Optimal timing for plastic surgical procedures for common congenital anomalies:A review

Apart from listening to the cry of a healthy newborn,it is the declaration by the attending paediatrician in the labour room that the child is normal which brings utmost joy to parents.The global incidence of children born with congenital anomalies has been reported to be 3%-6%with more than 90%of these occurring in low-and middle-income group countries.The exact percentages/total numbers of children requiring surgical treatment cannot be estimated for several reasons.These children are operated under several surgical disciplines,viz,paediatric-,plastic reconstructive,neuro-,cardiothoracic-,orthopaedic surgery etc.These conditions may be life-threatening,e.g.,trachea-oesophageal fistula,critical pulmonary stenosis,etc.and require immediate surgical intervention.Some,e.g.,hydrocephalus,may need intervention as soon as the patient is fit for surgery.Some,e.g.,patent ductus arteriosus need‘wait and watch’policy up to a certain age in the hope of spontaneous recovery.Another extremely important category is that of patients where the operative intervention is done based on their age.Almost all the congenital anomalies coming under care of a plastic surgeon are operated as elective surgery(many as multiple stages of correction)at appropriate ages.There are advantages and disadvantages of intervention at different ages.In this article,we present a review of optimal timings,along with reasoning,for surgery of many of the common congenital anomalies which are treated by plastic surgeons.Obstetricians,paediatricians and general practitioners/family physicians,who most often are the first ones to come across such children,must know to guide the parents appropriately and convincingly impress upon the them as to why their child should not be operated immediately and also the consequences of too soon or too late.

Congenital Bilateral Proximal Radio-Ulnar Synostosis in a Nigerian Child

Congenital radio-ulnar synostosis (CRUS) is a rare skeletal malformation of the upper limb and the most common congenital functional disorder of the elbow joint, causing limitation in forearm rotational movements, which may lead to difficulties with some activities of daily living. We reported a 4-year-old girl with congenital bilateral proximal radio-ulnar synostosis who presented with functional discomfort due to limitation of the prono-supination movements of the forearms and abnormal gestures when handling objects. She has clinical and radiological features of congenital radio-ulnar synostosis (CRUS). However, the parents declined corrective surgery because of wrong perception of the condition to be normal. This case highlighted the poor health seeking behavior and wrong illness perception prevalent in the developing countries.

R-I subtype single right coronary artery with congenital absence of left coronary system: A case report

BACKGROUND Isolated single coronary artery is a rare congenital anomaly.R-I subtype single coronary artery is even rarer.In this subtype,a very large right coronary artery extends in the coronary sulcus to the anterior base of the heart where it produces the left anterior descending coronary artery.Currently,only a few case reports are available in the literature for this anomaly.CASE SUMMARY Here,we report the case of a 62-year-old woman who presented to the cardiology clinic with decreased exercise tolerance and poor blood pressure control.The patient underwent coronary angiography(CAG)and emission computed tomography(ECT).CAG images revealed a single gigantic right coronary artery(R-I type)arising from the right coronary sinus with branches supplying the left coronary territory.The ECT results confirmed myocardial ischemia at the location of the absent left coronary artery.The ECT findings confirmed that ischemia was consistent with the vascular loss location in CAG images.In such anomalies,there is a compensatory widening of the coronary artery lumen.Medical treatment was administered,and the patient was discharged.CONCLUSION Isolated single coronary arteries are associated with ischemia and potentially fatal acute coronary events.Hence,controlling risk factors is critical.

