Unilateral nasal agenesis and ipsilateral supernumerary nostril: A case report and literature review

Background: Nephropathies are generalizations that indicate kidney disease or damage, which eventually can lead to some type of kidney failure. A rare condition in dogs, renal agenesis can be bilateral or unilateral, the former being incompatible with postnatal life. Unilateral renal agenesis is commonly asymptomatic unless the opposite kidney is involved. Ureteral agenesis ipsilateral to the absent kidney may also be seen, the ureter may be absent or malformed with a blunt end ending in connective tissue. The aim of the present report is to describe a case of renal agenesis in an adult female dog. Case: A mixed-breed, spayed bitch, approximately five years old, was seen for consultation and preoperative exams, in order to perform an elective periodontal cleaning procedure. The animal had a history of ovariosalpingohysterectomy without previous laboratory tests. Imaging tests were also performed. In the ultrasound examination, the absence of the right kidney was found, and the other organs had a normal appearance. A second ultrasound and radiography performed later showed the absence of a functional right kidney and its remains. In both exams, the left kidney was within an apparent normality. During the radiographic examination, no silhouette or structure consistent with the right kidney was observed. Discussion: The etiopathogenesis of renal agenesis in companion animals is still uncertain, but genetic predisposition has been identified mainly in Beagle dogs, as well as in Shetland Sheepdogs and Doberman Pinschers. In agreement with the case report presented, the kidney should be retroperitoneally compressed against the dorsal abdominal wall, more cranial in relation to the left kidney, predominantly in the lumbar region, projecting cranially to the intrathoracic part of the abdomen. As renal function can be sustained when up to 75% of the organ is affected, unilateral agenesis can go unnoticed during the animal's life, being found by chance in routine examinations or during necropsy. This is due in most cases to the compensatory hypertrophy of the other kidney, with an increase in the glomerular filtration rate, which explains why biochemical parameters of renal evaluation remain in the normal range. This also corroborates the present report, because although there was no compensatory hypertrophy of the left kidney, the diagnosis was only made in routine exams, that is, the patient had no clinical signs consistent with kidney disease. The study of nephrology in companion animals is of extreme clinical importance, because due to genetics or the prolongation of life of these individuals, dysfunctions and diseases related to the urinary system are increasingly present in the routine of care, in significant numbers and severity. Although unilateral renal agenesis is compatible with life, this dysfunction can lead to functional overload of the remaining kidney, which can lead to renal failure. In veterinary nephrology there is still room for discussion and learning and the way to develop better strategies to address this problem is through study and research in the area. It is extremely important to assess the health of the urinary system of companion animals, especially in preoperative exams, allowing for adequate care and procedures to maintain the patient's health and good quality of life.

Background. Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is a rare congenital condition marked by agenesis or hypoplasia of the upper female reproductive tract. While it is typically recognized for its reproductive implications, renal anomalies - such as unilateral renal agenesis or ectopic kidneys - are also frequent but often underdiagnosed. Recent findings suggest shared embryological origins between the genital and urinary systems, supporting the need for a broader diagnostic and therapeutic perspective. Case Description. We describe two cases of women with MRKH syndrome diagnosed in adolescence, in whom renal anomalies were detected only in adulthood. Both patients developed hypertension and progressive renal dysfunction in their 30s, revealing previously unrecognized congenital malformations: one with renal dysplasia, the other with unilateral agenesis and compensatory hypertrophy. These cases emphasize how renal involvement in MRKH can remain silent for years and highlight the importance of early and continuous uro-nephrological surveillance. Conclusion. MRKH syndrome should be considered a systemic disorder involving both reproductive and renal systems. The traditional classification into Type I and Type II may not reflect the full clinical spectrum. A multidisciplinary approach - including gynecology, urology, and nephrology - is essential for timely detection and management of renal complications. Regular follow-up, even in asymptomatic patients, can prevent or delay chronic kidney disease. Greater awareness of renal risks in MRKH is vital to improving long-term outcomes and ensuring truly comprehensive care.

Zinner syndrome (ZS) is a rare urogenital condition characterised by the triad of unilateral renal agenesis, ipsilateral seminal vesicle cyst, and ipsilateral ejaculatory duct obstruction, resulting from malformation during early embryogenesis of the mesonephric (Wolffian) duct. The authors present a 35-year-old male who was being evaluated for chronic hepatitis B virus infection. He was referred to the urology outpatient clinic on account of incidental ultrasound finding of solitary right kidney. General physical examination revealed a healthy-looking young male with a flat abdomen and no palpable enlarged organs. Digital rectal examination revealed normal sized prostate with no palpable pararectal masses. MRI of the pelvis revealed a triad of unilateral renal agenesis, ipsilateral seminal vesicle cyst, and ipsilateral ejaculatory duct obstruction. The clinical diagnosis was asymptomatic ZS. He is on yearly follow-up at the urology outpatient clinic for lower urinary tract symptoms, pelvic pain, painful ejaculation, features of infertility, and pelvic ultrasound. If any of these symptoms occur, he will be treated with an α-adrenergic receptor blocker, drainage of the seminal vesicle cyst, and appropriate treatment for infertility. He is also on active surveillance for viral hepatitis by the gastroenterology team. In conclusion, prompt referral and comprehensive radiological imaging investigations of patients with unilateral agenesis of the kidney will lead to increased identification and report of patients with ZS. There is paucity of literature reports on ZS in the authors’ environment, and this case report, to the best of the authors’ knowledge, is the first from Nigeria.

