Persistent Müllerian Duct Syndrome (PMDS): a Rare Anomaly the General Surgeon Must Know About

Persistent Müllerian duct syndrome is a rare condition occasionally encountered in men with normal phenotype but with presence of Müllerian duct structures. In India, owing to neglect and lack of facilities, we encounter this condition in adult males. We encountered on the same day in the operation theatre two phenotypic males aged 40 years and 10 months who had inguinal hernia on one side along with contralateral undescended testis. Both patients intraoperatively had uterus with fallopian tubes and underwent subtotal hysterectomy with preservation of vas. Repair of inguinal hernia with fixation of the testis in the scrotum was done. Though rare, every surgeon operating upon inguinal hernia or undescended testes or cryptorchidism needs to know about the presence of the uterus in a phenotypic male patient at any age. High degree of suspicion and awareness is needed to diagnose this condition. Early treatment is needed to maintain fertility and to prevent the occurrence of malignancy in remnant müllerian structures.     

Colonic Volvulus Associated with Chilaiditi’s Syndrome: Report of a Case

Hepatodiaphragmatic interposition of the intestine, known as Chilaiditis syndrome, is a rare and often asymptomatic anomaly, typically found as an incidental radiographic sign. We report a case of Chilaiditis syndrome associated with transverse colon volvulus, predisposed by segmental agenesis of the right lobe of the liver. A 45-year-old man presented with a 2-day history of abdominal pain, nausea, vomiting, and constipation. Plain chest X-ray and abdominal computed tomography showed colonic interposition and segmental agenesis of the right lobe of the liver. Laparotomy revealed a clockwise volvulus of the transverse colon associated with interposition and incarceration of the colon through the space of the agenetic segment of the liver. The transverse colon, which was adherent to the agenetic space in the liver and diaphragm, was dissected away and repositioned, and the volvulus was reduced. To our knowledge, this is only the sixth reported case of a colonic volvulus associated with Chilaiditis syndrome and the first case associated with segmental agenesis of the right lobe of the liver.     

Persistent Müllerian duct syndrome: a surgical approach

Laparoscopy revealed a left inguinal testis and a right abdominal testis. Surgery revealed uterus-like structures. The bilateral testes showed primitive testis without ovarian tissue. Physical examination showed a normal and an empty scrotum with a nonpalpable gonad. Chromosome analysis revealed 46,XY. Pathological findings demonstrated the immature testis and the immature uterus.     

Sarcomatoid Carcinoma of Penis with Bilateral Inguinal Metastases—A Case Report and Review of Literature

Sarcomatoid carcinoma or carcinosarcoma of the penis is a rare entity with 36 cases reported in the literature. A 61-year-old male presented with swelling in the glans and shaft of the penis with bilateral inguinal lymph node metastases. He underwent total penectomy with bilateral inguinal block dissection. Histopathology revealed sarcomatoid carcinoma of the Penis with bilateral inguinal lymph node metastases. The patient later developed bilateral lung metastases in 6 months and was succumbed to death. Sarcomatoid carcinoma or carcinosarcoma is a rare malignancy with poor prognosis.     

Lemierre’s Syndrome Associated with Septic Pulmonary Embolism: A Case Report

An 18-year-old female patient suffering from a painful right-sided neck mass was admitted to our emergency service. Computed tomography of the neck revealed thrombosis of the right internal jugular vein. Computed tomography of the chest indicated septic pulmonary emboli in both lungs. Blood and oropharyngeal cultures showed proteus that was sensitive to cefepime and amicasin. Chemotherapy was administered for 12 days, however, her complaints continued with fulminant progression. An urgent decompression and excessive debridement were performed. Although Lemierre syndrome is a well-known cause of internal jugular vein thrombosis, association with proteus culture is very rare.     

Persistent Müllerian duct syndrome diagnosed by laparoscopic examination: a case report

A 2-year-old boy presented for the evaluation of left nonpalpable testis. Laparoscopic examination revealed right migratory testis without any abnormal appearance and a uterine-like structure with an immature gonad in rectovesical fossa. Right spermatic duct merged into the uterine-like structure on the right side. The immature gonad was removed by laparoscopic procedure, while the uterine-like structure was not removed to preserve the right spermatic duct. Right orchidopexy was also performed. Histopathologic diagnosis for the removed gonad was hypoplastic testis. After the chromosomal analysis, 46 XY karyotype, we diagnosed this case as persistent Müllerian duct syndrome. Persistent Müllerian duct syndrome diagnosed by laparoscopic examination is very rare. To the best of our knowledge, this is the third case in Japan.     

