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PubMed1983

Congenitally corrected transposition in a patient with situs inversus, atrioventricular discordance and mesocardia (I,D,D).

The clinical, hemodynamic and angiographic features of a patient with situs inversus, atrioventricular discordance, corrected transposition of the great arteries (I,D,D) and mesocardia are presented. A review of the literature concerning corrected transposition of the great arteries in situs inversus is included. The incidence of associated cardiovascular and electrocardiographic anomalies is compared with that found in corrected transposition of the great arteries in situs solitus; special surgical aspects of the above cases are also discussed.

Journal Article↗
PubMed1986

Acute abdomen in a patient with situs inversus. A case report.

The case of a man with situs inversus who presented with acute abdomen is reported. Acute left-sided appendicitis was considered before operation, but at laparotomy an omental abscess of unknown aetiology was drained. The appendix, localized in the left iliac fossa, was removed but was normal on histological examination.

Abdomen, Acute↗
PubMedDate not supplied

Young's syndrome associated with situs inversus totalis.

Primary ciliary dysfunction causes recurrent sino-pulmonary infections and shares the pathophysiology of the reproductive tract. A 34-year old man with bronchectasis and situs inversus totalis was investigated because of azoospermia. Endocrinological evaluations were all normal and scrotal exploration revealed the distended caput, but atrophic body to tail, of the epididymis. Light microscopy of testicular biopsy showed normal spermatogensis and motile sperms were easily obtained by testicular sperm extraction. Electron microscopy demonstrated normal sperm structure, whereas 65% of nasal cilias showed defect of central microtubules. This case, which bears a resemblance of Kartagener's syndrome (situs inversus totalis), is considered a unique variant of Young's syndrome.

Adult↗
PubMed1987

X-linked laterality sequence: situs inversus, complex cardiac defects, splenic defects.

The association of abdominal situs inversus, complex cardiac defects, and alterations in development of the spleen represents a developmental field complex with variable expression of altered laterality. Familial and inherited cases documenting respectively autosomal recessive and dominant inheritance have been reported. We report on the first family in which X-linked recessive inheritance of this defect has been documented.

Child, Preschool↗
PubMed1990

Congenital aglossia and situs inversus.

Lingual agenesis is a rare anomaly caused by failed embryogenesis of the lateral lingual swellings during the fourth and fifth gestational weeks. Most reported cases have been part of oromandibular limb hypogenesis syndromes. A review of the literature reveals two previously reported cases of congenital aglossia and situs inversus. A case of lingual agenesis associated with micrognathia and situs inversus is reported in a newborn presenting with upper airway obstruction at birth.

Humans↗
PubMed1995

Follicular thyroid carcinoma and mediastinal goiter in a case of situs inversus viscerum.

A 55-year-old woman with total situs inversus viscerum was subjected to total thyroidectomy for a well-differentiated follicular thyroid carcinoma. After surgery the patient remained euthyroid for two months and serum TSH did not rise. A whole body scan with iodine-131 (131I) showed mediastinal uptake. A chest computed tomography visualized a sequestered substernal goiter. Upon the patient's refusal to undergo surgery, she was treated with 150 mCi of 131I. A further dose of 100 mCi of 131I was administered six months later. Ten years after treatment, the patient is still alive and disease-free.

Adenocarcinoma, Follicular↗
PubMed1998

[Radical D2,D3 lymph node excision of stomach carcinoma in situs inversus totalis and malrotation of the colon].

The combination of Situs inversus totalis (SIT) with incomplete rotation of the colon is an extremely rare anomaly and especially of interest when presented with advanced gastric cancer. The routine en-bloc resection of gastric cancer with D2,D3 lymphadenectomy according to the recommendations of the Japanese Research Society for Gastric Cancer [5] can, as in this case, present tactical operative problems, due to the mirror-image anatomy and potential vessel anomalies, which might complicate the operative procedure. The preoperative diagnosis of SIT is not only essential to allow an adequate operative approach but furthermore to prevent vessel injury.

Adenocarcinoma↗
PubMed2001

Case report: off-pump total myocardial revascularization for dextrocardia and situs inversus.

A 42-year-old man with dextrocardia and situs inversus underwent successful off-pump total myocardial revascularization using the technique popularized by Tector [Tector 1994, Tector 1996]. The free left internal mammary artery (LIMA) was anastomosed to the in-situ right internal mammary artery (RIMA) at the level of the right-sided left atrial appendage, then anastomosed sequentially to the first diagonal branch (D1) of the left anterior descending artery (LAD), and to the LAD. The in-situ RIMA was sequentially anastomosed to the first and the second obtuse marginal branches (OM1 and OM2) of the circumflex coronary artery. A saphenous vein was then anastomosed to a diffusely diseased posterior descending branch (PDA) of the right coronary artery (RCA). The patient was asymptomatic and in excellent condition three months after surgery. In economically depressed regions of the world, such as the Gaza Strip, off-pump complete revascularization is an excellent alternative to cardiopulmonary bypass.

Adult↗
PubMed1994

Double lung transplantation in situs inversus with Kartagener's syndrome. Paris-Sud University Lung Transplant Group.

En bloc double lung transplantation with bilateral bronchial anastomoses was successfully performed in three patients with complete situs inversus and end-stage Kartagener's syndrome. Dextrocardia was not a technical problem for institution of cardiopulmonary bypass, but a large azygos vein draining the systemic venous return was systematically preserved. The major technical difficulty was restoration of airway continuity, because patients with situs inversus have an inverse direction and length of the main stem bronchi. The right and left main bronchi of the recipients were approached in the aortocaval sinus and transected approximately at 1.5 cm from the carina. The donor right main stem bronchus was divided at its origin and the donor left main stem bronchus was divided proximal to the upper lobe takeoff. The different bronchial angulation was not an obstacle, and airway continuity was reestablished twice with an end-to-end anastomosis and once with a telescopic technique. Because of the midline position of the left atrium and pulmonary artery, the anastomoses with the respective recipient's structures were made as in patients with situs solitus. One patient required a right lower lobectomy because the position of the right side of the heart interfered with lobar expansion. One patient died of obliterative bronchiolitis 36 months after the operation. The remaining two are alive and doing well after 48 and 6 months, respectively.

