Cervical Lymphatic Malformation Research Articles

Risk factors for functional state deterioration in patients with lymphatic malformation occuring in the neck region

Introduction. The improvement and prediction of functional status in patients with lymphatic malformations of the head and neck, particularly in neck masses, remains a pressing socio-economic concern. As of the publication of this article, no comprehensive scientific studies have explored the cause-and-effect relationships that impact the functional status of this specific patient demographic.Aim. To elucidate the cause-and-effect relationships impacting the functional status of patients with cervical lymphatic malformations and to develop a predictive model for their functional decline.Materials and methods. A retrospective cohort study was conducted, involving a detailed analysis of the functional status of 115 paediatric and adolescent patients aged 1 month to 17 years, treated for cervical lymphatic malformations at the Dmitry Rogachev National Medical Research Center for Pediatric Hematology, Oncology, and Immunology, from May 2012 to December 2022. The analysis utilised the Cologne Disease Score (CDS), varying according to the histological type of the lymphatic malformations (microcystic, macrocystic, or mixed) and the staging of the de Serres classification.Results and discussion. The study involved 115 patients with a median age of 2.1 years (ranging from 0.4 to 5.5 years). It was determined that the ‘Mixed lymphatic malformations type,’ ‘Stage V of the de Serres classification,’ and ‘Need for surgical treatment’ were clinically and statistically significant prognostic factors for the deterioration of functional status in these patients, reducing the CDS scores by 0.976 to 4.514 points, respectively. These findings supported the development of a predictive model for worsening functional status in this group.Conclusion. The predictive model formulated during this research accounts for the histological type, location, and treatment modality, and is recommended for clinical application within specialised medical institutions.

The Duplicity of Incidents: Cervical Lymphatic Malformation in two Newborns

We report on two neonates born the same day, both with an isolated cervical lymphatic malformation. Cervical masses were detected by ultrasound late in the third trimester. Following interdisciplinary case conferences, a caesarean section in the presence of a neonatal team was the chosen delivery mode in both cases. Delivery and transition of the newborns were uneventful. The suspected diagnosis was confirmed by postnatal MRIs, which demonstrated neither associated malformations nor compression of vital structures. Therefore, an expectant approach was chosen for the newborn with the smaller lesion. The other newborn featured a sizeable lymphatic malformation, and due to consecutive head tilt, sclerotherapy was initiated in its second week of life. Our case report outlines the challenges of a rare connatal malformation. Guidelines are often missing. Individual decisions regarding delivery mode, diagnostics and therapy have to be made on an interdisciplinary basis and patients as well as parents need counseling and support over a long period. All the more significant is good, interdisciplinary collaboration between the involved disciplines.

EXIT-to-airway for obstructing fetal neck masses: a singular methodology for monochorionic and dichorionic twin pregnancies

INTRODUCTION: Fetal airway obstruction in one twin of a diamniotic pregnancy presents unique challenges. Very few cases of Ex-Utero-Intrapartum-Treatment (EXIT) procedures for twin pregnancy have been reported and only in dichorionic pregnancies. We report a singular methodology for EXIT-to-airway procedures in two pregnancies involving monochorionic and dichorionic twins. CASE PRESENTATION: Two cases of EXIT-to-airway in twin pregnancies were performed in 2018-2019 at a regional fetal treatment center. Case 1 involved a giant cervical teratoma in a monochorionic-diamniotic twin pregnancy with preterm labor at 29 weeks. Case 2 involved a dichorionic-diamniotic pregnancy with a large cervical lymphatic malformation with preterm labor at 36 weeks. In each case, the polyhydramnios caused the affected twin’s amniotic sac to be the presenting sac for the surgical approach. Bronchoscopy and successful intubation was completed after 22 and 10 minutes of uteroplacental bypass, respectively. The bystander twins were delivered second without intubation and resuscitated without perinatal distress. CONCLUSION: EXIT-to-airway appears to be a reasonable option for twins including monochorionic pregnancies, via delivery of the affected twin first followed by delivery of the bystander twin. Thoughtful preparation and counseling by an experienced multidisciplinary team permits an EXIT-to-airway approach for twin pregnancies even in an emergent setting.

