Fredy Nehme

Hirsute esophagus: An unusual cause of dysphagia

A 56-year-old man presented with a history of progressive dysphagia and chocking episodes over the previous few weeks. He had ~a history of tonsillar squamous cell carcinoma 10 years ago treated with induction chemotherapy followed by concurrent chemotherapy and radiation therapy. Nine years later, he was diagnosed with laryngeal squamous cell carcinoma treated with total laryngectomy and ipsilateral neck dissection. He then underwent reconstructive surgery with pectoralis major myocutaneous flap. Barium esophagram revealed filling defects along the reconstructed cervical esophagus (Fig. 1). Esophagogastroduodenoscopy revealed an intact skin graft with a tight proximal esophageal stricture with epithelial surface-bearing hair (Fig. 2). The esophageal stricture was impassable by the endoscope. Balloon wire-guided dilation was carried out resulting in improvement of his dysphagia. The patient subsequently had multiple recurrences of stricturing and excessive hair growth requiring dilation and removal of hair masses every 2 months on average. Locally recurrent squamous cell carcinoma of the head and neck is associated with significant morbidity and occurs in up to 30% of previously treated patients. Salvage surgery with partial or total laryngectomy is considered the treatment of choice and is often followed by free flap reconstruction surgery. Hirsute esophagus is a unique complication of reconstructive surgery of the pharynx and esophagus characterized by the classic triad of dysphagia, hair-spitting and choking spells. The outer epithelial surface of the skin flap becomes the inner surface of the reconstructed esophagus and may continue to bear hair. Usually, the hair follicles atrophy within 1 year but, rarely, excessive hair growth occurs. In such cases, periodic endoscopic hair removal is needed. In the present case, esophageal stricturing and hairy esophagus repeatedly co-occurred, raising speculation on the etiology of strictures being hairy esophagus, esophageal inflammation as a result of food retention within the hairy esophagus, or the aftermath of surgical scarring.

Gastrointestinal Amyloidosis: Review of the Literature

Gastrointestinal amyloidosis (GIA), a protein deposition disorder, represents a complex common pathway that encompasses multiple etiologies and presentations. It represents a significant diagnostic and treatment challenge. The disease results from the deposition of insoluble extracellular protein fragments that have been rendered resistant to digestion. GIA can be acquired or genetic, and most commonly results from chronic inflammatory disorders (AA amyloidosis), hematologic malignancy (AL amyloidosis), and end-stage renal disease (Beta-2 amyloidosis). The deposition of these abnormal proteins interferes with gastrointestinal tract (GI) organ structure and function, most notably in the liver and small bowel. Presentation from GI involvement includes cirrhotic sequelae, abdominal pain, malabsorption, and GI bleeding. Diagnosis hinges on pathologic examination of affected tissue, with classic green birefringence under polarized light. Abdominal fat pad and rectal mucosal biopsy have been described as sites of higher sensitivity for diagnosis. Serum amyloid P scintigraphy is near 90% sensitive for diagnosis of AA amyloidosis. Patients should be considered for further evaluation to rule out additional organ involvement, notably cardiac and renal. Treatment hinges on an adequate suppression of the predisposing inflammatory disorder, or malignancy, followed by supportive therapy. Prognosis varies depending on the etiology of the disease, with the AL subtype showing worse outcomes, as well as those with hepatic involvement.

Primary Hepatic Angiosarcoma Mimicking Multifocal Liver Abscess with Disseminated Intravascular Coagulation and Hemoperitoneum

Primary hepatic angiosarcoma (PHA), a rare and aggressive malignancy, has rarely been reported to present with disseminated intravascular coagulation with liver hemorrhage. Incidence is estimated at 0.5–2.5 cases per 10,000,000. To our knowledge, it has not been reported to mimic liver abscess with a septic presentation. Advanced imaging techniques may aid in the diagnosis, though biopsy with microscopy and immunohistochemistry is the mainstay. Prognosis is very poor, with a 5-year survival rate estimated at 6.4%. We present the case of a 70-year-old man who presented with sepsis thought to be due to a multifocal liver abscess, who did not respond to drainage and antibiotics. This atypical course led to further workup which subsequently revealed a diagnosis of PHA.

Isolated Ileal Stricture Secondary to Antigen-Negative GI Histoplasmosis in a Patient on Immunosuppressive Therapy.

We present a case of antigen-negative disseminated histoplasmosis manifesting as an isolated ileal stricture in a patient on chronic infliximab and methotrexate. Diagnosis can be challenging due to imperfect tests, and this condition should remain in the differential, even with negative testing. Mortality of untreated disseminated histoplasmosis can be as high as 80%.

