A Chinese adolescent girl with Fechtner-like syndrome

We describe a 15-y-old girl with Fechtner-like syndrome, who is the first Chinese reported to have this rare syndrome. She presented with left homonymous hemianopia and neuroimaging revealed haemorrhage in both parietal and occipital lobes. Peripheral blood smear showed macrothrombocytopenia and intracytoplasmic inclusion bodies inside leucocytes. Thrombocytopenia and proteinuria responded to intravenous immunoglobulin and pulsed methylprednisolone. This case illustrates that life-threatening haemorrhage can occur in patients with Fechtner syndrome. Although there was no effective treatment reported in the literature, high dose steroid and immunoglobulin seemed to be useful in our patient. Our patient also had nephritic-nephrotic syndrome with renal insufficiency, which is unusual in adolescent female patients.     

SUICIDE ATTEMPT DUE TO METOCLOPRAMIDE-INDUCED AKATHISIA

Akathisia as a side-effect of metoclopramide has received increasing attention in consultation-liaison psychiatry in recent years. A case of metoclopramide-induced akathisia resulting in a suicide attempt is reported in order to highlight the suffering of such patients and the factors that lead to misdiagnosis.     

Evaluation of a Hong Kong Chinese version of a self-administered questionnaire for assessing symptom severity and functional status of carpal tunnel syndrome: cross-cultural adaptation and reliability.

OBJECTIVES: To evaluate the application of a translated version of an established self-administered questionnaire for carpal tunnel syndrome on Chinese patients in Hong Kong. DESIGN: Evaluation of an instrument tool. SETTING: Department of Orthopaedics and Traumatology, Queen Mary Hospital, Hong Kong; Holistic Medical Centre, Aberdeen, Hong Kong. PARTICIPANTS: Patients with carpal tunnel syndrome, translators. MAIN OUTCOME MEASURES: The adaptation was based on forward-backward translation from English to Chinese (Hong Kong) and vice versa. Meetings with translators, investigators, and patients were organised to generate an acceptable version of the questionnaire. A pilot study was carried out on 20 patients and subsequently minor adjustments were added. Fifty patients were recruited to validate the reliability and internal consistency of the questionnaire. RESULTS: The ordinality of response agreed with the original instrument. Test-retest reproducibility showed no significant difference between tests. The Pearson correlation coefficient ranged from 0.83 to 0.93. Internal consistency was good, at 0.85. CONCLUSION: Through the validation of the Hong Kong Chinese version of the questionnaire, we are able to produce an assessment tool for the local patients. Furthermore, we are able to create a platform for: (i) a cross-national and cross-cultural epidemiological comparison as well as a means of (ii) evaluating different types of treatments.     

ACARDIAC TWIN FETUS WITH SEVERE HYDROPS FETALIS AND BILATERAL TALIPES VARUS DEFORMITY

Twin reversed-arterial-perfusion syndrome (TRAPS) is a rare complication of monochorionic twin pregnancies. TRAPS is characterized by the hemodynamic dependence of a “recipient” twin from a “pump” twin. The “recipient” twin exhibits lethal abnormalities, such as acardia and acephaly. Circulatory failure of the normal twin derives from the existence of arterio-arterial and veno-venous anastomoses within the placenta that allow retrograde perfusion of the acardiac twin by blood coming from the normal twin. Acardiac twinning is the most extreme manifestation of the twin-twin transfusion syndrome. This occurs in 1 in 100 monozygotic twin pregnancies and 1 in 35,000 births. We report a case of diamnionic monochorionic female twins in which the acardiac twin demonstrated severe hydrops fetalis and bilateral talipes varus deformity. Cesarean section was performed on a 27-year-old hypertensive gravida 2, para 1 mother for fetal indications at 32 6/7 weeks gestation. The acardiac fetus had a two-vessel umbilical cord measuring 43.5 cm in length and 0.8 cm in diameter. The proximal end inserted into the root of the normal twin's umbilical cord in an acute angle forming a “v” close to the placental disc. Structures rostral to the thorax were absent except for a round mass of flesh with three small buds in place of the head and neck, and bilateral upper extremities. Only the kidneys, right adrenal, small and large intestine, and rudimentary urinary bladder were present. Both feet demonstrated talipes varus deformity. The fetus was severely hydropic. The subcutaneous fat measured 4.5 cm in maximum thickness. The normal twin had a protracted course complicated with respiratory distress syndrome, moderate secundum atrial septal defect with left to right shunt, and thrombocytopenia of prematurity. The baby was eventually discharged after approximately 1 month. At the time of this report, 5 months postpartum, the neonate is growing and developing normally. To our knowledge, this is the first report of severe hydrops fetalis and talipes varus deformity in an acardiac twin.     