Robotic Surgery Through a Transhairline Approach for Cervical Lymphatic Malformation in the Pediatric Population

Robotic Surgery Through a Transhairline Approach for Cervical Lymphatic Malformation in the Pediatric Population

Cervical lymphatic malformations amenable to transhairline robotic surgical excision in children: A case series.

Lymphatic malformations are rare benign malformations that predominantly occur in the head and neck region. The advent of surgical robots in head and neck surgery may provide beneficial outcomes for pediatric patients. Here, we describe our experiences with transhairline incisions for robot-assisted surgical resection of cervical lymphatic malformations in pediatric patients.In this prospective longitudinal cohort study, we recruited consecutive patients under 18 years of age who were diagnosed with congenital cervical lymphatic malformations and scheduled for transhairline approach robotic surgery at a single medical center. We documented the docking times, console times, surgical results, complications, and postoperative follow-up outcomes.The studied patients included 2 with mixed-type lymphatic malformations and 2 with macrocystic-type lymphatic malformations. In all 4 patients, the incision was hidden in the hairline; the incision length was <5 cm in 3 patients but was extended to 6 cm in 1 patient. Elevating the skin flap and securely positioning it with Yang retractor took <1 hour in all cases. The mean docking time was 5.5 minutes, and the mean console time was 1 hour and 46 minutes. All 4 surgeries were completed endoscopically with the robot. The average total drainage volume in the postoperative period was 21.75 mL. No patients required tracheotomy or nasogastric feeding tubes. Neither were adverse surgery-associated neurovascular sequelae observed. All 4 patients were successfully treated for their lymphatic malformations, primarily with robotic surgical excisions.Cervical lymphatic malformations in pediatric patients could be accessed, properly visualized, and safely resected with transhairline-approach robotic surgery. Transhairline-approach robotic surgery is an innovative method for meeting clinical needs and addressing esthetic concerns.

Utility of three-dimensional modeling of the fetal airway for ex utero intrapartum treatment.

Recent technological developments in three-dimensional (3D) printing have created new opportunities for applications in clinical medicine. 3D printing has been adopted for teaching and planning complicated surgeries, including maxillofacial, orthopedic reconstructions, and airway manipulation for one-lung ventilation or airway stenting. We present here the first use of such technology to print a model from in utero imaging for intrapartum treatment planning. A 32-week fetus presented with congenital high airway obstruction syndrome (CHAOS) due to a large cervical lymphatic malformation. An ex utero intrapartum treatment (EXIT) procedure was planned to allow delivery of a viable infant. We printed a 3D model of the fetal airway by printing separate elements: mandible, tongue, mass, larynx, and trachea from the fetal MRI. The elements were stuck together maintaining correct anatomical relationships. Airway planning was then performed in consultation with a pediatric ear nose and throat (ENT) surgeon. 3D modeling in utero presents many challenges: the resolution of the 3D model generated from a fetal MRI is less crisp than from CT images, fetal position may be variable and not in a defined anatomical plane, movement artifact occurs. Nevertheless, pre-procedure simulations with the aid of 3D modeling promoted team cooperation and well-prepared management of the fetus during EXIT.

