Correction of congenital diaphragmatic hernia in utero IX: fetuses with poor prognosis (liver herniation and low lung-to-head ratio) can be saved by fetoscopic temporary tracheal occlusion

BACKGROUND/PURPOSE: Fetuses with congenital diaphragmatic hernia (CDH) who have a "poor prognosis" with postnatal treatment now can be identified on the basis of liver herniation, early diagnosis (before 25 weeks' gestation) and a low lung-to-head ratio (LHR). Because complete in utero repair proved unsuccessful for this group, the strategy of temporary tracheal occlusion was developed to gradually enlarge the hypoplastic fetal lung. The purpose of this study is to compare the outcome of patients in the poor-prognosis group treated by one of three methods: (1) standard postnatal care, (2) fetal tracheal occlusion via open hysterotomy, and (3) the recently developed video-fetoscopic (Fetendo) technique of tracheal occlusion without hysterotomy. METHODS: In the past 3 years, 34 of 86 fetuses with an isolated left CDH met criteria for the poor-prognosis group. Thirteen families chose postnatal treatment at an extracorporeal membrane oxygenation (ECMO) center, 13 underwent open fetal tracheal occlusion, and eight underwent fetoscopic tracheal occlusion. RESULTS: The survival rate was 38% in the group treated by standard postnatal therapy, 15% in the open tracheal occlusion group, and 75% in the Fetendo group. There were less postoperative pulmonary complications noted in mothers who underwent the Fetendo procedure versus the open tracheal occlusion. All but one Fetendo clip patient had a striking physiological response demonstrated by sonographic enlargement of the small left lung that was documented postnatally by plain radiographs and its subjective appearance during repair of the CDH. In contrast, only 5 of the 13 open tracheal occlusion patients demonstrated lung growth. CONCLUSION: Fetuses with a left CDH who have liver herniation and a low LHR are at high risk of neonatal demise and appear to benefit from temporary tracheal occlusion when performed fetoscopically, but not when performed by open fetal surgery.     

An unusual presentation of multiple congenital melanocytic nevi with a limb distribution

We report a newborn in whom multiple small congenital melanocytic nevi (MN) were noted on the right side involving the scapular area, shoulder, upper arm and forearm. Such a limb distribution of small congenital MN has never been reported in the literature.     

The lateral chest radiograph in the detection of pericardial effusion: a reevaluation

The purpose of this study was to evaluate the usefulness of 10 previously published plain film signs for diagnosing pericardial effusion and to determine whether the posteroanterior (PA) or lateral chest radiograph was the better view for detecting pericardial effusion. A retrospective study of 100 consecutive adult patients with pericardial effusions confirmed by echocardiography and/or computed tomography was undertaken. Five signs were found to be useful in detecting pericardial effusion on plain films, and the lateral chest radiograph was found to be better than the PA view in detecting pericardial effusion. The water-bottle configuration, widening of the carinal angle, and the differential-density sign were helpful in diagnosing pericardial effusion on the PA view. However, these signs were only seen in moderate-to-large effusions. The displaced epicardial fat pad sign and the posteroinferior bulge sign on the lateral view improved the detection of moderate-to-large pericardial effusions, and were also present in many of the cases with small pericardial effusions. Recognition of specific signs of pericardial effusion on the lateral chest radiograph may significantly improve the plain film detection of pericardial effusion.     

Dacryocystitis associated with malignant lymphoma of the lacrimal sac

BACKGROUND: Tumors of the lacrimal sac are unusual, and lymphomas of the lacrimal sac are quite rare. Four patients with a history of well-differentiated, small cell lymphoma or chronic lymphocytic leukemia presented with either acute or chronic dacryocystitis and epiphora due to lymphomatous infiltration of the lacrimal sac. METHODS: All four patients underwent dacryocystorhinostomy with lacrimal sac biopsy. All tissues underwent complete histopathologic evaluation including immunohistochemical studies for cell surface markers and, in addition, were compared with previous biopsies performed for the initial diagnosis of lymphoma. FINDINGS: All biopsies demonstrated small cell well-differentiated lymphoma on histologic and immunofluorescent examination. No patient demonstrated orbital involvement on computed tomography. One patient had previously diagnosed chronic lymphocytic leukemia and one patient demonstrated diffuse lymph node involvement on postoperative systemic evaluation. All four patients underwent additional chemotherapy. No recurrence of dacryocystitis or epiphora occurred. CONCLUSION: Four patients presented with dacryocystitis secondary to lacrimal sac lymphoma. Lymphomatous lacrimal sac infiltration is an unusual cause of dacryocystitis. Biopsy of the lacrimal sac plays a diagnostically important role in dacryocystorhinostomy even in the absence of obvious tumorous involvement of the lacrimal sac mucosa.     

