Management of intrathoracic and cervical anastomotic leakage after esophagectomy for esophageal cancer: a systematic review.

Background: Anastomotic leakage (0-30%) after esophagectomy is a severe complication and is associated with considerable morbidity and mortality. The aim of this study was to determine which treatment for anastomotic leakage after esophagectomy have the best clinical outcome, based on the currently available literature. Methods: A systematic literature search was performed in Medline, Embase, and Web of Science until April 2017. All studies reporting on the specific treatment of cervical or intrathoracic anastomotic leakage following esophagectomy with gastric tube reconstruction for esophageal or cardia cancer were included. The primary outcome parameter was postoperative mortality. Methodological quality was assessed by the Newcastle-Ottawa Quality Assessment Scale. Results: Nineteen retrospective cohort studies including 273 patients were identified. Methodological quality of all studies was poor to moderate. Mortality rates of intrathoracic anastomotic leakages in the treatment groups were as follows: conservative (14%), endoscopic stent (8%), endoscopic drainage (8%), endoscopic vacuum-assisted closure system (0%), and surgery treatment group (50%). Mortality rates of cervical anastomotic leakages in the treatment groups were as follows: conservative (8%), endoscopic stent (29%), and endoscopic dilatation (0%). Discussion: Due to small cohorts, heterogeneity between studies, and lack of data regarding leakage characteristics, no evidence supporting a specific treatment for anastomotic leakage after esophagectomy was found. A severity score based on leakage characteristics instead of treatment given is essential for determining the optimal treatment of anastomotic leakage. In the absence of robust evidence-based treatment guidelines, we suggest customized treatment depending on sequelae of the leak and clinical condition of the patient. PrDepartment of Surgery, Radboudumc, P.O.B. 9101/618 NLactical advices are provided. Trial registration: Registration number PROSPERO: CRD42016032374.

Successful combined surgical approach in a rare case of retrotracheal goitre in a patient with anatomical impediments.

Diving goitres can descend the cervical region expanding directly into the thoracic cavity. In most cases, diving goitres extend into the anterosuperior compartment, but they may also extend behind the trachea. We herein present a case of a male patient with retrotracheal goitre and history of left thyroid lobectomy and median sternotomy for thoracic aortic aneurysm repair with graft placement. After detailed preoperative evaluation, the patient underwent surgical resection of the mass through a combined approach; the existing cervical incision and a right posterolateral mini-thoracotomy. The postoperative course of the patient was uncomplicated. One year after surgery, the patient is asymptomatic and disease-free. (Folia Morphol 2018; 77, 1: 166-169).

Right thoracic ectopic kidney in a child with breathing difficulties--sonographic diagnosis.

Congenital intrathoracic ectopic kidney is a rare congenital abnormality that is usually found as an incidental lesion on chest radiographs. We report the case of a 6-month-old male with a 1-day history of breathing difficulties whose chest radiograph revealed a soft tissue right basilar mass. Further investigation utilizing ultrasound revealed a thoracic kidney. This case demonstrates the importance of sonography as a diagnostic tool in identifying thoracic kidneys in the pediatric age group.

Update on congenital spinal deformities: preoperative evaluation.

STUDY DESIGN: Review. OBJECTIVE: To review and outline the preoperative evaluation and approach in assessing children with congenital vertebral malformation. SUMMARY OF BACKGROUND DATA: Congenital vertebral malformations encompass a broad spectrum of conditions. A high association of renal, cardiac, and intraspinal anomalies with congenital vertebral malformation has been well documented in the literature. Vertebral malformation with involvement of the thoracic cage may lead to the development of thoracic insufficiency. The natural history, the character, and location of the deformity ultimately influence the propensity for progression and the necessity for treatment. Multiple factors should be considered before treatment with the goal of treatment aimed at providing the best possible care to be able to optimize the child's overall function and potential for growth. METHODS: Narrative and review of literature. CONCLUSION: Congenital scoliosis is a multifaceted condition. The presentation of the condition can be quite varied from those presenting with an isolated hemivertebrae to those with severe malformations, complicated by multiple medical conditions. A thorough preoperative evaluation is necessary before the institution of any treatment protocol. The presence of any medical condition must be addressed; the treatment should be tailor-made for each patient putting into consideration the patients' age and the effects of treatment on pulmonary function at maturity.