949 EXIT-to-airways for fetal neck mass: extending the procedure to diamniotic-monochorionic twin gestation

Fetal airway obstruction in one twin of a diamniotic pregnancy presents unique challenges especially when a single placenta is shared between the fetuses. Very few cases of Ex-Utero-Intrapartum-Treatment (EXIT) in twin pregnancies have been reported, and only in dichorionic pregnancies. We report a single methodology for EXIT-to-airway procedures in monochorionic and dichorionic twins. We review two EXIT-to-airway in twin pregnancies with a giant cervical mass in one fetus. Two cases of EXIT-to-airway in twin pregnancies were performed in 2018-2019 at a regional fetal treatment center. A multi-disciplinary evaluation was completed, and treatment options developed to accommodate parental goals balancing the risks for both fetuses. Case 1 involved a dichorionic-diamniotic pregnancy with a large cervical lymphatic malformation with labor at 36 weeks. Case 2 involved a giant cervical teratoma in a monochorionic-diamniotic twin pregnancy with preterm labor at 29 weeks. In each case, the polyhydramnios caused the affected twin’s amniotic sac to be the presenting sac for the surgical approach. Continuous monitoring of both fetuses was obtained via pulse-oximetry and intermittent transthoracic echocardiography and continuous transuterine echocardiography. Bronchoscopy and intubation were completed after 10 and 22 minutes of uteroplacental bypass respectively. In each case, the affected fetus was transferred to adjacent operating room for complete mass resection. The unaffected twins were delivered as second without intubation and resuscitated without any perinatal distress. Twins and mothers recovered without complications. EXIT-to-airway appears to be a reasonable option for the safe transition of complicated twins even in the setting of significant prematurity in a monochorionic pregnancy. Continuous monitoring permits safe algorithm-based airway management of the affected twin first and subsequent delivery of the unaffected twin. Thoughtful preparation by an experienced multidisciplinary team permits an EXIT-to-airway approach for twin pregnancies in an emergency setting.

Neonatal cervical lymphatic malformation involving the fetal airway the setting of emergency caesarean section

Abstract Objectives Lymphatic malformations are benign vascular anomalies comprised of lymphatic elements and most commonly occur in the neck. They may be diagnosed prenatally on ultrasound, and, in addition to confirming the diagnosis, fetal MRI can delineate the presence and extent of airway involvement. A variety of therapies are available for the treatment of lymphatic malformations including medical management, sclerotherapy, and surgery. Case presentation We report a case of a 28 year-old pregnant female diagnosed with a fetal neck mass by ultrasound at 21 weeks gestation. A fetal MRI was performed which demonstrated a multiseptated cystic neck mass most suggestive of a lymphatic malformation. The mass was noted to be intimately associated with the fetal airway. Follow up ultrasounds showed continued growth of the lesion throughout pregnancy. The baby was born by EXIT procedure and the lymphatic malformation was ultimately treated by a combination of medical treatment, sclerotherapy, and surgery. Conclusions Cervical lymphatic malformations have a classic imaging appearance and can enlarge throughout gestation as well as after delivery. The treatment plan typically involves a multidisciplinary approach.

A large cervical lymphatic malformation in a neonate successfully treated with Kampo medicine

Abstract Introduction: Recently the application of Kampo, or Japanese traditional medicine, to lymphatic malformation (LM) has been reported, but there are no reports of its effects against neonatal LM. We herein report a case of a neonatal huge cervical LM successfully treated with eppikajutsuto and ogikenchuto without tracheostomy or sclerotherapy. Patient concerns: A baby boy with a huge cyst in his right neck was delivered by Caesarean section without a prenatal diagnosis. The cyst extended and slightly compressed the trachea on the 7th day after birth. Diagnosis: The patient was diagnosed with LM by ultrasonography. Interventions: Although we planned sclerotherapy with OK-432 followed by tracheostomy, his parents refused tracheostomy. Therefore, we started the administration of eppikajutsuto (0.5 g/kg/d) on the 26th day of life instead of sclerotherapy. As the cyst was slightly enlarged at the beginning of eppikajutsuto administration, we increased the dose of eppikajutsuto to 0.75 g/kg/d and added ogikenchuto (0.4 g/kg/d), expecting greater regression of the lesion than with the initial regimen. Outcomes: After a few days, the cyst started to diminish in size. Six months later, magnetic resonance imaging showed remarkable regression of the LM without tracheal compression. Lessons: The combination of eppikajutsuto and ogikenchuto was thus found to be effective in the treatment of a neonatal LM. This is the first report to demonstrate that the dose-escalation of eppikajutsuto may therefore be effective without any severe adverse effects, even in neonates.

