Ehlers-Danlos syndrome – vascular type (ecchymotic variant): cutaneous and dermatopathologic features

Ehlers-Danlos syndrome – vascular type, the only lethal form, is rarely reported in dermatology literature. It is characterized by translucent, atrophic skin, easy bruising, arterial, intestinal and/or uterine fragility manifesting as varicose veins, aneurysms and vascular/visceral/uterine rupture. As its dermatopathologic features are not well elucidated, diagnosis is often made after a catastrophic complication or at autopsy. This 36 year-old non-consanguineous male had brown-black plaques with atrophy and frequent ulceration over legs and dorsal feet and tortuous varicose veins around ankles for the past 15 years. Perivenous skin was translucent and hypopigmented. He had multiple and ecchymotic keloids and small atrophic, pityriasis versicolor-like lesions over trunk. He did not have hypermobile/hyperextensible skin and joints and showed no systemic or investigative abnormality. Histopathologic features of atrophic lesion included blood extravasation in atrophic epidermis/dermis, focal clustering and dilatation of blood vessels, malformed vessel walls, abundant hemosiderin in the dermis and homogenously stained/whorled patterned collagen especially around blood vessels. Pathology of keloidal lesion showed new collagen and vascular fragility. These histopathologic features appear of diagnostic value especially in patients who have compatible clinical findings but cannot afford confirmation by biochemical testing for abnormal synthesis of type III procollagen or identification of mutations in the COL3A1 gene.     

Oropalatal band (maxillo-mandibular band). off.

A rare case of a maxillo-mandibular band in a 21 year female patient is reported. Possibility of its being a remnant of buccaphary geal membrane is discussed in the light of clinical and histological findings.     

Diagnostic significance of estimation of serum apolipoprotein A along with alpha-fetoprotein in alcoholic cirrhosis and hepatocellular carcinoma patients

The serum apolipoprotein A (Apo A) and alpha-fetoprotein (AFP) were evaluated in histologically verified 30 cases of alcoholic cirrhosis and 18 cases of hepatocellular carcinoma (HCC). The latter were also divided into subgroups depending on the presence or absence of associated cirrhosis. Serum Apo A levels were found to be significantly decreased in cirrhotics (p less than 0.001) compared to controls and non-cirrhotic HCC patients. In 22 cases of alcoholic cirrhosis (AFP less than 10 ng/ml) and 12 cases of HCC (AFP greater than 600 ng/ml), the AFP levels itself were diagnostic, but in the remaining cases, AFP levels (100-600 ng/ml) were not able to differentiate between cirrhosis and malignancy. In this later group of patients with low pathological range of AFP, serum Apo A levels found to be significantly decreased in alcoholic cirrhotic patients (p less than 0.001) compared to HCC patients. Thus, estimation of Apo A levels may be helpful to interpret the AFP values at lower pathological range due to suspected liver pathology.     

Lobster-claw syndrome.

Lobster-Claw syndrome is a rare autosomal dominant, hand-foot malformation with Oro-dental features. This is a rare condition and from the available reports so far, cases being reported are less than 1%. Most reports have focused on the hand-foot deformity of this syndrome. This paper highlights the typical Oro-dental features associated with this syndrome such as retained deciduous teeth, hypodontia and variation in crown size, arch length and arch width.     

ORL-HNS training in South Africa

An out line of the scheme of training specialists in Otorhinolaryngology-Head and Neck Surgery (ORL-HNS) in South Africa is given and the difference with Indian training discussed     

Ambulatory Surgery Patients May Be Discharged before Voiding after Short-acting Spinal and Epidural Anesthesia

Background: Voiding before discharge is usually required after outpatient epidural or spinal anesthesia because of concern about bladder overdistention and dysfunction. Shorter duration spinal and epidural anesthesia may allow return of bladder function before overdistention occurs in low-risk patients (those younger than age 70, not having hernia, rectal, or urologic surgery, and without a history of voiding difficulty), and predischarge voiding may not be necessary.

Methods: After institutional review board approval and informed consent, 201 low-risk ambulatory patients were prospectively studied in either a standard or accelerated pathway after undergoing spinal or epidural anesthesia with procaine, lidocaine, 2-chloroprocaine, or less than 7 mg bupivacaine; epinephrine was not used in any anesthetic. Standard pathway patients (n = 70) were required to void before discharge. Accelerated pathway (n = 131) patients were not required to void. (After randomization of an initial 163 patients to one of the two tracks, 38 additional patients were assigned to the accelerated pathway.) If accelerated pathway patients voided, they were discharged when all other discharge criteria were met. If they did not spontaneously void after block resolution, a bladder ultrasound (BUS) was performed. If the BUS indicated a urine volume of less than 400 ml, the patients were discharged and instructed to return to the emergency department if they were unable to void within 8 h of discharge. If the BUS indicated a urine volume of greater than 400 ml, the patients were reassessed in 1 h and were discharged if they could void spontaneously. If they could not void spontaneously, they were catheterized to facilitate discharge. All patients were contacted the next day to assess the return of normal bladder function.

Results: All standard pathway patients voided without difficulty, and were discharged in 153 +/- 49 (SD) min. 62 patients in the accelerated pathway voided spontaneously after resolution of their block and were discharged in 127 +/- 41 min. 46 patients were discharged with a BUS less than 400 ml in 120 +/- 42 min. 23 patients had a BUS greater than 400 ml: of these, 20 patients voided within an hour and were discharged in 162 +/- 45 min. Three were catheterized after 1 h, and were discharged in 186 +/- 61 min. Mean discharge time for all patients in the accelerated pathway was 22 min shorter than the standard pathway (P = 0.002). No patients had difficulty voiding or returned to the hospital for urinary problems. None reported new urologic symptoms.