Pediatric Cullen gangrene: Case report of postoperative pyoderma gangrenosum in a preterm infant with a complex gastrointestinal malformation.

INTRODUCTION: Pyoderma gangrenosum (PG) is a rare condition in infants, children and adolescents. The postoperative variant of this rapidly progressive ulceration is known as Cullen gangrene. Only a few case reports of pediatric Cullen gangrene have been published. PRESENTATION OF CASE: We present a male infant (gestational age 31 + 6 weeks) with gastroschisis and intrauterine volvulus. After multiple operations due to his complex gastrointestinal malformation, the patient showed classical symptoms of postoperative PG (PPG) 4 weeks after birth. Diagnosis could be made early in an interdisciplinary approach, differentiating intraabdominal infection (vancomycin resistant enterococcus (VRE), candida albicans) from epifascial occurrence. Treatment with high-dose steroids and immunoglobulins was initiated immediately and led to rapid recovery. DISCUSSION: The unusual combination of a complex gastrointestinal malformation and an intraabdominal infection with VRE and candida albicans made the diagnosis difficult. However, early diagnosis is crucial in the treatment of Cullen gangrene. CONCLUSION: This is the first case report of Cullen gangrene presenting in an infant (at 4 weeks of age).

From pullout-techniques to modular elastic stable chest repair: the evolution of an open technique in the correction of pectus excavatum.

BACKGROUND: Traditionally open procedures have been replaced by minimally invasive techniques in the correction of pectus excavatum. Efforts to improve the extent of mobilization of the chest wall and its stabilization have led to constant modifications. There is currently no consensus about the best procedure for correction of pectus excavatum. METHODS: Based on the contributions of a single institution for the last 60 years, we present the various strategies used for the correction of pectus excavatum and the evolution of operational procedures. These approaches are compared with those performed internationally at similar periods. RESULTS: Resections with external extension achieved moderate results and were modified in 1962 to the "Shred" method. The establishment of the "Strut" method in 1963 and, in 1977, its extension with the erection of the lower rib arches significantly improved patient outcomes. The "minimization" of the procedure in 2006 was accompanied by an increase in wound healing disorders and recurrent deformities. Since 2010, elastic stable chest repair (ESCR) has provided lossless mobilization and sternal elevation for healing costosternal pseudarthrosis and allowed correction of complex recurrences with excellent cosmetic-functional results. Strong asymmetric or broad-base deformities can now be stabilized using a modular hybrid technique of transsternal bar and locked plates. CONCLUSIONS: ESCR marks the end of the 60-year development of an open procedure and, after loss-free mobilization of the chest wall by elastic-stable biomechanical management, optimizes the possibility of anatomical reconstruction of the chest wall during initial and re-interventions, achieving a permanent, physiologically stable remodeling of the chest wall.

Elastic stable chest repair and its hybrid variants in 86 patients with pectus excavatum.

BACKGROUND: Complex and mature funnel chest deformities are traditionally managed with open surgical procedures. Elastic stable chest repair (ESCR) has been used successfully and safely for relapse corrections. Does pure plate osteosynthesis in ESCR allow comparable corrective potency and implant safety as hybrid methods with metal bars? METHODS: Data from 86 patients with open funnel chest correction between 2011 and 2015 were analyzed in this retrospective study. Exclusion criteria included being under 12 years of age, and having a history of septic wound healing disorder or other malignant diseases. Main groups consisted of ESCR and hybrid techniques, subgroups were primary and recurrence correction. Correction results and follow-up examinations at six and 12 weeks and at 1 year were statistically analyzed. RESULTS: A total of 38 ESCR and 48 hybrid methods were analyzed. Bar implantation was required in 77% (recurrence 34%) of patients. All patients received plates with different combinations e.g., longitudinal-sternal, costosternal and costo-sterno-costal. In all groups, follow-up uptake showed a funnel chest correction result at the anatomical level with healthy values according to the Haller index (ESCR 4.36-2.84, hybrid 6.99-2.74, P<0.001). No material dislocations were observed in any subgroup. CONCLUSIONS: ESCR and hybrid techniques represent promising and safe therapeutic approaches.

Does an external chest wall measurement correlate with a CT-based measurement in patients with chest wall deformities?

BACKGROUND: Measurements in chest wall deformities are typically conducted using a thorax caliper or a CT scan of the chest wall. This paper focuses on the possible correlation between these two methods to validate the reliability of the thorax caliper, minimize radiation exposure, and limit the usage of expensive imaging techniques. METHODS: We evaluated 95 consecutive patients (77 pectus excavatum (PE), 17 pectus carinatum (PC), 1 mixed deformity) who received surgical correction of the anterior chest wall. The results of the external chest wall measurements and the CT-based measurements were statistically compared. RESULTS: A significant correlation between the two measurements was observed in PE and PC at the highest point of the deformation. The strongest correlation was noted in PE. We also noted a correlation between the transverse diameter of the external measurement and the inner thoracic diameter of the CT scan but not for the sagittal diameters in the upper parts of the sternum. CONCLUSIONS: Thorax caliper measurements are suitable for determining the sagittal thoracic diameter at the maximum level of the deformity and the transverse diameter with an accuracy comparable to that of CT measurements. Since these values key, the thorax caliper is reliable for monitoring and documenting chest wall malformations. LEVEL OF EVIDENCE: Study of diagnostic test. Testing previously developed diagnostic criteria in a consecutive series of patients and a universally "gold" standard-Level I.

