RESUMEN
Conjoined twins represent a rare but fascinating congenital condition, the aetiology of which remains obscure. Over the past four decades, the paediatric surgeons at Red Cross Children's Hospital have been involved in the management of 46 pairs of conjoined twins, of which 33 have been symmetrical and 12 asymmetrical. Seventeen symmetrical twins have undergone separation with 22 children (65%) surviving; all of the live asymmetrical twins survived separation. We describe the important features of this unique cohort, outline our approach to management and present the results of this approach. We consider some of the ethical and moral dilemmas we have confronted, and discuss the prenatal diagnosis, obstetric implications and postnatal care of these children, including the relevant investigations and anaesthetic and surgical management. Specific aspects related to the cardiovascular system, hepatobiliary and gastrointestinal tracts, urogenital tract, central nervous system and musculoskeletal system are highlighted.
Asunto(s)
Enfermedades en Gemelos/epidemiología , Hospitales de Condado/estadística & datos numéricos , Hospitales Pediátricos/estadística & datos numéricos , Gemelos Siameses , Adolescente , Adulto , Diagnóstico Diferencial , Enfermedades en Gemelos/diagnóstico , Enfermedades en Gemelos/cirugía , Procedimientos Quirúrgicos Electivos/métodos , Femenino , Humanos , Incidencia , Lactante , Recién Nacido , Persona de Mediana Edad , Embarazo , Diagnóstico Prenatal , Pronóstico , Estudios Retrospectivos , Sudáfrica/epidemiologíaRESUMEN
UNLABELLED: The liver transplant programme for infants and children at Red Cross War Memorial Children's Hospital is the only established paediatric service in sub-Saharan Africa. Referrals for liver transplant assessment come from most provinces within South Africa as well as neighbouring countries. PATIENTS AND METHODS: Since 1987, 81 children (range 6 months-14 years) have had 84 liver transplants with biliary atresia being the most frequent diagnosis. The indications for transplantation include biliary atresia (48), metabolic (7), fulminant hepatic failure (10), redo transplants (3) and other (16). Four combined liver/kidney transplants have been performed. Fifty-three were reduced-size transplants with donor/recipient weight ratios ranging from 2:1 to 11:1 and 32 children weighed less than 10 kg. RESULTS: Sixty patients (74%) survived 3 months-14 years post-transplant. Overall cumulative 1- and 5-year patient survival figures are 79% and 70% respectively. However, with the introduction of prophylactic intravenous ganciclovir and the exclusion of hepatitis B virus (HBV) IgG core Ab-positive donors, the 1-year patient survival is 90% and the projected 5-year paediatric survival is > 80%. Early (< 1 month) post-liver-transplant mortality was low. Causes include primary malfunction (1), inferior vena cava thrombosis (1), bleeding oesophageal ulcer (1), sepsis (1) and cerebral oedema (1). Late morbidity and mortality was mainly due to infections: de novo hepatitis B (5 patients, 2 deaths), Epstein-Barr virus (EBV)- related post-transplantation lymphoproliferative disease (12 patients, 7 deaths) and cytomegalovirus (CMV) disease (10 patients, 5 deaths). Tuberculosis (TB) treatment in 3 patients was complicated by chronic rejection (1) and TB-drug-induced subfulminant liver failure (1). CONCLUSION: Despite limited resources, a successful paediatric programme has been established with good patient and graft survival figures and excellent quality of life. Shortage of donors because of infection with HBV and human immunodeficiency virus (HIV) leads to significant waiting-list mortality and infrequent transplantation.
