compared with 8% of patients treated with doses of less than 68 Gy. Definitive radiotherapy for STS should be considered in clinical situations where no acceptable surgical option is available. Higher radiation doses yield superior tumor control and survival. A rise in complications occurs in patients who receive doses of 68 Gy or more, which provides a therapeutic window for benefit in these patients.

The embryology of the common arterial trunk

The developmental background of common arterial trunk still remains to be elucidated. In this anomaly both the coronary, systemic and pulmonary circulation arise from a common arterial stem with a common orifice. The contribution of the embryonic ectomesenchymal neural crest seems to be essential for a normal separation of the aorta, pulmonary trunk and the adjoining myocardium lined outflow tract. Experimental neural crest ablation studies in chicken have proved that there is a role for neural crest cells. Several molecular biological studies, however, have shown that we are not solely dealing with the influence of neural crest. Many genes and gene cascades have been manipulated in mice and have shown that e.g. the endothelin cascade, Foxc1 and Foxc2, and more recently Sema3C and neuropilin-1 are factors, that if disturbed, can lead to common arterial trunk often in combination with aortic arch anomalies such as interruption type B. In humans common arterial trunk is one of the characteristics of the 22q11 deletion syndrome. In the mouse several of these genes, located on the syntenic chromosome 16 have been studied for this effect, but most result in an embryolethal phenotype. Neural crest tracing studies in both chicken–quail chimeras and transgenic neural crest reporter mice have revealed that there is a difference in neural crest contribution to the aortic and pulmonary truncal wall. The orifice of the common arterial trunk reflects this finding in that the often abnormally high positioned coronary orifices reside in the aortic part of the orifice. Combining current data the common denominator for common arterial trunk seems to be a defective interaction of neural crest, endothelial cells and the surrounding mesenchyme. Disturbances of genes in any of the three cell types can lead to the malformation.

Anatomic relationship of the coronary orifice and truncal valve in truncus arteriosus and their surgical implication

350 The Journal of Thoracic and Cardiovascular Surgery • February 2002 A trileaflet configuration is considered essential to maintaining the competence of a chorda-less semilunar valve.1 Bicuspid aortic and pulmonary valves are prone to stenosis, and quadricuspid valves are susceptible to regurgitation. Truncal insufficiency occurs more frequently in quadricuspid than tricuspid valves, but no cases have occurred in bicuspid valves.2 One method of restoring competence in truncal insufficiency is to excise one of the quadricuspid truncal valves (QTV) with its wall.2 To use that supernumerary cusp and its truncal wall, we reviewed the reported truncal valve anatomy in relation to the coronary orifice and conceived the following procedure for reconstruction.

Correction of truncus arteriosus with autologous arterial flap in neonates and small infants

This study describes the results of techniques using the autologous truncal wall and part of the pulmonary artery for correction in anticipation of the growth of the pulmonary tract in patients with truncus arteriosus. Seven consecutive patients with truncus arteriosus were reviewed. The posterior wall of the pulmonary tract was obtained by anastomosing the lower edge of the truncal arteriotomy to the upper corner of the ventriculotomy from the truncus in types I and II. Anterior translocation of the pulmonary artery was performed in a type III. A pericardial patch with or without a monocusp was placed to complete the right ventricular outflow tract. There were two hospital deaths, one of which was unrelated to a cardiac problem. Postoperative right-to-left ventricular peak pressure ratio was less than 0.55. There was one left pulmonary stenosis due to monocusp adherence in the late postoperative period. The sizes of the pulmonary tract at anastomosis were between 107% and 166% of the normal value between 7 months and 3.8 years of follow-up. The use of autologous arterial wall instead of a conduit is recommended for the repair of truncus arteriosus to expect growth of the pulmonary tract.

Truncus arteriosus with interrupted aortic arch: Successful correction using autologous flap

A newborn baby with type II truncus arteriosus and type B interrupted aortic arch was successfully treated by creating a pulmonary tract using autologous flap made from truncal wall without excision of the pulmonary artery and by reconstructing the aortic arch with direct anastomosis. This method provided excellent hemodynamics with wide reconstruction of the pulmonary tract without conduit.

Surgical Treatment for the Type II and III Truncus: Complete Division of the Truncal Root with Primary Repair Using Absorbable Suture

A simplified technique for repair of type II and III truncus arteriosus applicable in neonates is described. The method is particularly useful in those patients in whom the pulmonary artery orifices arise from the posterior wall of the truncal root. Advances in two-dimensional echocardiography have provided an accurate means of determining the exact anatomy preoperatively. At operation, isolation of the pulmonary orifices on a button of posterior truncal wall is accomplished by complete division of the main truncal root. Primary closure of the new ascending aorta is made possible by extensive mobilization of the aorta and pulmonary orifices. Early postoperative follow-up indicates that the use of a new absorbable suture for primary repair of the aortic root allows for normal growth.

Spontaneous Intra-Uterine Amputation

1. One case of spontaneous intra-uterine amputation and one case of spontaneous amputation occurring shortly after birth as a result of slough at a point of constriction have been presented. 2. Both cases presented illustrate associated constricting bands and subsequent osseous overgrowth of the amputation stump. 3. Spontaneous intra-uterine amputation, frequently associated with constricting bands of the other extremities, may be due to focal faulty development and is probably not due to amniotic bands or umbilical-cord encirclement. This type of late congenital limb absence differs from the absence due to limb-bud arrest. It is to be remembered that the limb buds appear on the truncal wall at four weeks after ovulation. The sequence of growth is from proximal to distal. The cartilaginous miniature of the extremity is complete at seven weeks after ovulation. 4. Osseous overgrowth of the stump in the spontaneous intra-uterine amputation is a phenomenon paralleling the overgrowth seen in children after a traumatic amputation. 5. The exact etiology of both types of congenital limb absence is not known. A genetic origin is probable. To date, there have been no reported studies regarding the possible role of chromosomal abnormalities in these entities.