Chief Medical Officer's Report - Evidential Base and Clinical Practice Aspects of Congenital Cardiac Services (August 2013) (PDF 77KB)

Report on Evidential Base and Clinical Practice Aspects of Congenital Cardiac Services The principle drivers that should determine the optimal arrangements for the provision of congenital cardiac services, including  paediatric and adult cardiac surgery, for the population of Northern Ireland is how best those services can be configured to ensure the safest possible care that is of the highest quality possible in order to optimise outcomes and experience for patients and carers. Of necessity, this requires consideration of all requisite supporting services and arrangements to ensure access across the continuum of care. Such a configuration should support safe, high quality service provision on an on-going basis i.e. ensure sustainability as far as can be determined. In addressing this issue, consideration to the changing profile of population need and the evolving nature specialist services is required.  

Minimally invasive repair of Morgagni hernia - A multicenter case series

Children may benefit from minimally invasive surgery (MIS) in the correction of Morgagni hernia (MH). The present study aims to evaluate the outcome of MIS through a multicenter study. National institutions that use MIS in the treatment of MH were included. Demographic, clinical and operative data were analyzed. Thirteen patients with MH (6 males) were operated using similar MIS technique (percutaneous stitches) at a mean age of 22.2±18.3 months. Six patients had chromosomopathies (46%), five with Down syndrome (39%). Respiratory complaints were the most common presentation (54%). Surgery lasted 95±23min. In none of the patients was the hernia sac removed; prosthesis was never used. In the immediate post-operative period, 4 patients (36%) were admitted to intensive care unit (all with Down syndrome); all patients started enteral feeds within the first 24h. With a mean follow-up of 56±16.6 months, there were two recurrences (18%) at the same institution, one of which was repaired with an absorbable suture; both with Down syndrome. The application of MIS in the MH repair is effective even in the presence of comorbidities such as Down syndrome; the latter influences the immediate postoperative recovery and possibly the recurrence rate. Removal of hernia sac does not seem necessary. Non-absorbable sutures may be more appropriate.

Dynamic self-regulating prosthesis in inguinal hernia repair

Inguinal hernia repair is one of the most common surgical procedure performed in Western countries and it consumes a lot of healthcare resources. Several types of different mesh are now disposable and tension-free techniques represent the “golden standard”. In our study, fifty male patients were operated on for inguinal hernia and a PAD (i.e., dynamic self-regulating prosthesis) used for the repair of the inguinal defect: this technique demonstrated to be safe, effective and easy to perform.

Modified patch repair of femoral hernia after inguinal herniorrhaphy

Background and aim. It has been reported that femoral hernias are rather common after a previous repair of inguinal hernia. We herein present a modified patch repair technique for large femoral hernias that develop after a Lichtenstein operation for ipsilateral inguinal hernia. Patients and methods. The modified technique for femoral hernia was applied to three patients who had a Lichtenstein repair for inguinal hernia. All patients were male. Hernia sac is dissected completely and sent back into to the preperitoneal space. Special attention should be given to the prevascular component of the sac. It is dissected as deep as possible into the preperitoneal space over the femoral vein. The defect is quite wide in this particular type of femoral hernia following Lichtenstein repair. A prosthetic patch that matches the defect is prepared. The medial edge of the mesh is configured to correspond to the pubic corner and lacunar ligament. The lateral margin of the patch is cut to create several petals for inverting the mesh above and medial to the femoral vein to prevent prevascular herniation. The mesh is secured to inguinal ligament, ilioinguinal tract, lacunar ligament, and Cooper ligament. Few sutures are put on the pubic corner and lacunar ligament. Results. One patient was discharged after two hours, other two stayed overnight. Readmission because of seroma development was recorded in two cases where standard polypropylene meshes were used. No complication was observed in the other patient who received lightweight meshes. No early recurrences were recorded after 4, 9, and 30 months. Conclusion. Femoral recurrence after previous inguinal hernia repair seems to be a specific entity. It has a prevascular component and the hernia defect can be much larger than that of a primary femoral hernia. A patch repair with infra-inguinal approach can be a valuable alternative with low complication rate.

Small bowel occlusion due to giant perineal hernia: abdominal approach with plastic perineal reconstruction

A perineal hernia is defined as a protrusion of peritoneal or extraperitoneal content through a pelvic floor defect. A 64-year-old woman with a bowel occlusions due to a giant postoperative perineal hernia was admitted to our hospital. We describe abdominal approach with plastic perineal reconstruction.

Incisional hernia in Day Surgery: our personal experience

Incisional hernia is one of the most common complications of laparotomy. Its repair with prosthesis has enabled a considerable improvement in the outcome, significantly reducing recurrences. This study analyses the results of open hernioplasty with mesh performed as a Day Surgery procedure in 42 patients between November 2008 and October 2010. The results were good, with low postoperative morbidity and recurrences (2.4%).

