Multiple abdominal cocoons: an unusual presentation of intestinal obstruction and a diagnostic dilemma.

Sclerosing encapsulating peritonitis (SEP) or abdominal cocoon is a rare acquired condition with an unknown aetiology. It is characterized by encapsulation of the small bowel by a fibrous membrane and can lead to intestinal obstruction. We present the case of a 42-year-old gentleman with a history of hepatitis C, tuberculosis, and previous abdominal surgery, who presented with subacute intestinal obstruction. Surgical exploration of the abdomen revealed that the entire contents were enclosed into three distinct sacs by a dense fibrous membrane. Excision of the sacs was performed followed by adhesiolysis. This is believed to be the first reported case of multiple cocoons within the abdominal cavity. The case is discussed with reference to the literature.

The management of chronic osteomyelitis using the Lautenbach method.

We describe our medium-term results for the management of chronic osteomyelitis in long bones using the Lautenbach procedure. Seventeen consecutive patients (18 segments) were treated prospectively. Osteomyelitis had been present for a mean of 12.5 years (1 to 31). A discharging sinus was present in all cases. Nine of the associated fractures had failed to unite and a further two needed correction of malunion. The Lautenbach procedure involves debridement, intramedullary reaming and the insertion of double-lumen tubes to establish both a local antibiotic delivery system and cavity analysis for volume and culture. The end-point of treatment is when the irrigate produces three consecutive clear cultures with improvement in the blood indices and obliteration of the cavity volume. The mean length of treatment was 27 days (14 to 48). One patient required a second procedure and another local debridement for recurrence of the infection. Two patients had Papineau grafting because of cortical defects. All the patients have subsequently remained free from infection. After treatment 11 had internal or external fixation for treatment of non- or malunion or a joint replacement, including two successful limb-lengthening procedures. Two further patients, while cured of infection, underwent amputation for other reasons. The mean length of follow-up was 75 months. This procedure allows precise control over the osteomyelitis until objective assessment suggests that infection has been cleared and the cavity obliterated. We recommend this procedure for long-standing complex cases in which basic techniques using debridement and antibiotics have failed.

Frostbite: epidemiology at high altitude in the Karakoram mountains.

During a 10-year period (December 1984 to December 1994), 1500 cases of frostbite were treated at a tertiary care medical facility. They were all males with their ages ranging from 17 to 43 years. All the patients sustained the frostbite injury in the northeastern part of Pakistan known as the Karakoram range of mountains. They included a large number of porters and guides employed by various mountaineering expeditions (approximately 250-300 expeditions per year) in that region, as well as local inhabitants. This retrospective study included the heights at which frostbite occurred (range 11,000-22,000 feet above sea level). Of the patients, 15% (n = 225) got frostbitten within 1 h of exposure, whereas the majority (71%) had an exposure of 1-3 h. The effect of seasonal variations (relative hypothermia) on the extent and depth (degree) of frostbite and the distribution of lesions as per body surface subunits (areas) was noted and found to be statistically significant with P < 0.05 for both. The occurrence of frostbite at various heights showed a very steep upward curve beyond a height of 17,000 feet above sea level. This has been termed the 'cut-off' point for frostbite by the authors, the increase depicting the true picture of 'high altitude frostbite'. Tobacco smoking and peripheral vascular disease were found to be important contributing factors. The feet were involved most frequently (64%) followed by the hands (32%), the head and neck region (3%) and the perineum (1%). Independent effects of the height (relative hypoxia) on the depth of frostbite lesion (degree) and on the involvement of multiple body areas (surface subunits) showed significant correlation with P values well below 0.05 for each. Of cases, 92% (n = 1386) had second- or third-degree frostbite necessitating definitive surgical intervention. Total frostbite-related mortality spanned over 10 years was 11%.

Frequency of consanguinity and its effect on congenital malformation--a hospital based study.

A consecutive sample of 5,000 families of Armed Forces personnel was analysed to study the prevalence of consanguineous marriage and its effect on the prevalence of hereditary gross (physical) malformations. The overall frequency of inbreeding was 76% with a coefficient of 0.04151 which is probably the highest figure reported in literature. The overall prevalence of congenital malformations in children of related parents was 40% (1530 out of 3820) and in non-related parents 26% (305 out of 1180) (p = < 0.01). Considering the prevalence of malformations with the types of relationship of parents, the inter-marriage with first cousins, alone, was highly significant (p = < 0.01).

Antibiotic prophylaxis in paediatric surgery.

Effect of antibiotic prophylaxis was studied in 400 children undergoing various types of surgery. Patients were divided into different classes according to the type of operation and each was further randomized into a routine or trial group. The routine group received antibiotics for prolonged periods. In the trial group, "clean" cases (class A) did not get any antibiotics. The "clean-contaminated" (class B) and "contaminated" cases (class C) received peri-operative antibiotics only. Frankly infected cases were not included in this trial. There were 131, 213 and 56 cases in classes A, B and C respectively; of these 13 (3.25%) cases were diagnosed as infected, four in the trial group and nine in the routine group. It was found that a short course of peri-operative antibiotics was equally, if not more effective. Prolonged courses of antibiotics were not only useless and expensive but could also be harmful. For clean cases there is no need for antibiotic prophylaxis. Children behave no differently and these results may be extrapolated to adults.