BackgroundTotal anomalous pulmonary venous connection accompanied by unilateral lung agenesis and Goldenhar syndrome is extremely rare.Case presentationWe present a case of total anomalous pulmonary venous connection accompanied by unilateral lung agenesis and Goldenhar syndrome in a patient who was diagnosed based on transthoracic echocardiography and computed tomography. We observed complete absence of the lung, the bronchial tree, and vascular structures on the right side, with abnormal drainage of the left pulmonary veins into the innominate vein. The patient showed clear clinical evidence of pulmonary venous obstruction and underwent surgery 3 days after birth. The pulmonary venous chamber containing the vertical vein was anastomosed to the left atrium using 7–0 PDS running sutures via a median sternotomy. Echocardiography and computed tomography performed 1 year postoperatively revealed no pulmonary venous obstruction.ConclusionWe report a rare case of total anomalous pulmonary venous connection accompanied by unilateral lung agenesis and Goldenhar syndrome, which was successfully repaired 3 days after birth. A median sternotomy is a safe and effective approach for surgical repair of congenital heart disease with unilateral lung agenesis. Repair of the supra cardiac total anomalous pulmonary connection using the vertical vein is feasible in patients with a small pulmonary venous chamber.

A 64-year-old man with chronic atrial fibrillation was referred to our hospital for resection of a biopsy-proven adenocarcinoma of the right lung. A preoperative coronary angiogram demonstrated large, tortuous collateral vessels arising from the proximal right (Figure 1) and left circumflex (Figure 2 …

Background:Congenital unilateral lung agenesis is a rare condition in which there is complete absence of one lung, in addition to the associated bronchus and pulmonary vasculature. It can be an isolated finding or present in conjunction with cardiac, gastrointestinal, genitourinary and skeletal malformations. Due to the increasing physiological demands on the body during gestation and parturition, this condition poses significant risks in individuals with this conditionCase:A 26-year-old G1P0 with known right lung agenesis was managed with a multidisciplinary approach by obstetrics, maternal–foetal medicine, pulmonology and cardiology specialists. The patient subsequently had an uncomplicated pregnancy and vaginal deliveryConclusion:Multidisciplinary care led to an optimal pregnancy outcome in a woman with congenital unilateral lung agenesis. Women with this condition with normal baseline function have a good prognosis in pregnancy.

BACKGROUNDCongenital scrotal agenesis (CSA) is very rare. There are 11 cases of congenital scrotal agenesis or absence reported in the literature, most of which are bilateral and accompanied by cryptorchidism. Only two cases of which are unilateral scrotal agenesis and not accompanied by cryptorchidism. This is the first reported case of unilateral scrotal agenesis with cryptorchidism and scrotoplasty.CASE SUMMARYA 2-year-old boy was admitted to our hospital with left cryptorchidism and ipsilateral CSA. An innovative method was used in the patient where a scrotal skin pedicle from the right part of scrotal skin was transplanted to the left side. At the same time, descent orchiopexy was performed. At the 4-mo follow-up, the left testicle was located in the scrotum and the size and shape were normal.CONCLUSIONFor unilateral CSA with ipsilateral cryptorchidism, contralateral scrotal pedicle transplantation and descent orchiopexy appear to be a successful surgical option.

Unilateral pulmonary artery agenesis (UPAA) is a rare congenital anomaly that may remain undiagnosed until adulthood, when patients present with exertional dyspnoea, recurrent respiratory infections, hemoptysis, or pulmonary hypertension. A 55-year-old female presented with exertional dyspnoea and hypoxemia. Clinical evaluation and echocardiography revealed pulmonary arterial hypertension, cor pulmonale with preserved left ventricular systolic function. Chest radiography demonstrated cardiomegaly with prominent but otherwise normal hilar shadows. Computed tomography pulmonary angiography revealed complete absence of the right pulmonary artery, with the right lung supplied by extensive systemic collaterals. Diffuse mosaic attenuation was noted, reflecting chronic hypoperfusion. The patient was managed conservatively with oxygen therapy and pulmonary vasodilator therapy, including ambrisentan and tadalafil, resulting in clinical stabilization. Unilateral pulmonary artery agenesis is a rare but important cause of pulmonary hypertension and cor pulmonale in adults. Computed tomography pulmonary angiography plays a pivotal role in diagnosis by accurately delineating vascular anatomy and collateral circulation, enabling appropriate management and prevention of complications.