Pregnancy and Congenital Uterine Anomalies: Case Series

Background: Congenital Uterine Anomalies are malformations of the Uterus which occur during embryonic life and result from the abnormal formation, fusion or resorption of the Mullerian ducts. Most of them are asymptomatic and diagnosis is done incidentally or during examinations performed for other purposes. We report three cases of women with pregnancies in malformed uteri. Aim: To depict the diagnostic challenges and therapeutic aspects of management of pregnancies in congenitally malformed Uteri. Case Presentation: The first case was a 22-year-old student who came to consult for a second opinion on the management of an ectopic pregnancy. A 2D Ultrasound done prior revealed an ectopic pregnancy but failed to specify its location in a rudimentary uterine horn. Management with a Multi-dose Methotrexate regimen was initiated but progress was not favorable. She came to us for a second opinion on management. Diagnostic laparoscopy was done and revealed an ectopic pregnancy in a rudimentary uterine horn. This was confirmed by histopathology. Management consisted of resection of the rudimentary horn and a right total salpingectomy. The second case was that of a woman who presented with spotting in early pregnancy. An Obstetric 2D ultrasound done revealed a bicornuate Uterus with a Gestational sac in one horn. She was placed on progesterone supplementation for 2 weeks, with regular antenatal contacts. She had an elective cesarean section at 39 weeks. Surgery revealed a complete Bicornuate Uterus. The post-operative period was uneventful with no complications. The third case was that of a woman with a past history of five successive spontaneous abortions, who presented with spotting at about 8 weeks of gestation. An Obstetric Ultrasound done revealed a Bicornuate Uterus and an embryo in one cornus. She was admitted, given her poor obstetric history, for about 14 days and placed on progesterone supplementation till 20 weeks of gestation. Antenatal contacts were regular and she had an emergency cesarean section at 36 weeks. There were no complications in the post operative period. Conclusion: The high degree of diagnostic accuracy makes 3D ultrasound the diagnostic modality of choice. Nevertheless, 2D and Hysterosalpingography can be used as well. Management of pregnancies in women with congenital Uterine anomalies varies per case as presenting symptoms and outcomes with pregnancies are not alike. When diagnosed out of pregnancy, and depending on the type of anomaly, surgical management may be recommended.

Nasal Dermoid Cyst with Sinus Tract Intranasal Bone: A Case Report

Dermoid cysts are benign tumors originating from germ cells, which can form in various locations, including the nasal area in rare cases. They are of unknown exact etiology, but it is suggested that it is due to abnormal tissue migration during early embryonic development. Nasal dermoid cysts albeit rare, can present in various forms such as sinuses, fistulas, or intracranially extending tracts. They can be asymptomatic and incidentally discovered or present with a visible external mass or sinus that is either painful, infected or cosmetically concerning. If nasal dermoid cysts with an intra-nasal bone sinus tract are left untreated, they can lead to life-threatening complications. This report describes the case of a 6-year-old girl with a nasal dermoid cyst connected to a superficial punctum by an intra-nasal tract. She had undergone surgical excision of a nasal swelling previously diagnosed as a dermoid cyst. One year later, she returned to our clinic with a recurrence of the nasal swelling. Imaging tests revealed a nasal dermoid cyst with a tract extending to the nasal tip, without intracranial expansion. The cyst, along with the entire tract, was successfully removed surgically, and the postoperative follow-up indicated no complications. Histopathology confirmed the diagnosis of a dermoid cyst. This case underscores the significance of considering the dermoid tract in nasal cyst cases and the necessity of its complete removal to prevent recurrence.

Unilateral Choanal Atresia in Adults: A Case Series

Choanal atresia (CA) is a rare occlusion of the posterior choanae. Unilateral cases have been reported more than bilaterally, and it’s more often right-sided in those patients. According to the literature, mixed bony-membranous atresia is the most common type. There is a high incidence of craniofacial and visceral anomalies associated with congenital choanal atresia. Therefore, investigation for associated congenital anomalies is an important step before the surgery. We report 2 cases of incidental finding of unilateral choanal atresia in a 21- and 17-year-old with nasal discharge being the only complaint in the former and nasal obstruction with headache in the latter. The patients were then scheduled for day-surgery as a case of choanal atresia for transnasal, endoscopic repair and posterior septectomy. The patients were discharged home on the same day with the absence of restenosis or other complications.

