Tubulo-Interstitial Nephritis and Uveitis - TINU Syndrome

Paz Yitzhaki

Dept. of Medicine A, Rambam Medical Center, Haifa

Acute tubulo-interstitial nephritis and uveitis (TINU syndrome) in a 53-year-old woman is reported. This rare syndrome was described 27 years ago by Dobrin et al. Since then about 50 cases have been described. The syndrome can appear at any age but most patients are under 20 years; about 75% are females. Clinical characteristics include fatigue, general malaise, weight loss, fever, night sweats, anorexia, nausea and vomiting, pallor, nocturia, polyuria, arthralgia and skin rash. Ocular involvement usually includes anterior uveitis but is sometimes posterior; in most cases the uveitis is bilateral.

The characteristic laboratory findings are anemia, rapid sedimentation rate, decreased glomerular filtration rate with increased serum creatinine and urea. Total protein is increased because of polyclonal gammopathy and elevated b2-microglobulin. Urinalysis characteristically reveals proteinuria and b2-microglobulinuria. The histopathologic features on renal biopsy are characteristic of tubulo-interstitial nephritis. Uveitis can precede, accompany or follow onset of the nephropathy.

The pathogenesis and etiology of the syndrome are as yet unknown. Treatment consists of large doses of corticosteroids, but the necessity for treatment is unclear, since there is evidence of spontaneous improvement. Although the prognosis of the nephropathy is favorable and most cases are reversible, the uveitis tends to recur.

Presurgical Neuropsychological assessment in Epilepsy: The Wada Test

Fani Andelman, Miri Neufeld, Irith Reider-Groswasser, Uri Kremer, Yoram Segev, Itzhak Fried

Functional Neurosurgery and EEG and Epilepsy Units, Section of Neuroradiology; and Pediatric Neurology Unit; Tel Aviv-Sourasky Medical Center, Tel Aviv

37 candidates for epilepsy surgery underwent the intra-carotid amytal procedure (IAP; also known as the Wada test) to determine hemispheric speech dominance and memory capacity. 31 demonstrated left hemisphere speech dominance, 2 showed evidence for bilateral language and 4 demonstrated right hemispheric language dominance.

Our study supports a correlational relationship between handedness, lesion laterality and age of onset of seizures, as reported in earlier studies. Left-handed patients with a left hemisphere lesion whose seizures began to an early age had a strong tendency for reversed language dominance.

Asymmetry of at least 20% in performing the memory test was taken as the cutoff score for demonstrating laterality of lesions. The asymmetry score correctly predicted laterality of lesions in all 28 patients; 6 did not have asymmetry scores and 3 were examined for language only. None of the patients who successfully passed the Wada memory test had any significant postsurgical memory deficits; 1 had transient reduction in verbal memory and 4 who did not pass the test were not operated on for this reason.

Our results demonstrate the importance of the Wada test in determining cerebral speech dominance, in predicting post-surgical amnesia, and support its usefulness in predicting laterality of seizure focus in candidates for temporal lobectomy.

Management of Compound High Energy Injuries of the Limbs 

A. Lerner, I. Weisz, G. Nierenberg, D. Horer, H. Stein

Dept. of Orthopedic Surgery A, Rambam Medical Center, and Technion Medical Faculty, Haifa

We treated 49 patients with high energy injuries of the limbs between 1.1.94-31.12.97. They had sustained 61 fractures, 50 of which were open; 14 had bilateral injuries, and 3 had lost a limb. All fractures were stabilized on arrival with an A-O tubular external fixator. Soft tissue loss was covered by a combination of split skin graft and tissue flaps following repeated surgical debridement.

After 10 days, or when all soft tissue defects were covered, the cantilever external fixation frame was exchanged for a hybrid ring which provided three-dimensional stability and allowed early full weight bearing and joint mobilization. The hybrid ring frame did not interfere with the care of soft tissue injuries. Furthermore, it kept the risk of developing deep infections to a bare minimum. At follow-up after a median of 20 months, 1 patient had developed osteomyelitis but all had returned to independent function.

Laser Treatment of Airway Obstruction in Infants and Children

Y. Efrati, S.M. Sarfaty, S. Kromholz, G. Eshel, M. Weinberg, I. Vinograd

Depts. of Pediatric Surgery, Otolaryngology, Anesthesia and Pediatric Intensive Care, Assaf Harofeh Medical Center, Zerifin (Affiliated with Sackler Faculty of Medicine, Tel Aviv University)

Airway obstruction during infancy and childhood requiring surgical ablation is rare, and surgical intervention poses a significant challenge. During recent decades, appropriate endoscopic instrumentation, together with advanced laser beam technology have provided new operative modalities for such patients.