Comparison of hand-sewn and stapled esophagogastric anastomosis after esophageal resection for cancer: a prospective randomized controlled trial.

OBJECTIVE: The objective of this study was to compare the hand-sewn and stapled methods in esophagogastric anastomosis. SUMMARY BACKGROUND DATA: After esophageal resection for cancer, the relative merits of the hand-sewn and the stapled methods of esophagogastric anastomosis, especially regarding leakage and stricture rates, have not adequately been studied. METHODS: A prospective randomized controlled trial was undertaken in 122 patients with squamous cell cancer of the thoracic esophagus who underwent a Lewis-Tanner esophagectomy. Patients were stratified according to esophageal size, based on the diameter of the divided esophagus (< or > or = 30 mm) and then were randomized to have either a hand-sewn or a stapled anastomosis. RESULTS: The mean total operating times (standard error of the mean) when the hand-sewn and the stapled methods were used were 214 (4) minutes and 217 (3.4) minutes, respectively (p = not significant [NS]). The respective in vivo proximal resection margins (standard error of the mean) were 8 (0.4) cm and 7.6 (0.4) cm (p = NS). Leakage rates were 1.6% and 4.9% (p = NS). Excluding hospital deaths, patients with leakage or anastomotic recurrence, and those who received radiation therapy to histologically infiltrated resection margin, anastomotic stricture was found in 5 (9.1%) of 55 patients in the hand-sewn group and 20 (40%) of 50 in the stapler group (p = 0.0003). The difference in stricture rates was significant in small as well as large esophagi. Anastomotic recurrence developed in only one patient in each group. CONCLUSIONS: The authors conclude that both methods were safe, but the stapled technique resulted in more stricture formation.     

Proximal gastric vagotomy. The preferred operation for perforations in acute duodenal ulcer.

Simple closure, the conventional operation for perforated acute duodenal ulcers, is associated with symptomatic relapse in a large proportion of patients. In order to assess the role of immediate definitive surgery, 78 fit patients with perforated acute ulcers were prospectively randomized to undergo either closure alone or proximal gastric vagotomy with closure (PGV). Patients taking potentially ulcerogenic drugs or who had severe stress were excluded from the study. Both groups were comparable with respect to age, sex, general medical health, duration of perforation, length of ulcer history, and presence of duodenal scarring. There was no hospital mortality. Minor complications occurred in 7.3% after closure and 10.8% after PGV. At 3 years follow-up, the cumulative recurrence rates were 36.6% and 10.6% after closure and PGV, respectively (p = 0.001). Eighty-five per cent of recurrences after closure were symptomatic, and half of them required reoperation. Duodenal scarring itself did not appear to influence the outcome after closure. PGV was not associated with dumping, diarrhea or other unwanted side effects. Although less than that in chronic ulcers, there is a substantial risk of symptomatic relapse after closure of perforated acute duodenal ulcers. With judicious patient selection, PGV effectively reduces this risk without incurring disabling side effects associated with other ulcer operations.     

Risk analysis in resection of squamous cell carcinoma of the esophagus

A study of risk factors that affect morbidity and mortality in 523 patients with squamous cell cancer of the esophagus who had one-stage resection was undertaken. The 30-day and hospital mortality rates were 5.0% and 15.5%, respectively. Pulmonary complications, malignant cachexia, and surgical complications accounted for 42%, 25%, and 21% of hospital deaths, respectively. Major pulmonary complications occurred in 23% of patients. Multivariate analysis identified six factors that predicted major pulmonary complications: age, mid-arm circumference, percent of predicted FEV₁, abnormal chest radiograph, amount of blood loss, and palliative resection. Three risk groups of pulmonary complications were identified: low, median, and high risk group with complications in 3%, 17%, and 43% of patients, respectively. Significantly, patients with curative resection had a lower hospital mortality rate (9%) than those with palliative resection (20%), p=0.001. Patients with stage I, IIa, or IIb disease had a lower hospital mortality rate (9%) than those with stage III or IV disease (18%), p=0.026. Multivariate analysis identified six factors that predicted hospital death: age, mid-arm circumference, history of smoking, incentive spirometry, number of stairs climbed, and amount of blood loss. Three risk groups of hospital death were identified: low, median, and high risk groups with death in 7%, 30%, and 38%, respectively. Anastomotic leakage rate was 4%. Technical faults were identified in 53% of patients with leakage. Together with other surgical complications, a presumed or apparent technical error was noted in 63% of patients. The identification of high-risk patients and prevention of technical faults can help improve surgical outcome.