Adult↗
PubMed2003

Adult onset hydrocephalus and situs inversus: new autosomal dominant syndrome?

Familial adult onset hydrocephalus is unusual and its genetic basis is unknown. We report a two-generation family with three members having hydrocephalus, two of them with complete situs inversus. Laterality syndromes were considered in the differential diagnosis and are unlikely. We suggest that this family represents a previously unrecognised form of autosomal dominant late onset hydrocephalus with situs inversus.

Adolescent↗
PubMed1988

Solitary or predominantly right-sided varicocele: a possible sign of situs inversus.

Among more than 850 patients undergoing outpatient sclerotherapy of the internal spermatic vein, only 1 unilateral and 1 predominantly right-sided varicocele were encountered. In both patients with this finding a total situs inversus was present, with inversion of normal anatomy, enlarged "right" internal spermatic vein draining into the renal vein, and the "left" internal spermatic vein entering the inferior vena cava directly. Total situs inversus should be considered whenever right-sided varicocele is solitary or predominant.

Adolescent↗
PubMed1994

[An experience of successful valve repair for acquired mitral and tricuspid regurgitation in dextrocardia, situs inversus, bilateral vena cava, and hemiazygos continuation].

A 56-year-old woman was admitted to our department because of congestive heart failure. Chest X-ray showed dextrocardia with situs inversus. The echocardiography revealed severe mitral regurgitation due to prolapse of posterior leaflet and secondary Venography demonstrated the bilateral superior vena cava, huge coronary sinus and hemiazygos continuation with hypoplastic inferior vena cava. Mitral valve regurgitation was repaired by Carpentier's and Burr's technique, and tricuspid valve regurgitation was repaired by Kay's technique. Her postoperative course was uneventful. Valve surgery for acquired valvular lesion in dextrocardia with situs inversus is rare. Although valve repair is easily performed even in mirror-image heart as well as in normal, levo-position heart, preoperative evaluation of systemic venous return is important in the case of dextrocardia with systemic venous anomalies.

Azygos Vein↗
PubMedDate not supplied

Familial dextrocardia, divergent strabismus and situs inversus of optic disc.

A family is described in which three of four siblings have major congenital cardiac defects in association with ocular abnormalities. The eldest sibling has isolated dextrocardia and the second has total situs inversus. The fourth child has both atrial and ventricular septal defects, with pulmonary hypertension and right ventricular hypertrophy. The mother and third sibling have normal hearts but exhibit a number of ocular defects. The principal ocular anomalies demonstrated in this family are divergent strabismus, bilateral situs inversus of the optic disc, and myopia. The hereditary aspect of these multiple findings is discussed.

Child↗
PubMed2004

Situs inversus totalis: giant hiatal hernia repair by laparoscopic Collis gastroplasty and Nissen fundoplication.

We report the repair of a giant hiatal hernia by laparoscopic Collis gastroplasty and Nissen fundoplication in a patient with situs inversus totalis, highlighting the unique anatomic challenges in this case. The 52-year old female patient had Kartageners syndrome, a giant hiatal hernia, and a history of chronic severe gastroesophageal reflux disease with uncontrolled regurgitation. The laparoscopic procedure was accomplished with five ports placed in a mirror-image configuration, reversed from our standard positions. After visual confirmation of the complete reversal of the intraabdominal anatomy, we performed a modified Collis gastroplasty and Nissen fundoplication. Significant technical challenges were encountered intraoperatively. To the best of our knowledge, this report is the first of its kind in the literature. The use of advanced laparoscopic techniques is highly adaptable to unusual anatomy. Laparoscopic hiatal hernia surgery is feasible in patients with situs inversus.

Female↗
PubMedDate not supplied

Situs inversus totalis and corrected transposition of the great arteries [I,D,D] in association with a previously unreported vascular ring.

A 3-month-old girl with "noisy breathing" was found to have situs inversus totalis, corrected transposition of the great arteries [I,D,D], and a vascular ring. The ring was composed of a left aortic arch with normal branching pattern and a right ligamentum arteriosum that extended from a diverticulum off the descending aorta and coursed retroesophageal and to the right to join the pulmonary artery. There was no circumflex component of the aorta or aberrant subclavian artery. The descending aorta was left sided. Compression of the esophagus and trachea was noted on contrast esophagram, magnetic resonance imaging (MRI), and at the time of surgery to divide the vascular ring. In association with her corrected transposition, the patient also was shown to have a mild Ebstein's deformity of the right-sided (systemic) atrioventricular valve and electrocardiographic evidence of Wolfe-Parkinson-White syndrome. The combination of situs inversus totalis, corrected transposition of the great arteries [I,D,D], and an aortic arch anomaly has not been previously reported. In addition, the aortic arch anomaly suggested by MRI imaging and confirmed at surgery has previously only been postulated to exist but to our knowledge never reported.

Echocardiography, Doppler, Color↗
PubMed1993

A rare case of isolated atrial discordance with primary atrial situs inversus.

A newborn with isolated atrial discordance complicated by primary atrial situs inversus and dextrocardia is reported. She received a Mustard's corrective procedure with a good clinical outcome. To our knowledge, this is the third report of a patient with isolated atrial discordance and viscero-atrial discordance or ambiguus.

Dextrocardia↗