Persistent müllerian duct syndrome: How to deal with the müllerian duct remnants — a review

Persistent müllerian duct syndrome (PMDS) is usually a surprise finding either during orchidopexy or during routine inguinal hernia repair in male patients. Often, the surgeon would face a dilemma about what is to be done with the remnants of müllerian duct, i.e. the fallopian tubes, uterus and proximal vagina. Till recently, it was advised to retain these structures whenever it was felt that the complete excision of these structures may jeopardise the blood supply and integrity of the vas deferens. Recent reports of malignancy in these retained structures would justify more aggressive approach. In our patients with PMDS, we have removed the mucosa of the retained müllerian structures, without compromising the integrity and vascularity of the vas deferens, thus reducing the chances of malignancy.     

Persistent müllerian duct syndrome associated with irreducible inguinal hernia, bilateral cryptorchidism and testicular neoplasia: a case report

Persistent müllerian duct syndrome is a rare form of male pseudohermaphroditism. A case is reported of normal male appearance with bilateral cryptorchidism and a right irreducible inguinal hernia. On exploration, an uterus with two fallopian tubes and a testicle were found in the hernia sac. The uterus, fallopian tubes and left testicle were en bloc removed. Right orchidopexy and hernia repair were performed. In conclusion, if there is an adult bilateral cryptorchidism, surgeons should take into consideration a persistent müllerian duct syndrome.     

Wünderlich Syndrome in Renal Transplant Recipients: A Case Report and Literature Review

BackgroundWünderlich syndrome, or spontaneous atraumatic renal hemorrhage, is a clinical entity rarely described in the native kidney of patients who have undergone renal transplant. Although its manifestation is quite similar in reported cases, it may present few symptoms, from bleeding of unidentified etiology to dramatic pictures associated with hypovolemic shock. There are few reports of spontaneous hemorrhage of a native kidney after kidney transplantation.Case reportWe present a 38-year-old male patient who developed hemorrhage of a ruptured native kidney after a late renal transplantation. We analyze what has been reported in the literature and highlight the possibility of this complication after kidney transplantation. Imaging exams and surgical specimen demonstrated the presence of ruptured angiomyolipoma in the patient's native right kidney. The postoperative period was not complicated, with a benign clinical evolution.ConclusionAlthough rare in patients who have undergone renal transplant, it is justified to suggest Wünderlich syndrome in the presence of acute flank pain, abdominal tenderness, and signs of internal bleeding (Lenk's triad), with unexplained hemoglobin drop.     

Congenital Bilateral Lower Lip Pits Associated with Fistulae of the Minor Salivary Glands: Case Report of the Principal Van Der Woude Syndrome’s Trait

Background

Van der Woude syndrome (VWS; OMIM 119300) is an autosomal dominant developmental malformation presenting with bilateral lower lip pits related to cleft lip, cleft palate, and other malformations in about half the patients. These congenital lip pits appear clinically as a defect in the vermilion border of the lip. They are commonly related to fistulae of minor salivary glands with or without excretion. Usually the cause of cosmetic defects, VWS is preferably treated through simple excision. This report aims to describe a case of VWS involving a patient who presented with isolated congenital bilateral lower lip pits related to fistulae of the minor salivary glands, which were treated successfully through simple excision.

Case Report

A 5-year-old girl with a diagnosis of congenital bilateral lip pits associated with fistulae of minor salivary glands was treated successfully through simple excision. The diagnosis was confirmed by clinical examination, sialography, and scintigraphy. A good aesthetic result was observed after 2 years of follow-up evaluation.

Conclusion

Physicians must be aware of VWS because, although underreported and frequently not diagnosed, it is the most common cleft syndrome.

     

Laparoscopic Treatment of Boerhaave’s Syndrome: a Case Report and Review of the Literature

Spontaneous rupture of the oesophagus (Boerhaave’s syndrome) is a rare life-threatening disease requiring urgent surgical management. Symptoms can masquerade many other clinical disorders like acute myocardial infarction, dissecting aneurysm or upper gastro-intestinal tract diseases. Without prompt diagnosis and treatment, Boerhaave’s syndrome has a very high mortality rate.

