Supernumerary testis or polyorchidism: A rare urogenital anomaly (case report and literature review).

INTRODUCTION AND IMPORTANCE: Polyorchidism, or supernumerary testis, is a rare urogenital congenital disorder. Because of its rarity, there is no approved standard treatment protocol for preserving or removing the extra testicle, yet orchiopexy is frequently performed as a preferred treatment in most medical facilities. CASE PRESENTATION: We present a 23-year-old single male with a bilaterally empty scrotum. He was unaware of his condition and had not seen a doctor before being admitted to our surgical unit. During his younger sibling's circumcision by a local circumcisionist (a medical staff member, idealy a nurse, whose duty is to perform circumcision, preferably at home), he saw something different (his emptey scrotum) and came to us with his problem. Laboratory findings revealed severe oligospermia, and tumor markers (Alpha fetoprotein, beta-human chorionic gonadotropin, and lactate dehydrogenase) were negative for malignancy. The patient underwent bilateral herniorrhaphy and orchiopexy of all six testicles (three in each inguinal canal) and had an uneventful recovery. CLINICAL DISCUSSION: As polyorchidism is not a common problem, its management remains a contentious issue due to the lack of evidence-based consensus. However, with the introduction of new imaging modalities and on-table frozen section biopsy, the decision to continue with orchiopexy or orchiectomy can be easily justified; however, conservative treatment is preferable in cases of no coexisting anomalies, particularly cryptorchidism. CONCLUSION: Polyorchidism could run unnoticed for years, especially if there is no direct and consistent access to a medical facility. In cases where polyorchidism is detected accidentally by imaging or during surgical exploration, the treatment must be justified accordingly.

Noninvoluting congenital hemangiomas with hypovolemic shock, anemia and prolonged jaundice in a neonate: a case report.

Noninvoluting congenital hemangioma is an extremely rare congenital anomaly in newborn babies and may complicate life-threatening events, including hemorrhage. We present a very rare case of noninvoluting congenital hemangiomas in an Asian and Afghan six-day-old girl that was detected at birth. The noninvoluting congenital hemangiomas were accompanied by hypovolemic shock, anemia, and prolonged jaundice during the first two weeks of life. These diseases were diagnosed by medical history, physical examination, blood analysis, and doppler ultrasonography of the skin lesions. Her hypovolemic shock and hemorrhage were improved after supportive treatment, and she was discharged from the hospital in a good condition. After four weeks of life, the hemangiomas did not regress or progress, suggesting noninvoluting congenital hemangiomas. Hemangioma may complicate severe hemorrhage and shock, as well as there may be an association between congenital hemangioma and prolonged neonatal jaundice.

Giant cervical lymphangioma encompassing the neck great vessels (carotid artery and jugular vein): a case report.

Lymphangiomas are benign congenital defects affecting the lymphatic system. These lesions commonly involve the head and neck, predominantly the posterior cervical triangle. Lymphangiomas cause obstructive symptoms in the upper airway and pose an esthetic concern to the patient. Clinically, these lesions are seen as cervical swelling, and a definite diagnosis is made by ultrasonography, computerized tomography scanning, and histopathologic analysis. Herein, the author present an unusual case of an 18-month-old child presenting with a huge cervical swelling on the right side with extension to the carotid triangle (encompassing neck great vessels) as well as unilateral neck and fascial disfigurement. The patient was treated surgically with the complete excision of the mass and followed up with an excellent esthetic result. Case presentation: An 18-month-old child was brought to our teaching hospital's pediatric surgery department with a huge cervical mass on the right side since birth. Following the completion of work-up with the laboratory and imaging (computerized tomography scan) diagnostic modalities, the patient was prepared for definite treatment. Our team approached the mass through a right neck hockey stick incision, and with the preservation of the neurovascular bundle, the mass was completely excised. The patient was followed up for 12 months on two occasions, with excellent esthetic results and no relapse. Conclusion: Lymphangiomas confined to the posterior cervical triangle are a common problem in children. Lesions extending to the anterior of the neck, especially those involving the neck neurovascular bundle, are uncommon entities. The decision to proceed with sclerotherapy or surgical excision should be justified, provided that, during the surgical procedure, the neurovascular bundle is preserved and none of the vital organs (neurovascular components) are compensated with the goal of a complete mass excision.

Right-side diaphragmatic eventration with atrial septa defect and cleft palate in an infant: a case report.

BACKGROUND: Congenital right-side diaphragmatic eventration with atrial septal defect and cleft palate is a rare congenital anomaly. CASE PRESENTATION: We present a rare case of congenital right-sided diaphragmatic eventration along with atrial septal defect, cleft palate, pneumonia, and undernutrition in a 3-month-old Asian and Afghan girl. The clinical features were observed in the third month of life, and the diagnosis of these anomalies was established by the patient's history, physical examination, chest X-ray, thoracic computed tomography, and echocardiography. Her condition was good after supportive treatment. Since the index case of diaphragmatic eventration was associated with congenital heart disease, cleft palate, and parental consanguinity, a genetic basis may have played an important role in the pathogenesis of this anomaly. CONCLUSION: Eventration of the diaphragm may be diagnosed in early infancy, and genetic factors may contribute to its pathogenesis.

