Twenty-year review of the surgical management of perianal Crohn's disease

PURPOSE: A retrospective analysis of 48 patients treated over a 20-year period (March 1973–April 1993) was undertaken to assess the results of our practice of early surgical intervention in suppurative complications of perianal Crohn's disease. METHODS: All patients were either seen in the office within the last six months or contacted by phone. RESULTS: The average age of our patients was 30 years at initial diagnosis. Thirty-four patients (71 percent) initially presented with intestinal disease and four (8 percent) with only perianal disease. Thirteen patients (27 percent) initially presented with simultaneous intestinal and perianal disease. The various fistulas at initial presentation included 8 intersphincteric (17 percent), 14 transphincteric (29 percent), 11 complex or multiple (23 percent), 5 rectovaginal (10 percent), and 2 unclassified, for a total of 40 patients. Eight patients (17 percent) presented with only an abscess. Eighty five percent of our patients healed after their first procedure, with an average time to heal of 2.8 months. Thirteen (27 percent) patients had recurrences after initial healing of their wounds. The mean time to recurrence after healing was 5.25 years. Fifty-four percent of our recurrences (7 patients) were treated by incision and drainage of an abscess only. Seven of 13 recurrences healed after the second procedure (54 percent), and 5 of 6 healed after a third procedure (83 percent). Only seven (14 percent) of our patients underwent a proctocolectomy during the study period, through September, 1993. Our overall probability of avoiding proctectomy and healing perineal wounds of 86 percent is consistent with published literature. CONCLUSIONS: Early aggressive surgical management of suppurative complications of perianal Crohn's disease before complex management problems ensue results in a high incidence of healing and a low risk of subsequent proctectomy.     

Intralesional ethanol in the treatment of unresectable liver cancer

Percutaneous ethanol injection (PEI) under ultrasonography guidance has been widely tried in not advanced hepatocellular carcinoma (HCC). Ten years after the introduction of PEI, some conclusions of its indications can be drawn. In our series, 210 cirrhotic patients were treated; 141 with multisession PEI in an outpatient clinic, 57 with single session PEI under general anesthesia, and 12 with both. The 1-, 3-, and 5-year survival rates (by Kaplan-Meier method) were 93%, 65%, and 41% for Child class A patients with single HCC 5 cm, and 88%, 47%, and 33% for patients with multiple HCC up to five lesions 5 cm. In these patients the local recurrence rate was 15% and the new lesion rate at the 5-year follow-up was 74%. One death due to hemorrhage from esophageal varices in a Child class C patient treated by single session PEI occurred. The large number of cirrhotic patients enrolled in ultrasonography screening programs has created a large demand for effective, safe, repeatable, low-cost treatment that can be offered at many centers. PEI meets all these requirements. PEI is proposed as the treatment of choice for the mentioned patients, excluding candidates for liver transplantation and surgical resection according to the predictive adverse factors currently in use. Single session technique widened the indications of traditional PEI to larger lesions.

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Resumen La inyección percutánea de etanol (IPE) bajo guía ultrasonográfica ha sido ampliamente ensayada en carcinoma hepatocelular (CHC) no avanzado. Transcurridos diez años luego luego de la introducción de la IPE, se pueden sacar conclusiones pertinentes a su indicación: En nuestra serie, se trataron 200 pacientes cirróticos, 141 de ellos con IPE en múltiples sesiones en forma ambulatoria, 57 con una sesión única de IPE bajo anestesia generaly y 12 con ambas modalidades. Las tasas de sobrevida a 1, 3 y 5 años (por el método de Kaplan-Meier) fueron 93%, 65%, 41% en los pacientes en clase Child-A con CHC único de 5 cm, y 88%, 47% 33% en CHC múltiple de hasta 5 lesiones de 5 cm. En estos pacientes la tasa de recurrencia local fue 15% y la tasa de nuevas lesiones a los 5 años de seguimiento fue de 54%. Se presentó una muerte por hemorragia de várices esofágicas en un paciente en clase Child-C tratado con una sesión única de IPE. El gran número de pacientes cirróticos incorporados a programas de tamizaje con ultrasonido ha creado una gran demanda por tratamientos eficaces, seguros, reproducibles y de bajo costo. El IPE llena todos estos requerimientos. Se propone el IPE como el tratamiento de escogencia para los pacientes arriba mencionados, haciendo exclusión de los candidatos para transplante hepático y para resección quirúrgica de acuerdo con los factores adversos de pronóstico actualmente en uso. La técnica de une sesión única ha ampliado las significaciones tradicionales del IPE para ser aplicada a lesiones de mayor tamaño.