From 1993 to 1995 we treated 40 infants and children, 26 males and 14 females, 13 days to 11 years old (mean 3.3 years) with Nd-YAG or CO² laser. Obstructing lesions included granulation tissue or polyps (16 cases), septa or webs (27), or benign tumors (4). 7 had more than a single lesion.

All were treated endoscopically under general anesthesia without any operative or postoperative deaths. Surgical intervention removed the obstruction and related symptoms in 34. In 6, laser treatment failed, necessitating additional surgical procedures. 3 had circumferential subglottic web. Operative complications included bleeding during removal of a hemangioma in 1 and recrudescence in another. Postoperative complications were transient respiratory failure and pneumonia in 6, all of which resolved with appropriate treatment.

This series proves that laser technology is feasible in the treatment of airway obstruction during infancy and childhood, and is safe and effective.

Sport-Related Maxillofacial Fractures

Yitzhak Shoshani, Navot Givol, Shlomo Taicher

Dept. of Oral and Maxillofacial Surgery, Sheba Medical Center, Tel Hashomer and Faculty of Dental Medicine, Tel Aviv University

The records of 537 patients with 750 maxillofaciafractures were reviewed and analyzed. 55 (10.2%) had sport-related injuries and the rest were due to othercauses. The sport-related group was predominantly male (ratio 9:1) with a mean age of 24.5 years. The mandible was most commonly injured (52.5%), followed by the zygomatic complex (32.8%). The incidence of complicated mid-facial fractures was only 2.8%, and of comminuted fractures 9%. This distribution of injuries is most likely due to the relatively low-energy of trauma associated with many sport activities. The relatively low mean age of the patients, most of whom had full dentition, allowed for conservative treatment of most of the mandibular fractures, using closed reduction.

Injuries were caused by 11 different sports. The highest incidence was soccer (45%), followed by skating (15%), basketball (9%) and horse-riding (9%). Contact sports were the cause of injuries in 72.2%, with the highest incidence of trauma due to impact with another player (60%). Better protection of the oral and maxillofacial region is needed, especially during high-contact sport.

Atypical Mycobacterial Cervical Lymphadenitis in Children

B. Klin, I. Boldur, J. Sandbank, Z. Schpirer, I. Vinograd

Depts. of Pediatric Surgery, Microbiology and Pathology, Assaf Harofeh Medical Center, Zerifin, and Sackler Faculty of Medicine, Tel Aviv University

Scrofula (mycobacterial cervical lymphadenitis) has been well-known for thousands of years. Atypical mycobacteria were first categorized by Timpe and Runyon in 1954. Treatment has varied over the centuries, from exclusion therapy in ancient Greece, through digitalis, iodide, chemotherapy, and surgical excision. The varied differential diagnosis and consequent diagnostic and therapeutic challenges make reassessment of this almost forgotten disease necessary.

21 patients with typical mycobacterial cervical lymphaden„itis seen in over the past 5 years were reviewed. Age distribution ranged from 1-14 years, with peak incidence at 4 years; 9 were boys and 12 girls. Most presented with nontender, palpable neck masses and minimal constitutional complaints. Adenopathy was unilateral in all cases but 2. Mycobacterium avium-intracellulare and M. fortuitum were the main causative organisms. All underwent excision of the affected nodes. Long-term follow-up has been uneventful, except for 1 case of local recurrence requiring re-excision.

This study emphasizes the marked variability in the clinical presentation of scrofula in children, stressing the importance of the differential diagnosis between tuberculous and atypical mycobacterial cervical lymphadenitis. The treatment of choice for the latter is complete excision of the affected nodes. Other treatment is followed by recurrence and unnecessary complications and should be avoided.

Retinal Lesion due to Excessive Exposure to Sunlight

Irene Krasniz, Itzchak Beiran, Benjamin Miller

Dept. of Ophthalmology, Rambam Medical Center and Bruce Rappaport Faculty of Medicine, Technion, Haifa

Retinal damage caused by direct exposure to the sun's rays is well recognized by the ophthalmic community. Although functional ability in solar retinopathy is usually regained within weeks, some suffer long-term visual impairment. Anatomic damage to the retina, even in those who regain full vision, is permanent. We describe 2 cases of solar retinopathy, 1 of which remained with permanent loss of vision. The role of medical education in preventing damage from solar retinal hazards is stressed.