Spine deformities in rare congenital syndromes: clinical issues.

STUDY DESIGN: A focused review of the literature with regard to the important system abnormalities of patients with spinal deformities associated with exotic congenital syndromes with additional data from the author's own experience in assessment of patients with rare syndromes treated for thoracic insufficiency syndrome. OBJECTIVES: The objectives of this study are to emphasize important medical considerations that influence the choice of surgical treatment of spinal deformity in patients with exotic congenital syndromes and point out preoperative strategies that reduce treatment morbidity and mortality of these patients. SUMMARY OF BACKGROUND DATA: Individual experience is limited in the treatment of spine abnormality in rare exotic syndromes and the medical aspects of these syndromes that may impact spinal treatment are seldom discussed in detail in the orthopedic literature. For a successful outcome in the treatment of spinal deformity in these unique patients, a working knowledge of the unique pitfalls in their medical care is necessary in order to avoid morbidity and mortality during their treatment. METHODS: The literature was reviewed for 6 exotic congenital syndromes with known or unreported spinal abnormalities and the author's personal 22-years experience of the treatment of thoracic insufficiency syndrome in the relevant congenital syndromes was summarized. RESULTS: Children with Marfan syndrome and spinal deformity may have serious cardiac abnormalities. Spontaneous dissection of the aortic root is a clear danger and patients should be monitored by serial echocardiograms. Prophylactic cardiac surgery may be necessary before spinal surgery is to be performed. Patients with Jeune syndrome have a high rate of proximal cervical stenosis and should undergo screening with cervical spine films at birth. Significant stenosis or instability may require decompression and cervical-occipital fusion. Arthrogryposis may be associated with a severe scoliosis and jaw contracture may make intubation difficult. Larsen syndrome may have early onset scoliosis that is very rigid and requires early intervention. Cervical kyphosis and subluxation may be lethal in these patients and screening radiographs are important. Upper airway abnormalities are an anesthesia concern. Jarcho-Levin syndrome is a thoracic volume depletion deformity due to shortness of the thorax, either a spondylocostal dysostosis variant or spondylothoracic dysplasia. The former has a chaotic congenital scoliosis with varied combination of missing and fused ribs. Although spondylocostal dysostosis has a benign reputation in the literature for respiratory complications, respiratory insufficiency is nevertheless common and 1 death is known from respiratory failure. Spondylothoracic dysplasia seldom has significant scoliosis, but has a mortality rate approaching 50% from respiratory complications due to thoracic insufficiency syndrome. In spite of severe restrictive respiratory disease, adult survivors of spondylothoracic dysplasia appear to do well clinically for unknown reasons. Cerebrocostomandibular syndrome has scoliosis, micrognathia, and thoracic insufficiency syndrome, due to an "implosion" deformity of the thorax from congenital pseudarthrosis of the posterior ribs. CONCLUSION: For optimal patient care, it is necessary to have a clear understanding of exotic congenital syndromes and how they may impact on both the presentation of spinal deformity and the response to treatment, as well as how they may introduce additional morbidity into standard treatment plans. It is clear that with this understanding that preoperative strategies can be employed to enhance the safety of spinal treatment for these unique children.

Factors of thoracic cage deformity that affect pulmonary function in adolescent idiopathic thoracic scoliosis.

