Progressive congenital kyphosis: report of five cases and review of the literature

For over 60 years congenital kyphotic deformities of the spine have been categorized into two distinct groups, depending on the developmental defect. Those arising from a failure of formation of the vertebral bodies were classified as type 1, while those arising from a failure of segmentation were referred to as type 2. Recognition of the progressive and unstable nature of the type 1 defects alerted physicians to the need for early operative stabilization through decompression and stabilization through instrumentation. As the embryogenesis of the spinal column was further investigated, and as diagnostic imaging methods of the spine improved, unstable congenital kyphoses were further subdivided. Progressive congenital kyphotic deformities now may accompany a host of vertebral column developmental defects as well as genetically mediated mesenchymal tissue defect syndromes. This paper presents 5 patients from The Children's Hospital of Philadelphia with progressive and symptomatic congenital kyphotic deformities of the spine. Two of these lesions resulted from defects of formation of the vertebral bodies, while one resulted from segmental spinal dysgenesis, maldevelopment of both the anterior and posterior vertebral elements. One patient's kyphotic deformity was a result of caudal regression syndrome, and the final case presented experienced a high thoracic kyphosis from a syndrome associated diffuse midline mesenchymal tissue abnormalities known as cerebrocostomandibular syndrome. All patients showed evidence of progressive cord compression and required neural element decompression, fusion, and instrumentation. The cases are discussed individually, and the developmental and clinical aspects of each are explored.     

Necrotizing sialometaplasia: literature review and presentation of five cases

Necrotizing sialometaplasia is a benign, ulcerative, inflammatory process of the minor salivary glands, primarily found in the hard palate. It is often mistaken clinically and histologically for a malignant tumor (that is, mucoepidermoid or squamous cell carcinoma). The lesion heals spontaneously in six to 12 weeks. It occurs primarily in persons between the ages of 40 and 60 and has been reported more frequently in men than women. In necrotizing sialometaplasia the squamous metaplasia and tissue necrosis is confined to the existing ductal and lobular pattern of the salivary glands, a unique characteristic that aids in diagnosis. Five additional cases are presented.     

Megalourethra: a report of four cases and review of the literature

Megalourethra is a rare congenital anomaly characterized by severe dilatation of the penile urethra. Four cases of congenital megalourethra were seen at Sir Padampat Mother and Child Health Institute, Jaipur, during the last 10 years. Three cases of scaphoid megalourethra had no other associated congenital anomalies and were treat-ed successfully without any complications, while one patient with a fusiform megalourethra had severe associated congenital anomalies and died. These cases are reported with a review of the literature.     

Endometrial adenocarcinoma associated with intrauterine pregnancy. A report of five cases and a review of the literature

Endometrial adenocarcinoma associated with pregnancy is a rare lesion; only 14 acceptable examples have been reported in the literature. This study describes five additional examples with a critical review of the previously published cases. Four of the five women were nulliparous and three had sought medical intervention for infertility. The tumors were all well-differentiated endometrioid adenocarcinomas; three had a focal to extensive papillary pattern and three had focal to extensive squamous differentiation. Four were diagnosed at the time of dilatation and curettage and one at the time of cesarean section for a 28-week, live infant. Follow-up was available for four of the five women. Two women underwent hysterectomy with bilateral oophorectomy and were alive and well 12 and 48 months after diagnosis. The woman who had the live birth and the remaining woman were treated by repeat curettage with or without progesterone therapy, and each woman has had two subsequent full-term pregnancies with live births. These women are alive and well 57 and 58 months after diagnosis. Women with focal, well-differentiated carcinomas can successfully maintain their fertility if followed by repeat curettage with or without progesterone therapy.     

Ureterovaginal fistula: a report of 12 cases and review of the literature

Ureterovaginal fistula is an uncommon complication of pelvic operations, seen most often after Wertheim's hysterectomy. We report 12 cases of ureterovaginal fistulas seen during a 20-year period, all of which followed operations for benign gynecologic conditions. Most patients had no urinary symptoms until the sudden onset of incontinence 1 to 4 weeks postoperatively. Diagnosis was established readily by a combination of excretory urography, cystography, cystoscopy, retrograde pyeloureterography and dye studies. In our series only 1 patient was treated by primary nephrectomy, while 11 underwent ureteroneocystostomy: 2 with a Boari flap and 9 by a direct method. Reconstruction failed in 2 patients, 1 of whom required a secondary nephrectomy.     

