Laparoscopic sleeve gastrectomy in a patient with situs inversus totalis and Kartagener syndrome: an unusual surgical conundrum.

Kartagener syndrome is a rare autosomal recessive condition. Approximately 25% of those with situs inversus totalis suffer the syndrome. With the rising overall number and indications for bariatric surgery, this condition will be increasingly recognised. We present a case of a 25-year-old woman with SIT and Kartagener syndrome who underwent a laparoscopic sleeve gastrectomy. As with all bariatric surgery, a multidisciplinary team approach was important in managing such a case. There were considerable cognitive challenges for the surgical team both preoperatively and during the procedure. The patient tolerated the operation well and was discharged 2 days after the surgery. At 12-months follow-up, the patient had achieved 125% excess weight loss. This case illustrates that an experienced surgeon can safely perform a laparoscopic sleeve gastrectomy on a patient with situs inversus totalis.

Gastric cancer in a situs inversus totalis patient with multiple intestinal and vessel variations related to gastrectomy surgery: A case report and literature review.

RATIONALE: Situs inversus totalis (SIT) is a rare congenital anomaly characterized by complete inversion of the abdominal and thoracic organs, and often involves multiple genetic mutations. The most suitable surgical technique for patients with multiple vessel and organ variations as well as SIT remains unclear. Furthermore, there has been insufficient clinical evidence that demonstrates which surgical techniques achieve the best outcomes. Finally, the standard of care has not yet been determined. We present the case of a 60-year-old man with SIT, who was diagnosed with moderately and poorly differentiated adenocarcinoma at the gastroesophageal junction. We further describe the advantage of using robotic-assisted laparoscopic surgery in patients with this anomaly. PATIENT CONCERNS: A 60-year-old man complained of pain in his upper abdomen for 3 months. Physical examination revealed an apex beat in the right fifth intercostal space, and vascular anomalies were noted on abdominal angiographic computed tomography. DIAGNOSES: Moderately and poorly differentiated adenocarcinoma at the gastroesophageal junction with SIT. INTERVENTIONS: Robot-assisted total gastrectomy with D2 lymph node dissection and hand-sewn Roux-en-Y anastomosis was performed. OUTCOMES: The postoperative course was uneventful, and the patient was discharged on the seventh postoperative day. LESSONS: Robotic surgery for gastric cancer is a safe and feasible alternative to laparoscopic surgery and it can be successfully used to treat gastric cancer in patients with SIT with multiple anatomic variations. As exemplified by our case, SIT might be accompanied by multiple anatomic variations. Detailed preoperative detailed imaging of the blood vessels and gastrointestinal tract is useful in these patients.

Síndrome de Kartagener: reporte de un caso y revisión de la literatura / Kartagener syndrome: case report and literature review

El síndrome de Kartagener es una enfermedad autosómica recesiva poco frecuente (uno de cada 32.000 nacimientos), caracterizada por la tríada de bronquiectasias, sinusitis crónica y situs tnversus. El artículo presenta el caso de un hombre de veinticuatro años de edad con dicha enfermedad, a partir del cual se revisa su fisiopatología, las estrategias diagnósticas y terapéuticas y su pronóstico.

Kartagener syndrome us a rare autosomal recessive disease (one ¿n every 32,000 births), oharactenzed by a triad of bronchiectasis, chroníc sinusitis and situs mversus. We present the case of a 24'veai'old male with this disease and we review the pathophysiology, prognosis as well as the main diagnostic and therapeutic strategies.

Extracardiac Fontan in apicocaval juxtaposition.

Extracardiac total cavopulmonary connection between the inferior vena cava and right pulmonary artery has become the most popular Fontan modification nowadays, yielding good results. However, certain situs anomalies along with systemic venous drainage variations give rise to an issue about the best way for extracardiac conduit routing. Three cases of apicocaval juxtaposition are described. The situs was inversus with the apex and inferior vena cava on the same side (left), whereas the superior vena cava (Glenn) was located on the right in all cases. The literature is revisited and technical options are discussed. Surgery was carried out with excellent short- and midterm results.

Tetralogy of fallot with dextrocardia and situs inversus in a 7-year-old boy.

Tetralogy of Fallot is one of the most frequently diagnosed congenital cardiac abnormalities; however, its association with dextrocardia and situs inversus is reported only rarely. We report a case of these findings in a 7-year-old boy, describe the patient's postoperative course, and review the medical literature relevant to these combined conditions.

Experience with bidirectional cavopulmonary anastomosis and modified Fontan operation in patients with single ventricle and concomitant visceral heterotaxy.

