Helen T. Winer-Muram

Adult Presentation of Heterotaxic Syndromes and Related Complexes

(Definitions) Situs solitus indicates the normal position of the cardiac atria and viscera. Situs solitus with levocardia is the normal situation, with <1% incidence of congenital heart disease (Table 1). Levocardia denotes a left-sided heart and a left cardiac apex; it does not give any indication of cardiac structure or body situs (Fig. 1A). Situs inversus is the mirror-image location of the atria and abdominal viscera (Fig. 1B). Heterotaxia is the abnormal arrangement of organs and major blood vessels different from the orderly arrangement of either situs solitus or situs inversus. In situs ambiguus or heterotaxia, the relationship of the atria and viscera is inconsistent. Isomerism, a form of heterotaxia, is a term used to describe symmetric morphology, i.e., both sides of the viscera, and both lungs are nearly identical to one another (Fig. 2). Right isomerism or asplenia syndrome is characterized by situs ambiguus with bilateral right-sidedness (Fig. 2A). Left isomerism or polysplenia syndrome is a second type of situs ambiguus characterized by bilateral left-sidedness (Fig. 2B). In this review, all patients having situs ambiguus with either right isomerism (asplenia syndrome) or left isomerism (polysplenia syndrome) are regarded as having cardiac malposition, an inappropriate cardiac position.

Computed Tomography Demonstration of Lipomatous Metaplasia of the Left Ventricle Following Myocardial Infarction

Replacement of myocardium by fat, particularly of the right ventricle, is often diagnosed as arrhythmogenic right ventricular dysplasia. At autopsy, however, 68% of scars associated with chronic ischemic heart disease have shown fatty metaplasia in the scar. Four patients with a past history of previous myocardial infarctions and computed tomography demonstration of fatty change in left ventricular regions of hypokinesis and infarction are presented. It is proposed that these findings represent ischemic fatty metaplasia, an alternative etiology of fatty tissue replacing myocardium.

Topical treatment of life threatening haemoptysis from aspergillomas.

BACKGROUND: The efficacy of topical treatment of 12 episodes of severe life threatening haemoptysis from a pulmonary aspergilloma in 11 patients is reviewed. METHODS: A retrospective review was performed on five white and six African-American patients of mean age 49 years. The underlying diseases were bronchiectasis, sarcoidosis, tuberculosis, or histoplasmosis. The patients were prospectively considered poor surgical risks because they had a forced expiratory volume in one second (FEV1) of < 50% predicted and an arterial oxygen tension (PaO2) of < 7.95 kPa breathing room air. As surgical intervention was not possible, local intracavitary instillation of sodium or potassium iodide was performed. Two patients were previously treated with amphotericin B. In one patient sodium iodide alone was used and in the remaining eight potassium iodide alone was instilled. The transcricothyroid approach was used in six patients and the percutaneous approach in five. RESULTS: Haemoptysis ceased within 72 hours in all patients after the instillation of sodium or potassium iodide. There was no morbidity or mortality, and side effects included slight irritation on instillation of the iodide solution and occasional cough which was easily controlled. All patients were alive at least one year later. CONCLUSION: Intracavitary treatment is a viable option in the poor risk patient with life threatening haemoptysis from an aspergilloma.

Thoracic complications of tuberculosis

Tuberculosis is a disease that has plagued humankind for centuries. In spite of excellent antituberculous chemotherapy, a number of important complications of tuberculosis are still commonly seen. Some of these are caused by the disease, and others are the sequelae of therapy. Thus some are seen immediately, and others occur months or even years after the onset of disease. These complications may involve the lungs, mediastinum, pleura, chest wall, or any combination of these structures. This article reviews the pathophysiology, diagnosis, and treatment of the many complications of thoracic tuberculosis.