Traumatic abdominal wall hernia. Case report

Despite the high incidence of abdominal traumas, traumatic abdominal wall hernias (TAWHs) remain rare probably because of elasticity of the abdominal wall. The TAWH is due to blunt abdominal trauma with disruption of the abdominal wall muscles and fascia with intact overlying skin. TAWH can be classified into high energy injures (generally motor vehicle accidents) and low energy injures (impact on a small blunt object). Common example of the latter type is a fall onto a bicycle handlebar. The mechanism of the trauma includes sudden increase of intra-abdominal pressure and extensive shear forces applied to the abdominal wall. The diagnosis of TAWH is difficult in the Emergency Room because during the primary diagnostic process most attention is directed toward the detection of internal injures and TAWH can be missed. In this article we report a case of TAWH caused by a work accident (an heavy steel tube fallen onto the abdominal wall of the patient from a height of five meters) with delayed diagnosis.

Visual results with the use of prismatic lenses in the treatment of congenital nystagmus: a clinical case

Purpose: It is important to establish a differential diagnosis between the different types of nystagmus, in order to give the appropriate clinical approach to every situation and to improve visual acuity. The nystagmus is normally blocked when the eyes are positioned in a particular way. This makes the child adopt a posture of ocular torticollis that reduces the nistagmiformes movements, improving the vision in this position. A way to promote the blocking of the nystagmic movements is by using prismatic lenses with opposite bases, to block or minimize the oscillatory movements. This results in a vision improvement and it reduces the anomalous head position. There is limited research on the visual results in children with nystagmus after using prisms with opposing bases. Our aim is to describe the impact on the visual acuity (VA ) of theprescription prism lenses in a nystagmus patient starting at 3 months of age. Methods: Case report on thirty month old caucasian male infant, with normal growth and development for their age, with an early onset of horizontal nystagmus at 3 months of age. Ophthalmic examination included slit lamp examination, fundus, refractive study, electrophysiological and magnetic resonance tests, measurement of VA over time with the Teller Acuity Cards (TAC ) in the distance agreed for the age. At age ten months, the mother noted a persistent turn to the right of the child’s head, which became increasingly more severe along the months. There’s no oscillopcia. At 24 months, an atropine refraction showed the following refractive error: 0D.: -1,50, OS: -0,50 and prismatic lens adapting OD 8 Δ nasal base and OE 8 Δ temporal base. Results: Thirty month old child, with adequate development for their age, with onset of idiopatic horizontal nystagmus, at 3 months of age. Normal ocular fundus and magnetic ressoance without alterations, sub-normal results in electrophysiological tests and VA with values below normal for age. At 6 months OD 20/300; OE 20/400; OU 20/300. At 9 months OD 20/250; OE 20/300; OU 20/150 (TAC a 38 cm). At 18 months OD 20/200; OE 20/100; OU 20/80 (TAC at 38 cm), when the head is turned to the right and the eyes in levoversão, the nystagmus decreases in a “neutral” area. At 24 month, with the prismatic glasses, OD 20/200 OE 20/100, OU20/80 (TAC at 54 cm, reference value is 20/30 – 20/100 para OU e 20/40 – 20/100 monocular), there was an increase in the visual acuity. The child did visual stimulation with multimedia devices and using glasses. After adaptation of prisms: at 30 months VA (with Cambridge cards) OD e OE = 6/18. The child improved the VA and reduced the anomalous head position. There is also improvement in mobility and fine motricity. Conclusion: Prisms with opposing bases., were used in the treatment of idiopathic nystagmus. Said prisms were adapted to reduce the skewed position of the head, and to improve VA and binocular function. Monitoring of visual acuity and visual stimulation was done using electronic devices. Following the use of prismatic, the patient improved significantly VA and the anomalous head position was reduced.

De Garengeot hernia with acute appendicitis

Aim. The presence of the appendix within a femoral hernia sac is a rare condition known as De Garengeot hernia. We report a case of De Garengeot hernia with concomitant appendicitis and a brief review of the literature on the pathogenesis, diagnosis and treatment of this uncommon condition. Case report. A 33 year-old woman was admitted to our Surgical Unit with acute-onset pain and swelling in the right groin region. Clinical signs and ultrasound imaging suggested the presence of a strangulated femoral hernia and the patient was operated on in emergency setting. An inflamed appendix was discovered within the hernia sac. Appendectomy via McBurney incision and prosthetic repair of the femoral ring were performed. The postoperative course was uneventful and at the 2 week and 1 year follow-up no signs of wound infection and no hernia recurrence were found. Conclusion. Since clinical signs are non-specific and radiological findings may often be misinterpreted, appendicitis within a femoral hernia sac is often an incidental finding during an emergency operation for strangulated femoral hernia. Appendectomy-associated hernia repair may be performed with or without prosthesis depending on the extent of surgical field contamination.