Atypical ameloblastoma: report of 3 cases and a review of the literature

The aim of our study was to test the hypothesis that the better absorption of sorbitol when ingested with glucose could be related to a delayed gastric emptying. We tested the effect of the ingestion of glucose and lipids on the gastric emptying and intestinal absorption of sorbitol in six healthy volunteers, using gastric scintigraphy and hydrogen breath test. After an overnight fast, subjects ingested in random order, on 48-h test periods separated by at least one week, the following solutions: (a) 20 g sorbitol alone; (b) 20 g sorbitol and 20 g glucose; (c) 20 g sorbitol and 9 g lipids. Isotopic acquisitions were taken for 3 h following the ingestion of sorbitol labelled with 111Indium. Hydrogen concentration was measured in end-expiratory samples during 5 h, and the areas under the breath hydrogen curve, reflecting the amounts of sorbitol unabsorbed in the small bowel, were compared between periods. Mean area under the curve was 397 +/- 159 when sorbitol was ingested alone, and this was significantly lower when ingested with glucose or lipids (313 +/- 181 and 337 +/- 135, respectively; P < 0.05). The three curves of sorbitol gastric emptying differed significantly from each other, the gastric emptying being the slowest for sorbitol plus lipids, and the fastest for sorbitol taken alone. We found a positive correlation between the half-emptying time and the hydrogen areas under the curve (r = 0.46, P = 0.05). In conclusion, our study demonstrates that adding glucose or lipids to a solution of sorbitol slows the gastric emptying of sorbitol, resulting in a better intestinal absorption of sorbitol.     

Malignant histiocytosis-like B-cell lymphoma, a distinct pathologic variant of intravascular lymphomatosis: a report of five cases and review of the literature

Malignant histiocytosis (MH)-like B-cell lymphoma (BCL) is a neoplastic proliferation of large B cells clinically characterized by fever, hepatosplenomegaly, haemophagocytosis and abnormal laboratory data, without lymphadenopathy or skin lesions. Interestingly, most cases have been reported in Asian patients, and it is unclear whether MH-like BCL is biologically distinct from conventional large B-cell lymphomas. We report five Japanese patients with MH-like BCL. Biopsied specimens of bone marrow, liver and/or spleen showed infiltration of neoplastic B cells accompanied by haemophagocytosing histiocytes. Lymphoma cells were positive for CD19, CD20 and HLA-DR surface antigens, and negative for CD5 and CD10. In four cases elevated serum levels of interleukin (IL)-6 and the soluble IL-2 receptor isoform were noted, but not IL-1beta, IL-2 or tumour necrosis factor-alpha. Autopsies of two cases were pathologically diagnosed as intravascular lymphomatosis (IVL). Based on these observations, the current and nine previous cases reported as MH-like BCL in Japan were re-evaluated. They appear to form a peculiar variant of IVL, characterized by bone marrow involvement at presentation, haemophagocytic syndrome, and a rapidly aggressive clinical course, but rarely neurological complications or skin lesions. This variant may merit separate consideration because of the problems posed in the initial diagnosis and therapeutic approaches.     

Distal digital keratoacanthoma: a report of 12 cases and a review of the literature

Twelve cases of distal digital keratoacanthoma (DKA) affecting the subungual area or the proximal nail fold are reported. The distal phalanx of the toe was affected in three cases. Spontaneous resolution occurred in one; one other recurred after surgery. We also discuss the link between DKA and incontinentia pigmenti subungual tumours; these entities are indistinguishable.     

Laryngeal melanosis: report of four cases and literature review

Tumors grafted into mice may modify the proliferation of normal cell populations. In this paper, we have studied the evolution of mitotic activity (MA) in duodenal-crypt enterocytes of ES12a hepatocarcinoma-bearing mice; a total of 87 28-day-old female animals of the C3H/S strain were used after standardization for circadian-periodicity analysis. The mice were distributed into two groups: those remaining intact and those receiving tumor grafts. Each group was then divided into six batches (n = 6-10), one of which was sacrificed every 4 h over a period of one day. A dose of colchicine (2 micrograms/g) was administered to each animal 4 h before killing. Samples of duodenum were fixed in 10% (v/v) buffered formalin and processed for assessment of mitotic activity. The number and topographic localization of the colchicine-arrested metaphases were recorded among the entero-cytes within 20 longitudinal sections of the duodenal crypts in each animal. From these data the mitotic indices over the total crypt-cell population as well as within each previously-established zone were determined along with mean +/- SEM for each experimental group. The statistical significance of the differences among the data were analyzed by Student t test. The results show that the presence of ES12a tumor inhibits the mitotic activity of the duodenal-crypt enterocytes and produces an apparent temporal shift in the peak and trough within the circadian curve for this growth parameter.     