Congenital coronary artery fistulas complicated with pulmonary hypertension: Analysis of 211 cases

AIM To compare the behavior of pulmonary hypertension(PHT) associated with coronary artery fistulas(CAFs) between the Asian and Caucasian subjects.METHODS CAFs may be complicated with PHT secondary to leftto-right shunt. Literature review limited to the English language. A total of 211 reviewed patients were collected. Of those, 111 were of Asian and 100 were of Caucasian ethnic origin. The mean age of the Asian and the Caucasian groups of patients were 48.9(range 19-83) and 49.9 years(range 16-85), respectively. In both groups, right heart catheterization was the most commonly(95%) used method for determining pulmonary artery pressure. RESULTS From all of the reviewed subjects, PHT was found in 49 patients(23%), of which 15 were Asian and 34 were Caucasian. In 75% of PHT subjects, mild to moderate PHT was reported and 76% of the fistulas had a vascular mode of termination. Treatment was surgical in 61%, followed by percutaneous therapeutic embolization(27%) and finally conservative medical management in 12% of PHT subjects. PHT was associated with a slight female gender predominance. The majority demonstrated mild to moderate PHT. PHT was reported more frequent in the Caucasian compared with the Asian ethnicity group. The majority of fistulas in patients with PHT had a vascular mode of termination. The results of this review are intended to be indicative and require cautious interpretation.CONCLUSION The likelihood for a CAF patient to develop PHT is presented when possessing the following features, with a Caucasian female having a fistula with a vascular mode of termination.

Diagnosis of Congenital Aortic Arch Anomalies in Chinese Children by Multi-Detector Computed Tomography Angiography

Summary： The purpose of this study was to evaluate the value of multi-detector computed tomography （MDCT） angiography for the diagnosis of congenital aortic arch anomalies and present the radiological images of congenital aortic arch anomalies in Chinese children. MDCT angiography and transthoracic echocardiography （TTE） were applied for the diagnosis of congenital aortic arch anomalies in 362 Chi- nese children between May 2006 and December 2011 （age ranges from 5 days to 12 years; mean age, 3.3 years）. Surgery and/or catheter angiography （CA） were conducted in all patients to confirm the final diagnosis. In the 362 Chinese children with congenital heart anomalies, congenital aortic arch anomalies were definitely diagnosed in 198 children and 164 children ruled out by operation and/or （CA）. Among the 198 children with anomalies, coarctation of aorta （CoA）, interruption of aortic arch （IAA）, fight aor- tic arch, aberrant right subclavian artery and double aortic arch were diagnosed in 134, 32, 20, 10 and 2 children respectively, and there were 6 cases with uncommon congenital aortic arch anomalies： 2 had double aortic arch including 1 with five branches of the aortic arch, 2 had isolation of the right sub- clavian artery with two patent ductus arteriosus （PDA）, 1 had an isolation of the common carotid artery with a PDA, and 1 had double PDA with a single ventricle and pulmonary artery atresia. Among the 32 children with IAA, 28 were of type A, and 4 were of type B. The diagnostic sensitivity, specificity and accuracy of MDCT angiography for congenital aortic arch anomaiies were 100% （198/198）, 98% （161/164） and 99% （359/362）, respectively. The diagnostic sensitivity, specificity and accuracy of TTE were 92% （182/198）, 81% （133/164） and 87% （315/362）, respectively. In conclusion, MDCT angiogra- phy is a reliable, noninvasive imaging technique for the diagnosis of congenital aortic arch anomalies in children. Sometimes, even more information can be obtained from this technique than from conven- tional angiography.

Classification of facial nerve aberration in congenital malformation of middle ear:Implications for surgery of hearing restoration

Objectives: Facial nerve aberration is the most troublesome situation in congenital malformations of middle ear.The aim of our study is to investigate its imaging and clinical features as well as relevant choice of surgical techniques for hearing improvement.Methods: A retrospective study involving review of clinical data of 227 patients(256 ears) with congenital middle ear anomaly was undertaken, including preoperative computed tomography(CT) data, surgical records and videos.Results: Aberration involving intratemporal facial nerve was found in 82/256 ears(32.03%) with congenital middle ear anomaly. The most common forms of aberration included overhanging over the oval window(50/82 ears, 60.98%), bifurcation(3/82 ears, 3.66%) and transverse over the promontory(3/82 ears, 3.66%), counting for 68.29%(56/82) of the cases with facial nerve aberration. Concomitant stapes malformation was found in 76/82 ears(92.68%) and atresia or stenosis of the oval window in 27/82 ears(32.93%). In 9/82 ears(10.98%) both stapes and oval window was absent. Elective surgeries for the purpose of hearing improvement included stapodotomy + piston implantation, labyrinthotomy, labyrinthotomy + total ossicular replacement prosthesis(TORP) implantation and Vibrant Soundbridge(VSB) implantation.Conclusion: The majority of facial nerve aberration in congenital malformation of middle ear involves displacement of facial nerve, in addition to concomitant malformations of the stapes and/or oval window, which may influence the choice of surgery for hearing improvement. VSB implantation may be considered as a useful option.