Asunto(s)
Hospitales de Condado/estadística & datos numéricos , Hospitales Pediátricos/estadística & datos numéricos , Fallo Hepático Agudo/cirugía , Trasplante de Hígado/estadística & datos numéricos , Adolescente , Niño , Preescolar , Femenino , Supervivencia de Injerto , Humanos , Lactante , Trasplante de Hígado/mortalidad , Masculino , Estudios Retrospectivos , Sudáfrica/epidemiología , Tasa de Supervivencia/tendencias , Resultado del TratamientoRESUMEN
BACKGROUND: The liver transplant program for infants and children at the Red Cross Children's Memorial Hospital is the only established pediatric service in sub-Saharan Africa. Since 1985, 250 infants and children have been assessed and 155 accepted for transplantation. METHODS: Since 1987, 76 children (range 6 months to 14 years) have had 79 liver transplants, with biliary atresia being the most frequent diagnosis. The indications for transplantation include biliary atresia (n = 44), metabolic (n = 7), fulminant hepatic failure (n = 10), redo transplants (n = 3), and other (n = 15). Three combined liver/kidney transplants have been performed. Forty-nine were reduced-size transplants with donor: recipient weight ratios ranging from 2:1 to 11:1, and 29 children weighed < 10 kg. RESULTS: Fifty-six (74%) patients survived 3 months to 12 years posttransplant. Cumulative 1- and 5-year patient survival data are 79% and 70%, respectively. However, with the introduction of prophylactic intravenous gancyclovir and the exclusion of hepatitis B virus (HBV) IgG core Ab-positive donors, the projected 5-year pediatric survival has been >80%. Early (<1 month) post-liver-transplant mortality was low, but included: primary malfunction (n = 1); inferior vena cava thrombosis (n = 1); bleeding esophageal ulcer (n = 1); and sepsis (n = 1). Late morbidity and mortality was mainly due to infections: de novo hepatitis B (5 patients, 2 deaths); Epstein-Barr virus (EBV)-related posttransplantation lymphoproliferative disease (12 patients, 7 deaths); and cytomegalovirus disease (10 patients, 5 deaths). Tuberculosis (TB) treatment in three patients was complicated by chronic rejection (n = 1) and TB drug-induced subfulminant liver failure (n = 1). CONCLUSIONS: Despite limited resources, a successful pediatric program has been established with good patient and graft survival figures and excellent quality of life. Shortage of donors due to HBV and human immunodeficiency virus (HIV) leads to significant waiting list mortality and infrequent transplantation.
Asunto(s)
Trasplante de Hígado/métodos , Adolescente , Niño , Preescolar , Control de Enfermedades Transmisibles/métodos , Femenino , Hospitales Pediátricos , Humanos , Terapia de Inmunosupresión/métodos , Lactante , Hepatopatías/clasificación , Hepatopatías/cirugía , Trasplante de Hígado/mortalidad , Masculino , Complicaciones Posoperatorias/epidemiología , Complicaciones Posoperatorias/prevención & control , Cruz Roja , Estudios Retrospectivos , Sudáfrica , Análisis de SupervivenciaRESUMEN
UNLABELLED: Liver transplantation for infants and children has been available in South Africa at a single centre, the only established service in Sub-Saharan Africa, for more than a decade. Current concerns have shifted from an initial target of early post-transplant survival to quality of life in the long-term. MATERIALS AND METHODS: Since 1985, 225 infants and children have been assessed, with 146 accepted for transplantation. Sixty-nine have had 71 orthotopic liver transplants (OLTx). Biliary atresia was the most frequent diagnosis (54%) followed by acute liver failure (ALF) (15%). Waiting list mortality has remained high (23%), particularly for the ALF group (50%). Forty-three were reduced size transplants with donor: recipient weight ratios ranging from 2:1 to 11:1. Twenty-seven were <10 kg. RESULTS: Fifty (74%) survive 1 month-12 years post-transplant. Actuarial survival after 1996 since HBV core antibody positive donor livers were refused and prophylactic IV ganciclovir used has been >82%. Early post-OLTx mortality was low (5%), one primary non-function, one IVC thrombosis, one PV thrombosis, but late morbidity and mortality (20%) was mainly due to viral infection: de novo hepatitis B (five patients, three deaths), EBV-related post-transplantation lymphoproliferative disease (PTLPD) (eight patients, six deaths) and CMV disease (11 patients, five deaths). Tuberculosis prophylaxis, required in six cases, resulted in major morbidity in two and mortality in one. Poor compliance played a significant role in seven deaths. Hypertension requiring medication along with some compromise of renal function has been present in all but two patients. However, all those of school-going age (25) attend school normally and remain in good health and only three of the survivors have abnormal liver function tests. CONCLUSIONS: Successful liver transplantation is possible in a developing country with limited resources. Scarcity of virus-free donors (HBV and HIV) leading to waiting list mortality and infrequent re-transplantation along with long-term consequences of immunosuppression (infection, lymphoma and renal toxicity) remain problems. Intense education of the caregiver and close follow-up, particularly of those living at long distances has partly addressed the compliance problem.