We report a case of perforation of the distal oesophagus. A 40-year-old male patient presented at the emergency department with a classic history of acute epigastric pain and dyspnoea after an episode of vomiting. On clinical examination we found a firm, tender abdomen and cervical subcutaneous emphysema. Boerhaave’s syndrome was suspected on a clinical basis and was confirmed by thoraco-abdominal CT scan, showing an apparent pneumomediastinum and fluid at both lung bases. The patient underwent surgical repair of the distal oesophageal tear by laparoscopy. A mediastinal drain was left behind and a feeding gastrostomy was established. After initial improvement, the patient developed fever and dyspnoea. A thoracic CT scan revealed left-sided empyema. A thoracoscopic drainage of pus was performed and antibacterial and antifungal treatment was adapted. The patient recovered well and was discharged from the hospital 34 days after admission.     

Transaortic Mitral Valve Repair Combined with a Modified Bentall Procedure in a Patient with Marfan’s Syndrome: Report of a Case

We report a case of transaortic mitral valve repair combined with aortic root and arch replacement in a patient with Marfans syndrome. Preoperative computed tomography and echocardiography showed acute aortic dissection (DeBakey type 1), severe aortic regurgitation, annuloaortic ectasia, and mild mitral regurgitation (MR). We performed artificial chordae implantation to the anterior mitral leaflet (AML) through the aortic root, followed by insertion of an aortic composite graft and replacement of the aortic arch. The patient is well 55 months after the operation, with minimal MR. We think that the transaortic approach is a good alternative for exposure and correction of the AML and its apparatus in special circumstances.     

Müllerian duct remnant involving Wolffian system: a case report and literature review

A 14-month-old boy with repeated left acute epididymitis was admitted to our department. Ultrasonography detected a midline round cystic mass in a retrovesical region. This was easily opacified by cystourethrography and seen to have a free communication with the posterior urethra. Since urethroscopy revealed a passable orifice in the center of the verumontanum, while a cystic-wall biopsy specimen showed squamous epithelium, we considered this cystic lesion to be an enlarged prostatic utricle. Vasography showed that the bilateral vasa was implanted directly into this cystic lesion, and was the possible cause of his left epididymitis. Ligature of the left vas deferens was performed to prevent left epididymitis. An enlarged prostatic utricle involving the vasa is a rare presentation.     

Elevated Basal Calcitonin Levels in a Patient with a Hürthle-Cell Carcinoma of the Thyroid and Neuroendocrine Differentiation: Report of a Case

A thyroid nodule with elevated plasma levels of calcitonin is usually suggestive of a medullary thyroid carcinoma (MTC); however, thyroid and extrathyroid conditions have been reported with elevated plasma calcitonin levels in the absence of MTC. We report the case of a patient with a thyroid nodule and an elevated basal plasma calcitonin level of 315pg/ml (normal value <100pg/ml) who underwent a left hemithyroidectomy. Interestingly, histopathological examination revealed a Hürthle-cell carcinoma with positive neuroendocrine (NE) markers such as calcitonin and synapthophysin, but not with chromogranin staining. Thus, we discuss the phenomenon of non-NE tumors showing positivity for NE markers.     

Has Shouldice Repair in a Selected Group of Patients with Inguinal Hernia Comparable Results to Lichtenstein,TEP and TAPP Techniques?

Background

In the new international guidelines only the mesh-based Lichtenstein, TEP and TAPP techniques are recommended. This present analysis of data from the Herniamed Registry compares the outcome for Shouldice versus Lichtenstein, TEP and TAPP.

Methods

Propensity score matching analyses were performed to obtain homogeneous comparison groups for Shouldice versus Lichtenstein (n = 2115/2608; 81.1%), Shouldice versus TEP (n = 2225/2608; 85.3%) and Shouldice versus TAPP (2400/2608; 92.0%).

Results

The most important characteristics of the Shouldice patient collective were younger patients with a mean age of 40 years, a large proportion of women of 30%, a mean BMI value of 24 and a proportion of defect sizes up to 3 cm of over 85%. For this selected patient collective, propensity score matched-pair analysis did not identify any difference in the perioperative and one-year follow-up outcome compared with TAPP, fewer intraoperative (0.5 vs. 1.3%; p = 0.009) but somewhat more postoperative complications (2.3 vs. 1.5%; p = 0.050) compared with TEP and advantages with regard to pain at rest (4.6 vs. 6.1%; p = 0.039) and on exertion (10.0 vs. 13.4%; p < 0.001) compared with the Lichtenstein technique.

Conclusion

For a selected group of patients the Shouldice technique can be used for primary unilateral inguinal hernia repair while achieving an outcome comparable to that of Lichtenstein, TEP and TAPP operations.

