Definitive Surgical Correction Research Articles

Surgical treatment of Shone’s syndrome and patent ductus arteriosus in an adult

BackgroundShone’s syndrome is a rare complex congenital anomaly. The classical definition consists of four anomalies: supravalvular mitral membrane, parachute mitral valve (PMV), subaortic stenosis, and coarctation of the aorta (CoA). Few studies have been reported on Shone’s syndrome in adults, particularly the primary surgical correction of the anomalies.Case presentationA 22-year-old female patient presented with chest distress and tachypnea. Echocardiography and CT revealed supravalvular mitral membrane, PMV, Bicuspid aortic valve stenosis, CoA and patent ductus arteriosus. She underwent primary definitive surgical correction successfully and was discharged from hospital with symptoms free.ConclusionsOur case report highlights the importance of echocardiographic evaluation in the diagnosis of Shone’s syndrome. The surgical strategy should be tailored according to both the patient’s profile and the surgeon’s personal surgical experience. Extra-anatomical bypass procedure is an appropriate technique for adult patients with long-segment coarctation and concomitant cardiac lesions. The outcomes of the case study indicate that the primary definitive surgery is encouraging.

Balloon-Assisted Rib Graft Placement in Endoscopic Posterior Cricoid Split Procedure.

View Video S1 Laryngoscope, 132:212–214, 2022

Malrotation of Small Bowel with Midgut Volvulus in a COVID-19 Positive Young Male: A Rare Case Report

Malrotation of small bowel with midgut volvulus has a very low incidence of 0.2% in adults. Symptomatic malrotation in neonates occur in one in 6000 live births. The normal 270 degrees counter-clockwise gut rotation around the axis of superior mesenteric artery is absent in such cases. A 20-year-old male presented with intermittent colicky abdominal pain since four to five months, post-prandial bilious vomiting since one week and obstipation since two days. He also had difficulty breathing since two days. Abdominal examination revealed vague lump in right lower abdomen with no signs of peritonitis. Radiological investigations Ultrasonography (USG) and Contrast Enhanced Computed Tomography (CECT) abdomen demonstrated over distended stomach with dilated D1 and D2 showing changes in relation to superior mesenteric artery and superior mesenteric vein with rotation of mesentery in whirlpool pattern. His Reverse Transcriptase-Polymerase Chain Reaction Test (RT-PCR) was positive for Coronavirus Disease-2019 (COVID-19). Conservative trial for two days was followed by surgical exploration which revealed gut malrotation with mid-gut volvulus. Ladd’s procedure was performed. Malrotation with midgut volvulus is an acute surgical emergency that demands high index of suspicion in an adult. Associated COVID-19 pneumonia can increase mortality. Patient was followed-up after three weeks with fruitful results, proving that an early diagnosis and definitive surgical correction of malrotation with midgut volvulus is essential.

Minimally Invasive Repair of Adult Slipped Rib Syndrome Without Costal Cartilage Excision

Slipped rib syndrome (SRS) is a painful disorder caused when 1 or more of the 8th-10th false ribs become abnormally mobile. Established treatment modalities include analgesia, intercostal nerve injection, and costal cartilage excision. No definitive surgical correction of SRS without cartilage excision has been previously described. We aimed to determine whether a nonexcisional repair technique in affected adults could demonstrate significant relief from SRS using standardized outcome measures. We performed a retrospective review of cases of SRS treated at our institution in 2019. We obtained data by survey before and after sutured 10th rib fixation, using a self-reported rating scale from 0-10 at defined intervals. Surveyed outcome measures were compared pre- and postoperatively using the Wilcoxon signed rank sum test. The use of neural modulating, narcotic, and nonsteroidal antiinflammatory drug medications was also compared pre- and postoperatively using McNemar's test where applicable. SRS was diagnosed using clinical examination alone in 42 adults and repaired in 29 patients. Median postoperative improvement in pain at 1 and 6months was 75% (P < .001) and 80% (P < .001), respectively. Improvements in other outcome measures were similar. In patients who took pain medications preoperatively, narcotics were discontinued at 1 month by 100%, neural modulators by 86%, and nonsteroidal antiinflammatory drugs by 92% (all Pvalues < .001). Pain medication use remained minimal at 6 months in 23 (79%) of patients completing follow-up. Minimally invasive slipped rib repair in adults provides significant relief of SRS, offering a useful alternative to costal cartilage excision. It is well tolerated and effective.