Childhood mesenteric cyst: A rare intra-abdominal entity with literature review.

INTRODUCTION AND IMPORTANCE: Mesenteric cysts are uncommon intra-abdominal benign masses that appear in childhood with varying degrees of clinical manifestations, ranging from being asymptomatic to presenting as an acute abdomen. The diagnosis is made incidentally during the work-up for other abdominal pathologies such as acute appendicitis, bowel obstruction, etc. The treatment is mostly surgical and varies depending on the clinical type of the lesion. CASE PRESENTATION: A 26-month-old child was referred to our teaching hospital's pediatric surgery department with an abdominal mass. The patient had previously complained of constipation and been treated symptomatically with laxatives. Ultrasonography reported ovarian cysts confirmed by computerized tomography scanning (as a misdiagnosis report of an ovarian cyst instead of a mesenteric cyst), done outside the hospital in a private diagnostic center. CLINICAL DISCUSSION: The patient was prepared for operation and during the surgical procedure, she was found to have a duplex cyst, which was mostly incorporated in the mesentery of the distal 1/3 of the transverse mesocolon. The cyst was enucleated through a lower midline laparotomy incision without bowel resection and anastomosis. The histopathological analysis of the specimen confirmed a mesenteric cyst. CONCLUSION: Mesenteric cysts are rare lesions in children and should be considered when approaching any intra-abdominal mass. Except for the rare cases where intestinal resection and/or partial cyst excision are required, all mesenteric cysts can be excised while preserving intestinal integrity and vascular supply.

A challenging high-risk surgery for necrotizing pneumonia in a right bilobed lung.

BACKGROUND: Necrotizing pneumonia is rare in children and is one of the most serious complications of a lung infection caused by antibiotic failure. We present a 12-year-old leukopenic child with a long-lasting lung infection, presenting as having a lung hydatid cyst, but diagnosing with necrotizing pneumonia in the right bilobed lung. Failure to medical treatment and ongoing leukopenia justified surgical intervention with positive results. CASE PRESENTATION: The patient was referred to our teaching hospital's pediatric surgery department. He had previously been diagnosed with intestinal tuberculosis (TB) and received anti-TB treatment. On referral to our hospital, the patient was suffering from restlessness, frequent coughing, fever, vomiting, and diarrhea. Following the completion of the clinical work-up, a blood test revealed leukopenia (white blood cell count of 2100/microliter), a normal platelet count, and a lesion in the right lung. Computerized tomography scanning (CT-Scan) image reported a lung hydatid cyst. In the pediatrics ward, a broad-spectrum antibiotics regimen with triple-antibiotic therapy (linezolid, vancomycin, and metronidazole) was instituted and continued for a week with no response, but worsening of the condition. In the pediatric surgery ward, our decision for surgical intervention was due to the failure of medical treatment because of a pulmonary lesion. Our team performed right lung upper lobe anterior segment wedge resection due to necrotizing pneumonia and followed the patient 45 days post-operation with a reasonable result. CONCLUSION: Living in remote rural areas with low resources and inaccessibility to proper and specialized diagnostic and treatment centers will all contribute to an improper diagnosis and treatment of lung infection. In total, all of these will increase the morbidity and mortality due to lung necrosis in the pediatric population, regardless of their age. In low-resource facilities, high-risk patients can benefit from surgical intervention to control the ongoing infection process.

Left lung aplasia with patent ductus arteriosus in a term newborn baby: a case report.

Pulmonary aplasia is a very rare congenital anomaly identified by the absence of lung parenchyma or vessels. This malformation mostly accompanies other anomalies, and chest computed tomography (CT) is a useful diagnostic tool. We present a rare case of left lung aplasia with patent ductus arteriosus and neonatal sepsis in a term newborn baby who had respiratory distress. The clinical features were observed during the first week of life, and the diagnosis of these anomalies was established by clinical, laboratory, chest x-ray, thoracic CT and echocardiographic findings. After the management of neonatal sepsis and patent ductus arteriosus, he was discharged from the hospital in good condition. Since the index case of lung aplasia was associated with congenital heart disease and had strong parental consanguinity, a genetic basis may have been involved in the pathogenesis of pulmonary aplasia.

Congenital megalourethra.

Background: Congenital megalourethra is a urogenital anomaly characterized by a cystic dilatation and elongation of the penile urethra resulting from the absence and hypoplasia of the corpus spongiosum and corpus cavernosum, or anterior urethral valve. There are two clinical types: scaphoid and fusiform. Generally, the etiology is unknown, but it is thought to be a defect in mesodermal development. Fewer than 100 cases have been reported in the literature, and the exact incidence is unclear. In most cases, the surgical procedure is challenging and requires extensive reconstructive and/or replacement surgery. Case presentation: We present a 6-month-old boy suffering from a cystic dilatation of the penile urethra along with urine dribbling during micturition since birth. The patient was diagnosed with the scaphoid type of megalourethra and was operated on using reduction urethroplasty. On the 21st post-operative day, we removed the Foley catheter and followed the patient on two occasions (the 45th post-operative day and the 6th post-operative month) with excellent results. Conclusion: The anagement of megalourethra depends on the clinical type. Meticulous surgical technique, the use of fine suture materials with careful handling, and fixation of the vascularized flap are the main principles of an acceptable result. Observation of erectile function and fertility require long-term follow-up.