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Resumen Se emprendió un estudio sobre los factores de riesgo que afectaron la mortalidad en 523 pacientes con carcinoma escamocelular del esófago sometidos a resección en una etapa en nuestra institución.Las tasas de mortalidad a 30 días y de mortalidad hospitalaria fueron 5% y 15%. Las complicaciones pulmonares, caquexia maligna y quirúrgica representaron 42%, 25% y 21% de las muertes hospitalarias, respectivamente. Complicaciones pulmonares mayores fueron registradas en 23% de los pacientes.El análisis multivariado identificó seis factores que predicen complicaciones pulmonares mayores: edad, circunferencia del brazo, porcentaje del FEV1 predecible, radiografía de tórax anormal, pérdida de sangre durante la operación y resección de tipo paliativo. Se identificaron tres grupos de riesgo de desarrollar complicaciones pulmonares: bajo, medio y alto, con tasas de 3%, 17% y 43% de los pacientes, respectivamente. Los pacientes que recibieron resección curative exhibieron una significativamente menor tasa de mortalidad hospitalaria (9%) en comparación con los que recibieron resección paliativa (20%), p=0.001. Los pacientes con enfermedad en estados I, IIa, IIb exhibieron menor mortalidad hospitalaria (9%) en comparación con los estados III o IV (18%), p=0.026. El análisis multivariado identificó seis factores que predicen mortalidad hospitalaria: edad, circunferencia del brazo, historia de tabaquismo, espirometría de incentivo, número de escalones que puede ascender y pérdida de sangre durante la operación. Se identificaron tres grupos de riesgo de mortalidad hospitalaria: bajo, medio y alto, con tasas de 7%, 30% y 38% respectivamente.La tasa de fuga anastomótica fue 4% y se identificaron defectos técnicos en 53% de los pacientes. Junto con otras complicaciones quirúrgicas, se observó un error técnico presumible o aparente en 63% de los pacientes.La identificación de los pacientes de alto riesgo y la prevención de los errores técnicos pueden ayudar a mejorar el pronóstico.

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Résumé Dans cette étude, on a étudié les facteurs de risque influençant la morbidité et la mortalité chez 523 patients ayant un cancer épidermoïde de l'oesophage et ayant eu une résection en un seul temps. La mortalité à 30 jours et la mortalité hospitalière ont été respectivement de 5% et de 15.5%. Les complications pulmonaires, la cachexie maligne et les complications chirurgicales ont été responsable respectivement de 42%, 25% et 21% des décès hospitaliers. Une analyse multifactorielle a permis d'identifier six facteurs prédictifs des complications pulmonaires: l'âge, la circonférence brachiale, la prévision du volume expiratoire forcé en une seconde, les anomalies de la radiographie thoracique, la quantité de sang perdu, et le caractère palliatif de la résection. Trois groupes, dont le risque de complications pulmonaires a été classé faible, moyen et élevé, ont été identifiés. Le taux de complications dans ces groupes ont été respectivement de 3%, 17% et 43%. Les patients ayant eu une résection à visée curative avaient une mortalité hospitalière significativement plus basse (9%) comparée à celle des patients ayant eu une résection à visée palliative (20%) (p=0.001). Les patients ayant des maladies de stades I, IIa, IIb avaient une mortalité plus basse (9%) que ceux qui avaient des stades III ou IV (18%), (p=0.026). L'analyse multifactorielle a permis d'identifier six facteurs prédictifs de la mortalité hospitalière: l'âge, la circonférence brachial, des antécédents de consommation excessive du tabac, la spirométrie, le nombre d'escaliers que le patient peut monter, et la quantité de sang perdu. Trois groupes de patients, dont le risque de mortalité hospitalière a été classé faible, moyen, et élevé, ont eu des décès dans respectivement 7%, 30% et 38% des cas. Le taux de fistule a été de 4%. Une faute technique a été identifiée chez 53% des patients ayant eu une fistule. Une faute technique apparente ou présumée a été identifie chez 63% des patients ayant eu soit une fistule soit une complication chirurgicale. L'identification des patients à haut risque et la prévention des fautes techniques peuvent contribuer à améliorer le pronostic après chirurgie.

     

Relationship between intracranial pressure and intracranial volume in craniosynostosis.

Premature fusion of cranial sutures in craniosynostosis has been thought to lead to craniostenosis, which in turn may lead to increased intracranial pressures. In 41 consecutive patients with craniosynostosis, intracranial pressure and intracranial volume were measured. Of the 41 patients, 38 (92.6%) had raised intracranial pressure but only 4 (9.7%) had a decreased skull volume. In the present study, there is no correlation between intracranial volume and intracranial pressure. This study confirms that the measurement of intracranial volume, a non invasive procedure, cannot be used to assess intracranial pressure and to avoid an invasive procedure.