ASSOCIATION OF HIRSCHSPRUNG DISEASE WITH WAARDENBURG SYNDROME AND ROLE OF GENE STUDIES: A REVIEW OF 2 CASES

Background: Waardenburg-Shah syndrome type 4 is an association of Waardenburg syndrome with Hirsch sprung disease. Three disease-causing genes have been identified so far: Endothelin receptor type B encoding the endothelia-B receptor, EDN3 encoding an endothelia receptor ligand and Sry-like HMG bOX10 (SOX10) encoding the SOX10 transcription factor. Case Report: This is a review of 2 cases with variable onset of presentation and extent of aganglionic segment. Intervention/Outcome: In case 1, primary pull-through, as definitive surgical correction was done as a single procedure, whereas in case 2, required ileostomy with a plan of definitive surgery later on. Message: Mutation studies are helpful in characterization of the syndrome and counseling to the family. Furthermore, prognosis depends on the length of the ganglionic segment.

Redo pullthrough for Hirschsprung disease.

Pullthrough procedures for Hirschsprung diseases typically have favorable results. However, some children experience long-term postoperative complications comprising stooling disorders, such as intermittent enterocolitis, severe stool retention, intestinal obstruction, as well as incontinence. Reoperative Hirschsprung Disease surgery is complex. This begins with the workup after the initial presentation following primary pullthrough, continues with the definitive surgical correction with redo pullthrough, and ends with long-term follow-up of individuals. The decision tree can be varied with each patient. The operating pediatric surgeon must be able to utilize different operations and treatment options available. While lesser procedures may provide relief in a select population, those with residual aganglionosis or transition zone pathology or mechanical problems will likely require a redo pullthrough. Thus, the diagnostic workup, treatment plan, and definitive surgical care should be coordinated, and executed by an experienced, specialized team at a pediatric referral center.

Penile fracture with disruption of both cavernosal bodies and complete urethral rupture in a 15-years-old male: Delayed surgical approach

Penile fracture is defined as the traumatic rupture of the tunica albuginea of the corpus cavernosa usually associated to trauma during sexual intercourse or masturbation. Historically penile fracture has been managed conservatively, but contemporary management includes early surgical exploration. The case presents a 15-year-old male who suffered a blunt penile trauma and was first managed with cystostomy and no penile exploration. Five months after trauma was submitted to definitive surgical correction of both, urethral rupture and bilateral corporal fracture. The proposed surgical techinique was a diamond-shape corpora anastomosis. Surgery did well and after 3 years he presented no late complications.

Laryngeal clefts

Laryngeal clefts

Living with Cleft Lip and Palate: The Treatment Journey

To better understand how individuals with cleft lip and palate (CLP) perceive and experience their treatment process and how these perceptions and experiences change over the life course. Qualitative in-depth semistructured interviews with 11 adults with nonsyndromal complete CLP. Individuals from three Canadian cities were recruited by convenience and theoretical sampling through AboutFace International. The number of participants was determined by the principle of theoretical saturation. The experience of individuals with CLP through the treatment process changes over the life course. In childhood and early adolescence, most individuals experience stigma, negative self-perception, and as a result were more prone to perceiving the treatment process not only as unbearably burdensome but also as fueling their feeling of "defectiveness." In adulthood, participants' self-perception improved, partly because of definitive surgical correction, leading them to realize treatment benefits and reappraise the treatment process as satisfactory rather than burdensome. Subsequently, some individuals pursued further surgeries hoping for additional psychological gains, in lieu of psychosocial interventions addressing the underpinnings of residual feelings of "defectiveness." This led to dissatisfaction and frustration when the procedures did not lead to the hoped-for psychological gains. The results emphasize the importance of self-perception in determining how participants perceive several important aspects of the treatment experience throughout the life course. Further studies should focus on how to incorporate self-perception as an important variable and outcome in the treatment process.