Resting energy expenditure, macronutrient utilization, and body composition in term infants after corrective surgery of major congenital anomalies: a case-study

Introduction - Knowledge on the metabolic changes and nutritional needs during the postsurgical anabolic phase in infants is scarce. This analysis explores the associations of resting energy expenditure (REE) and macronutrient utilization with body composition of full-term infants, during catch-up growth after corrective surgery of major congenital anomalies. Methods - A cohort of full-term appropriate for-gestational-age neonates subjected to corrective surgery of major congenital anomalies were recruited after gaining weight for at least one week. REE and macronutrient utilization, measured by respiratory quotient (RQ), were assessed by indirect calorimetry using the Deltatrac II Metabolic Monitor ®. Body composition, expressed as fat-free mass (FFM), fat mass (FM) and adiposity defined as percentage of FM (% FM), was measured by air displacement plethysmography using the Pea Pod ®. Results - Four infants were included at 3 to 5 postnatal weeks. Recommended energy and macronutrient intakes for healthy term infants were provided. Through the study, the median (min-max) REE (Kcal/Kg FFM/d) was 70.8 (60.6-96.1) and RQ was 0.99 (0.72-1.20). Steady increases in both body weight and FFM were associated with initial decrease in FM and adiposity followed by their increase. Low RQ preceded decrease in adiposity. Conclusion - The marked adiposity depletion, not expected during steady weight gain in the postsurgical period, prompts us to report this finding. The subsequent adiposity catch-up was associated with relatively high REE and RQ, suggesting preferential oxidation of carbohydrates and preservation of lipids for fat storage.

Congenital adrenal hyperplasia: focus on the molecular basis of 21-hydroxylase deficiency

Congenital adrenal hyperplasia (CAH) is an autosomal recessive disorder caused by defects in one of several steroidogenic enzymes involved in the synthesis of cortisol from cholesterol in the adrenal glands. More than 90% of cases are caused by 21-hydroxylase deficiency, and the severity of the resulting clinical symptoms varies according to the level of 21-hydroxylase activity. 21-Hydroxylase deficiency is usually caused by mutations in the CYP21A2 gene, which is located on the RCCX module, a chromosomal region highly prone to genetic recombination events that can result in a wide variety of complex rearrangements, such as gene duplications, gross deletions and gene conversions of variable extensions. Molecular genotyping of CYP21A2 and the RCCX module has proved useful for a more accurate diagnosis of the disease, and prenatal diagnosis. This article summarises the clinical features of 21-hydroxylase deficiency, explains current understanding of the disease at the molecular level, and highlights recent developments, particularly in diagnosis.

Minimally invasive repair of Morgagni hernia - A multicenter case series

Children may benefit from minimally invasive surgery (MIS) in the correction of Morgagni hernia (MH). The present study aims to evaluate the outcome of MIS through a multicenter study. National institutions that use MIS in the treatment of MH were included. Demographic, clinical and operative data were analyzed. Thirteen patients with MH (6 males) were operated using similar MIS technique (percutaneous stitches) at a mean age of 22.2±18.3 months. Six patients had chromosomopathies (46%), five with Down syndrome (39%). Respiratory complaints were the most common presentation (54%). Surgery lasted 95±23min. In none of the patients was the hernia sac removed; prosthesis was never used. In the immediate post-operative period, 4 patients (36%) were admitted to intensive care unit (all with Down syndrome); all patients started enteral feeds within the first 24h. With a mean follow-up of 56±16.6 months, there were two recurrences (18%) at the same institution, one of which was repaired with an absorbable suture; both with Down syndrome. The application of MIS in the MH repair is effective even in the presence of comorbidities such as Down syndrome; the latter influences the immediate postoperative recovery and possibly the recurrence rate. Removal of hernia sac does not seem necessary. Non-absorbable sutures may be more appropriate.

Profilaxis antibiótica en la reparación de la hernia inguinal con prótesis: estudio clínico controlado utilizando profilaxis antibiótica en hernioplastias con prótesis, efectuando en los hospitales cantonales del Sígsig y Paute de la provincia del Azuay, 2000-2001

Se realiza un ensayo clínico controlado de Profilaxis antibiótica en la reparación hernia inguinal con prótesis en los hospitales cantonales de Sigsig y Paute, Azuay, Ecuador en el 2000-2001, con el fin de determinar el uso de antibiótico profilaxis en la hernioplastia inguinal utilizando la técnica de Lichteinten [hernioplastia con prótesis] como alternativa quirúrgica, cuando se realiza el abordaje anterior abierto en hernias primarias. Se estudiaron 60 pacientes divididos en dos grupos comparables en cuanto a edad, sexo, procedencia, ocupación, tipo de hernia y su localización. Al grupo de estudio se le administró 2 horas antes del acto quirúrgico 500 mg. de Ciprofloxacina vía oral, realizándose controles a las 72 horas, 8 días y al mes de la intervención quirúrgica, para valorar signos de infección de la herida quirúrgica y posibles complicaciones. La infección de la herida se presentó en un paciente [3.3 por ciento correspondió al grupo que no recibió profilaxis. En el grupo de estudio el seroma se presentó el 6.7 por ciento y el hematoma en el 3.3 por ciento. En el grupo control seroma u orquitis presentaron el 6.7 por ciento, hematoma el 3.3 por ciento y recurrencia de la herida el 3.3 por ciento. No existiendo diferencia al comparar los grupos [p mayor 0.05]. La estadia hospitalaria en ambos grupos fue menor a 24 horas en más del 80 5 de los casos, pudiendo convertirse al prótesis de hernia inguinal en un procedimiento ambulatorio