Internal jugular vein haemodialysis catheter-induced right atrium endocarditis--case report and review of the literature

OBJECTIVE: To determine the distribution and prevalence of sarcoptic mange in wombats, particularly the common wombat (Vombatus ursinus). DESIGN: Questionnaire survey in two parts. PROCEDURE: Questionnaires were distributed to biologists, rangers, animal carers and naturalists. Part 1 of the questionnaire aimed to determine the present distribution of sarcoptic mange in wombats (103 responses). Part 2 invited respondents to assess the prevalence of sarcoptic mange in wombats over a 3 month period (four responses). Information on wombats from 66 localities was received. Each locality represented an area of about 2500 km2. RESULTS: Mange was observed at 93% of localities surveyed and Sarcoptes scabiei was present in common wombats at 52% of localities. Sarcoptic mange was highly prevalent (22%) in two common wombat populations in Victoria. Anecdotal evidence suggested that mange epizootics are sporadic, cause significant morbidity and mortality and have a substantial effect on local abundance. The respondents did not report sarcoptic mange in either northern hairy-nosed wombats (Lasiorhinus krefftii) or southern hairy-nosed wombats (Lasiorhinus latifrons). CONCLUSIONS: Sarcoptic mange occurs in common wombat populations throughout the range of the common wombat including Tasmania and Flinders Island. While mange epizootics are sporadic, they have the potential to threaten the long-term survival of small, remnant populations.     

Non-alcoholic steatohepatitis. Report of five cases and review of the literature

We describe three men and two women, aged 18-50, with an occasional finding of increased aspartate and alanine aminotransferase and gamma-glutamyl transpeptidase levels in the absence of any drug treatment and past or current alcohol abuse. Two patients were overweight (body mass index 29 and 32, respectively) and physical examination was normal in all but one case. Tests for hepatitis A, B and C, Epstein-Barr virus, cytomegalovirus, toxoplasma and autoimmune hepatitis were negative and metabolic diseases (Wilson's disease, haemochromatosis, alpha-l-antitrypsin deficiency) were excluded by specific tests. Ultrasound liver scan revealed massive steatosis in all patients. Liver histology showed diffuse steatosis and parenchymal inflammation in all cases, with concomitant fibrosis and Mallory bodies in three of them. Findings were consistent with non-alcoholic steatohepatitis, a rare condition with potential progression to cirrhosis in a minority of cases. This disease, for which no treatment is currently available, must be considered in all subjects with elevated aminotransferases, in the absence of known causes of liver damage.     

Uretero-arterial fistulas: report of 2 cases and review of the literature

To investigate whether iloprost, a stable analog of prostacyclin, is useful for the prevention of posttraumatic spinal cord injury, we examined its effects on compression trauma-induced spinal cord injury in rats. Spinal cord injury was induced by applying a 20-g weight for 20 minutes to the spinal cord at the level of T-12, resulting in motor disturbances in the hindlimbs. These motor disturbances, evaluated using Tarlov's index, were markedly attenuated in rats with nitrogen mustard-induced leukocytopenia. Administration of iloprost also attenuated the motor deficits. Histological examination revealed that intramedullary hemorrhages observed 24 hours after trauma were significantly attenuated in leukocytopenic animals and in animals that received iloprost. The accumulation of leukocytes at the site of trauma, evaluated by measuring tissue myeloperoxidase activity, significantly increased with time following the trauma, peaking at 3 hours postinjury. Spinal cord myeloperoxidase activity in sham-operated animals did not increase postoperatively. Leukocyte depletion and administration of iloprost reduced the accumulation of leukocytes in the damaged spinal cord segment 3 hours posttrauma. These findings indicate that iloprost attenuates motor disturbances induced by spinal cord trauma and that its therapeutic efficacy can be partly explained by its inhibition of leukocyte accumulation at the traumatized site.     

