Amulya K. Saxena

Chest-deformities: a proposal for a classification

BackgroundIn this article we assess the significance of classifying chest-deformities based on morphological findings in type-related treatment and its results.Data sourcesRecent publications on chest-deformities in children and youth were retrieved from PubMed and Medline and from our clinical and intraoperative findings.ResultsChest-deformities are diagnosed by thoraxmeasurements using a flexible meter projected on a graph-paper by MR/CT investigations and color coded videorasterstereography. In addition an ultrasound guided mediastinal analysis is performed on the heart, the great vessels and mediastinal organs. These investigations could determine meticulously the morphology of the sternum, the sterno-costal segments and the costal arch, enabling to find different chest wall deformities, i.e., 11 different types. The clinical and surgical significance of such a classification can be shown by comparing postoperative results of non-classified chest-deformities with those of classified. Preoperatively non-classified chest-deformities often have postoperative asymmetric shapes, partial local recurrences, costal arch eversions and a platythorax. Such a classification can be used to analyze and predict socalled “secondary associated alterations” of the vertebral column or mediastinal organs.ConclusionsDetermining the specific type of a thorax deformity could be considered a type-related physiotherapy as conservative treatment or vacuum treatment and if surgery is indicated a type-related surgical correction can be performed. A type-related and adapted surgical correction can prevent subsequent mitral valve prolapse, recurrent infections, vertebral disturbances caused by kypho-scoliosis and increasing psychological irritation. Typing chest-deformities are an additional and essential help for the surgeon to perform specific surgical procedures: detorsion of the sternum, correction of the sterno-costal region, the costal arch bow and the kind of chest wall immobilization by metal struts. It can also compare the postoperative results more accurately in similar types of chest-deformities.

Pigeon chest: comparative analysis of surgical techniques in minimal access repair of pectus carinatum (MARPC)

BackgroundAfter minimally invasive repair for pectus excavatum (MIRPE), similar procedures for pectus carinatum were developed. This study aimed to analyse the various published techniques of minimal access repair for pectus carinatum (MARPC) and compare the outcomes.Data sourcesLiterature was reviewed on PubMed with the terms “pectus carinatum”, “minimal access repair”, “thoracoscopy” and “children”.ResultsTwelve MARPC techniques that included 13 articles and 140 patients with mean age 15.46xa0years met the inclusion criteria. Success rate of corrections was nxa0=xa0125,xa0aboutxa089% in cumulative reports, with seven articles reporting 100%. The complication rate was 39.28%. Since the pectus bar is placed over the sternum and has a large contact area, skin irritation was the most frequent morbidity (nxa0=xa020, 14.28%). However, within the complication group (nxa0=xa055), wire breakage (nxa0=xa021, 38.18%) and bar displacement (nxa0=xa010, 18.18%) were the most frequent complications. Twenty-two (15.71%) patients required a second procedure. Recurrences have been reported in four of twelve techniques. There were no lethal outcomes.ConclusionsMARPC techniques are not standardized, as MIRPE are, so comparative analysis is difficult as the only common denominator is minimal access. Surgical morbidity is high in MARPC and affectsxa0>xa02/3rd patients withxa0aboutxa015% requiring surgery for complication management.

Minimally invasive resection of adrenal masses in infants and children: results of a European multi-center survey

BackgroundMinimal access adrenal surgery (MAAS) for adrenal pathologies is the standard for many pediatric surgical centers. However, the literature offers few reports and minimal evidence from small case series. The aim of this study was to evaluate the outcomes of pediatric MAAS through a multi-center data analysis.MethodPediatric patients who underwent MAAS between January 2002 and December 2013 were retrospectively included. Data analysis was conducted using Spss software (Welchs t-test, X-square, Fisher tests, multiple regression model).ResultsSix European centers participated, 68 patients were included with mean age of 5.2xa0years (2 months–16 years). Lesion volume was of 18.1xa0cc (0.78–145.6), with a mean diameter of 2.8xa0cm (1.1–6.5). Localization was 50% left-sided masses, 45.6% right-side masses, and 4.4% bilateral. Histological examination revealed 36 neuroblastomas, 15 adenomas, nine pheochromocytomas, three ganglioneuromas, two ganglioneuroblastomas, one bilateral hyperplasia, one adrenocortical carcinoma, an alveolar sarcoma, and a calcification. Surgical access was transperitoneal in 63 (92.6%) and retroperitoneal in 5 (7.4%). Mean operative time was 170u2009±u200987xa0min (285u2009±u200930xa0min for bilateral lesions). Mean hospital stay was 4.2u2009±u20092.5xa0days. Complications included blood loss requiring transfusion in five patients (7.4%) and a diaphragmatic tear. Infiltration of surrounding structures correlated with intraoperative complication rate (pu2009=u20090.027) and operative time (pu2009<u20090.01). No mass rupture, conversion, or post-operative complications were observed. Median follow-up was 52xa0months (1–161). Two recurrences occurred in patients with pheochromocytoma. Age, weight, symptoms, characteristics at imaging, chemistry, volume, or histology, did not influence operative time, hospital stays, or complication rate.ConclusionsPediatric MAAS was safe adopted for masses up to 145.6xa0cc, with a very low rate of complication. Conversion to open is not necessary even in the presence of infiltrations. MAAS should represent the first-line treatment for selected cases in centers experienced in laparoscopy.