Symptoms in Pectus Deformities: A Scoring System for Subjective Physical Complaints.

Background The literature is silent on the relationship between symptoms and the Haller index. Nor is there a classification of the severity of the physical complaints. Materials and Methods Retrospectively, data from 128 patients (102 funnel, 25 pigeon chest patients, and 1 mixed type) were evaluated. To objectify the symptoms, we developed a score to describe the level of physical ailments. This score includes 10 different symptoms as well as the situation or frequency in which they occur and an impact factor. This depends on how much they affect everyday life. Results Pectus excavatum patients express physical complaints more frequently than pectus carinatum patients who actually suffer more from psychological stress. We could not find a correlation between the Haller index and symptoms or levels of ailment. Conclusion Pectus deformities are likely to cause physical and psychological complaints. Since the subjective symptoms did not show any correlation to the chest severity index, they are supposed to be independent from the deformity's extent.

Elastic Stable Chest Repair as a Means of Stabilizing the Anterior Chest Wall in Recurrent Pectus Excavatum with Sternocostal Pseudarthrosis: An Innovative Fixation Device.

INTRODUCTION: Open surgical procedures in the treatment of pectus excavatum (PE) involve predetermined incisions in the parasternal cartilage and the bony ribs. For some procedures, the ribs are even dissected from the sternum for better sternal mobilization and thus better elevation of the funnel. Secure restoration of the sternocostal junction is then required, with the consequence that healing may be quite impaired. Patients may also subsequently suffer from sternocostal nonunion, for example, pseudarthrosis, and dislocated ribs, as well as pain and a recurrence of PE. MATERIALS AND METHODS: Patients underwent another open surgery with revision of the pseudarthrotic sternocostal junctions and sufficient mobilization of the anterior chest wall, followed by an open reduction and internal fixation using Matrix Rib titanium plates (Synthes, Oberdorf, Switzerland). This procedure consisted of elevating the anterior chest wall and fixing the ribs to the sternum. In 2011 and 2012, we studied this procedure, known as elastic stable chest repair (ESCR), in a series of 20 patients. The patients underwent clinical and ultrasound examinations and X-ray radiographs after the operation, after 6 weeks, and at 3- and 12-month intervals. RESULTS: Follow-up showed high patient tolerance, although a loose plate was observed in one patient and a broken plate in three patients. A stable union was achieved for all sternocostal pseudarthroses. PE improved highly significantly (p < 0.001), as the Haller index decreased from 3.6 (range: 2.7-6.6, standard deviation [SD]: 0.92) to 2.7 (range: 2.0-3.7, SD: 0.42). Pain in the anterior chest wall was significantly reduced after the operation in the majority of cases. All but one patient was mobilized already the day after the operation. CONCLUSIONS: ESCR in recurrent PE achieved functional stabilization of the anterior chest wall combined with satisfactory results.

Sternocostal dislocation following open correction of pectus excavatum-"stairway phenomenon": complication management by means of sternocostal locking titanium plate osteosynthesis.

INTRODUCTION: Some open surgical methods describe complete sternocostal dissection with subsequent resynthesis. Lack of consolidation with painful sternocostal instability and retrosternal dislocation of sternal rib tips are possible complications. MATERIALS AND METHODS: Seven patients with symptomatic unilateral sternocostal dislocation were included in this study. After diagnosis, confirmed by three-dimensional computed tomography (CT), patients underwent open surgery. All affected ribs were reset and fixed to the sternum with the aid of titanium implants. RESULTS: The patients had, on average, unilateral dislocation of 3.4 rib joints with 15.4 mm retrosternal dislocation, which was fixed with 2.3 plates. A titanium splint was also employed. The recurrent nature of the problem made procedures very time-consuming (average operation time: 3 hours 25 minutes). One patient suffered strong intraoperative bleeding requiring transfusion of blood products and access enlargement. Follow-up examinations showed high patient satisfaction (grade of 1.7; rating scale 1-6). Remaining rib instabilities were observed just as infrequently as were material failures. The sternocostal rib splint in the costal cartilage became loose and was removed. One patient exhibited a pectoral muscle asymmetry. No other complications were observed. CONCLUSION: The term "stairway phenomenon" describes the dislocation of sternocostal joints. Observed after open pectus excavatum correction it can trigger substantial physical complaints. Thus, preserving those joints during pectus repair is strongly recommended. Locking titanium plates are a safe alternative to sternocostal suture fixation and is characterized by high patient satisfaction.