Choledochal cysts in adults: a report of two cases and review of the literature

A choledocal cyst is a dilation of some component of the biliary tract that may include both intra- and extra-hepatic sites. They are classified into six types, all of which are relatively rare. Previously, choledochal cysts were treated with biliary-enteric bypass procedures. The current recommendation is to attempt complete excision to minimize the known risk of malignancy and the development of recurrent cholangitis or pancreatitis that may occur in patients with these cystic lesions. Two cases are discussed in which type I choledochal cysts presented. One was removed from a 31-yr-old man who presented with vague abdominal complaints the other from a 32-yr-old man who presented with pancreatitis. The epidemiology, diagnosis, surgical treatment, and risk of cancer in choledochal cysts is described.     

Pulsatile tinnitus associated with a high jugular bulb and slow venous blood flow

A case of permanent pulsatile tinnitus of the left ear in a patient with hypertriglyceridemia is reported. The combined radiological study with computed tomography, magnetic resonance imaging, and digital angiography excluded a glomus tumor and revealed an enlarged, high-position jugular bulb with slow blood flow. Causes of pulsatile tinnitus are discussed. We conclude that imaging techniques play a major role in the diagnosis of head and neck vascular abnormalities.     

Diagnosis of traumatic carotid-cavernous sinus fistula by monitoring venous oxygen saturation in the jugular bulb: report of two cases

OBJECTIVE AND IMPORTANCE: A traumatic carotid-cavernous sinus fistula (CCF) is rarely diagnosed early and may sometimes be missed until clinical signs and symptoms appear. The continuous monitoring of cerebral venous oxygen saturation may reveal the presence of a CCF by means of a fiberoptic catheter that records very high oxygen saturation values when positioned in the jugular bulb. CLINICAL PRESENTATION: We report two cases of early diagnosis of CCFs unexpectedly revealed by monitoring the jugular bulb for venous oxygen saturation values that approximated arterial saturation values. One case was diagnosed on Day 3 after admission, and the other was diagnosed shortly after cannulation of the ipsilateral jugular bulb. INTERVENTION: Confirmation of the diagnosis of CCF was obtained by angiography. Intravascular treatment was performed in one case. CONCLUSION: These cases add another diagnostic role to cerebral venous oxygen saturation monitoring. When high cerebral venous oxygen saturation values rapidly or abruptly reach arterial oxygen saturation, the presence of a CCF must be considered and confirmed by arterial angiography.     

Pentazocine fibrous myopathy: report of two cases and literature review

Two patients are described in whom the analgesic, pentazocine (Talwin), injected intramuscularly over long periods of time, resulted in a fibrous myopathy of the injected muscles. Although early testing of pentazocine indicated a low abuse potential and no skin or muscle complications, reports of such abuse and complications have since appeared in the literature, a review of which is included.     

Carotid sinus syndrome. Report of five cases and review of the literature

Five cases of carotid sinus syndrome are presented. The syndrome is defined by spontaneous attacks of dizziness and fainting which can be reproduced by graded pressure on one carotid sinus. Three forms of the clinical syndrome, cardioinhibitory, vasodepressor, and cerebral, are discussed. The hyperactive carotid sinsu reflex, in which there is ventricular asystole lasting at least 3 seconds or a decrease of more than 50 mm Hg in systolic and diastolic blood pressure, should be differentiated from this syndrome. Treatment modalities include reassurance, drugs, radiotherapy, cardiac pacemakers, and surgical approaches. Carotid sinus syndrome should be considered in the differential diagnosis of unexplained syncope, arteriovenous block, or inappropriate sinus bradycardia.     

Spontaneous intracranial hypotension: report of four cases and review of the literature

Spontaneous intracranial hypotension is an unusual syndrome of postural headache and low cerebrospinal fluid pressure without an established cause. We present four cases, analyze those previously reported in the literature, examine the MRI, CT, angiographic and cisternographic finding and discuss the clinical picture, proposed pathophysiologic mechanisms and potential treatment.     

Extradural myxopapillary ependymoma: report of two cases and review of the literature

The cauda equina is the most frequent location for ependymomas, particularly the myxopapillary variant, which generally arises from the filum terminale. These tumors have a characteristic histopathologic pattern and are usually easily recognized. The occurrence of these tumors in an extradural, sacrococcygeal, or subcutaneous location may prove challenging, particularly in the absence of any obvious central nervous system connection. We describe two such extradural cases, one with multiple regional and distant metastases and the other with multiple recurrences. The origin of these tumors from subcutaneous sacrococcygeal ependymal rests is postulated on the basis of earlier reports. Clinical and histopathological features are described and a review of the literature is presented.