Morphological Features of Complex Congenital Cardiovascular Anomalies in Fetuses:as Evaluated by Cast Models

Accurate prenatal diagnosis of complex congenital cardiovascular anomalies, vascular ones in particular, is still challenging. A fetal cardiovascular cast model can provide a copy of the cardiac chambers and great vessels with normal or pathological structures. This study was aimed to demonstrate three-dimensional anatomy of complex congenital cardiovascular anomalies in fetuses by means of corrosion casting. Twenty fetuses with prenatal-ultrasound-diagnosed complex cardiovascular anomalies were enrolled in this study（19 to 35 gestational weeks）. Fetal cardiovascular cast models were made by a corrosion casting technique. The specimens were injected with casting material via the umbilical vein, and then immersed in strong acid after casting fluid was solidified, to disclose the geometries of cardiovascular cavities. Nineteen cast models were successfully made from 20 specimens. The casts distinctly showed the morphological malformations and spatial relationship between cardiac chambers and great vessels. One hundred and eleven abnormalities were revealed by casting in the 19 specimens, including 34 abnormalities located in the cardiac chambers（3, 4 and 27 anomalies in the atria, atrioventricular valves and ventricles, respectively）, and 77 in the great vessels（28, 20, 24 and 5 anomalies in the aorta and its branches, the pulmonary artery, the ductus arteriosus and the major veins, respectively）. Corrosion casting can display three-dimensional anatomy of fetal complex cardiovascular anomalies. This improves our understanding of related pathomorphology and prenatal diagnosis.

‘Children Kidney Care Centers’:Rationale,requirements and recommendations for best facilities and better future

Specialized centers are needed for nephrology and urology care of children.The justifications are the specialized nature of care needed and the growing incidence and prevalence.Children with chronic kidney disease(CKD)are at risk of morbidity,mortality,and decreased quality of life.Current pediatric practice structures are apparently poorly suited for the increasing demands of chronic disease in children.Kidney diseases account for around 8%-10%of total outpatients and 12%of admissions to the pediatric ward in hospitals.The major causes of pediatric CKD in registries are congenital anomalies of the kidney and urinary tract(around 50%),followed by inherited nephropathies and glomerulonephritis.The nephrologist’s role is important for specialized investigations and treatment.Urologist’s services are essential for the wide variety of conditions from birth to early adult age for complete cure and complementing medical management.Children have a right to treatments and to resources that are as sophisticated and advanced as those available to adults.Simple and sophisticated care for all children with ailments of the kidneys and related structures is important for ensuring‘health for all’.The availability of‘Child Kidney Care Centers’will go a long way in improving the lives of affected children.

Our experience with management of congenital urological pathologies in adulthood:What pediatric urologists should know and adult urologists adopt in pediatric practice experience

Purpose:To summarize our experience in the management of congenital anomalies in the kidney and urinary tract(CAKUT)in adults.Materials and methods:We conducted a retrospective chart review of all adult patients who underwent primary surgical intervention for CAKUT between 1998 and 2021.Results:The study included 102 patients with a median age of 25(interquartile range,23-36.5).Of these,85(83.3%)patients reported normal prenatal ultrasound,and the remaining 17(16.7%)patients were diagnosed with antenatal hydronephrosis.These patients were followed-up conservatively postnatally and were discharged from follow-up because of the absence of indications for surgical intervention or because they decided to leave medical care.All studied adult patients presented with the following pathologies:67 ureteropelvic junction obstructions,14 ectopic ureters,9 ureteroceles,and 6 primary obstructive megaureters,and the remaining 6 patients were diagnosed with vesicoureteral reflux.Forty-three percent of the patients had poorly functioning moieties associated with ectopic ureters or ureteroceles.Notably,67%of patients underwent pyeloplasty,9%underwent endoscopic puncture of ureterocele,3%underwent ureteral reimplantation,6%underwent endoscopic correction of reflux,7%underwent partial nephrectomy of non-functioning moiety,and the remaining 9%underwent robotic-assisted laparoscopic ureteroureterostomy.The median follow-up period after surgery was 33 months(interquartile range,12-54).Post-operative complications occurred in 5 patients(Clavien-Dindo 1-2).Conclusions:Patients with CAKUT present clinical symptoms later in life.Parents of patients diagnosed during fetal screening and treated conservatively should be aware of this possibility,and children should be appropriately counseled when they enter adolescence.Similar surgical skills and operative techniques used in the pediatric population may be applied to adults.