Ultrasonographic diagnosis, classification, and treatment of cervical lymphatic malformation in paediatric patients: a retrospective study

BackgroundTo explore the imaging features, key diagnostic points, classification, treatment, and prognosis of cervical lymphatic malformation.MethodsOverall, 320 patients diagnosed with cervical lymphatic malformation were retrospectively analysed in our hospital between 1 January 2014 and 31 December 2017. Imaging modalities included colour Doppler ultrasound, magnetic resonance imaging, and contrast-enhanced computed tomography. Cervical lymphatic malformations were classified by cyst diameter. Treatments included interventional therapy, surgery, and expectant treatment.ResultsCervical lymphatic malformation was identified in 320 of 1192 patients with lymphatic malformation. Four were excluded due to misdiagnosis by ultrasonography. Cervical lymphatic malformation was classified as mixed, macrocystic, and microcystic in 184 (57.5%), 117 (36.56%), and 19 (5.94%) patients, respectively. Sixty-four (20%), ten (3.12%), seven (2.19%), and three (0.94%) patients experienced intracystic haemorrhage, infection, concurrent intracystic haemorrhage and infection, and calcification, respectively. Among 260 (81.25%) patients who underwent interventional sclerotherapy, 163 (50.94%) received it once and 96 (30%) received it two or more times. Twenty-eight (8.75%), five (1.56%), and 27 (8.44%) patients underwent surgical resection, interventional sclerotherapy plus surgery, and expectant management, respectively.ConclusionsUltrasonography is useful for diagnosing definite cervical lymphatic malformation. Interventional therapy is the first choice for children with confirmed cervical lymphatic malformation.

Long-term symptom control following resection of cervical lymphatic malformations: a case series

BackgroundPrevious literature has reported on the incidence of short-term complications following resection of cervical lymphatic malformations (LMs) in children, however no research has yet investigated the long-term symptomatic course in these patients. This study aims to provide families and providers with an understanding of expectations for long-term symptom control, specifically in association with subsequent upper respiratory infections (URIs).MethodsA retrospective chart review produced a case series of patients who underwent resection of cervical LM at a pediatric tertiary care center between 2007 and 2016. Demographic data, disease characteristics, operative details, and postoperative care were evaluated. Telephone surveys were conducted to ascertain the course of postoperative symptoms at the surgical site.ResultsForty-three patients responded to the telephone survey. Thirty-seven (86.0%) had at least one postoperative surgical site symptom during subsequent URIs, with 28 (65.1%) reporting redness, 34 (79.1%) reporting swelling, and 18 (41.9%) reporting pain. Patients who experienced any of these symptoms universally indicated that they developed soon after the surgical resection, and over half reported that they improved over time. Postoperative seroma was associated with swelling during subsequent URIs (p = 0.04). Patients age 7 or were greater were more likely than those under 7 to report pain with URIs (p = 0.006). All 8 patients with drain placement for at least 2 days reported swelling during subsequent URIs. The incidence of the queried symptoms did not vary significantly based on sex, stage, histology, surgical subsite, or presence of residual disease.ConclusionWhile preoperative symptoms associated with cervical LMs are also frequently encountered postoperatively, particularly in some patient subgroups, improvement over time should be expected.Level of evidence4

Left Hemifacial Lymphatic Malformation in a Nine-Year-Old Boy

Left Hemifacial Lymphatic Malformation in a Nine-Year-Old Boy

The Ex Utero Intrapartum Treatment (EXIT) Procedure in Huge Congenital Cervical Lymphatic Malformation

The Ex Utero Intrapartum Treatment (EXIT) Procedure in Huge Congenital Cervical Lymphatic Malformation

Oral Sirolimus: An Option in the Management of Neonates with Life-Threatening Upper Airway Lymphatic Malformations.

Background: Mechanistic target of rapamycin (mTOR) inhibitors are being used off-label showing promising results in patients with vascular anomalies. Children with lymphatic malformations (LMs) involving the airway benefit from sirolimus therapy soon after birth, reducing the need of tracheostomy. Available information about efficacy and side effects in neonates remains poor. We present seven newborns with severe head and neck LM showing response to sirolimus with no significant toxicity. Methods and Results: We performed a retrospective review of neonates with head and neck LM who received sirolimus between January 2014 and May 2018 with upper airway involvement needing ventilatory support. We analyzed type of LM, involved anatomical area, symptoms and response to sirolimus, including dosage, blood levels, response, side effects, and complications. Seven neonates received primary treatment with sirolimus in the context of cervical LM. Sirolimus was started at the recommended dose of 0.8 mg/m2/12 h and adjusted to maintain blood levels between 4 and 12 ng/mL. Median follow-up was 32 months (4-43) with a median treatment duration of 12 months (3-43). One patient had complete resolution of the malformation, one had complete resolution of symptoms, and five had partial resolution of the malformation with significant improvement in their respiratory conditions. Two patients required additional subtotal surgical resection and one tracheostomy. Four patients remain under treatment. Toxicity was not observed. Conclusions: Sirolimus is a safe drug in neonates and can be considered the first therapeutical option in newborns at high risk of respiratory failure before sclerosis or surgery. Close follow-up is mandatory to identify side effects at long-term use.