BackgroundZinner syndrome is a rare Wolffian duct anomaly presenting as a triad of unilateral renal agenesis, ipsilateral seminal vesicle cyst and ipsilateral ejaculatory duct obstruction. The condition is due to a developmental anomaly leading to malformation of the mesonephric duct. Magnetic resonance imaging is considered to be the gold standard for the diagnosis of Zinner syndrome. Due to the limited number of cases reported in the literature, the associations of Zinner syndrome are not well established. Case presentationIn this case report, we are presenting a case of a 38-year-old male with dysuria, intermittent pain in the scrotum and lower abdomen and painful ejaculation for the past six months. Imaging findings led to the diagnosis of Zinner syndrome, with unilateral testicular agenesis and an aberrant course of the left common iliac artery.ConclusionsApart from the known associations of Zinner syndrome, testicular agenesis in a patient with Zinner syndrome has not been reported in the literature and is an important addition to the limited literature available.

Unilateral lung agenesis, a rare congenital deformity, is secondary to abnormal embryonic development, leading to a shift of the mediastinum and remaining lung. In our case reports, we describe the diagnostic courses of two pediatric patients with unilateral lung agenesis admitted to our hospital in 2014. We present and compare the findings in transthoracic echocardiography with that in CT. Echocardiography played a pivotal role in revealing pulmonary deformities and leading to the diagnosis. © 2016 Wiley Periodicals, Inc. J Clin Ultrasound 44:514-517, 2016.

Unilateral pulmonary artery agenesis is a rare congenital malformation, typically observed in infancy or childhood, but rarely in adulthood. An elderly female patient admitted to our hospital with a chief complaint of acute chest pain is reported here. The patient experienced a sudden onset of chest pain during physical exertion (folding quilts) 4 days before admission, which progressively worsened. Thoracoabdominal computed tomography angiography (CTA) was performed to rule out acute aortic syndromes, revealing agenesis of the right pulmonary artery with systemic collateral circulation supplying the right lung. A further examination of the clinical history and symptoms uncovered a past medical history of chronic pulmonary disease lasting several decades. The patient's clinical manifestations had consistently presented as symptoms of common conditions such as chronic bronchitis, bronchiectasis, pneumonia, and pulmonary tuberculosis, and a definitive diagnosis of Isolated Unilateral Pulmonary Artery Agenesis (IUAPA) had not been established, nor had the association between this disorder and chronic pulmonary lesions been previously considered. Although follow-up examinations confirmed that the present episode of chest pain resulted from an osteoporotic vertebral fracture, further in-depth research is necessary to fully understand the relationship between the absence of the pulmonary artery and the chronic pulmonary lesions. This report, together with the literature review, discusses the key characteristics, misdiagnosis challenges, and strategies for improving the diagnosis of IUAPA.

Agenesis of the internal carotid artery with transcavernous anastomosis presenting with an anterior communicating artery aneurysm—A case report and review of the literature

Introduction and importance:Zinner syndrome (ZS), a rare congenital defect, involves three specific conditions on one side of the body: renal agenesis (missing one kidney), cyst of the seminal vesicle, and ejaculatory duct obstruction. It arises from developmental irregularities in the mesonephric duct. MRI is the definitive diagnostic tool. Its rarity in young patients underscores the uniqueness of our case.Case presentation:A male student, 19 years of age, presented with dysuria, urethral pain, a burning sensation during urination, and painful nocturnal emissions for two years. A physical examination showed no abnormalities, with normal vital signs and laboratory results. Imaging showed right-sided renal agenesis, left kidney hypertrophy, a cyst near the right seminal vesicle, and ejaculatory duct obstruction, with a suspected hemorrhagic seminal vesicle cyst.Clinical discussion:ZS is a rare congenital condition frequently without symptoms but may present with lower urinary and genital tract symptoms in the second or third decade. A review of 200 cases showed a 0.046% incidence rate, with right-sided abnormalities more common. Seminal vesicle cysts are the most frequent cystic lesions. Diagnosis typically involves MRI, though CT and ultrasound were used historically. Management options include surgery, aspiration, or observation. Conservative management was successful for our patient, with symptoms improving over time.Conclusions:ZS is an extremely rare condition with unilateral agenesis of the kidney, an ipsilateral cyst of the seminal vesicle, and obstruction of the ejaculatory duct. Early diagnosis and heightened awareness are essential for effective treatment and better outcomes in affected patients due to its rarity.

Congenital anomalies of the male reproductive tract are significantly rare and are often discovered incidentally during infertility workups. This report identifies a rare case of a young male with oligospermia who is found to have congenital fusion of the spermatic cords, left unilateral seminal vesicle agenesis, as well as a left grade III varicocele. Workup established a lack of cystic fibrosis transmembrane conductance regulator (CFTR) mutation or urinary abnormalities usually associated with male anatomical reproductive alterations. A unilateral seminal vesicle agenesis is exceedingly rare and may lead to both obstructive and nonobstructive subfertility.

The supernumerary nostril is an extremely rare congenital anomaly of duplication. A review of the literature shows that only 17 cases have been reported in the world. The supernumerary nostril in association with facial clefting is even more rare, with only three cases reported so far. We are reporting a case of supernumerary nostril in association with complete unilateral cleft lip without cleft palate, which happens to be the first case of its kind to be reported. Most supernumerary nostrils are situated superior to the normal nostrils and very few of them are situated at the same level or below the normal nostrils. In this case, the supernumerary nostril was placed lateral to the normal nostril.