Transitional Care in Patients With Hirschsprung Disease: Those Left Behind.

BACKGROUND: The long-term effects of Hirschsprung disease are clinically variable. An improved understanding of challenges patients may face as adults can help inform transitional care management. OBJECTIVE: To explore the outcomes and transitional care experiences in adult patients with Hirschsprung. DESIGN: Cohort study. SETTING: Single center. PATIENTS: All patients treated for Hirschsprung between 1977 and 2001 (aged older than 18 years at the time of survey distribution in July 2018-2019). Eligible patients were sent validated multidomain surveys and qualitative questions regarding their transitional care. MAIN OUTCOME MEASURES: Status of transitional care, bowel function, and quality-of-life assessment. Qualitative analysis of transitional care experience. RESULTS: Of 139 patients, 20 had received transition care (10 had at least 1 visit but had been discharged and 10 were receiving ongoing follow-up). These patients had inferior bowel function and quality-of-life scores at follow-up. Twenty-three patients (17%) had issues with soiling at the time of discharge, and 7 patients received transitional care. Of these 23 patients, 9 (39%) had a normal Bowel Function Score (17 or more), 5 (22%) had a poor score (less than 12), and 1 had since had a stoma formation. Eighteen patients (13%) had active moderate-severe issues related to bowel function, only 5 had been transitioned, and just 2 remained under ongoing care. Importantly, when these patients were discharged from our pediatric center, at a median age of 14 (interquartile range, 12-16) years, 10 of 17 patients had no perceptible bowel issues, suggesting a worsening of function after discharge. LIMITATIONS: The retrospective design and reliance on clinical notes to gather information on discharge status as well as patient recall of events. CONCLUSIONS: There remains a small but significant proportion of Hirschsprung patients for whom bowel function either remains or becomes a major burden. These results support a need to better stratify patients requiring transitional care and ensure a clear route to care if their status changes after discharge. See Video Abstract . ATENCIN DE TRANSICIN EN PACIENTES CON ENFERMEDAD DE HIRSCHSPRUNG, LOS QUE SE QUEDAN ATRS: ANTECEDENTES:Los efectos a largo plazo de la enfermedad de Hirschsprung son clínicamente variables. Una mejor comprensión de los desafíos que los pacientes pueden enfrentar cuando sean adultos puede ayudar a informar la gestión de la atención de transición.OBJETIVO:Explorar los resultados y las experiencias de atención de transición en pacientes adultos con Hirschsprung.DISEÑO:Estudio de cohorte.AJUSTE:Unico centro.PACIENTES:Todos los pacientes tratados por Hirschsprung 1977-2001 (edad >18 años en el momento de la encuesta, Julio de 2018-2019). A los pacientes elegibles se les enviaron encuestas multidominio validadas, así como preguntas cualitativas sobre su atención de transición.PRINCIPALES MEDIDAS DE RESULTADOS:Estado de la atención de transición, función intestinal y evaluación de la calidad de vida. Análisis cualitativo de la experiencia de cuidados transicionales.RESULTADOS:De 139 pacientes, 20 habían recibido atención de transición (10 tuvieron al menos una visita pero habían sido dados de alta y 10 estaban recibiendo seguimiento continuo). Estos pacientes tenían puntuaciones inferiores de función intestinal y calidad de vida en el seguimiento. Veintitrés (17%) pacientes tuvieron problemas para ensuciarse en el momento del alta y 7 recibieron atención de transición. De estos, 9/23 (39%) tenían una puntuación de función intestinal normal (≥17), 5/23 (22%) tenían una puntuación baja (<12) y un paciente había tenido desde entonces una formación de estoma. Dieciocho (13%) pacientes tenían problemas activos de moderados a graves relacionados con la función intestinal, solo cinco habían realizado la transición y solo 2 permanecían bajo atención continua. Es importante destacar que cuando estos pacientes fueron dados de alta de nuestro centro pediátrico, a una edad promedio de 14 [RIQ 12-16] años, 10/17 no tenían problemas intestinales perceptibles, lo que sugiere un empeoramiento de la función después del alta.LIMITACIONES:El diseño retrospectivo y la dependencia de notas clínicas para recopilar información sobre el estado del alta, así como el recuerdo de los eventos por parte del paciente.CONCLUSIÓN:Sigue existiendo una proporción pequeña pero significativa de pacientes con Hirschsprung para quienes la función intestinal permanece o se convierte en una carga importante. Estos resultados respaldan la necesidad de estratificar mejor a los pacientes que requieren atención de transición y garantizar una ruta clara hacia la atención si su estado cambia después del alta. ( Traducción-Dr. Yesenia Rojas-Khalil ).

Choledochal malformations: global research, scientific advances and key controversies.