Cervical lymphatic malformations: Prenatal characteristics and ex utero intrapartum treatment

The ex utero intrapartum treatment (EXIT) is utilized to transition fetuses with prenatally diagnosed airway obstruction to postnatal life. We describe the unique clinical course, diagnosis, treatment, and outcomes of patients with cervical lymphatic malformation (CLM) managed with EXIT. Review of fetuses with diagnosed CLM was delivered by EXIT (2001-2018) in a tertiary referral fetal center. Outcomes included survival, tracheostomy at discharge, neonatal course after delivery, and pulmonary hypoplasia. Data are reported as median [range] and rate (%). Out of 45 patients delivered by EXIT, 10 were delivered for CLM: seven had polyhydramnios, one had nonimmune hydrops, five delivered preterm, and three were emergency EXITs. The EXIT time and estimated blood loss were 125minutes (95, 158) and 900mL (500, 1500), respectively. Airway was secured in all. There was one neonatal death (day 8) with prematurity, sepsis, and pulmonary hypoplasia. Three out of nine were discharged with a tracheostomy. In CLM, close monitoring for structural neck involvement and development of polyhydramnios are important and may be an indication for EXIT as the optimal delivery mode. An experienced multidisciplinary team is a key factor for an effective approach to the obstructed airway in CLM.

216: Cervical lymphatic malformations: Prenatal imaging characteristics and neonatal outcomes of ex- utero intrapartum treatment

216: Cervical lymphatic malformations: Prenatal imaging characteristics and neonatal outcomes of ex- utero intrapartum treatment

Thirty-day perioperative outcomes in resection of cervical lymphatic malformations

IntroductionLimited information exists regarding short-term morbidity in the resection of lymphatic malformations. In order to make informed collaborative medical decisions, clinicians and families would benefit from information on 30-day outcomes and the expected course associated with surgical excision of lymphatic malformations. MethodsA retrospective chart review was conducted to develop a case series of patients who underwent resection of lymphatic malformation at a pediatric tertiary care center between June 1, 2007 and September 30, 2016. Demographic data, disease characteristics, operative details, post-operative care, and adverse events in the 30-day post-operative period were analyzed. Primary outcomes included facial nerve dysfunction, seroma formation, re-admission, and overall rate of any complications. Secondary outcomes included operative time, duration of stay, and duration of drain placement. ResultsForty-nine excisions were performed in 46 patients (21 male, 25 female). Median age was 5 years. All but 7 cases were performed as the initial primary intervention. Median operative time was 96 min (range 22–224). Higher stage lesions (3-5) were associated with a longer operative time (p = .03). Median length of stay was 2 days (range 0–35). Higher stage lesions were associated with an increased length of stay (p = .0004). Median duration of drain placement was 2 days (range 0–14), and was longer in higher stage lesions (p = .0002). Higher stage lesions (p = .002) and cases ultimately found to have residual disease (p = .019) were associated with an increased overall rate of complications; there was no association between cyst type and rate of complications. Seroma formation (31%) and transient facial nerve weakness (26%) were the two most common complications observed. There was no association between stage or cyst type and likelihood of seroma formation. Seromas resolved after a median duration of 3 months and transient facial nerve weakness resolved after a median duration of 2 months. 3 patients required return to the OR and 1 patient was readmitted. ConclusionThe overall rate of adverse events after surgical excision of cervical lymphatic malformations is relatively low. Increased rates of complications can be expected with higher stage. Similarly, for these higher stage lesions, a longer operative time, hospital stay, and duration of drain placement can be expected.