Asunto(s)
Trasplante de Hígado/estadística & datos numéricos , Análisis Actuarial , Adolescente , Antivirales/uso terapéutico , Atresia Biliar/epidemiología , Niño , Preescolar , Femenino , Estudios de Seguimiento , Ganciclovir/uso terapéutico , Hospitales Pediátricos , Humanos , Lactante , Fallo Hepático Agudo/epidemiología , Trasplante de Hígado/mortalidad , Estudios Longitudinales , Masculino , Infecciones Oportunistas/epidemiología , Tamaño de los Órganos , Cooperación del Paciente/estadística & datos numéricos , Cruz Roja , Sudáfrica/epidemiología , Análisis de Supervivencia , Donantes de Tejidos/estadística & datos numéricos , Listas de EsperaRESUMEN
The subject of malrotation and midgut volvulus in infancy and childhood is reviewed from the perspective of experience with 138 patients evaluated in a published series and a further 82 cases seen since. Embryology, historical aspects, clinical presentation, investigation, surgery, and outcome are discussed. The diagnosis of malrotation and volvulus should always be kept in mind when assessing any infant or child with symptoms of vomiting and pain, particularly when the vomiting is bile-stained. The diagnosis cannot be excluded if diarrhea is present, when clinical findings are bland, or even with normal abdominal radiographs. A contrast meal and follow-through should be done and, if correctly interpreted, should be diagnostic. Ultrasound examination may be helpful but is not secure enough to exclude the diagnosis. Laparotomy or laparoscopy is the only way to be sure. Malrotation with its propensity for volvulus is truly a time bomb lying within.
Asunto(s)
Vólvulo Intestinal/diagnóstico , Intestinos/anomalías , Dolor Abdominal/etiología , Bilis , Niño , Femenino , Humanos , Lactante , Recién Nacido , Vólvulo Intestinal/complicaciones , Vólvulo Intestinal/cirugía , Intestinos/embriología , Intestinos/patología , Masculino , Vómitos/etiologíaAsunto(s)
Duodenostomía/métodos , Yeyunostomía/métodos , Yeyuno/anomalías , Yeyuno/cirugía , Anomalías Congénitas/clasificación , Anomalías Congénitas/epidemiología , Anomalías Congénitas/cirugía , Duodenostomía/estadística & datos numéricos , Duodenostomía/tendencias , Humanos , Incidencia , Yeyunostomía/estadística & datos numéricos , Yeyunostomía/tendencias , Análisis de Supervivencia , Resultado del TratamientoRESUMEN
Liver transplantation for infants and children has been available in South Africa for more than a decade. Current concerns have shifted from an initial target of early post-transplant survival to quality of life in the long term. Since 1985, 175 infants and children have been assessed, with 104 accepted for transplantation. Fifty have had orthotopic liver transplants (OLTx), 48 (3 retransplants) in Cape Town and 2 abroad. Biliary atresia was the most frequent diagnosis (52%) followed by acute liver failure (ALF) (16%). Waiting list mortality has remained high (15%), particularly for the ALF group (50%). Thirty-four patients survive 1 month--14 years post-transplant. Early post OLTx mortality was low (7%) but late morbidity and mortality (23%) were mainly due to viral infection: de novo hepatitis B (5 patients, 2 deaths), EBV-related post-transplantation lymphoproliferative disease (PTLPD) (6 patients, 4 deaths) and CMV disease (9 patients, 4 deaths). Tuberculosis prophylaxis, required in 6 cases, resulted in major morbidity in 1. Hypertension requiring medication along with some compromise of renal function has been present in all but 2 patients. However, all those of school-going age (20) attend school normally and remain in good health and only 3 of the survivors have abnormal liver function tests. Successful liver transplantation is possible in a developing country with limited resources. Scarcity of virus-free donors (HBV and HIV) leading to waiting list mortality and infrequent retransplantation along with long-term consequences of immunosuppression (infection, lymphoma and renal toxicity) remain problems.