Diffuse polymicrogyria associated with an unusual pattern of multiple congenital anomalies including turribrachycephaly and hypogenitalism

A newborn male infant born to consanguineous parents was found to have diffuse polymicrogyria associated with an unusual pattern of congenital anomalies including microbrachycephaly, turricephaly, blepharophimosis, microstomia with maxillary retrusion and mandibular prognathism, micropenis with cryptorchidism, camptodactyly and adducted thumbs, and a progeroid appearance. The combination of manifestations in our patient represents a unique form of polymicrogyria with congenital anomalies, probably representing a new syndrome.     

Bronchioalveolar adenocarcinoma associated with a chylous ascites. An unusual case

A case of bronchioloalveolar carcinoma with long evolution associated to chylous is reported. The natural clinical history of the adenocarcinoma follows with an unusual dissemination as an atypical manifestation of lymphatic metastasis.     

An unusual presentation of congenital infantile myofibromatosis arising from the interspinous ligament

The authors describe an extremely rare presentation of congenital infantile myofibromatosis. A full-term newborn boy presented with a thumb-sized subcutaneous mass on the mid-spinal line between the 2nd and 3rd lumbar spinous processes. A solid tumor arising from the interspinous ligament was resected. Microscopic and immunohistochemical studies revealed myofibromatosis.     

Airway muscle in infants with congenital diaphragmatic hernia: response to treatment

BACKGROUND/PURPOSE: Airway muscle hyperactivity and chronic lung disease frequently follow congenital diaphragmatic hernia (CDH) treatment. The aim of this study was to compare the quantity of airway muscle and alveolar ductal artery muscle in CDH infants after various treatments. METHODS: Five groups were studied postmortem: CDH, died within 24 hours, without high ventilatory assistance (n = 3); CDH, various extracorporeal membrane oxgenation (ECMO) durations, without high ventilatory assistance (n = 4); CDH, various ECMO durations, with high ventilatory assistance (n = 7); no CDH, without high ventilatory assistance (n = 12); and no CDH, with high ventilatory assistance and bronchopulmonary dysplasia (BPD) (n = 5). Sections from standardized fixed lungs were immunohistochemically stained for alpha-smooth muscle actin. Muscle surrounding conducting airways from small preterminal bronchioles to bronchi was quantitated in both the ipsilateral and contralateral lungs with computerized image analysis. Similarly, muscle mass was quantitated in alveolar ductal arteries. RESULTS: CDH infants with low ventilatory assistance, regardless of postnatal age, had the same quantity of airway muscle as low ventilatory assistance controls. Infants with CDH and prolonged high ventilatory assistance had significantly more muscle throughout the conducting airways, similar to BPD infants without CDH, even though the CDH infants had significantly less exposure to high ventilatory assistance. With both low and high ventilatory assistance, the quantity of muscle in both the ipsilateral and contralateral lungs was similar. In contrast, small acinar arteries in CDH infants have increased muscle mass at birth. This muscle is decreased by ECMO but persists in CDH infants with high ventilatory assistance. CONCLUSIONS: The authors show that postnatally, CDH infants acquire increased muscle quantity throughout the conducting airways, in both the ipsilateral and contralateral lungs, with relatively short exposure to high ventilatory assistance. The normal decrease in acinar arterial mass that occurs postnatally is delayed in CDH infants with high ventilatory assistance.     

Renal tuberculosis associated with renal adenocarcinoma: unusual association

We present a patient 50 years old, complaining of right lumbar pain and miccional syndrome for three weeks. After analytic and x-ray studies, renal tuberculosis and renal cell carcinoma was the diagnosis. We started with specific antibiotic therapy and a radical nephrectomy was developed. According with the literature revised, just three cases has been published before. Because is an unusual clinic presentation we present these clinical note, we discuss the pathogenic and therapeutic procedures.     