     

Laryngeal Squamous Cell Carcinoma in a 13 Year-Old Child Associated with Human Papillomaviruses 16 and 18: A Case Report and Review of the Literature

Squamous cell carcinoma (SCC) of the larynx is extremely rare in adolescents and typically has an aggressive nature. The mechanism of laryngeal oncogenesis is complex and little is known about the role that human papillomavirus (HPV) plays in SCC in adolescents. We report a case of invasive laryngeal SCC that co-expressed HPV DNA subtypes 16 and 18 in a 13 year-old boy. Detection of HPV DNA types 6, 11, 16, 18, 31, 33, and 51 was performed by in situ hybridization, with confirmation by polymerase chain reaction. Immunohistochemical staining with p16 and HPV 16/18 revealed diffusely positive staining in the tumor cells. Coinfection by HPV DNA types 16 and 18 has not been previously reported, but our case suggests that HPV is a risk factor in developing laryngeal SCC in children and adolescents. Future studies evaluating HPV in the pathogenesis of these lesions is recommended to determine its prognostic significance.     

Blind hemibladder, ectopic ureterocele, or Gartner’s duct cyst in a woman with Müllerian malformation and supposed unilateral renal agenesis: a case report

Genital anomalies associated with unilateral renal agenesis are generally due to agenesis or hypoplasia of the entire urogenital ridge or distal mesonephric aberrations. However, renal adysplasia could also occur in association with anomalies of the ventral urogenital sinus. The patient presented didelphys uterus in the superior uterine segment, a septate cervix, and a simple vagina. After transvaginal puncture and injection of a contrast agent into the bulge observed in the right vaginal wall, a filled sac or cavity was detected, possibly a hemibladder. This structure continued upward with a possible dilated tortuous ureter that filled retrogradely. Magnetic resonance imaging also showed the presence of the right blind paravaginal sac. Right hemitrigone and ureteral orifice were absent in the cystourethroscopy. No right kidney was found, despite the use of multiple imaging techniques. Blind hemibladder, ectopic ureterocele, and Gartner’s duct cyst seem to be a possible diagnosis associated to Müllerian malformations and supposed unilateral renal agenesis. Therefore, Müllerian anomalies without combined mesonephric alteration could be associated with conditions of the ventral urogenital sinus, including blind hemibladder or ectopic ureterocele with secondary renal dysplasia.     

Fever,Malaise, and Dyspnea in a Diabetic Heart Transplant Patient: A Case Report

Patients with solid-organ transplants usually present at the emergency department with nonspecific symptoms. The physician should consider a great variety of syndromes and diseases, given the greater risk that solid-organ transplant patients carry because of immunosuppression and transplant-related conditions. Myocardial infarction caused by cardiac allograft vasculopathy must be always suspected and ruled out, even when initial symptoms do not orientate in that direction. We present a case that conjugates signs that can be present in different pathologies. It shows that fever is not always related to infection or rejection but could also appear in acute cardiac allograft vasculopathy. It emphasizes the need of a multi-disciplinary team led by a heart transplant specialist when dealing with this sort of clinical case.     

An 80 year old hermaphroditic with Hugh Brenner Tumour: a Rare Case Report and Review

Introduction

Hermaphroditism is a general term used to describe a sexual development disorder usually diagnosed at an early stage in sexual development. However, this is a report of an 80 year old sexual ambiguity patient with right adnexal Brenner Tumour.

Resection of Over 290 Polyps During Emergency Surgery for Four Intussusceptions with Peutz–Jeghers Syndrome: Report of a Case

A 41-year-old male patient with aggravated epigastralgia and nausea was admitted to Central Aizu General Hospital in February 1997. His past history showed a colonic polyp and anemia in the fourth decade. The patient looked healthy, but showed abdominal distension and tenderness, and pigmented lips. A plain abdominal X-ray revealed a dilation of the small intestine with niveau. Computed tomography disclosed multiple target signs. An emergency laparotomy clarified four intussusceptions of the small intestine with numerous polyps. Three were successfully reduced, while one jejunal intussusception was resected. Due to a fear of recurrence, a total of over 290 polyps were removed. His illness was diagnosed to be Peutz–Jeghers syndrome with a histology of hamartomatous polyps. He thereafter did well for 6 years, when he underwent an ileal resection for another intussusception caused by a newly grown lipoma. He was able to retain his job, but anemia and hypoproteinemia due to the proliferation of polyps necessitated treatments at the outpatient clinic. In May 2005, he underwent a third emergency laparotomy for an intussusception, followed by a resection of the ileum and 54 polyps. Since then he has been able to lead a normal life.