Asunto(s)
Hospitales Pediátricos , Trasplante de Hígado/métodos , Niño , Supervivencia de Injerto , Mortalidad Hospitalaria , Hospitales Pediátricos/estadística & datos numéricos , Humanos , Trasplante de Hígado/efectos adversos , Trasplante de Hígado/mortalidad , Trasplante de Hígado/estadística & datos numéricos , Trasplante de Hígado/tendencias , Morbilidad , Atención Perioperativa/métodos , Calidad de Vida , Cruz Roja , Derivación y Consulta/estadística & datos numéricos , Sudáfrica/epidemiología , Análisis de Supervivencia , Resultado del Tratamiento , Listas de EsperaRESUMEN
In October 1994 we changed our management of neonatal Hirschsprung's disease from a staged procedure after initial colostomy to a neonatal single-stage Soave endorectal pull-through (SERPT) without a colostomy. We report on our experience with the first 12 neonatal SERPTs, comparing them retrospectively with a consecutive series of 12 patients managed with initial colostomy, followed by a SERPT months later. A neonatal SERPT proved to be an easier and quicker procedure than the same procedure in the older child. Patients could be discharged earlier and fewer complications were encountered; in addition a colostomy was avoided. The cosmetic result and parental acceptance were excellent. At the same time the cost was less than half compared with the staged group. At this stage of follow-up functional results are comparable with previous staged pull-through procedures.
Asunto(s)
Colon/cirugía , Enfermedad de Hirschsprung/cirugía , Recto/cirugía , Anastomosis Quirúrgica/métodos , Femenino , Estudios de Seguimiento , Costos de la Atención en Salud , Enfermedad de Hirschsprung/diagnóstico , Humanos , Lactante , Recién Nacido , Masculino , Complicaciones Posoperatorias , Reoperación , Estudios Retrospectivos , Estomas Quirúrgicos , Resultado del TratamientoAsunto(s)
Quiste del Colédoco/cirugía , Páncreas/cirugía , Conducto Colédoco/cirugía , Humanos , MétodosRESUMEN
The accidental ingestion of corrosive agents is a major cause of oesophageal strictures in children. The mainstay of treatment is repeated dilatations. Despite this, a significant number of patients eventually require oesophageal bypass. We reviewed the records of all cases managed with this condition at the University of Cape Town teaching hospitals between 1976 and 1994. Dilatation therapy alone was successful in 14 out of 39 patients (41%). Morbidity of failed dilatation therapy included repeated hospital admissions over an average 11.5 months and 17 dilatations each. Oesophageal perforations occurred in 7 cases (18%). Early factors predictive of failure of conservative treatment were: delay in presentation of more than 1 month; severe pharyngo-oesophageal burns requiring a tracheostomy; oesophageal perforation; and a stricture longer than 5 cm on radiological assessment. The size of dilators accepted during early bougienage also correlated with eventual outcome. These criteria may be useful in predicting which patients will not respond to repeated dilatations. Early surgical intervention in such cases will prevent fruitless dilatations and related complications.
Asunto(s)
Ácidos/efectos adversos , Quemaduras Químicas/cirugía , Colon/trasplante , Estenosis Esofágica/cirugía , Esófago/cirugía , Hidróxido de Sodio/efectos adversos , Anastomosis Quirúrgica , Quemaduras Químicas/etiología , Preescolar , Estenosis Esofágica/inducido químicamente , Esofagoscopía , Femenino , Estudios de Seguimiento , Humanos , Lactante , Masculino , Estudios Retrospectivos , Rotura , Resultado del TratamientoRESUMEN
Impalpable testes constitute up to 20% of cases of undescended testes and present both a diagnostic and a therapeutic problem. Knowledge of testicular site prior to orchidopexy is useful in planning surgical strategy. Misdirected blind inguinal exploration can be avoided. Many imaging techniques are time-consuming and expensive and can neither confidently locate an impalpable testis nor exclude its absence. Recently laparoscopy has been advocated as a safe and efficient method of identifying presence and site of an impalpable testis. The first stage of a divided-vessel orchidopexy may also be performed simultaneously. Fifty children with 60 impalpable testes--40 unilateral and 10 bilateral--underwent laparoscopic examination prior to groin exploration. In 5 (8%) an inguinal testis was found which had been missed on pre-operative palpation. The testis was absent in 23 (38%), of whom 13 had a limited negative groin exploration because cord structures were seen entering the internal ring. In 30 (50%) an intra-abdominal testis was identified; 14 were relocated into the scrotum with a standard orchidopexy, 16 required a divided-vessel orchidopexy. In 2 (4%) intra-abdominal testicular remnants were removed. There was no operative morbidity. We conclude that laparoscopy is a useful technique in the management of children with impalpable testes. Open perumbilical insertion of the trochar is a safe method of introducing the telescope into the peritoneal cavity. In addition to accurately identifying a testis in 60%, 40% were spared the pain and morbidity of an extensive groin exploration.