A systematic review of the management and outcomes of cecal and appendiceal volvulus in children

Appendiceal volvulus (AV) and cecal volvulus (CV) are rare conditions and there is no consensus regarding the best surgical approach. This study reviewed CV and AV management and outcomes in children.

Instruments for Correction of Chest Wall Deformities

With the introduction of the minimal access surgical approach in 1998, this method gained popularity in the treatment of pectus excavtum deformities, with a global acceptance of this method. The instruments for performing the minimal access repair of pectus excavatum have thus been standardized with a few modifications that differentiate the products between the major suppliers. On the other hand, the large series of repairs of CWDs using the open Willital’s technique and its refinement to the less invasive PLIER technique, (Pectus Less Invasive Extrapleural Repair) which is well suited for severely asymmetric forms of pectus deformities, combined forms of pectus excavtum-carinatum type of deformities, have utilized a standardized metal strut in over 2500 patients. This chapter will hence cover the instruments for minimal access repair of pectus deformities, the metal struts used for the Willital and PLIER technique as well as two other instrument systems for the stabilization of chest wall- the Strasbourg Thoracic Osteosyntheses System and titanium implants for ribs repairs.

The Willital-Hegemann Procedure

Willital-Hegemann is an open procedure to repair Chest Wall Deformities. It is performed using a midline chest incision, following which the costal cartilages are excised bilaterally to free the sternum. The sternum and the flail chest wall segments are stabilized by 3 metal struts are to support the reconstructed chest wall. In patients with severe asymmetry as well as those presenting with complex forms, a 4th strut could be employed. Corrections have been performed on over 1262 patients with severe asymmetric forms of pectus excavatum and pectus carinatum or combined pectus excavatum-carinatum deformities using this technique with excellent result. Excellent aesthetic results and low rates of recurrences after the procedure indicate the need to offer this method of surgical correction patients with severe asymmetric and complex chest wall deformities.

Pros and Cons of the Nuss-Procedure

Successful pectus repairs with conventional open surgical techniques were offered at some Centers throughout the world that were able to further develop these techniques and expertise due to high volume of patients being treated. Centers will low volumes of patients struggled to replicate these results, with the consequence that either these procedures were completely abandoned from their surgical armament or indications were not given to operate these patients. Hence, during the 1980’s and 1990’s Centers that were presenting data on larger series of pectus repairs were often criticized for their indications for surgery rather than the good results obtained after correction. With this scenario, there was lack of enthusiasm in low volume Centers and the majority of global Centers to develop their pectus services and justify reasons for surgical indications.

Poland’s Syndrome

Poland’s syndrome is a rare congenital disorder characterized by unilateral chest wall hypoplasia, ipsilateral hand abnormalities, and a variety of associated anomalies. When considering and planning the surgical options, the extent of the chest wall deformity and the degree of muscular involvement must be carefully taken into account based on a case by case basis. Surgical corrections could be offered as a single or two stage procedures. Reconstruction or stabilization of the hypoplastic ribs can be achieved by open repair or minimal access surgical repairs, however in case of aplastic ribs techniques using bone grafts or a prosthetic mesh patch are advocated. Muscle flaps, breast prosthesis and lipomodeling are possible options to offer reconstructive for muscle deficiency and breast hypoplasia and to accomplish an acceptable esthetic result. Due to the established association between Poland’s syndrome and tumors patients with Poland’s syndrome should be screened and not be lost to follow-up as they make the transition between medical practitioners from the pediatric age to the adult.

Pleural and Pericardial Associations After Minimal Access Pectus Repair

Pleural effusions are among the most common complications seen after minimal access repair of pectus excavatum with surgical intervention required in a large proportion of them. Besides discussing the etiology of pleural effusions, this chapter reviews the literature regarding incidences and required interventions. Additional to pleural morbidity, rare conditions as pericardial effusions and the postpericardiotomy syndrome have been described.

Minimal Access Repair of Pectus Excavatum

Pectus excavatum occurs in an estimated 1 in 300–500 births, with a male predominance of 3:1. Worsening of the chest’s form and the onset of symptoms are usually reported during rapid bone growth seen in puberty and early adolescence. This topic focuses on the operative technique known as the minimally access repair of pectus excavatum (MARPE). Because of the early excellent results obtained with this technique and because of the less radical nature of the open surgical procedures, the popularity of this approach has grown. This chapter deals with the technical and operatives steps of performing a MARPE. Technical modifications in MARPE have also been highlighted and discussed.