The object of our study was to analyze the results of bidirectional cavopulmonary anastomosis (BCPA) and modified Fontan operations (MFO) in patients with a functionally single ventricle and heterotaxy syndrome and to reveal risk factors for these surgical interventions. During 1983-2010, 681 patients underwent BCPA or MFO. Thirty-nine had heterotaxy syndrome. The median follow-up period after BCPA and MFO was nine and 1.5 years, respectively. Risk factors for lethal outcomes were determined by logistic regression analysis. Hospital mortality after BCPA and MFO was 7.9% and 12.5%, respectively and did not significantly differ from patients without heterotaxy. The most frequent hospital complications were heart failure, pleural effusions, and arrhythmias. Late mortality after BCPA and MFO was 8.7% and did not significantly differ from patients without heterotaxy. Late deaths were caused by congestive heart failure or pulmonary thromboembolism. The main non-lethal complication was arrhythmia. Patients have significantly improved their functional class at follow-up. The independent risk factor for lethal outcomes after BCPA and MFO was preoperative regurgitation at atrioventricular valves (P=0.012). BCPA and MFO in patients with a functionally single ventricle and heterotaxy syndrome allow to significantly improves their quality of life. Preoperative regurgitation at atrioventricular valves worsens surgical results.

Cavopulmonary pathway modification in patients with heterotaxy and newly diagnosed or persistent pulmonary arteriovenous malformations after a modified Fontan operation.

OBJECTIVE: Pulmonary arteriovenous malformations are an important but uncommon complication of cavopulmonary connection, particularly in patients with heterotaxy. Absence of hepatic venous effluent in pulmonary arterial blood seems to be a predisposing factor. Pulmonary arteriovenous malformations are most common after superior cavopulmonary anastomosis, but may develop, progress, or persist in 1 lung after Fontan completion if hepatic venous blood streams completely or primarily to the contralateral lung. METHODS: Among 53 patients with heterotaxy and inferior vena cava interruption who underwent a modified Fontan procedure from 1985 to 2005, 8 had unilateral streaming of hepatic venous flow and clinically significant pulmonary arteriovenous malformations after hepatic venous inclusion and underwent reconfiguration of the cavopulmonary pathway. In all 8 patients, the hepatic vein-pulmonary artery pathway was contralateral to and offset from the pulmonary artery anastomosis of the single or dominant superior vena cava. Pathway reconfiguration included pulmonary arterial stenting (n = 2), revision of the superior vena cava-pulmonary artery connection (n = 1), construction of a branched hepatic vein-pulmonary artery conduit (n = 2), and surgical or transcatheter construction of a direct hepatic vein-azygous vein pathway (n = 5). RESULTS: Hepatic vein-azygous vein connection led to improvement in 4 of 5 patients; other approaches typically did not lead to improvement. CONCLUSIONS: Resolution of hypoxemia after cavopulmonary pathway reconfiguration in patients with unilateral pulmonary arteriovenous malformations and hepatic venous flow-streaming after Fontan completion supports the importance of hepatic venous effluent in the pathogenesis of pulmonary arteriovenous malformations and the practice of cavopulmonary pathway revision in such patients. Completion or reconfiguration of the Fontan circulation with direct hepatic vein-azygous vein connection may provide the most reliable mixing and bilateral distribution of hepatic venous blood in this population of patients.

Functional state of patients with heterotaxy syndrome following the Fontan operation.

BACKGROUND: Children born with heterotaxy syndromes have poorer outcomes compared with children born with comparable cardiac lesions requiring similar surgical palliation. Heterotaxy has been reported as a separate risk factor for mortality and increased morbidity in a series of Fontan operations reported from single centres. Little is known, however, about the functional state of surviving patients with heterotaxy following a Fontan operation. METHODS: In the multicentric cross-sectional study carried out by the Pediatric Heart Network of 546 survivors of the Fontan procedure, the patients, aged from 6 to 18 years, underwent evaluation by echocardiography, exercise testing, electrocardiography, magnetic resonance imaging, and functional health status questionnaires compiled by the patients and their parents. Heterotaxy was identified in 42 patients (8%). Medical and patient characteristics were compared between those with heterotaxy and the remaining 504 patients who did not have heterotaxy. RESULTS: Patients with heterotaxy had their Fontan procedure performed at a later age, with a median of 3.9 years versus 2.8 years (p=0.001) and had volume-unloading surgery performed later, at a median age of 1.4 versus 0.9 years (p=0.008). These patients had significantly different ventricular and atrioventricular valvar morphology, as well as a higher incidence of systemic and pulmonary venous abnormalities. They had a higher incidence of prior surgery to the pulmonary veins, at 21 versus 0.4%. The type of Fontan procedure was different, but no difference was detected in length of stay in hospital, or the number of postoperative complications. Sinus rhythm was less common, at 44 versus 71%, (p=0.002), and history of atrial arrhythmias more common, at 19 versus 8%, (p=0.018) in those with heterotaxy. Echocardiography revealed a greater degree atrioventricular valvar regurgitation, lower indexed stroke volume, and greater Tei index. Exercise performance, levels of brain natriuretic peptide in the serum, and summary and domain scores from health status questionnaires, were not different from those not having heterotaxy. CONCLUSIONS: The study illustrates a profile of characteristics, medical history, functional health state, and markers of ventricular performance in patients with heterotaxy after the Fontan procedure. Despite obvious anatomic differences, and some differences in echocardiography and heart rhythm, there were no important differences in exercise performance or functional health state between these patients and other survivors of the Fontan procedure.