Asunto(s)
Criptorquidismo/diagnóstico , Criptorquidismo/cirugía , Laparoscopía/métodos , Testículo/cirugía , Niño , Preescolar , Humanos , Lactante , Laparoscopios , MasculinoRESUMEN
Patients with congenital diaphragmatic hernias (CDH) usually present soon after birth with respiratory distress. Occasionally presentation is delayed. Twelve patients with delayed presentation (outside the neonatal period) of a total of 91 with CHD were seen over 17 years (1977-1994). Case records were reviewed to identify reasons for late presentation, evidence of morbidity, treatment and outcome. Age at presentation ranged from 6 weeks to 30 months (mean 8 months). Ten patients were aged 6 months or younger and there was an equal number of boys and girls. Seven cases were left-sided, 4 right-sided and 1 was anteriorly placed and bilateral. Ten patients presented acutely, 5 with small-bowel obstruction and 5 with respiratory distress, but all of the latter had a history of previous recurrent chest infection. Diagnosis was confirmed by radiographic examination of the chest in 10, barium meal in 1 and a 5-month-old patient had an inappropriate laparotomy and gastrojejunostomy for duodenal obstruction at an outlying hospital. Two had documented normal chest radiographs 3 and 6 months prior to diagnosis. Patients who presented with gastro-intestinal symptoms were older (mean age 1 year) compared with those with predominantly respiratory symptoms (mean age 19 weeks). None had any evidence of bowel strangulation. All were repaired via an abdominal approach. A hernial sac was found in half the cases (4/7 left and 2/5 right). Five had non-rotation of bowel. Primary repair without patch was carried out in all. One child with lung hypoplasia and multiple cardiac abnormalities died; the others recovered well. Delay in presentation of CDH is not uncommon (14% in this series). Most patients will have respiratory symptoms. A normal previous chest radiograph does not exclude the diagnosis. All patients with recurrent chest infection should have a contrast meal investigation early on. Long-term prognosis is favourable and postoperative morbidity is minimal, despite late presentation.
Asunto(s)
Hernia Diafragmática/diagnóstico por imagen , Hernias Diafragmáticas Congénitas , Preescolar , Femenino , Hernia Diafragmática/cirugía , Humanos , Lactante , Masculino , Radiografía , Factores de TiempoRESUMEN
This paper records our experience in the management of 25 sets of conjoined twins seen over a 32 year period (1964 - 1996). The twins were classified into 14 complete and symmetrical sets and 11 incomplete or heteropagus. The 14 symmetrical sets included 9 thoracopagus, 2 ischiopagus, 1 craniopagus and 1 omphalopagus twins. In the incomplete heteropagus group there was 1 ischiopagus, one twin being anencepahlic, 2 dipygus, 5 parasitic, 2 fetus-in-fetu and I cranial and caudal. The management is detailed case by case. Overall 10 of 14 symmetrical sets underwent attempts at separation with 16 surviving the procedure, but there were 3 late deaths. In the incomplete group 10 of 11 were operated on with 9 survivors. The importance of a multi-disciplinary approach, the extensive investigations required pre-operatively to define areas of organ and bony conjunction, congenital anomalies of each twin and surgical teamwork is emphasized. Specific problems encountered were identified. In thoracopagus twins the hearts were of paramount importance as conjuction was usually fatal, being associated with major congenital defects. The greater the extent of thoracic cage fusion the greater the chance of associated severe anomaly. Skin expansion to assist coverage of the defects after separation was of great assistance, as was the use of collagen coated vicryl. Evaluation of the liver and pancreatico-biliary systems with isotope excretion scanning was crucial to pre-operative planning. Where there was fusion of the duodenum a single pancreatico-biliary system could be expected and prior strategies for separation and Roux-en-Y enteric drainage of both pancreatic and biliary secretion should be planned. Gastro-oesophageal reflux led to considerable morbidity in both twins of a thoraco-omphalopagus set. In ischiopagus and dipygus conjoined twins bilateral posterior iliac osteotomies were an essential component to anatomic reconstruction of the pelvic ring and wound closure. Also in this group, due to the frequency and extent of shared genital, urinary and ano-rectal structures, long-term morbidity was expected and a component of this might be due to spinal cord tethering, or as in one of our cases, a progressive hydrosyringomyelia. Timing of separation was ideally set at between 5 and 9 months with 6 to 8 weeks of prior tissue expansion but earlier operation was frequently required because of cardio-respiratory problems or organ failure in one twin. In most cases the goal of obtaining separate, independent and intact individuals was achieved.