EUS diagnosis of ectopic opening of the common bile duct in the duodenal bulb: A case report

Among the various congenital anomalies of the biliary system,an ectopic opening of the common bile duct (CBD) in the duodenal bulb is extremely rare. ERCP is essential for diagnosing the anomaly. A 55-year-old male was admitted to hospital for severe right upper quadrant abdominal pain,followed by fever, chills, elevated body temperature and mild icterus. The diagnosis of ectopic opening of CBD in the duodenal bulb was established on endoscopic ultraso-nography (EUS),which clearly demonstrated dilated CBD, with multiple stones and air in the lumen, draining into the bulb. A normal pancreatic duct, which did not drain into the bulb, was also observed. This finding was confirmed on ERCP and surgery.As far as we know, this is the first case of this anomaly diagnosed by EUS. Ectopic opening of the CBD in the duodenal bulb is not an incidental finding, but a pathologic condition which can be associated with clinical entities such as recurrent or intractable duodenal ulcer, recurrent biliary pain,choledocholithiasis or acute cholangitis. Endoscopic ultrasonography features allow preoperative diagnosis of this anomaly and can replace ERCP as a first diagnostic tool in such clinical circumstances. Embryology of the anomalies of the extrahepatic biliary tree has been also reviewed.

Polysplenia syndrome with preduodenal portal vein detected in adults

Polysplenia syndrome, defined as the presence of multiple spleens of almost equal volume, is a rare condition involving congenital anomalies in multiple organ systems. We report this anomaly in a 41-year-old female who underwent a left lateral sectionectomy due to recurrent cholangitis and impacted left lateral duct stones. Polysplenia syndrome with preduodenal vein was diagnosed preoperatively by computed to-mography (CT) and surgery was done safely. Although the polysplenia syndrome with preduodenal portal vein (PDPV) in adult is rarely encountered, surgeons need to understand the course of the portal vein and exercise caution in approaching the biliary tract.

Ipsilateral hemifacial microsomia with dextrocardia and pulmonary hypoplasia:A case report

BACKGROUND Hemifacial microsomia(HFM)is a rare congenital malformation characterized by a combination of various anomalies,including the face,ears,eyes,and vertebrae.Prenatal diagnosis for HFM is possible,and quite accurate ultrasound can detect obvious defects.The etiology is still unknown,although some hypotheses have been proposed,including gene mutation,chromosome anomaly,and environmental risk factors.However,there are few reports of pulmonary hypoplasia and dextrocardia in HFM.CASE SUMMARY A 2-year-old boy presented to the ear reconstruction department of our hospital complaining of deviation of the face to the right side and auricular anomaly.Physical examination revealed facial asymmetry,preauricular skin tags,and concha-type microtia with stricture of the external auditory canal on the right side.Head magnetic resonance imaging showed bilateral semicircular canal dysplasia and bilateral internal auditory canals stenosis.Audiometric examination showed bilateral severe sensorineural hearing loss.Chest radiography and computed tomography showed dextrocardia and right pulmonary hypoplasia.CONCLUSION This case presented a rare finding and an unusual association of 3 malformations,ipsilateral HFM,pulmonary agenesis,and dextrocardia.

Multiseptate gallbladder with anomalous pancreaticobiliary ductal union: A case report

Multiseptate gallbladder, characterized by the presence of multiple septa dividing the gallbladder lumen, is a very extremely rare congenital anomaly of the gallbladder. On the other hand, anomalous pancreaticobiliary ductal union is also one of the congenital anomalous biliary diseases and thought to be related with choledochal cyst or biliary tract malignancies. In this paper, we describe a unique and first patient of multiseptate gallbladder with anomalous pancreaticobiliary ductal union and a review of the literature. To clarify more characters of the multiseptate gallbladder, examination of a larger patient population will be needed and further studies will be required.