Heterotaxy and complex structural heart defects in a mutant mouse model of primary ciliary dyskinesia.

Primary ciliary dyskinesia (PCD) is a genetically heterogeneous disorder associated with ciliary defects and situs inversus totalis, the complete mirror image reversal of internal organ situs (positioning). A variable incidence of heterotaxy, or irregular organ situs, also has been reported in PCD patients, but it is not known whether this is elicited by the PCD-causing genetic lesion. We studied a mouse model of PCD with a recessive mutation in Dnahc5, a dynein gene commonly mutated in PCD. Analysis of homozygous mutant embryos from 18 litters yielded 25% with normal organ situs, 35% with situs inversus totalis, and 40% with heterotaxy. Embryos with heterotaxy had complex structural heart defects that included discordant atrioventricular and ventricular outflow situs and atrial/pulmonary isomerisms. Variable combinations of a distinct set of cardiovascular anomalies were observed, including superior-inferior ventricles, great artery alignment defects, and interrupted inferior vena cava with azygos continuation. The surprisingly high incidence of heterotaxy led us to evaluate the diagnosis of PCD. PCD was confirmed by EM, which revealed missing outer dynein arms in the respiratory cilia. Ciliary dyskinesia was observed by videomicroscopy. These findings show that Dnahc5 is required for the specification of left-right asymmetry and suggest that the PCD-causing Dnahc5 mutation may also be associated with heterotaxy.

Obstruction of the duodenum by a preduodenal portal vein in situs inversus

Mirror image transposition of abdominal and thoracic viscera is termed situs inversus. Duodenal obstruction in situs inversus is rare. A preduodenal portal vein, though not uncommon in situs inversus, rarely causes duodenal obstruction. Where obstruction by a preduodenal portal vein is diagnosed, a duodeno-duodenostomy is the recommended treatment. A duodenal diaphragm and other more common causes of duodenal obstruction should also be excluded in these patients.

Heterotaxy patients with total anomalous pulmonary venous return: improving surgical results.

BACKGROUND: Survival after repair of total anomalous pulmonary venous return in patients with heterotaxy syndrome has consistently been reported in the literature to be far inferior to the results obtained in nonheterotaxy patients. The outcomes of heterotaxy patients at Texas Children's Hospital undergoing repair of total anomalous pulmonary venous return were examined and compared with those of nonheterotaxy patients. METHODS: Between July 7, 1995, and November 22, 2005, 122 consecutive patients underwent primary repair of total anomalous pulmonary venous return. Characteristics were as follows: mean age, 0.35 +/- 1.8 years; mean weight, 4.3 +/- 2.8 kg; single ventricle, 40 (33%); heterotaxy, 38 (31%); pulmonary atresia, 13 (11%). Concomitant procedures included pulmonary artery banding in 4 (3%), systemic-to-pulmonary shunt in 14 (12%), bidirectional Glenn in 11 (9%), and other in 16 (14%). RESULTS: With a mean follow-up of 2.6 +/- 2.5 years including all patients, overall 30-day and 5-year survival was 93% and 86%, respectively; for heterotaxy patients it was 84% and 79%; and for nonheterotaxy patients it was 96% and 89%. Heterotaxy and nonheterotaxy survival curves were not significantly different (p> 0.05). Overall, there were 12 (9.8%) patients who required reoperation for pulmonary vein stenosis, at a mean of 5.1 +/- 5.5 months postoperatively. Seven (58%) reoperations were in heterotaxy patients. Rate of pulmonary vein reoperation is statistically greater (p < 0.05) for heterotaxy patients than nonheterotaxy patients. At last follow-up, all patients were in New York Heart Association class II or less. Using Cox proportional hazards models, pulmonary atresia and need for systemic-to-pulmonary shunt were risk factors for death (not heterotaxy). Single ventricle, operation after July 2000, and need for systemic-to-pulmonary shunt were risk factors for pulmonary vein reoperation. CONCLUSIONS: At midterm follow-up, heterotaxy patients undergoing total anomalous pulmonary venous return repair have a higher pulmonary vein reoperative rate but a comparable overall survival to nonheterotaxy patients.