Primary Squamous Cell Carcinoma of the Pancreas as a Cause of Biliary Obstruction

Primary squamous cell carcinoma of the pancreas (SCCP) is a rare neoplasm, given a lack of naturally occurring squamous cells within the pancreas, accounting for only 0.2% of all pancreatic cancers. The etiology is unknown. Symptomatology is non-specific and similar to other pancreatic neoplasms. No non-invasive testing can adequately rule in SCCP, and workup should proceed similarly to any pancreatic mass. Tissue sampling is required for diagnosis and guidance of further management, most commonly by endoscopic ultrasound with fine needle aspirate. SCCP is more aggressive than adenocarcinoma of the pancreas with a median survival of three and ten months for those treated with palliative and surgical intent, respectively. The optimal treatment regimen remains unknown, though the uses of radiation therapy, platinum-based regimens, gemcitabine, and 5-FU have all been reported with favorable results. We present a case of primary SCCP in an 81-year-old female who presented with jaundice.

Small Bowel Obstruction Secondary to Ileal Mucosa-Associated Lymphoid Tissue Lymphoma

First described in 1983 by Isaacson and Wright [1], mucosaassociated lymphoid tissue (MALT) lymphomas comprise around 8 % of all non-Hodgkin lymphomas [2]. They arise in a wide range of extranodal sites, most commonly in the gastrointestinal tract. The majority of the MALT lymphomas within the gastrointestinal tract involve the stomach and are usually associated with Helicobacter pylori infection [3]. Rarely, they have been reported to arise within the colon, small bowel, and rectum [4]. We describe a case of ileal MALT lymphoma presenting as small bowel obstruction.

Gastrointestinal histoplasmosis ileal stricture successfully treated with through-the-scope balloon dilation in a patient with hyperimmunoglobulin M syndrome

Gastrointestinal histoplasmosis is common in patients with disseminated disease affecting both immunocompetent and immunocompromised patients. However, it is often unrecognized due to a lack of specific signs and symptoms. It has only rarely been reported to cause small bowel obstruction, during which surgical treatment was nearly always necessary. Little is known about the usefulness of endoscopic therapy in gastrointestinal histoplasmosis associated strictures. We report the case of a 32-year-old man with a history of hyperimmunoglobulin M syndrome who presented with small bowel obstruction secondary to disseminated gastrointestinal histoplasmosis. Treatment was successful with a through-the-scope balloon dilator in combination with medical therapy. This report adds to the limited data available on the benefit of endoscopic therapy in infectious strictures, particularly gastrointestinal histoplasmosis.

Primary duodenal follicular lymphoma with late disseminated nodal relapse responsive to rituximab monotherapy: A case report

Gastrointestinal follicular lymphoma is a rare malignancy accounting for only 1-3.6% of primary non-Hodgkin lymphomas of the gastrointestinal tract and it is a relatively new clinical entity that was recently classified as a distinct variant of systemic follicular lymphoma. Therefore, data regarding long-term outcome are currently lacking. In addition, a consensus on the management of this disease has not been established and treatment strategies are derived from systemic follicular lymphoma. We herein report the case of a 51-year-old female patient diagnosed with duodenal follicular lymphoma who had nodal relapse nearly 5 years after complete remission. The patient was successfully treated with a 4-week course of rituximab during the initial diagnosis and relapse. To the best of our knowledge, this is the first case report to demonstrate the efficacy of a 4-week course of rituximab during both the initial diagnosis and nodal relapse. The aim of this report was to add to the limited available data on the treatment of gastrointestinal follicular lymphoma.

A Rare Cause of Primary Aortoenteric Fistula: Streptococcus parasanguinis Aortitis

Primary aortoenteric fistula is a rare cause of upper gastrointestinal bleed but can lead to significant mortality if the diagnosis is delayed. Aortitis, characterized by inflammation of the aortic wall, is a rare cause of aortoenteric fistula. We present a case report of a 72-year-old male patient with infectious aortoenteric fistula secondary to Streptococcus parasanguinis, along with a review of the literature. This case demonstrates the importance of early diagnosis and aggressive surgical treatment of aortoenteric fistulae and recognizing infectious aortitis as a potential etiology.

Spontaneous Splenic Infarcts in a Cirrhotic Patient with Primary Biliary Cirrhosis

Spontaneous splenic infarction has been rarely reported as a complication of cirrhosis and portal hypertension. We describe the case of a 67-year-old female with past medical history of primary biliary cirrhosis presenting for a 1-day history of left upper quadrant pain. Investigations were in favor of splenic infarcts secondary to portal hypertension. The patient improved with conservative management and no recurrence was noted on further follow-up. Splenic infarction must be kept in mind when a patient with cirrhosis presents with left upper quadrant abdominal pain without a clear source.