The Scrotoschisis about a Case in the Pediatric Surgery Department of the Donka National Hospital

Introduction: Scrotoschisis is a very rare congenital defect of the scrotum characterized by the exteriorization of one or two testes. We report a case of right scrotoschisis in a newborn as well as a review of the literature for an approach of probable etiology. Patient and Observation: A newborn baby of 8 hours of life, weighing 3200 g was referred to our department for a right scrotal defect with exteriorization of the testis associated with fluid swelling of the left bursa. The 18-year-old mother, primiparous and primigeste followed all the prenatal consultations with eutocic delivery. After clinical investigation the diagnosis of right scrotosisis and left hydrocele was retained. Surgical treatment was carried out by primary closure after orchidopexy and exploration of the contralateral bursa, the content of which was calcified meconium bathed in a yellowish liquid. The post-operative consequences were simple. Conclusion: Scrotoschisis is an extremely rare birth defect whose etiology is poorly understood. However, it could be the cause of meconium peritonitis secondary to a scarred fatal intestinal perforation.

Anomalous Origin of the Right Coronary Artery from the Left Sinus Valsalva with Coronary Ectasia

The abnormal origin of the right coronary artery from the left aortic sinus coursing between the aorta and the pulmonary trunk is a rare congenital anomaly. It may remain asymptomatic or may result in cardiac morbidity or mortality. In the past, an anomalous origin of the right coronary artery from the left sinus of Valsalva was considered a benign finding;it is now evident that this anomaly can be associated with atypical chest pain, myocardial ischemia, and sudden death. We thought that;the diagnosis of left sinus Valsalva aneurysm, described by transtorasic echocardiography and transeusophageal echocardiography, might need demonstrated advanced scanning techniques.

Duodenal duplication cyst causing severe pancreatitis:Imaging findings and pathological correlation

We here report a case of a 18-year-old man with a history of recurrent abdominal pain and a previous episode of severe acute pancreatitis. Abdominal ultrasonography, contrast enhanced multislice computer tomography, endoscopic retrograde cholangiopancreatography, endoscopic ultrasonography and magnetic resonance imaging demonstrated a cystic mass lesion. Only on delayed phase magnetic resonance images after GadoliniumBOPTA injection, it was possible to demonstrate the lesion's relationship with the biliary tree, differentiating the lesion from intraluminal duodenal diverticulum, and to achieve the diagnosis of duodenal duplication cyst, a recognized rare cause of acute pancreatitis. The diagnosis was confirmed by histology.

Assessment of fetus during second trimester ultrasonography using HDlive software:What is its real application in the obstetrics clinical practice?

AIM To show imaging results from application of fourdimensional(4D) ultrasound lightening technique(HDlive^(TM)) in clinical obstetrics practice. METHODS Normal and abnormal fetuses at second and third trimester of pregnancy undergoing routine scan with 4D HDlive^(TM)(5DUS) in the rendering mode are described. Realistic features of fetal structures were provided by 5DUS in the rendering mode. Normal anatomy as well as pathology like cleft lip, hypoplastic face, micrognathia, low-set ears, corpus callosum, arthrogryposis, aortic arch, left congenital diaphragmatic hernia are highlighted in this study. Anatomical details of the fetuses were provided by 5DUS with higher quality imaging modality compared to those obtained using conventional 2D/3D ultrasound. RESULTS Realistic views of fetal anatomy details were displayed by means of 5DUS in the rendering mode, with high image quality obtained either in low-risk or in high-risk obstetrics population. Corpus callosum, esophagus, and aortic arch were obtained in normal fetuses. Cleft lip, cleft lip and palate, micrognathia, hypoplastic face, lowset ears, arthrogryposis, left congenital diaphragmatic hernia, exomphalos, and clitoris hypertrophy were clearly rendered by 5DUS application.CONCLUSION The use of 5DUS in the rendering mode, when clinical available, was diagnostic in a variety of congenital anomalies, aided understanding of the parents-tobe and improved prenatal counseling and perinatal management.