Biliary atresia with situs inversus: an experience shared.

Biliary Atresia (BA) is a well-known entity and can present with multiple congenital anomalies. BA is one of the most common conditions in which pediatric liver transplant is performed. Identification of Biliary atresia with situs inversus (SI) has not been documented in Pakistan. We report two such cases. First was an eighty-day-old baby boy, icteric from day of birth. On further evaluation had dextrocardia, SI, gross hydronephrosis (HN) of left kidney and stasis at pelvi ureteric junction (PUJ). Liver biopsy showed biliary cirrhosis secondary to extra hepatic biliary atresia (EHBA). The second baby presented at two months of age. Ultrasound abdomen and hepatobiliary scintigraphy confirmed liver in left hypochondrium (SI) and findings suggestive of BA. Echocardiography confirmed SI with mesocardia. In this paper we have described the association of BA with SI in two patients presenting at the pediatric Gastroenterology, hepatology and nutrition unit.

Reflection of structural abnormality in the axoneme of respiratory cilia in the clinical features of immotile cilia syndrome.

In immotile cilia syndrome (ICS), respiratory symptoms show a wide range of severity. Various types of axonemal abnormalities related to ICS are known, but it is unclear whether the symptom severity is related to the type of abnormality. We sent a questionnaire concerning respiratory symptoms to ICS patients. Another type of the questionnaire regarding the clinical features and axonemal abnormality of ICS was sent to the physicians in charge. Analysis of the results showed that in the 48 patients responding, those lacking outer dynein arms (LO) in cilia were more likely to be associated with situs inversus (SI) than those lacking inner dynein arms (LI). Sputum symptoms were more serious in those associated with SI than those unassociated with SI, as was the case in those associated with LO compared to those with LI. Based on these results, it may be concluded that the type of dynein arm defect in cilia is related to the clinical features in ICS.

Anestesia para colecistectomía laparoscópica en una paciente con situs inversus totalis / Anesthesia for laparoscopic cholecystectomy in a female patient with situs invesus totalis

Se presenta el manejo anestésico de una paciente del sexo femenino con situs inversus totalis, sometida a colecistectomía laparoscópica. El ultrasonido abdominal confirmó el diagnóstico de colecistitis, así como el situs invesus con el hígado y la vesícula biliar en el lado izquierdo y el brazo en el lado derecho. El electrocardiograma y la placa de tórax demostraron dextrocardia. Se administraron Propofol y fentanyl para la inducción de la anestesia, para el mantenimiento se empleó isoflurano y una dosis subsecuente de fentanyl. La relajación muscular se mantuvo con una infusión continua de atracurium. Se uso ventilación controlada con un ventilador de volumen empleando oxígeno al 100 por ciento, el volumen corriente y la frecuencia se ajustaron de acuerdo a las cifras de PETCO2 (Presión de CO2 al final de la espiración), para mantener valores entre 31 y 35 mmHg. La anestesia transcurrió sin complicaciones y la emersión fue satisfactoria. La paciente no presento complicaciones postoperatorias y fue dada de alta al día siguiente. El Situs Inversus Totalis que no se acompaña de otras alteraciones no es contraindicación para procedimientos laparoscópicos

Kartagener's syndrome.

Kartagener's syndrome is characterized by situs inversus, chronic paranasal sinusitis and bronchiectasis. The pathogenesis of this rare condition is an ultrastructural defect that results in immotile cilia. Radiologic features may include findings consistent with bronchiectasis.

Electrophysiologic delineation of the specialized atrioventricular conduction system in two patients with corrected transposition of the great arteries in situs inversus (I,D,D).

Electrophysiologic delineation of the atrioventricular conduction system at surgery is described in two patients with corrected transpostion of the great arteries in situs inversus. Intra-atrial electrograms were recorded in one patient from sites immediately adjacent to the coronary sinus located in the left-sided right atrium. The intraventricular portion of the atrioventricular conduction system was identified in both patients along the posterior and inferior margin of the ventricular septal defect, in contrast to the superior and anterior location found in corrected transposition of the great arteries in situs solitus. In contrast to the superior and anterior location found in corrected transposition of the great arteries in situs solitus. The course of the conduction system in the hearts of these two patients and a possible relationship to the cardiac loop and dual origin of the atrioventricular node is discussed. These cases illustrate the usefulness of segmental diagnosis of congenital heart disease and of electrophysiologic identification of the specialized atrioventricular conduction system at surgery.