The concurrence of facioauriculovertebral spectrum and the Rokitansky syndrome

A 20-year-old, black woman presented with primary amenorrhea. The patient was the product of an uncomplicated twin pregnancy. The parents were not consanguineous and were of average height. The proband had one sister and two brothers, all normal and of average stature. The sister reportedly had normal menstruation. The patient was short throughout her childhood. She graduated from high school but repeated the eleventh grade. She was 4 feet, 9 inches tall and weighed 98 pounds. Breasts and pubic hair were Tanner stage 5. Head circumference, arm span, and intercanthal distance were normal. The head and face were asymmetric. The right side of the head and face was smaller than the left, with the head tilted to the right (Fig. 1). The supraorbital ridges were flat, and palpebral fissures slanted upward. Both ears were small; the right ear was malformed and rudimentary. There was a small right auditory meatus, and the tympanic membrane could not be visualized. The thoracic cage was malformed as a result of severe scoliosis (Fig. 2). External genitalia were normal, but the vaginal orifice was absent and the uterus could not be felt on rectal examination. Neurological examination revealed limited extension of elbows and abduction of the arms (cubitus valgus) bilaterally. The hands and feet were normal except for hyperconvex fingernails. Every dermatoglyphic digital pattern was an ulnar loop, and both axial triradii were proximal.

Pulmonary CT Findings in Behçet Disease

A 32-year-old man with Behçet syndrome presented with hemoptysis and abnormal chest radiography. A 3 cm opacity was present in the apical posterior segment of the left upper lobe and the hila were enlarged with lobulated contours. Computed tomography revealed bilateral pulmonary artery aneurysms, a complication of Behçet syndrome. Computed tomography is recommended for the evaluation of patients with Behçet syndrome who have abnormal chest radiography.

Pet-associated lung diseases.

With the development of new diagnostic techniques, several diseases have emerged in recent years as zoonotic or potentially zoonotic. Even so, most pet-associated illnesses are infrequent, and therefore, health care providers may not be familiar with their manifestations or with the appropriate preventive measures. This article reviews the epidemiology, diagnosis, therapy and prevention of lung disorders acquired from dogs and cats.

An Analysis of the Predictability of Hypoxemia Following Vertical Banded Gastroplasty

Postoperative (post-op) hypoxemia is unpredictable, often undetected by physical examination, sometimes fatal. We studied 45 morbidly obese patients with an average age of 37, including 16 smokers, having vertical banded gastroplasty (VBG) for useful preoperative (pre-op) predictor(s) of post-op hypoxemia during the first five days following VBG. Patient blood gases (arterial blood oxygen, PaO2, in mmHg), pre-op and five post-op days (POD), after 30 min in room air were: pre-op, 85 ± 9; POD1, 63 ± 9*; POD2, 61 ± 9*; POD3, 63 ± 10*; POD4, 63 ± 9*; POD5, 64 ± 11*. (*p < 0.05, Students t-test compared with pre-op). Linear regression showed no practical, predictive value for PaO2 for age, Body Mass Index (BMI), pulmonary function tests (PFTs), smokers or preop PaO2. Post-op atelectasis occurred in 84% of patients, mostly the posterior basilar regions on chest X-ray. No patient developed clinically diagnostic pneumonia. VBG patients experienced profound hypoxemia post-op, the lowest on POD2. There is no reliable method to predict which patient may develop severe hypoxemia. It is, therefore, extremely helpful to uniformly monitor PaO2 post-op in morbidly obese patients.

Quality assurance review: Improving diagnostic accuracy of percutaneous needle biopsy

Adequate pathologic slide preparation of percutaneous fine-needle biopsy (PFNB) specimens is necessary to maximize sensitivity and yield a tissue diagnosis. We present a quality control review of our biopsy technique and results in 71 aspiration biopsies done in 59 patients. Immediate cytology slide preparation in the radiology suite enabled us to increase our diagnostic yield from 53% to 84%, and allowed us to preserve PFNB, rather than open biopsy, as the primary diagnostic modality. We recommend regular review of biopsy techniques to confirm adequate diagnostic yields of these procedures.

Uterine Myomas in Pregnancy

: Pregnancy complications occur in many patients who have uterine myomas. Patients in whom the placenta is implanted near a myoma are more likely to be affected. Early pregnancy bleeding, premature rupture of membranes and postpartum hemorrhage are the problems most frequently seen. Sonographic assessment identifies these patients who are at risk and in whom special precautions are advisable.