phenylephrine-hydrochloride and Precursor-Cell-Lymphoblastic-Leukemia-Lymphoma

Rhino-orbital-cerebral mucormycosis (ROCM) is a rare fulminant opportunistic fungal infection that despite relevant treatment has high mortality. We present a case of a 3-year-old girl with acute lymphoblastic leukemia and ROCM, who was treated successfully with excessive surgery, systemic antifungal treatment with amphotericin B (AmB), posaconazole, and terbinafine as well as hyperbaric oxygen. Surgery included, beside extracranial and intracranial removal of infected areas, endoscopic sinus and skull base surgery with local AmB installation and in addition placement of an Ommaya reservoir for 114 intrathecal administrations of AmB. In addition, we review the literature of ROCM in pediatric patients with hematological diseases.

Topics: Amphotericin B; Antifungal Agents; Brain; Child, Preschool; Female; Humans; Hyperbaric Oxygenation; Mucormycosis; Naphthalenes; Nose; Opportunistic Infections; Orbit; Precursor Cell Lymphoblastic Leukemia-Lymphoma; Terbinafine; Triazoles

To determine if simultaneous, bilateral lateral rhinotomies for medial maxillectomies would result in central skin or bone loss in pediatric patients with invasive fungal disease.. Retrospective chart review.. Tertiary care children's hospital.. Three children underwent surgery between April 1996 and June 1998. Ages at treatment ranged from 11 to 14 years. All had bilateral, biopsy-proven invasive fungal disease of the lateral walls of the nose. All were undergoing chemotherapy for acute lymphocytic leukemia (ALL) or acute myelocytic leukemia (AML).. Bilateral lateral rhinotomies for medial maxillectomy. Two of 3 also had simultaneous total septectomy.. Skin survival and patient survival.. All three patients had bilateral simultaneous medial maxillectomy for invasive fungal disease in the presence of profound pancytopenia secondary to treatment of leukemia. One patient had minor nasal edema postoperatively, but none showed any loss of the central nasal skin or facial skeleton. All patients survived the invasive fungal disease with follow-up of at least 24 months. All patients underwent multiple debridements after the original surgery, and 3 of 6 eyes had permanent epiphora requiring dacryocystorhinostomies.. Bilateral simultaneous lateral rhinotomies are safe in children and did not result in any central skin loss. Aggressive bilateral surgery for invasive fungal disease is compatible with a good esthetic outcome and long-term survival.

Topics: Adolescent; Child; Female; Humans; Immunocompromised Host; Leukemia, Myeloid, Acute; Male; Maxillary Sinus; Maxillary Sinusitis; Mycoses; Nose; Precursor Cell Lymphoblastic Leukemia-Lymphoma; Retrospective Studies

Cleft lip and palate is among the most common congenital anomalies. Its association with major blood disorders has rarely been reported. The purpose of this study was to report two patients who had major blood diseases associated with cleft lip and palate.. From June 1995 to December 1997, there were 2700 patients with cleft lip, cleft palate, or both who received treatment at Chang Gung Memorial Hospital. Two of them were found to have major hematological disorders. In both cases, the disorder was detected by preoperative blood cell counts and white cell differentiation. Case 1 was a 21-year-old woman patient with repaired right cleft lip. She was admitted for alveolar bone grafting and closure of oronasal fistula. Abnormal presentation of blast cells was found, and subsequent bone marrow study confirmed acute lymphocytic leukemia. Case 2 was a 26-year-old man with left secondary cleft lip nasal deformity scheduled to receive staged reconstructive operations. An elevated platelet count was found and subsequently confirmed to represent essential thrombocytosis. In both cases, reconstructive operations for the cleft-related deformities were performed.. Association of major hematological disorders and cleft lip, palate, or both is rare and is reported herein.

Topics: Adult; Alveolar Process; Blood Cell Count; Bone Transplantation; Cleft Lip; Cleft Palate; Contraindications; Diagnostic Tests, Routine; Female; Humans; Male; Nose; Oral Surgical Procedures; Precursor Cell Lymphoblastic Leukemia-Lymphoma; Thrombocytosis

Topics: Child; Epistaxis; Fatal Outcome; Hemostatic Techniques; Humans; Necrosis; Nose; Precursor Cell Lymphoblastic Leukemia-Lymphoma