Surgical excision and hyoid suspension without tracheostomy for large cervical lymphatic malformation

Abstract Lymphatic malformation is a rare congenital lesion caused by lymphatic abnormality. Herein is a case of extensive pediatric lymphatic malformation involving almost entire neck from skull base to mouth floor. Surgical excision of the huge mass was performed without tracheostomy. Repeated attempts on post-operative extubation resulted in cyanosis and apnea due to narrow postlingual space. Hyoid suspension was performed to improve postlingual insufficiency and apnea events were eventually resolved. Clinical Trial Registration: None.

Surgical excision is the treatment of choice for cervical lymphatic malformations with mediastinal expansion

BackgroundLymphatic malformations (LMs) in the mediastinum are uncommon. However, cervical LMs may expand into the mediastinum. The aim of this study was to review our experience with the management of patients with LMs involving the mediastinum and to propose a treatment algorithm to guide the management of these rare malformations. Material and MethodsThis was a descriptive retrospective chart review of all patients with LMs involving the mediastinum treated at our Institution between 2009 and 2015. We collected demographic data, data on investigations, management, and complications of the treatment, as well as outcomes at follow-up. Complications were defined and described according to the Clavien-Dindo classification. The clinical outcome was assessed using a clinical assessment scale. ResultsThe cohort consisted of seven patients. Airway compromise at the time of diagnosis was seen in 4 patients. Three patients had anatomical compression of the trachea and two patients had sub-total compression of the right lung. All three patients with tracheoscopy-verified compression of the trachea had compromise of the distal trachea, and a tracheostomy would not have been protective. All patients received sclerotherapy. The median time with mechanical ventilation at the neonatal intensive care unit after each sclerotherapy was eleven days (range 8–31). Each patient received sclerotherapy in median three times (range 1–9). Five of the patients (71%) were operated with excision of the LM in the mediastinum. Two of the patients were operated primarily and three patients were operated after major complications to sclerotherapy. The patients treated with sclerotherapy and the operated patients had comparable amount of mild complications, Clavien-Dindo grade I-II complications. Severe complications, Clavien-Dindo grade III-IV, were seen five times more commonly after sclerotherapy than after surgery. The clinical outcome was excellent for the operated patients and fair to good for the patients receiving only sclerotherapy. ConclusionPatients with cervical LM involving the mediastinum represent a high-risk group with respect to the severity of complications following sclerotherapy. The swelling is unpredictable and requires extended observation at an intensive care unit with ventilation support. Tracheostomy does not prevent tracheal compression in mediastinal LM, as the malformation may compress trachea distal to the stoma. Surgical resection of the LM in the mediastinum is recommended, with the possibility of intra-operative sclerotherapy as an adjunctive. Level of EvidenceIV.

Diagnosis and surgical treatment of cervical macrocystic lymphatic malformations in infants.

The treatment of lymphatic malformations (LMs) represents a great clinical challenge. The present study reported on the treatment of 68 infants with cervical macrocystic LMs using surgical resection. The cases were retrospectively analyzed. All patients underwent pre-operative ultrasonography, computed tomography (CT), and magnetic resonance imaging (MRI) examinations. The surgery was performed under general anesthesia with endotracheal intubation. Ultrasonograms showed that 24 cases were monolocular, 44 were multilocular, 16 had no echo, 20 had a uniform low-level echo and 32 had a non-uniform low-level echo. CT showed non-enhancing low-attenuating cystic lesions and attenuation values of 10–45 HU. The magnetic resonance images of the LMs showed a low signal intensity on T1-weighted imaging (WI) and a high signal intensity on T2-WI. Complete resection was achieved in 56 patients, subtotal resection in eight and partial resection in four. Two complications were noted, including reversible paresis of the marginal mandibular branch of the facial nerve and a surgical-site infection. One patient in whom partial resection was achieved had recurrence at ~2 months after the surgery. Ultrasonography, CT and MRI clearly demonstrated the size, shape, extent and adjacent structures of LMs, which aided in surgical planning and assessment of potential risks. Surgical excision increased the chances of cure and was relatively safe for infants aged <1 year. Location and extent, rather than age, were determined to be the most important factors for successful surgical treatment.