Choledochal malformations (CMs) represent a spectrum of relatively rare and complex congenital anomalies, characterized by abnormal dilatation of the biliary tract in the absence of any acute obstruction. Today, almost 20% of CMs can be detected in-utero using maternal ultrasonography. Formal scientometric analysis was used to identify where modern CM research is taking place and perhaps where our attention should be directed in the future. Thus, this article offers a comprehensive review of recent scientific advances relating to CMs including the current understanding of etiology and classification, whilst also discussing key controversies such as risk of malignant transformation and the role of newer modalities of surgical treatment. Although laparoscopic excision of CMs and biliary reconstruction is nowadays feasible and safe, care should be taken before dispensing with standard open techniques, which have minimal complication rates and proven long-term benefit.

Correction to: Choledochal malformations: global research, scientific advances and key controversies.

The last sentence in the first paragraph under subheading "Minimally invasive treatment" was incorrect in the original publication.

Learning from lawsuits: Ten-years of NHS litigation authority claims against 11 surgical specialities in England.

INTRODUCTION: Medico-legal claims are a drain on NHS resources and promote defencive practice. The litigious burden of surgery in England has not been previously described. This paper describes trends over ten years of claims made against the NHS across 11 surgical specialities. MATERIALS AND METHODS: Data were requested for all claims received by the NHS Litigation Authority (NHSLA) from 2004 to 2014. Surgical specialities included cardiothoracic, general, neurosurgery, obstetric, oral and maxillofacial (OMFS), orthopaedic, otorhinolaryngology, paediatric, plastic, urology and vascular surgery. A literature review of peer-reviewed publications was performed with search terms 'NHSLA' and 'Surgery'. RESULTS: The NHS paid out approximately £1.5 billion across 11 surgical specialities from 2004 to 2014. Orthopaedic, obstetric and general surgery received the largest number of claims per year, and paediatric surgery the least. The mean time from registration of claim with the NHSLA to settlement was 25.5 months (range 17.8 months-35 months). Neurosurgery was responsible for the highest average amount paid per claim, and OMFS the lowest. Failure/delay in treatment and/or diagnosis and failure to warn/adequately consent were the three leading types of claim. 806 never events were successfully claimed for during the ten-year period. DISCUSSION AND CONCLUSION: Sharing information and good practice should be a priority for surgical professionals. Lessons learnt from medico-legal claims are transferrable in strategic planning. This pan-speciality report has demonstrated considerable burden on the NHS and should promote improvement in practice on an individual level in addition to providing systems based recommendations to NHS and international organisations.

Growth in children with choledochal malformations: effect of the Roux loop.

PURPOSE: Excision and biliary reconstruction using a Roux loop is the current standard for choledochal malformation (CM). This is un-physiological, delivering bile beyond the duodenum and excluding a significant length of the jejunum from intestinal absorption. We investigated whether this had an effect on post-operative growth. METHODS: Retrospective case-note analysis of children surgically treated for CM. Growth variables were converted to standard deviation scores (SDS) and compared against population norms. P < 0.05 was significant. RESULTS: From 1994 to 2014, 135 children (<16 years) were identified. Median age at surgery was 3.3 (IQR 1.5-7) years. Morphology included: type 1 Cystic (n = 54, 40%), type 1 Fusiform (n = 58, 43%) and type 4 (intra and extra-hepatic) (n = 22, 16%). There was pre-operative growth failure [median weight SDS = -0.4 (-1.2 - 0.4), P = 0.0004] with a similar trend for height [SDS = -0.38 (-1.2 - 0.5), P = 0.08)]. This correlated with presentation bilirubin (r s = -0.24, P = 0.004), GGT (r s = -0.27, P = 0.002) and AST (r s = -0.27, P = 0.002) but not morphology (P = 0.82) or presentation (P = 0.4). Median follow-up was 1.9 (0.6-4.7) years, during which time both height (P = 0.73) and weight (P = 0.45) reverted to normal. CONCLUSION: This is the first report of growth in children with CM following a Roux-loop reconstruction and showed pre-operative growth failure probably attributed to a period of biliary obstruction but catch-up growth when corrected.

Acute testicular pain in children: collaboration in timely management.

Acute scrotal pain in a child is a urological emergency. Surgeons should be involved early and exclude torsion promptly, often by surgical exploration. This short report compares the time taken by different medical practitioners [Emergency Department (ED) doctors and surgeons] to assess children presenting to the ED with acute scrotal pain. A retrospective observational study was carried out in a district general hospital ED over a 2-year period. A total of 205 children presented with testicular pain, 48 of whom were clinically diagnosed with torsion in the ED. Surgeons reviewed patients with testicular pain quicker than ED doctors when they were referred directly from triage (P=0.019). Overall, review by a surgeon was more timely if the patient was referred directly from triage rather than after referral by the ED doctor (P<0.0001). A direct fast-track referral system to surgeons is needed in the ED to prevent unnecessary delays in definitive management of patients with testicular pain.

A modification of the collar-and-cuff sling to elevate the hand.

Elevation of the hand is routinely sought after surgery and with pathology such as inflammation and infection. Many models of sling have been described. The collar-and-cuff model is a traditional low-cost method that is easily learned and applied, is versatile, and customized to each patient. It is, however, frequently misapplied so that it immobilizes the arm but does not produce sufficient elevation. We describe a minor modification that achieves high elevation while retaining all other advantages.