Ciguatera fish poisoning.

Cases of ciguatera fish poisoning no longer are confined to endemic areas. This makes awareness of this entity important. The diagnosis usually is made by the presence of gastrointestinal symptoms, nausea, vomiting, and diarrhea, and of neurological symptoms such as paresthesias, paresis, and pruritus. The detection of ciguatoxin in the ingested fish by any of the available bioassays, will confirm the diagnosis. The treatment of this food poisoning is supportive, although intravenous mannitol is reported to be safe and effective. The prognosis is good and complete recovery is to be expected. However, relapses can occur, especially on re-exposure to the toxin.

Thoracic cord compression by extramedullary hematopoiesis in thalassemia.

Extramedullary hematopoiesis (EMH) refers to hematopoiesis occurring outside the medulla of bone. It may be physiologic or due to pathological conditions like hematopoietic disorders. EMH can involve liver, spleen, thorax, and lymph nodes. It can involve paraspinal tissues with extension and involvement of spinal canal. In our case, the diagnosis was confirmed by the history of the patient stating underlying hematological condition and by magnetic resonance imaging (MRI) findings showing large soft tissue masses in paraspinal areas with involvement of spinal canal and leading to cord compression.

Idiopathic hypertrophic cranial pachymenigitis - A long follow-up needed.

Idiopathic hypertrophic cranial pachymenigitis is a rare clinical condition caused by localized or diffuse inflammatory thickening of dura matter. Described here is a person having diffuse thickening of dura matter of base of skull and he was on follow-up treatment for 5 years with us. Diagnosis was done by excluding other conditions and with biopsy. The patient responded to steroid and the MRI picture, which is given serially, shows improvement.

Subcortical white matter abnormalities related to drug resistance in Wilson disease.

Wilson disease (WD) produces typical lesions in the brain, which can aid in diagnosis and therapy. The authors present a drug-resistant WD case with atypical cerebral lesions with marked involvement of white matter as visualized on MRI scans. The diagnosis was confirmed by identification of mutations in the ATP7B gene. The case demonstrates an uncommon pathology-related cerebral copper accumulation and emphasizes the importance of genetic screening in the diagnosis of WD.

Force transmission, compliance, and viscoelasticity are altered in the alpha7-integrin-null mouse diaphragm.

Alpha7beta1 integrin is a transmembrane structural and receptor protein of skeletal muscles, and the absence of alpha7-integrin causes muscular dystrophy. We hypothesized that the absence of alpha7-integrin alters compliance and viscoelasticity and disrupts the mechanical coupling between passive transverse and axial contractile elements in the diaphragm. In vivo the diaphragm is loaded with pressure, and therefore axial and transverse length-tension relationships are important in assessing its function. We determined diaphragm passive length-tension relationships and the viscoelastic properties of its muscle in 1-month-old alpha7-integrin-null mice and age-matched controls. Furthermore, we measured the isometric contractile properties of the diaphragm from mutant and normal mice in the absence and presence of passive force applied in the transverse direction to fibers in 1-month-old and 5-month-old mutant mice. We found that compared with controls, the diaphragm direction of alpha7-integrin-null mutants showed 1) a significant decrease in muscle extensibility in 1-year-old mice, whereas muscle extensibility increased in the 1-month-old mice; 2) altered muscle viscoelasticity in the transverse direction of the muscle fibers of 1-month-old mice; 3) a significant increase in force-generating capacity in the diaphragms of 1-month-old mice, whereas in 5-month-old mice muscle contractility was depressed; and 4) significant reductions in mechanical coupling between longitudinal and transverse properties of the muscle fibers of 1-month-old mice. These findings suggest that alpha7-integrin serves an important mechanical function in the diaphragm by contributing to passive compliance, viscoelasticity, and modulation of its muscle contractile properties.

Anorectal malformations in female children--10 years experience.

Despite enormous interest in the development of an ideal operative procedure to treat anorectal malformations, the situation is as confusing as it was 30 years ago. The reasons could be inconsistency of the nature of the lesions found in patients along with multi-system associated malformations primarily affecting the prognosis of available procedures. High imperforate anus is a complex anomaly that requires a combination of careful preservation of structures and precise anatomic reconstruction for optimal results. A retrospective study, comprising a consecutive sample of female children with anorectal malformations treated over a period of 10 years, is presented. Of 130 patients, 83% (n = 108) presented later than 3 months of age as they could pass stools through associated fistulae, of which the commonest type was found to be an ano- or recto-vestibular fistula (65%, n = 83). Ninety-four cases (72%) had what are traditionally known as "low" anorectal malformations (perineal fistulae, anteriorly placed anus and anorecto-vestibular fistulae). Ten percent (n = 14) had translevator, and 17% (n = 22) were found to have "high" lesions. Seventeen percent of patients (n = 22) had associated congenital malformations, predominantly in intermediate and high anorectal lesions, 40% of which pertained to the urogenital system. In total, 137 definitive operative procedures were done including Mollard's anterior perineal approach in 81 patients and posterior sagittal anorecto plasty (PSARP) in 38, enabling a comparison to be made of the functional results and complications in the two groups. Out of 24 post-operative complications noted in the series, 17 were found in the group who had Mollard's procedure carried out (including 3 deaths) as compared with 7 in these cases who had PSARP (two-sided P utilizing Fisher's exact test = 0.475).