Premature Birth and Cognitive Functioning in Adolescence

Ofra Lubetzky, Avi Weitzman, Izhak Gilat, Samuel Tyano

Levinsky College of Education and Geha Hospital, Tel Aviv and Sackler Faculty of Medicine, Tel Aviv University

Premature infants are considered a high-risk population for developing cognitive dysfunction. Studies have indicated lower cognitive performance among elementary school children born prematurely. We focused on cognitive functioning of such adolescents. This age was chosen because of its critical importance in the development of the individual.

50 adolescents aged 14-16 years born prematurely were compared with 50 born at full-term and matched for gender, age and socioeconomic status. All subjects attended regular schools and did not suffer severe neurological disorders. Cognitive functioning was measured by the Bender-Visual Motor Gestalt Test and by 3 subtests from the Wechsler Intelligence Scale for Children (revised WISC-R test).

Results revealed that prematurely born adolescents scored lower than those born at term on all measures of cognitive performance. The results are discussed in terms of their developmental meaning and of therapy for the prematurely born.

One-Stage Surgery for Hirschsprung's Disease in Children

Mark Weinberg, Baruch Klin, Itzhak Vinograd

Dept. of Pediatric Surgery, Assaf Harofeh Medical Center, Zerifin, and Sackler School of Medicine, Tel Aviv University

Traditionally Hirschsprung's disease has been treated by 2-or 3-stage procedures. During the past 6 years a 1-stage Duhamel procedure without stoma has become our treatment of choice for Hirschsprung's disease in neonates and young infants. Over a 6-year period, 15 infants and children with colonic Hirschsprung's disease were treated with the 1-stage Duhamel retro-rectal pull-through procedure without a stoma, with the Lester-Martin modification. All patients had the usual short segment aganglionosis, but 1 had a long segment which included the splenic flexure.

Early complications included wound infection in 1 and minor rectal bleeding in 3. Late complications included constipation in 1 and enterocolitis in 4. Long-term functional results were very good in all those operated except for 1 with rectal achalasia.

We conclude that Hirschsprung's disease can be successfully treated with a 1-stage pull-through operation, the child usually benefitting from the shorter hospital stay and the avoidance of a colostomy.

Elastofibroma, a Rare Cause of Snapping Scapula Syndrome

Ilan Cohen, Yehuda Kolender, Josephine Isakov, Aaron Chechick, Yitzhak Meller

Dept. of Orthopedic Surgery, Sheba Medical Center, Tel Hashomer and Depts. of Orthopedic Oncology and Pathology, Sourasky Medical Center, Tel Aviv

Scapular pain is a common complaint in daily orthopedic practice. A different type of scapular discomfort, the snapping scapula syndrome that occurs when smooth gliding motion of the scapula upon the chest wall is interfered with is much less common.

We studied the syndrome of periscapular pain and discomfort, and present a rare etiology: elastofibroma dorsi, a unique, benign, soft tissue-tumor with unique characteristics.

Elastofibroma appears deep to the lower scapular pole, is often bilateral, and consists of a mixture of collagen, elastic fibers and fibroblasts. We present 6 cases, in 3 men and 3 women aged 51-65.

Malignant Nodular Hidradenoma (Sweat Gland Tumor)

Anat Engel, Yaron Bar-Dayan, Santiago Engelberg, Yair Levi

Depts. of Medicine B, Pathology, and Disease Research Unit, Sheba Medical Center, Tel Hashomer and Sackler Faculty of Medicine, Tel Aviv University

Malignanhidradenoma is a very rare tumor that originates from sweat glands. We present a 61-year-old man with an ulcerated tumor in his right flank, 4 cm in diameter, that was excised with a wide free margin. Histopathologic study showed an ill-defined, epithelial neoformation, formed by lobules of clear polygonal cells in the deep dermis and subcutaneous tissue, diagnosed as malignant nodular hidradenoma.

1.5 years after excision there was enlargement of the right axillary and inguinal lymph nodes, which showed metastatic, adnexal neoplastic cells. Axillary resection and superficial dissection of the right inguinal nodes were performed. After 3 months the tumor had spread to other lymph nodes and acute obstructive renal failure required insertion of a pig-tail catheter into the right ureter. Radiotherapy was followed by chemo-therapy, but he died from end-stage metastatic disease in multi-organ failure.

Home Palliative Care of Terminal Cancer Patients, with Family Feedback

Hana Arad, Hana Geva, Valery Rosin, Ruth Kibrik, Isaac Kersz

Home Care Unit of Kupat Holim Haklalit, HaEmek Medical Center, Afula and Quality Improvement Unit, Rambam Medical Center, Haifa

Palliative care of terminal cancer patients is one of the tasks of our Home Care Unit. Increasing hospitalization costs have brought forward the decision to treat them at home, assuming that they would prefer to return and die in their natural surroundings, among family.