Childhood esophageal achalasia: Case report from Afghanistan with literature review.

INTRODUCTION AND IMPORTANCE: Esophageal achalasia is a motility disorder of the esophagus with unknown etiology characterized by the failure of lower esophageal sphincter relaxation. Diagnosis is made by barium esophagography, endoscopy, and esophageal manometery. Heller Esophagomyotomy along with Dor's fundoplication is the treatment of choice. Persisting undiagnosed cases may lead to malnutrition. CASE PRESENTATION: We present a case of an 8-year-old child suffering from dysphagia and regurgitation. The child was misdiagnosed and maltreated for the suspicion of respiratory tract and gastrointestinal problems in the local clinics. During this time, he remained unresponsive to the mentioned treatments, and the local physician advised him to have an upper gastrointestinal (GI) endoscopy, which revealed esophageal achalasia (EA). On admission to our pediatric surgery ward, the patient had coexistent parotitis, which was treated conservatively. Following recovery, the patient was prepared for surgery and underwent esophageal myotomy along with Dor's fundoplication. CLINICAL DISCUSSION: Esophageal achalasia is rare in children, but poses major health challenges to children if left untreated. Symptomatic treatment may mask the actual picture of the problem and last for years. Following surgery and discharge from the hospital in a three-month follow-up interval of time, our patient exhibited full recovery, with gaining 4 kg weight. CONCLUSIONS: Respiratory and gastrointestinal conditions with similar signs and symptoms should always be considered in differential diagnosis of esophageal achalasia, especially where there is no direct access to a pediatric specialized complex. On-time evaluation and treatment will further prevent children from malnutrition in long-lasting undiagnosed patients.

Choledochal cyst: A challenging diagnostic and therapeutic entity in low-resource settings.

BACKGROUND: Choledochal cyst is an uncommon congenital biliary tract abnormality of unknown etiology. Its classical symptoms are jaundice, abdominal pain, and right upper quadrant mass. However, the disease may present with a vague and non-specific chronic abdominal discomfort. Delay in diagnosis and management may increase the risk of complications particularly the malignancy, which can directly affect the prognosis and outcome. Complete excision of the extra-hepatic duct, cholecystectomy, and Roux-en-Y hepaticojejeunostomy through the open surgical intervention or laparoscopic procedure is the mainstay of treatment. CASE PRESENTATION: A 14-year-old male was presented to our hospital complaining of vague abdominal pain for 5 years. The radiologic imaging showed the features of a type IVa choledochal cyst. He underwent complete excision of the extra-hepatic duct, cholecystectomy, and Roux-en-Y hepaticojejeunostomy. Although the bile leakage occurred as a complication of the procedure, our team best managed the patient in the post-operative period until full recovery. DISCUSSION: The Choledochal cyst was first described by Vater, which is a congenital anomaly that sometimes may remain asymptomatic till adulthood. Surgical intervention is the mainstay of treatment. However, postoperative complications including bile leakage need a close follow-up of the patient. CONCLUSION: Diagnostic delay (antenatal and postnatal) and non-specific symptoms will mask the real feature of the disease, especially in low-resource settings. Technical facilities and professional care of the patient may preclude complications.

Bilateral open lip schizencephaly.

Schizencephaly is a central nervous system (CNS) developmental disorder characterized by abnormal cleft extending from the lateral ventricles to the cerebral cortex. Clinically, it occurs as trans-mantle, closed lip and open lip types which may be unilateral or bilateral. The exact cause of schizencephaly is not known but genetic disorders, exposure to teratogens, viral infections and maternal age are implicated. We present a case of bilateral open lip schizencephaly with some degrees of neurological disorders caused by increased intra-cranial pressure (ICP) due to ventriculomegaly. We applied ventriculo-peritoneal shunt (V-P shunt) to the patient with considerable improvement after post-operative follow-up.

A rare case report of prune belly syndrome with malnutrition.

Prune belly syndrome (PBS) is a rare congenital malformation of unknown etiology characterized by a triad of deficient abdominal wall musculature, undescended testicles and urinary tract malformations. Most of the patients have pulmonary, cardiac, skeletal and gastrointestinal tract anomalies. Lack of abdominal muscles leads to constipation due to inability to perform Valsalva maneuver, which helps push the stool out of the rectum during the defecation. Additionally, frequent respiratory tract infections, persisting constipation and urinary tract infections lead to the development of malnutrition in children. We report this case to raise the awareness of low socioeconomic and low-resource medical settings that malnutrition could be existed or caused by PBS. We also encourage the expansion of pediatric surgery and family medicine training to increase the number of specialist (family medicine) to report and refer PBS in earlier phase, while working in rural areas and remote provinces.