Anomalous Origin of the Left Pulmonary Artery from the Right Pulmonary Artery (Pulmonary Artery Sling)

PULMONARY artery (PA) sling is created by anomalous origin of the left PA from the posterior aspect of the right PA. The anomalous left PA courses over the right mainstem bronchus and then from right to left, posterior to the trachea or carina and anterior to the esophagus, to reach the hilum of the left lung. This compresses the esophagus and the lower trachea and right mainstem bronchus, producing feeding and respiratory problems, such as recurrent cough and respiratory infection, stridor and wheezing. In addition, associated anomalies of the tracheobronchial tree and/or of the cardiovascular system are present in about 50% of the patients.1,2In addition, bronchoscopy can show a pulsating stricture in the trachea. Medical care is supportive until the patient can undergo definitive surgical correction. Anesthetic management is the same as with any tracheal obstruction; spontaneous ventilation for bronchoscopy and endotracheal intubation bypassing the tracheal restriction for other surgeries. Hypoxemia and respiratory distress are treated with supplemental oxygen and endotracheal intubation. Surgical repair consists of transection of the left PA at its origin and implantation into the main PA anterior to the trachea. Tracheoplasty utilizing end-to-end anastomosis is often needed if the sling is not repaired early. In this case, cardiopulmonary bypass or extracorporeal membrane oxygenation may be required. The anatomy could be correctly diagnosed by magnetic resonance imaging in 97% and by angiography in 90% of patients3 (angiogram shows pulmonary trunk, right PA, and left PA).

Acute Renal Failure Caused by Bilateral Ureteral Herniation Through the Sciatic Foramen

Ureteral herniation is rare. Only a few cases of bilateral ureterosciatic herniation have been reported. We report the case of a 74-year-old woman with flank pain and acute renal failure. The initial ultrasound scan showed bilateral hydronephrosis. Follow-up computed tomography imaging demonstrated sciatic herniation of both ureters, causing bilateral hydronephrosis and hydroureter. The patient underwent bilateral retrograde ureterography and ureteral stent placement, with improvement in renal function to normal limits. Observation after stent removal demonstrated recurrent sciatic herniation of both ureters. Definitive surgical correction was performed by way of laparoscopic bilateral ureterolysis and sciatic notch hernia repair using mesh.

Endograft Failure in an Adult Patient with Coarctation and Bicuspid Aortic Valve

A 25-year-old man presented with associated bicuspid aortic valve and coarctation of the aorta. Following aortic valve replacement, he underwent endovascular stenting of his native coarctation. We describe early failure of the latter procedure that necessitated definitive surgical correction.

EReply. Re: Percutaneous closure of post-myocardial infarction septal defect

We thank Hajj-Chahine et al. for the the eComment on the topic of 'What is the best timing of surgery in patients with post-infarct ventricular septal rupture?' [1-2] Contrary to what the authors conclude, the message from the published review on the management of post-infarction ventricular septal rupture (PIVSR) is clear, If the patient is in cardiogenic shock, due to pulmonary to systemic blood flow ratio shunt rather than infarct size, immediate surgery should follow resuscitation measures and cardiac support. Additionally we state, If there is clinical deterioration, immediate surgery is indicated. The data from the literature suggests that the only clinical scenario for which one can delay surgery is in patients with complete haemodynamic stability (a small cohort for this condition), where the outcomes of surgery are demonstrably better as scar tissue at infarct site is matured to allow durable repair. This subgroup invariably involves haemodynamically stable patients with multiple co-morbidities and a high-risk of operative death. At 2-3 weeks a lower-risk elective procedure can be performed that allows myocardial scar tissue to form facilitating PIVSR repair. This group can only be judged on a case-by-case basis by the heart team for the most optimal intervention and its timing. Additionally the authors should note that the guidelines were published in 2004 [3]. Three out of the six papers we based our conclusions on were published since the guidelines. The largest one of this is the series by Arnoutakis et al. that analysed outcomes in 2876 patients from the STS national database where operative mortality was 54.1% if they were operated on within 7 days from acute myocardial infarction and 18.4% if the repair took place after that. As the time interval between AMI and PIVSR repair became longer, the odds of dying became progressively lower (<6 h: odds ratio [OR] = 6.18; 6-24 h: OR = 5.53; 1-7 days: OR = 4.59; 8-21 days: OR = 2.37, all P <0.01) [4]. With regard to the transcatheter options in such patients we have previously published a BET review on this topic that specifically asked, Which patients might be suitable for a septal occluder device closure of postinfarction ventricular septal rupture rather than immediate surgery? [5]. In addition to the case reports mentioned by Hajj-Chahine et al. the current published cases of percutaneous closure of PIVSR rupture stand at less than 150. These repairs fail for the same reason as surgical failures and the outcomes are not necessarily improved. Procedure-related complications included major residual shunting, left ventricular rupture and device embolization, occurring in up to 41% of patients [5]. Despite the minimally invasive nature of transcatheter techniques, mortality was very high, mainly attributed to disease severity and comorbidities. At present early definitive surgical correction remains the gold standard for PIVSR. Transcatheter techniques have a role in selected patients with simple defects that are 3.5 weeks post AMI) setting where an Amplatzer device may provide definitive treatment. In some patients who are too unwell to be expected to survive an operation, an Amplatzer device may provide stabilization before urgent surgery is undertaken [5]. Conflict of interest: none declared