Asunto(s)
Gemelos Siameses/cirugía , Anomalías Múltiples/mortalidad , Anomalías Múltiples/cirugía , Femenino , Estudios de Seguimiento , Humanos , Recién Nacido , Masculino , Sudáfrica/epidemiología , Factores de Tiempo , Gemelos Siameses/clasificaciónRESUMEN
Controversy persists with regard to the diagnosis and treatment of the acute scrotum in children. The differential diagnosis includes torsion of the testis, torsion of one of the appendices testis and epididymo-orchitis. Clinical differentiation is notoriously difficult and our policy has been to explore the scrotum of all who present with signs of an acute scrotum. The medical records of 199 boys aged less than 13 years who presented with an acute scrotum at Red Cross War Memorial Children's Hospital in Cape Town during the period 1970-1996 were retrospectively reviewed. Diagnosis was made clinically in all cases. Sixty-two boys (31%) were found on exploration to have torsion of the testis (mean age 6.3 years), the left side being affecting 2.5 times more frequently than the right (1 neonate had bilateral torsion), 62 (31%) had torsion of testicular appendages and 56 (28%) had epididymo-orchitis. Nineteen were grouped separately and 13 (6.5%) of these were treated conservatively. Boys needing an orchidectomy (N = 38) for testicular torsion presented three times later than those who did not (48 v. 16.5 hours). Successful conservation of the testis was directly related to the time interval of symptom development and surgical derotation. Boys with torsion of a testicular appendage presented later (mean 51 hours) and were older (mean 10 years) than boys in the other two groups. Those with epididymo-orchitis were younger (mean 3.3 years) and 40% were younger than 1 year. Only in one-third was there any clinical supportive evidence of the diagnosis. Surgical complications were infrequent. We advocate expedient surgical exploration and derotation with contralateral orchidopexy, since clinical differentiation between torsion of the testis, testicular appendages and epididymo-orchitis is inexact, even with highly specialised investigation such as Doppler ultrasound and radionuclide scanning, which may not be widely available, may delay definitive treatment and suggest a lack of urgency where time is of the essence.
Asunto(s)
Enfermedades de los Genitales Masculinos/diagnóstico , Dolor/etiología , Escroto , Enfermedad Aguda , Niño , Preescolar , Diagnóstico Diferencial , Epididimitis/diagnóstico , Epididimitis/terapia , Enfermedades de los Genitales Masculinos/terapia , Humanos , Lactante , Recién Nacido , Masculino , Orquiectomía , Estudios Retrospectivos , Escroto/cirugía , Torsión del Cordón Espermático/diagnóstico , Torsión del Cordón Espermático/terapia , Factores de TiempoRESUMEN
Abdominal aortic aneurysms are rare in children. Causes include mycotic aneurysms, vasculitides (eg, Takayasu's arteritis), connective tissue diseases (eg, Marfan's syndrome, Ehlers-Danlos syndrome, and tuberous sclerosis) and traumatic false aneurysms. Four cases are described. Case 1 was a 12-year-old boy who presented with an acute unheralded rupture of the subdiaphragmatic aorta accompanied by lower limb paralysis and ischemia. Attempted repair failed because of extensive friability of the large arteries. Histological evaluation confirmed cystic medial necrosis despite Marfanoid phenotype. Cases 2 and 3 were boys aged 12 and 11 with Takayasu's arteritis who presented with hypertensive encephalopathy and heart failure. Although both had involvement of the origins of the renal arteries, one aneurysm was predominantly suprarenal and the other infrarenal. Currently both children are being managed successfully with antihypertensive therapy. Case 4 was a 5-year-old girl who presented with hypertension and a pulsatile abdominal mass after treatment of infective endocarditis 18 months previously. Arteriography and three-dimensional computed tomography confirmed an aneurysm (6 x 5 x 4 cm) arising from the aorta and involving the right renal artery. Aneurysmectomy, removal of a small ischemic right kidney, and Gore-Tex grafting resulted in cure of the hypertension and uneventful recovery. The present series confirms that rupture is a fatal complication, renovascular complications are common, and medical control of hypertension is an essential part of management. Management strategies need to be highly individualized, and may be successful without surgical intervention. Close clinical and ultrasound follow-up of those managed nonoperatively is essential.