Management of non-unions with mono-lateral external fixation.

UNLABELLED: We reviewed a cohort of 107 patients (110 long bone segments) with fracture non-union, treated by mono-lateral external fixation in Sheffield between 1987 and 1996. There were 83 males and 24 females with a mean age of 36 years. Sixty-seven patients had high-energy injuries and there were 56 open fractures. There were 60 tibiae, 38 femora and the rest were upper limb long bones with a mean of 3.2 previous procedures. The mean duration of non-union was 23.4 months (range 3-123). There were 61 mono-focal procedures with 41 supported in neutralisation, 20 in compression and three in distraction. There were 49 bifocal procedures (33 compression distraction and 16 bone transport). Seventy-one segments required a bone graft. The success rate using the initial fixator was 90%. Seven patients required further fixation to achieve clinical and radiological union, bringing the healing rate to 95.5% (total n=105). There were five amputations, all in smokers and three of them were directly related to vascular failure. The mean hospital stay was 21 days and the mean number of operations per patients was 2.55. The mean time to bony union was 12.69 months (range 2.5-64). The mean length gain was 4.5 cm (range 1.5-12 cm), the mean angular correction achieved was 12 degree (range 2-39 degree). The bony and functional results were assessed at the end of treatment by system described by Paley and Catagni (JBJS 77A, 1995). CONCLUSIONS: Mono-lateral external fixation can provide stable fixation for the treatment of established non-unions. The fracture environment may be carefully controlled and angulation and length corrected simultaneously. Interestingly 11 out of 12 problem cases were in smokers.

The value of a prognostic scoring system in the rehabilitation of elderly patients with proximal femoral fractures.

AIM: To evaluate a prognostic scoring system for elderly patients with hip fractures, which can, on admission, predict on the basis of pre-injury prognostic factors the possible length of stay in hospital, progress of rehabilitation, level of self-caring and rehousing needs at discharge. METHODS: A prognostic scoring system, developed on the basis of pre-injury level of independence in activities of daily living (ADL) (Katz), medical comorbidities and Pfeiffer's mental scoring system was used (Svensson O et al. J Bone Joint Surg 1996; B78: 115-8). The study was conducted on a cohort of 63 patients prospectively recruited from consecutive admissions. RESULTS: Ninety percent of the patients (without comorbidities and mental score > 7; group 3/3) who were independently mobile pre-injury remained independently mobile at discharge (CI 95% 0.79-0.97). Pre-injury score was found to be significantly related to the level of ADL at 2 weeks after operation and at discharge from hospital (CI 95% 0.69-0.9). There was a pattern in duration of hospital stay in different groups (score 3/3 = 22 days, 2/3 = 31 days and 1/3 = 33 days). A pattern of community placement and housing after discharge was noticed in relation to the pre-injury score. CONCLUSION: 3/3 score groups are most likely to achieve independence with mobility and ADL on the acute orthopaedic ward and to be discharged directly to their pre-injury homes. They also were the least likely group to need rehabilitation in a dedicated orthogeriatric rehabilitation unit. 2/3 score groups are most likely to benefit from rehabilitation in a dedicated orthogeriatric rehabilitation unit. 1/3 and 0/3 score groups are least likely to benefit from rehabilitation in a dedicated orthogeriatric unit, in terms of achieving independence with mobility and ADL. They were also the most likely group to need long-term high dependency residential care. Early liaison with social and community services is essential for effective and timely planning of discharge.

Toothpick perforation of the duodenum.

An 82-year-old man developed gastrointestinal hemorrhage and polymicrobial sepsis from toothpick perforation of the duodenal bulb. We removed the toothpick endoscopically.

Wirsungorrhagia or hemoductal pancreatitis: report of a case and review of the literature.

An unusual case of gastrointestinal hemorrhage due to rupture of the splenic artery into the pancreatic duct in a patient with chronic pancreatitis is described. The diagnosis was suspected by observing blood coming from the papilla of Vater during duodenoscopy and corroborated by findings seen on splenic arteriography. Previously reported cases are reviewed. An etiology is discussed and a classification suggested. This obscure cause of gastrointestinal bleeding should be suspected when the more common causes of bleeding have been satisfactorily ruled out.