Vaginal malformations: a proposed classification based on embryological, anatomical and clinical criteria and their surgical management (an analysis of 167 cases)

Although it is virtually impossible to formulate a scheme that can satisfactorily collect all different types of vaginal malformations, a simple classification would be of a considerable value and would permit logical operative decisions. Many classifications of anomalies of uterus and vagina have been proposed: we find them unsatisfactory and confusing, being either too simple or too complex. We propose a new classification, focused only on vagina and based on embryological, anatomical, clinical and surgical criteria. In over 30years, 167 females with vaginal malformations have been observed in our department. Intersex cases were excluded. The encountered anomalies have been divided into six types and ten subtypes: type I vaginal agenesis (IA associated with uterine agenesis/17 Pts, IB isolated/1 Pt); type II vaginal atresia (IIA proximal/1 Pt, IIB distal/4 Pts); type III vaginal atresia with urethrovaginal fistula-urogenital sinus (IIIA proximal fistula-high sinus/42 Pts, IIIB distal fistula-low sinus/55 Pts); type IV vaginal atresia with transverse septum (IVA transverse septum/6 Pts, IVB imperforate hymen/17 Pts); type V disorders of müllerian ducts fusion (VA vaginal duplication/4 Pts, VB longitudinal septum/4 Pts); type VI cloaca/16 Pts. Of each type and subgroup of malformation the appropriate surgical correction is reported. Types of malformation and surgical treatment are analysed: IA vaginal reconstruction using a sigmoid conduit, IB vaginal reconstruction using a sigmoid conduit, atresic cervical resection and uterus-new vagina anastomosis according to Schmid; II perineal vaginal pull-through; IIIA anterior sagittal transanorectal vaginal pull-through, IIIB perineal flap vaginoplasty; IVA excision with abdominovaginal approach, IVB hymen incision; VA tubularization, VB septectomy via perineal approach; VI posterior sagittal anorectal-vaginal-urethroplasty. Most of the patients had good aesthetic and functional results. Type III showed relatively more complications: four redo operations (IIIA), four revisions of the vaginoplasty (IIIB). One patient is still waiting for definitive surgical correction. An early diagnosis is desirable to correct adequately vaginal malformations, which becomes mostly evident around puberty. The better timing for surgery is early age, to obtain better results and to avoid many of the psychological problems that arise at a later age.

Distraction osteogenesis a setup surgery

Surgical correction of craniofacial deformities and asymmetries remains to be an extreme challenge. There are numerous surgical procedures which could be performed in order to correct such deformities. Surgical correction consists of a combination of osteotomies and bone grafting techniques. However, bone grafting techniques have some disadvantages, which include donor site morbidity and graft resorption. Ever since the introduction of distraction osteogenesis (D.O) to the craniofacial region it has been used to correct many congenital and acquired facial deformities. D.O could be used as part of a staged surgical procedure , which may need to be reapplied according to the degree of deformity or it is sometimes used as a definitive surgical correction. We discuss the use of D.O as a set up surgical procedure which creates a more favourable surgical base for further surgical procedures like osteotomies and bone grafts to prevent surgical relapse and failure .We report two cases with facial asymmetry in which DO was used as a set up surgery .In addition , we discuss the indications and list the advantages of D.O in each case.

Long-term results after early definitive surgical correction of complete atrioventricular septal defect with two patch technique and complete cleft closure: ten years single centre experience

Long-term results after early definitive surgical correction of complete atrioventricular septal defect with two patch technique and complete cleft closure: ten years single centre experience

Outcomes of Thoracic Endovascular Aortic Repair for Aortobronchial and Aortoesophageal Fistulas