Asunto(s)
Aneurisma Infectado/cirugía , Aneurisma de la Aorta Abdominal/cirugía , Factores de Edad , Niño , Preescolar , Femenino , Estudios de Seguimiento , Humanos , Masculino , Pronóstico , Arteritis de Takayasu/terapia , UltrasonografíaRESUMEN
One hundred seventy-eight of 330 patients were recalled after undergoing surgery for histologically proven Hirschsprung's disease (HD). One hundred fifteen were older than 4 years at interview (Mean age, 10 years). This sample appeared to be representative of the whole in terms of demographic features such as ethnic group, sex, length of aganglionic segment, timing of presentation and surgery performed. Anthropomorphic indices for weight and height were comparable to norms, but many younger patients were below expected weight for age. In general, weight and height for age was regained with time. Nine patients had delayed developmental milestones, which were owing to specific causes in four. Nine patients had a poor functional outcome, of which two had neurological impairment. Satisfactory school performance was achieved in all but 19 (26%) of the remaining patients. Long-term functional results were comparable for the Soave and Duhamel procedures with less favorable results noted following the Swenson procedure. Assessment of complications demonstrated a significantly (P < .01) lower incidence of constipation, sexual dysfunction, and micturition disturbance following the Soave procedure when compared with the Duhamel and Swenson procedures. Neurological impairment and length of aganglionic segment beyond the rectosigmoid area appeared to influence functional outcome, as did persisting enterocolitis. Enterocolitis was observed in 16.6% of patients on presentation, but continued in only 6%. Constipation was particularly associated with the Duhamel procedure, and a higher incidence of micturition disturbance, abdominal distension, and cuff stricture was noted following the Swenson procedure. Functional assessment by three different scoring methods showed that 86 (74.7%) of the 115 patients over the age of 4 had excellent anorectal function and appeared to be well adjusted. Twenty-two patients (19.2%) had relatively minor long-term problems but seven (6.1%) had persistent fecal soiling with resulting psychosocial maladjustment.
Asunto(s)
Enfermedad de Hirschsprung/cirugía , Complicaciones Posoperatorias , Calidad de Vida , Canal Anal/fisiología , Niño , Preescolar , Defecación/fisiología , Escolaridad , Incontinencia Fecal/etiología , Estudios de Seguimiento , Crecimiento , Enfermedad de Hirschsprung/complicaciones , Humanos , Incidencia , Recto/fisiología , Estudios Retrospectivos , Ajuste Social , Resultado del TratamientoRESUMEN
Iatrogenic esophageal perforations in children are rare. To evaluate patterns of injury, clinical presentation, and treatment options for such patients, the authors reviewed the case records of 11 children who had sustained transmural injury to the esophagus during a dilatation procedure at their institution between 1967 and 1994. Strictures requiring dilatation were attributable to caustic ingestion in eight, esophageal atresia repair in two, and congenital stenosis in one. Eight were penetrating injuries, and three were disruptions. Two involved the cervical esophagus; and nine involved the thoracic esophagus. Pain, pyrexia, and tachycardia were early signs. Proximal thoracic perforations led to signs in the left chest region (effusion/pneumothorax); with distal perforation the signs were on the right side. Treatment along conventional lines (local drainage, gastrostomy, primary repair after early recognition, and antibiotic therapy) was successful, with two major complications, both empyemas. The most recent case, a disruption of a tracheoesophageal fistula stricture, was successfully treated with oral water irrigation and antibiotics only. Six had colonic interposition (all caustic ingestion), one required endoesophageal resection of a distal stricture, and four had resolution of the stricture, three without additional dilatation. The majority of children with iatrogenic injuries of the esophagus can be managed successfully by conservative measures and pleural drainage, with surgical procedures reserved for large disruptions of the esophagus, intraabdominal perforations, and cases that do not respond to appropriate conservative measures. Patients with caustic injury to the esophagus have a greater risk for the development of penetrating injury, and this may be one indicator of the severity of scarring. There are distinct clinical patterns of presentation, which depend on the level at which the esophagus is perforated. Dilatation disruption of a localized stricture has a good long-term prognosis for the esophagus and may even cure the stricture. The role of oral irrigation still must be fully evaluated where disruption has occurred as distinct from a penetrating injury.