Most of our patients are aged, recent immigrants from the Soviet Union, of low socioeconomic status; most live with their close families. Our care model combines social, cultural, economic, medical and nursing aspects. More patients choose to die at home, and that is where costs are minimal. Care management and characteristics of 44 terminal cancer patients, who died between January and October 1996, are described. Living with a family was not required for treatment at home. Length of care by the unit ranged from 1-48 weeks, with an average of 8.5 and a median of 6. 55% of patients were hospitalized, most (58%) for 5-9 days for noncancerous diseases, and then discharged home. 54% died at home, a third were hospitalized for 2-17 days before death. Compared to the average length of stay in palliative care oncology wards, 1044 days and more than NIS 500,000 were saved.

A telephone survey examined families' satisfaction with various components of care. 92% were satisfied with the home treatment. 79%-82% felt that the nurse and doctor of the team met their needs and expectations. Half the families were satisfied with the treatment of pain. Families in which treatment was 24 weeks or more were generally less satisfied than those with shorter treatment at home. We learned that an early entry into treatment is necessary; hospital referral criteria should consider to a greater extent the coping ability of families; nursing aid hours should be increased and professional emotional support added; additional pain control methods should be used. All these would strengthen families, improve quality of care, and contribute to additional savings by decreasing hospital stay.

Malignant Lymphoma of the Bladder

Y. Mor, Z. Dotan, J.H. Pinthus, I.S. Engelberg, J. Golomb, J. Ramon

Depts. of Urology and Pathology, Chaim Sheba Medical Center, Tel Hashomer

Urinary tract lymphoma is usually reported when the secondarily stem is affected by widespread non-Hodgkin lymphoma. We describe an 83-year-old woman who presented with secondary lymphoma of the bladder 3 years after diagnosis when it initially infiltrated her breast. Treatment included local transurethral excision followed by chemotherapy, during which she died of disseminated disease.

Treatment of Retroperitoneal Sarcoma

Dina Lev-Chelouche, Subhi Abu-Abeid, Mordechai Gutman, Yoram Kluger, Moshe Michovitch, Isaac Meller, Moshe Inbar, Joseph M. Klausner

Depts. of Surgery B, Orthopedic Oncology and Oncology, Tel Aviv - Sourasky Medical Center (Affiliated with Sackler Faculty of Medicine, Tel Aviv University)

Soft tissue sarcomas are exceedingly rare, making up less than 1% of all solid malignancies. In the retroperitoneum, they tend to be large when diagnosed and are a therapeutic challenge to the surgical oncologist. Our experience with 51 patients with retroperitoneal sarcomas operated on during the past 4 years is presented. 37 were primary and 26 presented as recurrent tumors. The group included many different histological sub-types, the majority being high grade tumors.

Complete resection was achieved in 84%, necessitating extensive surgery, but was not possible in 8 patients (16%) who underwent partial resection or biopsy only. There was 1 perioperative fatality (2%). 18 (35%) suffered complications, all of which were reversible. The estimated 5-year survival in the complete resection group is 40%, while none of those who underwent partial resection survived more than 2 years. There was significantly better survival in patients with primary, low grade sarcomas which were smaller than 8 cm, compared to those with high-grade, recurrent sarcomas larger than 8 cm. Local recurrence developed in 8 patients of the complete resection group (18%), 2 months to 3 years after surgery.

These data show that despite the concept of retroperitoneal sarcomas as being aggressive, invasive tumors with a poor prognosis, the prognosis is not unusually bad. With proper surgical technique, resectability may be high, with improved overall survival.

Portal and Mesenteric Vein Thrombosis after Splenectomy for Idiopathic Thrombocytopenic Purpura

Eldad Silberstein, Alexander Smolikov, Itzhac Levi

Surgery B Dept. and Nathanzon Institute of Radiology, Soroka Medical Center and Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer Sheba

Mesenteric and portal vein thrombosis are rare complications of splenectomy. Their presentation depends on the underlying disease. It is most frequent in lymphoproliferative disorders but extremely rare in trauma. We describe a 22-year-old man and a 49-year-old woman who underwent splenectomy for idiopathic thrombocytopenic purpura and developed mesenteric and portal vein thromboses. Both were treated successfully with anticoagulants for 3 months until the thrombi regressed, as shown by CT scan. During a year of follow-up they were asymptomatic.