Cardiac arrest before repair or extracorporeal membrane oxygenation cannulation does not increase the mortality rate associated with congenital diaphragmatic hernia

Despite recent advances in the management of high-risk congenital diaphragmatic hernia (CDH), mortality remains high. Deaths occur later because infants with inadequate pulmonary parenchyma are treated aggressively but eventually succumb to respiratory failure. In an attempt to identify absolute predictors of mortality the authors examined retrospectively their experience with CDH to determine if cardiac arrest before repair or initiation of extracorporeal membrane oxygenation (ECMO) invariably increased mortality. The authors reviewed the charts of 119 infants who had high-risk CDH treated between 1981 and 1994. They were divided into two groups: those that suffered cardiopulmonary arrest (CA, n = 21) before CDH repair or ECMO cannulation; and those that did not (NCA, N = 98). The authors compared mortality rate, ventilatory parameters, duration of, and complications on ECMO, as well as length of hospitalization between groups. Twenty-one infants suffered CA before initiation of ECMO support or CDH repair. Three infants (14%) suffered CA before arrival at our institution; seven (33%) after, and 11 (53%) both before and after arrival. There was no difference between the CA and NCA groups in terms of birth weight, gestational age, race and gender mix, or pregnancy and delivery complications. Five-minute Apgar scores were significantly lower in the CA group compared with the NCA group (4.6 v 5.7, P = .04). The CA group also had significantly worse "best postductal" blood gas and ventilatory parameters. There was no significant difference in length of hospitalization, time from admission to ECMO cannulation or CDH repair, or incidence of complications while on ECMO between the two groups. CA cases were more likely to require ECMO support (76% v 48%, P = .02) and to stay on ECMO for a more prolonged period than NCA cases (5.8 v 3.8 days, P = NS). However, there was no significant difference in overall survival between CA and NCA cases (43% v 51%, P = NS). Cardiopulmonary arrest before repair of CDH or ECMO cannulation is not a univariate independent predictor of mortality and therefore should not preclude these high-risk infants from maximum intensive care therapy, including ECMO cannulation.     

An unusual hyperplastic hepatocellular nodule in a patient with hepatitis C virus-related liver cirrhosis

Recent advances in diagnostic imaging techniques have increased the likelihood of detecting novel nodular lesions of the liver. We report here a case of unusual hyperplastic hepatocellular tumor found in a 70-yr-old woman with hepatitis C virus-related cirrhosis. A mass was incidentally detected in the right lobe by abdominal ultrasonography and confirmed by computed axial tomography (CT). Magnetic resonance imaging demonstrated that the tumor had hyperintense signal with a small hypointense region in the center and a thin, hypointense rim on T1-weighted image and a hypointense signal on T2-weighted image. CT during hepatic arteriography showed that the tumor was hypodense with a central hyperdense region, whereas CT during arterial portography revealed that the tumor was isodense and surrounded by a thin circular hypodense band with a central hypodense region. These radiographic findings suggested a diagnosis of dysplastic nodule with malignant foci of hepatocellular carcinoma. The patient underwent tumor resection. Macroscopically, the tumor, 45 x 45 x 30 mm in size, was encapsulated and had a central stellate-like scar with radiating septa. Histological examination showed a hyperplastic hepatocellular tumor without cellular, nuclear or structural atypia. The central fibrous scar contained abundant small, artery-like and vein-like vessels, whereas there were no normal portal triads but rather several portal tract-like structures lacking bile ducts in the parenchyma of the tumor. Some of the portal tract-like structures were composed of artery-like and vein-like vessels, and the others possessed vein-like vessels only. There were no bile ducts in the tumor. The nontumorous liver tissue had evidence of macronodular cirrhosis. Finally, this tumor was regarded as an unusual type of hyperplastic hepatocellular nodule encountered in cirrhotic liver, characterized by the presence of central stellate-like fibrosis and the lack of bile ducts. Although the pathogenesis of the hyperplastic lesion is unclear, it may represent a focal regenerative hepatocellular response to localized circulatory disorder.     