Asunto(s)
Perforación del Esófago/etiología , Adolescente , Antibacterianos/uso terapéutico , Quemaduras Químicas/terapia , Cateterismo/efectos adversos , Niño , Preescolar , Colon/trasplante , Drenaje , Empiema/etiología , Atresia Esofágica/cirugía , Perforación del Esófago/diagnóstico , Perforación del Esófago/cirugía , Estenosis Esofágica/congénito , Estenosis Esofágica/etiología , Estenosis Esofágica/terapia , Femenino , Fiebre/diagnóstico , Gastrostomía , Humanos , Enfermedad Iatrogénica , Lactante , Masculino , Dolor , Derrame Pleural/diagnóstico , Neumotórax/diagnóstico , Complicaciones Posoperatorias , Estudios Retrospectivos , Taquicardia/diagnóstico , Irrigación Terapéutica , Fístula Traqueoesofágica/terapia , Resultado del TratamientoRESUMEN
La cirugia ambulatoria se ha transformado en un importante componente dentro de la pra ctica quirurgica. En 1987 se crea una unidad de Hospital de Dia en el Hospital de Niños de la Cruz Roja en Sudafrica. Evaluamos la experiencia acumulada en un periodo de 8 años (1987-1994) y la relacionamos con la cantidad total de intervenciones quirurgicas, la preparacion preoperatoria, utilizacion diaria de las instalaciones y cuidados postoperatorios junto con las complicaciones. En ese periodo se operaron 20.545 pacientes con una edad promedio de 3 anos. Participaron 9 sub-especialidades. El asesoramiento y la preparacion prequirurgica redujeron el porcentaje de suspension el dia de la cirugia a menos del 6 por ciento con un uso efectivo de las horas programadas de quirofano del 90 por ciento. Los escasos recursos humanos de enfermeria fueron tambien optimizados (relacion Paciente/Enfermera=11/1). El seguimiento postoperatorio de todos los procedimientos inguino-escrotales arrojo un porcentaje de complicaciones menor al 4 por ciento. La mayor parte de los procedimientos se efectuaron en menos de 30 minutos y solo el 0,8 por ciento excedio 1 hora de tiempo quirurgico. El promedio de tiempo de internacion fue de 6 horas. No se presentaron mayores complicaciones y la aceptacion por parte de los padres y los pacientes fue excelente. Solo 16 niños requirieron hospitalizacion prolongada durante la noche. Presentamos recomendaciones para establecer y mejorar los servicios del Hospital de Dia. Se puede concluir que las comunidades y las autoridades de los paises en vias de desarrollo pueden establecer un servicio de Hospital de Dia efectivo practico y eficiente, con bajos costos, expendiendo las instalaciones para la practica de cirugia ambulatoria
Asunto(s)
Procedimientos Quirúrgicos Ambulatorios , Centros de Día , PediatríaRESUMEN
La cirugia ambulatoria se ha transformado en un importante componente dentro de la pra ctica quirurgica. En 1987 se crea una unidad de Hospital de Dia en el Hospital de Niños de la Cruz Roja en Sudafrica. Evaluamos la experiencia acumulada en un periodo de 8 años (1987-1994) y la relacionamos con la cantidad total de intervenciones quirurgicas, la preparacion preoperatoria, utilizacion diaria de las instalaciones y cuidados postoperatorios junto con las complicaciones. En ese periodo se operaron 20.545 pacientes con una edad promedio de 3 anos. Participaron 9 sub-especialidades. El asesoramiento y la preparacion prequirurgica redujeron el porcentaje de suspension el dia de la cirugia a menos del 6 por ciento con un uso efectivo de las horas programadas de quirofano del 90 por ciento. Los escasos recursos humanos de enfermeria fueron tambien optimizados (relacion Paciente/Enfermera=11/1). El seguimiento postoperatorio de todos los procedimientos inguino-escrotales arrojo un porcentaje de complicaciones menor al 4 por ciento. La mayor parte de los procedimientos se efectuaron en menos de 30 minutos y solo el 0,8 por ciento excedio 1 hora de tiempo quirurgico. El promedio de tiempo de internacion fue de 6 horas. No se presentaron mayores complicaciones y la aceptacion por parte de los padres y los pacientes fue excelente. Solo 16 niños requirieron hospitalizacion prolongada durante la noche. Presentamos recomendaciones para establecer y mejorar los servicios del Hospital de Dia. Se puede concluir que las comunidades y las autoridades de los paises en vias de desarrollo pueden establecer un servicio de Hospital de Dia efectivo practico y eficiente, con bajos costos, expendiendo las instalaciones para la practica de cirugia ambulatoria