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Résumé L'alcoolisation par injection percutanée (APC) sous échographie est utilisée pour traiter des carcinomes hépatocellulaires (CHC) peu volumineux. Dix ans après l'introduction de la technique d'APC, on peut tirer des conclusions quant à es indications. Dans notre expérience, 210 patients cirrhotiques ont été traités: 141 ont été traités par APC en ambulatoire, 57 en «séance unique» sous anesthésie et 12 par les deux. La survie à 1, 3 et 5 ans (méthode de Kaplan-Meier) était respectivement de 93, de 65 et de 41 % pour les patients ayant une lésion unique 5 cm, classés Child A, et de 88, de 47 et de 33% chez les patients ayant des lésions multiples mais inférieures au nombre de 5, toujours 5 cm. Chez ces patients, le taux de récidive locale était de 15% et le taux de nouvelles lésions à 5 ans, de 74%. Il y a eu un décès par hémorragie en rapport avec une rupture de varices oesophagiennes chez un patient Child C traité en une seule séance d'APC. Le nombre croissant de patients actuellement soumis, à une surveillance par échographie va de paire avec un traitement efficace, sûr, de coût réduit, et facilement disponible dans de nombreux centres. L'APC répond à toutes ces conditions. L'APC peut être proposée comme traitement de choix pour des patients répondant aux critères suscités, en excluant les candidats à la transplantation ou ceux qui peuvent être traités chirurgicalement. Les indications de la technique préconisée ici, en une seule séance, s'élargissent actuellement à des lésions plus volumineuses.

     

Inherited disorders of the extracellular matrix

Primary and secondary defects in extracellular matrix proteins emphasize the role of these proteins in neuromuscular disorders; mutations have been found in the genes for the laminin-alpha 2 chain, for all three alpha chains of collagen VI and for integrin alpha 2. Secondary alterations in protein expression occur in association with these primary defects, and in other disorders they implicate the extracellular matrix. Animal models are helping to elucidate the function of some of these proteins, to develop therapeutic strategies and to suggest candidate proteins for other neuromuscular disorders.     

Mesenteric cystic neoformations: Report of two cases

(Received for publication on Apr. 28, 1997; accepted on May 15, 1998)     

Lasting reduction in mesolimbic dopamine neuronal activity after morphine withdrawal

The activity of mesolimbic dopaminergic neurons was investigated in rats at various times after a chronic regimen of morphine, which produced, upon suspension, a marked somatic withdrawal syndrome. Single-cell extracellular recording techniques, coupled with antidromic identification from the nucleus accumbens, were used to monitor neuronal activity while behavioural observations allowed quantification of the somatic signs of morphine withdrawal. Temporal correlation of electrophysiological indices, such as firing rate and burst firing, with scores obtained through behavioural assessments proved negative, in that somatic signs were pronounced at 24 h after suspension of treatment and then subsided to control values at 72 h after the last morphine injection. In contrast, the firing rate and burst firing of mesolimbic dopaminergic neurons were found to be reduced at 1, 3 and 7 days after morphine withdrawal. After 14 drug-free days, electrophysiological analysis revealed an apparent normalization of various parameters. However, at this time, intravenous administration of morphine produced an increment of electrical activity which was significantly higher than that obtained in control (saline treated) rats. Further, administration of the opiate antagonist naltrexone, administered without prior morphine, at 3, 7 and 14 days after the last morphine administration, failed to alter dopaminergic neuronal activity. The results indicate: (i) that the activity of mesolimbic dopaminergic neurons remains reduced well after somatic signs of withdrawal have disappeared; (ii) after 14 days of withdrawal, the augmented magnitude of the electrophysiological response to exogenous morphine suggests an increased sensitivity of opiate receptors; and (iii) the lack of relationship between dopaminergic activity and somatic signs of withdrawal corroborates the notion that dopaminergic activity in the mesolimbic system does not participate in the neurobiological mechanisms responsible for somatic withdrawal. The present results may be relevant to the phenomenon of drug addiction in humans and consequent relapse after drug-free periods.     

Clinical spectrum and diagnostic difficulties of infantile ponto-cerebellar hypoplasia type 1

We present the clinical and histopathological features and the diagnostic difficulties encountered in five children affected by a motor neuron disorder other than spinal muscular atrophy. Investigations performed suggested the diagnosis of ponto-cerebellar hypoplasia type 1 (PCH-1). Severe respiratory difficulty was present at birth in two of these children; hypotonia, arthrogryposis, microcephaly and nystagmus were present in all. Early and progressive bulbar involvement with swallowing difficulties and stridor was also a common feature in these infants. Severe cognitive delay was invariably present. Brain magnetic resonance imaging showed ponto-cerebellar hypoplasia in four children while striking atrophy of the cerebellar vermis and cerebellar hemispheres were present in the fifth child. Electrophysiological and pathological investigations of proximal muscles performed at presentation in all these children were not conclusive, while the post-mortem studies, or the study of distal muscles during life, showed a clear neurogenic picture. Genetic studies excluded involvement of the SMN gene, or of other genes located on chromosome 5q, confirming that ponto-cerebellar hypoplasia type 1 is a different entity from typical proximal spinal muscular atrophy.     