To identify in-hospital and follow-up outcomes of thoracic endovascular aortic repair (TEVAR) for aortobronchial fistula (ABF) and aortoesophageal fistula (AEF). The authors reviewed all published cases of ABF and AEF undergoing TEVAR indexed in the MEDLINE, Cochrane Library CENTRAL, and EMBASE databases. After removal of duplicates, 850 articles were scrutinized for relevance and validity. Exclusion criteria included: (1) no clear description of the organs involved with the fistula, (2) no description of outcomes after TEVAR for ABF or AEF, or (3) no original data presented in the article. In this manner, 66 relevant articles were identified that included original data on 114 patients (76 men; mean age 63+/-1.5 years) with ABF (n = 71) or AEF (n = 43). Meta-analyses were performed to investigate outcomes of TEVAR for ABF and AEF. Patients with AEF presented more frequently with hypovolemic shock (33% versus 13%, p = 0.012) and systemic infection (36% versus 9%, p<0.001) compared to patients with ABF. In-hospital mortality was 3% (n = 2) after TEVAR for ABF and 19% (n = 8) after TEVAR for AEF (p = 0.004). Additional thoracic surgery in the first 30 days after TEVAR was performed in 3% (n = 2) of ABF patients and in 37% (n = 16) of AEF patients (p<0.001); 12 AEF patients who had received esophageal surgery in the first month after TEVAR showed lower fistula-related mortality during 6 months of follow-up compared to patients who did not receive additional esophageal surgery (p = 0.018). TEVAR is associated with superior outcomes in patients with ABF. Endovascular management of AEF is associated with poor results and should not be considered definitive treatment. TEVAR could serve as a bridge to surgery for emergency cases of AEF only, with definitive open surgical correction of the fistula undertaken as soon as possible.

Clinical indicators of ineffective airway clearance in children with congenital heart disease

To analyse the sensitivity and specificity of clinical indicators of ineffective airway clearance in children with congenital heart disease and to identify the indicators that have high predictive power. The precise establishment of nursing diagnoses has been found to be one of the factors contributing to higher quality of care and cost reduction in healthcare institutions. The use of indicators to diagnose ineffective airway clearance could improve care of children with congenital heart disease. Design. Longitudinal study. Participants consisted of 45 children, <or=1 year of age, with congenital heart disease, who had not had definitive or palliative surgical correction. Six assessments were made at 2-day intervals. Each clinical indicator was defined based on previously established operational criteria. Sensitivity, specificity and positive and negative predictive values of each indicator were calculated based on a model for the longitudinal data. A nursing diagnosis of ineffective airway clearance was made in 31% of patients on the first assessment, rising to 71% on the last assessment, for a 40% increase. Sensitivity was highest for Changes in Respiratory Rates/Rhythms (0.99), followed by Adventitious Breath Sounds (0.97), Sputum Production (0.85) and Restlessness (0.53). Specificity was higher for Sputum Production (0.92), followed by Restlessness (0.73), Adventitious Breath Sounds (0.70) and Changes in Respiratory Rates/Rhythms (0.17). The best positive predictive values occurred for Sputum Production (0.93) and Adventitious Breath Sounds (0.80). Adventitious Breath Sounds followed by Sputum Production were the indicators that had the best overall sensitivity and specificity as well as the highest positive predictive values. The use of simple indicators in nursing diagnoses can improve identification of ineffective airway clearance in children with congenital heart disease, thus leading to early treatment of the problem and better care for these children.

Transcatheter occlusion of a classical BT shunt with the Amplatzer Duct Occluder II

We report on a 57-year-old woman who underwent transcatheter occlusion of a residual classical Blalock-Taussig shunt with the Amplatzer(R) Duct Occluder II (ADO II) 35 years following definitive surgical correction for tetralogy of Fallot.

Pin-Site Myiasis: A Rare Complication of a Treated Open Fracture of Tibia

Pin-site myiasis is a rare complication of external fixation of open fractures of the tibia. Case report and literature review. A 32 year-old man with a history of alcohol and drug abuse presented with an open fracture of the middle third of the fibula and tibia (Gustilo type IIIA) after a motor vehicle crash. Definitive surgical correction of the fracture was made with the insertion of an external skeletal fixator, type Baummer, with 2 bars, 6 pins, and 12 rotules. After three weeks, he presented again with a primary complaint of purulent secretion and maggots at the distal pin site. On removal of the pin, significant destruction of peripheral tissue was evidenced, with a wound approximately 5 cm diameter, from which 105 maggots were extracted, being identified as Cochliomyia hominivorax. There have been no previous reports of myiasis involving an external skeletal fixation and pin, and only one similar case has been reported in a patient with a halo orthosis for the management of a gunshot wound of the neck. As in that case, presumably, in our patient, myiasis occurred via the small infected wound adjacent to the distal pin. Adequate treatment for myiasis requires complete removal of the maggots, as well as antimicrobial therapy.