STUDY DESIGN: This clinical study examined the association between pulmonary function and thoracic cage deformities in scoliosis. OBJECTIVE: To determine the factors in spinal and thoracic cage deformities that affect pulmonary function in scoliosis. SUMMARY OF BACKGROUND DATA: Pulmonary function in scoliosis has generally been evaluated in terms of lateral spinal curvature. No previous report has evaluated changes in pulmonary function taking into consideration measurements reflecting not only spinal curvature but also thoracic cage deformities, although scoliosis is a three-dimensional deformity. METHODS: A total of 109 patients (mean age, 14.2 years) with adolescent idiopathic right thoracic scoliosis (mean lateral spinal curvature, 37.7 degrees) had full assessment of pulmonary function and a radiographic evaluation from radiographs of the whole spine, Moiré topography, and thoracic computed tomography. RESULTS: Multiple regression analysis (stepwise method) was performed at each vertebral level from T3-T12 to identify the factor that most strongly affects %VC. The correlation coefficient was highest at T9 and next highest at T8, with values of 0.641 (r2 = 0.411, P < 0.0001) and 0.625 (r2 = 0.390, P < 0.0001), respectively. At T9, multiple regression analysis showed that the sagittal diameter of the thoracic cage and the total lung area were identified as factors that most strongly affect %VC. Similarly, the sagittal diameter of the thoracic cage and the rotation angle to the sagittal plane were identified at T8. CONCLUSIONS: The factors that reduced %VC were the sagittal diameter of the thoracic cage, total lung area and vertebral rotation at the T8 and T9 levels.

Pulmonary function assessment in an infant with Barnes syndrome: proactive evaluation for surgical intervention.

Our aim for this study was to report pulmonary mechanics in a neonate with a severe case of Barnes syndrome, a rare form of thoracolaryngopelvic dysplasia, and to use these data to guide ventilatory support and serve as a presurgical screening tool. A comprehensive pulmonary function evaluation was performed on a 36-day-old patient with Barnes syndrome who was being mechanically ventilated because of severe pulmonary distress secondary to thoracic dystrophy. The measurements consisted of respiratory volumes including functional residual capacity, ventilatory mechanics including compliance and resistance, and thoracoabdominal synchrony. Chest wall compliance was 64% below normal, and the thoracoabdominal motion was indicative of predominantly abdominal displacement during inspiratory breaths. The lungs were functioning at a low functional residual capacity, resulting in low lung compliance and increased pulmonary resistance. As a result of the evaluation, the patient was recommended for lateral thoracic expansion surgery and the ventilatory management was adjusted to focus on end-distending pressure support.

Dolor en el pecho: estrategia diagnóstica / Chest pain: diagnostic strategy

En este artículo se expone la estrategia diagnóstica a seguir ante un paciente con dolor en el pecho. Se hace énfasis en el diagnóstico semiográfico, anatómico y etiológico de este síntoma. En particular se expone la semiografía distintiva de los diferentes prototipos anátomo-clínicos de dolor en el pecho y el enfoque diagnóstico para resolver casos con dolor en el pecho de origen indeterminado. Por último, se hacen consideraciones éticas relacionadas con el manejo diagnóstico de este síntoma

New autosomal recessive syndrome with short stature and facio-auriculo-thoracic malformations.

Two sibs, a boy and a girl, from a Lebanese consanguineous family presented with short stature, microcephaly, ptosis, small, dysplastic, low set ears, short neck, and pectum excavatum and carinatum. In addition, the boy had a high arched palate, a cardiac malformation, and at the X-rays an absence of fusion of the posterior hemi-arches of C7 and a fusion between L5 and S1 with a sagittal-cleft vertebral body of L5, while his sister had a cleft lip/palate and at the X-rays an abnormal odontoid peg and a malformation of the articular facets between C1 and C2, and bilateral cervical ribs. Other laboratory and radiological investigations were normal. Sequencing of PTPN11 exons 2, 3, 4, 7, 8, 12, and 13 did not reveal any variations. Two other sibs presented almost the same dysmorphic features; one girl died at age 6(1/4) years after an acute episode of renal insufficiency, and one boy died at 40 days of age. Differential diagnosis is discussed and the possibility of the report of a new autosomal recessive syndrome with variable expressivity is raised.