Aesthetic considerations in facial reconstructive surgery: The V-Y flap revisited

The subcutaneous pedicled V-Y advancement flap (also known as the kite flap of Dufourmentel) has been described by many authors. Its versatility in reconstructive surgery is well known, both in facial reconstructive surgery as well as surgery of the trunk and extremities. Its advantages in achieving aesthetic reconstruction in specific facial regions have been less well emphasized in the literature. The flap can be designed within natural facial creases and heals inconspicuously because of its widely based subcutaneous or muscle pedicle which incorporates venous and lymphatic drainage over most of its length. The undesirable biscuiting or flap edema frequently observed with other conventional transposition flaps is avoided. We have found the V-Y flap particularly useful in reconstructing the lower eyelid/medial canthus, supra-alar, and nasolabial regions. Our experience with over 40 such flaps is described, and technical considerations in designing and elevating the flap are discussed.     

Chemotherapy as first treatment for primary malignant non-Hodgkin's lymphoma of the central nervous system preliminary data

Non-Hodgkin's lymphoma of the central nervous system (NHL-CNS) is thought to account for about 1 % of primary brain tumours. Radiation therapy has mainly been applied to treat cerebral lymphoma, but the low cure rate and the lack of enduring response have stimulated the search for alternatives. With the aim of postponing radiotherapy as long as possible, we tested the efficacy of a M-BACOD schedule administered immediately after histological diagnosis in 14 patients. After two M-BACOD courses 10 (71%) patients displayed an objective response (i.e. were apparently tumour-free when examined by CT). In 6 (60%) M-BACOD-responsive patients, radiotherapy was delayed for 5 months (without recurrences after a follow-up ranging from 9 to 18 months). Moreover, in 3 M-BACOD-responsive patients, no recurrence took place (even without radiotherapy) after a follow-up of 6–12 months. We conclude that radiation can be postponed after chemotherapy or delayed until tumor recurrence.This paper was presented at the 3rd Meeting of the European Neurological Society, Lausanne, 27 June–1 July, 1992     

Noradrenaline transport by rat heart sympathetic nerves: A re-examination of the role of sodium ions

Summary The effect of sodium ion on ³H-(–)-noradrenaline (0.0875 to 0.5 M) transport by rat heart atrial hemi-appendages incubated in vitro has been studied, and the following observations made: a) When sodium was omitted (choline and lithium substitution) there was no evidence for active noradrenaline transport, and only a component that did not show saturation kinetics up to 1 M noradrenaline, remained. b) Omission of sodium or addition of 4×10^–5 M desipramine inhibited noradrenaline transport to exactly the same extent, and their effects were not additive. Alprenolol did not reduce this sodium-independent transport, but tropolone lowered it somewhat. c) No evidence for corticosterone-sensitive noradrenaline transport (uptake-2) was found in this preparation at the low amine concentrations used. d) In control medium, the kinetic parameters of transport were: K _m: 0.59 ± 0.063 M and V _max: 2.44 ± 0.43 (pmoles/mg protein/min). With 26 mM sodium and the rest substituted by choline, K _m:2.26 ± 0.70 M (P0.001) and V _max: 2.74 ± 0.43 (pmoles/mg protein/min) (not significant). Also with 26 mM sodium, but with sucrose substitution, K _m: 0.76 ± 0.13 M (N.S.) and V _max: 1.06 ± 0.13 (pmol/mg/min) (P<0.05). Such results indicate that sodium only modifies the affinity of the transport system for noradrenaline, without changing V _max, and that changes in the latter are only a consequence of a reduction of the ionic strength. e) When noradrenaline transport was studied at different concentrations of external sodium, at constant ionic strength and with precautions to minimize the noradrenaline-releasing effect of low sodium, it was found that the data could be best represented by two hyperbolas placed in series. This suggests that the noradrenaline carrier has two sites for sodium, that do not interact with each other. When the same experiments were repeated in the absence of chloride, it was found that the noradrenaline transport system had lost virtually all its affinity for sodium. f) The effect of prolonged tissue incubation in the absence of sodium was found to produce a relatively small inactivation of noradrenaline transport. Such phenomenon was enhanced by raising the calcium concentration to 2 mM.