Survival of patients with congenital diaphragmatic hernia during the ECMO era: an 11-year experience

The pure TdI-1 polypeptide that blocks miniature endplate potentials (MEPPs) and abolishes or reduces endplate potentials (EPPs) below the action potential threshold was identified from the crude fraction of Tityus discrepans venom. The toxin is a potent reversible non-depolarizing muscle relaxant that blocks more than 95% of the EPP at a 2 microM (0.1 mg/ml) concentration. On a molar basis, TdI-1 is as potent as or more potent than many muscle relaxants since, at the concentration used, the toxin suppressed more than 95% of the EPP. Using matrix-assisted laser desorption time of flight (MALD-TOF) ionization mass spectrometry, TdI-1 was found to have an unusally large mol. wt for a scorpion toxin, close to 48,000. The N-terminal sequence of the first 23 residues of TdI-1 was also determined. The fragment differs from the N-terminal sequences of all 140 peptidic scorpion toxins found in the SWISSPROT and PIR databases using the search engine of the felix.EMBL-Heidelberg.de computer (European Molecular Biology Laboratory, Heidelberg, Germany.     

Percutaneous balloon pericardiotomy for patients with recurrent pericardial effusion: using a novel double-balloon technique with one long and one short balloon

Percutaneous balloon pericardiotomy is effective and less invasive for the treatment of recurrent pericardial effusion. This study suggests that the double-balloon method with 1 longer and 1 shorter balloon is the procedure of choice for percutaneous balloon pericardiotomy.     

Tracheal ligation: the dark side of in utero congenital diaphragmatic hernia treatment

This retrospective study investigates the clinical characteristics of gestational diabetes mellitus (GDM) (time of diagnosis, different treatment, metabolic parameters, etc.) in relation to prepregnancy body mass index (BMI) and the influence of BMI on neonatal outcome. 93 GDM women and 110 control subjects were divided into three groups in relation to their prepregnancy BMI: normalweight (Nw), overweight (Ow) and obese (Ob). GDM was diagnosed significantly (p < 0.01) earlier in Ow and Ob than in Nw. Preterm deliveries and cesarean sections resulted significantly (p < 0.01) increased in all BMI categories of GDM respect to matched controls. Prevalence of neonatal macrosomia was higher in GDM patients (44.6%) compared with normal controls (15.4%) and correlated (p < 0.01) with prepregnancy BMI in both groups. Nevertheless in each BMI category the prevalence of macrosomia was significantly higher in GDM patients. The body weight increase during pregnancy was not associated with neonatal macrosomia. This study shows that prepregnancy BMI is an important risk factor for GDM and is predictive for macrosomia specially in women suffering from GDM.     

Primary pericardial angiosarcoma with multiple lung and a solitary liver metastases: the clinical and anatomicopathological findings

A case of 66-year-old woman with cardiac tamponade and bilateral pulmonary infiltrates is reported. These infiltrates were "pseudonodulars" and confluent. We practice pericardiocentesis and a hemorrhagic fluid is pulled out. Its cytology shown not neoplastic cells. She died and the necropsy shown an angiosarcoma of pericardium with multiple pulmonary and alone hepatic metastases.     

Hepatic adenoma associated with oral contraceptive use: an unusual clinical presentation

A young woman who had taken contraceptive steroids for many years had the acute onset of abdominal pain because of central necrosis and hemorrhage into a hepatic adenoma. She had multiple lesions confined to one lobe of the liver. Persistent pyrexia and leukocytosis were also prominent clinical findings. She has had no evidence of recurrence of this problem during the seven years following right hepatic lobectomy. A review of the anabolic and contraceptive steroid-associated hepatic neoplasms is presented with comments directed toward the recognition of the critical clinical sequelae that can befall the patient with hepatic adenoma. Although all the patients in the steroid-treated group have tumors with benign and striking histologic similarity, microscopic evidence of malignant invasion of surrounding tissue is occassionally noted.     

Lower extremity purpura associated with new-onset autoimmune hepatitis: an unusual presentation

Adapalene is a new topical retinoid derived from naphthoic acid with a selective effect on the epidermis. It is indicated for treatment of acne vulgaris, alone or with other antiacneic topicals. It displays comedolytic and anti-inflammatory activities. The only frequent adverse event is a mild skin irritation during the first two weeks of treatment.